Full text of "Contributions from the Department of Neurology and the Laboratory of Neuropathology (reprints)"

See other formats

UKIV.OF

immm

irinivevsit^ of Pennsylvania

CONTRIBUTIONS

FROM THE

Department of Neurology

'' AND THE

Laboratory of Neuropathology

FOR THE YEAR 1908

(REPRINTS)
VOLUME IV

36 / ^//^

PHILADELPHIA

CONTENTS.

The Cerebral Centers for Taste and Smell and the Uncinate Group of Fits.

Charles K. Mills, M.D. i- H

The Epiconus Symptom-Complex in Cerebrospinal Syphilis.

William G. Spiller, M.D. ' '
Tumors of the Cauda Equina and Lower Vertcbrc-e. .William G. Spiller, M.D.
A New Diagnostic Sign in Recurrent Laryngeal Paralysis.

Alfred Reginald Allen, M.D.
The Relationship between the Spinal Cord, the Sympathetic System, and Thera-
peutic Measures S. D. Ludlum, M.D.

Exaggeration of the Patellar Tendon Reflexes in Acute Anterior Polio-

„-,yelJtis , William G. Spiller, M.D.

Injuries of the Spinal Cord. . .' Alfred Reginald Allen, M.D.

Syringomyelia with Syringobulbia John H. W. Rhein, M.D.

The Operative Treatment of Papilledema (Choked Disk), with Special Refer-
ence to Decompressing Trephining.

G. E. DE Schweinitz, M.D., and T. B. Holloway, M.D.
Hemianesthesia to Pain and Temperature, and Loss of Emotional Expression
on the Right Side, with Ataxia of the Upper Limb on the Left. The Symp-
toms probably due to a Lesion of the Thalamus or Superior Peduncles.

Charles K. Mills, M.D.
The Symptom-Complex of Occlusion of the Posterior Inferior Cerebellar

Artery : Two Cases whh Necropsy William G. Spiller, M.D.

Polioencephalitis Superior, with Report of a Case with Autopsy.

E. H. Krumbhaar, A.B.
The Symptom-Complex of Transverse Lesion of the Spinal Cord and Its Rela-
tion to Structural Changes Therein Alfred Reginald Allen, M.D.

A Case of Apraxia, with Autopsy : John H. W. Rhein, M.D.

Delayed Apoplexy (Spatapoplexie), with the Report of a Case.

Alfred Reginald Allen, M.D.

Psychotherapy: Its Scope and Limitations Charles K. Mills, M.D.

The Symptom-Complex of a Lesion of the Uppermost Portion of the Anterior
Spinal and Adjoining Portion of the Vertebral Arteries.

William G. Spiller, M.D.
Hemorrhage into Ventricles: Its Relation to Convulsions and Rigidity in

' Apoplectiform Hemiplegia Alfred Reginald Allen, M.D.

Acquired Spasticity and Athetosis William G. Spiller, M.D.

Softening of the Dentate Nuclei causing Symptoms of Cerebellar Tumor.

William G. Spiller, M.D.
A Brain Tumor Localized and Completely Removed, with some Discussion of the
Symptomatology of Lesions variously Distributed in the Parietal Lobe.

Charles K. Mills, M.D., and Charles H. Frazier, M.D.

iii

iv CONTENTS

Osseous Plaques of the Pia-Arachnoid and their Relation to Fain in Akro-

megaiy ^- Leopold, AI.D.

Pathologic Report of the Nervous System in a Case of Spondylose Rhizo-

melique John H. W. Rhein, M.D.

Acute Anterior Poliomyelitis: A Pathological Study of Three Cases.

Williams B. Cadwalader, M.D.

The Post-Graduate Student William G. Spiller, M.D.

Idiopathic Epilepsy Complicated by Motor Aphasia and Diplegia, with Necropsy.

Williams B. Cadw.\lader, M.D.
Cerebellar Symptoms in Hydrocephalus; with a Pathologic Report of a Case

Associated with Syringomyelia John H. W. Rhein, M.D.

Hemiplegia with Paralysis of the Neck Muscles from a Small Myelitic Lesion.

William G. Spiller, M.D.
The Association of Syringomyelia with Tabes Dorsalis.

William G. Spiller, M.D.

Adenolipomatosis, with the Report of a Case Charles K. Mills, M.D.

Tumor of the Gasserian Ganglion. A Report of Two Cases with Necropsy.

William G. Spiller, M.D.
The Duration of Life after Extensive Hemorrhage of the Brain.

William G. Spiller, M.D.
Tumor Malformations of the Central Nervous System.

William G. Spiller, M.D.
Inferior Polio-encephalitis in a Child of Four Years, with Recovery.

Charles F. Judson, M.D., and Horace Carncross, M.D.
Hydrocephalus S. D. Ludlum, M.D.

Most of the reprints obtained for this volume were destroyed by fire, and it has
been necessary to reset a large portion of the type.

THE CEREBRAL CENTERS FOR TASTE AND SMELL AND
THE UNCINATE GROUP OF FITS,

Based on the Study of a Case of Tumor of the Temporal
Lobe with Necropsy ^

By Charles K. Mills, M.D.
professor of neurology in the university of pennsylvania) neurologist to

THE PHIL'ADELPHIA GENERAL HOSPITAL.

The case which forms the text of this article was first studied by me
in April, 1904, although I had received communications regarding the
patient from her husband, a journalist, and had seen her once or twice
before this date.

The patient was a married woman, aged 34 at the time of my first examina-
tion. She was the daughter of first cousins. No history of epilepsy in the
family was known. One sister had chorea when a child, but entirely recovered
and grew to womanhood without any further evidence of nervousness. Another
sister has had some sort of spasm at intervals. The patient had had fair health
up to the age of 30 years, although she had suffered considerably from headache
at irregular, but somewhat frequent intervals from early childhood. Between
one and two years before coming under observation her headaches had become
rather severe, the pain being referred chiefly to the forehead, but sometimes
affecting the back of the head. The patient stated that she remembered when a
child of having had a spell or period of dizziness, which lasted perhaps a
week and was of uncertain origin. In 1899, about a year after the birth of
her first child, she began to have slight seizures of a peculiar character. In these
spells she was said to have nausea, to be somewhat confused, and to articulate
indistinctly or incoherently. They usually lasted about half a minute. She
did not at first become unconscious in them. In October, 1903, she became
unconscious in one of her seizures. The spells without unconsciousness or
spasm which she had continued to have at intervals from 1899 until 1903

^ From the Department of Neurology of the University of Pennsylvania. _
Read in the Section on Nervous and Mental Diseases of the American
Medical Association, at the Fifty-ninth Annual Session, held at Chicago, June,
1908. Published in the Journal of the American Medical Association, Sept. 12,
1908.

2 mills: centers for taste and smell

gradually became more frequent. She sometimes had several daily, and then a
week might elapse without any. In these seizures her face would turn of an
ashen hue and her expression become drawn. The convulsion which occurred
in October was preceded or ushered in by the phenomena of one of her usual
seizures. In this attack some spasm on the right side of the face was noted.
She slobbered and smiled as she recovered from this attack, and for a few
minutes after recovery could not recollect anything. She did not pass into
a sleep after this and other attacks, nor did she bite her tongue in any of them.
A week or two after this convulsion she began again to have her original form of
seizure.

Shortly after the patient first came under my observation in April, 1904, I
made several careful examinations of her. Later I saw her at intervals of
several weeks or months, occasionally examining her as regards her special
senses, sensation, motility, reflexes and mental state. Her husband, who was a
good observer, from time to time until her death, reported on her condition to
me, furnishing special data with regard to her seizures. I did not see her,
however, for about six months before her death. The patient, in describing her
attacks in the spring of 1904, said that she had a sensation of some kind in the
region of the stomach. This was soon followed by a sensation of taste and
then of smell. These were of a definite character in that they recurred in the
same way. She could not tell what the taste or smell was, that is she was not
able to refer it to any particular variety of taste or smell, although she some-
times thought that she was just about to be able to do this. She thought that
the smell was somewhat like that of a flower, but she could not name the
flower. These sensations were in some instances accompanied by smacking of
the lips, or as her husband expressed it, clapping of the lips and champing or
chewing movements. Immediately after these gustatory and olfactorv- aura,
she became more or less dazed, this confusion being worse at times ; in some
instances she would keep right on talking through the spell.

Examination for taste showed that on the back of the tongue she was able
to taste correctly. In the chorda tympani area of the tongue the patient said in
response to the test that she tasted something, but could not distinguish the
quality or flavor of what she tasted. Salt was appreciated as sour or acid on
both sides; sweet was not recognized as such, and bitter substances gave the
same taste as sweet. The tests were made with care, with washings of the
mouth and with intervals between them, so as not to cause confusion by the
mingling of different substances.

Smell was retained. At one of her visits, May 6, 1904. she gave an interest-
ing statement with regard to her abilitj- to smell. She said that a daj- or two
before she had been out in the woods and had tried to smell some little green
bunches of leaves, which she did not know by sight ; by smelling them she
thought they were sassafras but was not sure of it. She picked several flowers
and could smell their fragrance. In cooking she could smell very well, dis-
tinguishing the different substances which were being cooked. Examination of
her nostrils for motor power by having her sniff with them, with or without
the examiner's finger on them, showed undoubtedly that more force was exerted
by the right nostril than by the left. She spontaneonsly spoke of this difference.

mills: centers for taste and smell

She said that she could sniff much better with the right than with the left
side of the nose. The left nostril seemed smaller and thinner than the right.
The tongue was protruded in the median line, was not atrophied, and had no
fibrillary tremor. All its voluntary movements could be performed. The left
half of the tongue seemed to be a little smaller and flatter than the right. The
left pupil was slightly large than the right ; both pupils responded to light and
in accommodation. No nystagmus w-as present and no external ocular palsies.

Fig. I. Photograph of a section at the upper part of the basal ganglia,
showing the tumor invading the left thalamus, filling the front part of the
posterior horn of the lateral ventricle and implicating the extreme anterior part
of the median aspect of the occipital lobe.

Before coming under my care this patient had been seen by Dr. C. A. Oliver
of Philadelphia, who has kindly furnished me with the following report : " At
14 years of age the patient accidentally discovered that the vision of her left
eye was not so good as its fellow. At that time, glasses, which were worn
comfortably for six years, were ordered by a well-known ophthalmologist. In
1900 she saw another ophthalmologist who operated on the left externus, and
two j'ears later on the right externus. The exterior eye muscles were exercised
and iodid of potassium was given. When first seen by me, on Dec. 2, 1903, the

mills: centers for taste and smell

patient had normal vision for both form and color in each eye, the right eye
being far-sighted and astigmatic and the left eye near-sighted and astigmatic.
The left pupil was the larger (4V2 mm.), while that of the right was 3 mm. in
size. The irides were equally and freely mobile to light stimuli, accommodation
and convergence. The actions of the exterior ocular muscles were good in
every direction with proper muscle equilibrium. The visual fields were normal.
The patient's husband informed me that the patient had had epileptic seizures
with olfactory aura, and that odors tended to give rise to the ' spells.' A diag-

Fig. 2. Photograph of a section at a level of about 5 of an inch below that
represented in Figure i, showing the tumor extending to the anterior and median
surfaces of the occipital lobe and involving almost all of the lenticula.

nosis of focal epilepsy and antimetropia (the ophthalmic signs and symptoms
being negative), with a suspicion of a focal cerebral lesion, which was either a
tumor or a localized meningitis, was made. It was also noted that the patient
apparently improved after her tenotomies."

In May, 1904, careful examinations were made for sensation, motility and
reflexes in all parts of the body, the results being almost entirely negative.
Movements of the muscles supplied by the motor division of the trigeminus and
by the seventh nerve, with the exception of one nostril, as above indicated, were

mills: centers for taste ani-) smell 5

not impaired. No ataxia of station or gait was present and no paralysis and
no paresis or ataxia of the extremities. Sensation was everywhere preserved.
The reflexes also were preserved ; the knee jerks, however, were depressed, more
markedly on the left. This may have been due to the use of bromids, which had
been administered for several months. The bowels and bladder were unaffected.

Fig. 3. Photograph of a section at a level of about i of an inch below that
shown in Figure 2. The tumor fills all the anterior and median portions of the
temporal lobe (uncinate and hippocampal convolutions) extending into the mid-
dle part of the lobe, including the subcortex of the fourth temporal ; it also
slightly invades the extreme anterior part of the occipital lobe.

The general condition of the patient was good. Her mentality was fully re-
tained and no aphasia was present. The patient was perhaps at times a little
apathetic, but on the whole was in good spirits. She seemed at times in a state
of dreaminess.

My last examination, which was made in August, 1907, never showed any
paralysis, disorder of sensation nor hemianopsia. Her husband stated that she
was never paralysed in her face, arm, leg or in any part of her body. Inquiring
with regard to hemianopsia, he said that she never appeared to be blind or
partially so, either to the right or to the left. She did not run up against
things to either side. The only visual phenomenon which he observed was that
she said if she looked a certain way to one side she felt as though a ^pell
were coming on.

6 MILLS : CENTERS FOR TASTE AND SMELL

Under the influence of a combination of sodium iodid, sodium bromid,
Fowler's solution and fluid extract of conium, the number of the patient's attacks
considerably decreased. She continued, however, to have them at varying in-
tervals of days, weeks or 'months as long as she remained under my direct
observation, which was during 1904 and 1905. Intelligent reports of her attacks
and of her condition during the intervals were made by her husband during
this time, and for the record of her condition for a year before her death I
am entirely dependent on these reports.

Her husband reported that in August, 1906, she began to run down and that
her speech was incoherent, with decided aphasia. Whether this was in a tech-
nical sense aphasia is doubtful. The patient evidently had some difficulty in

.. I

Fig. 4. Photograph of the base of the brain, showing the tumor extending
as a pointed process over the left cerebral peduncle. The line shows the posi-
tion of the tumor.

remembering words and some slowness and hesitation of speech. Her general
health declined so that her weight dropped from 140 to 119 pounds. She came
home from her vacation in the summer a physical and almost a mental wreck.
She would get out of bed at night unable to tell which way to go to the door.
In September, 1906, all medicine was stopped and her old spells began to be more

mills: centers for taste and smell /

severe and frequent, but she recovered her general tone and her speech. When
she was what was termed " aphasic " she would forget words. She would

come into a room, for instance, saying " Where are the " and after some

hesitation she would say, " Oh, I mean the scissors." At other times she would
substitute wrong words. The patient recovered sufficiently from this so that
one would hardly notice any difference between her speech and that of a person
who was slightly forgetful of words. She went along about the same until
March, 1907. The spells usually occurred in the early morning and her husband
would be awakened by a clapping of her lips. One morning on hearing this
noise he spoke to and touched the patient, who then went into a general
convulsion.

She was last seen by me in August, 1907. She was at this time very sleepy
and drowsy. At the time of her visit to my office she lay down on the sofa
and went to sleep. She had at times been taking two or three doses of bromid
daily. Little change occurred in her condition until about December 26 or 27,
about two months before her death. She was taken at this time with what was
said to be influenza and she was treated for such principally with remedies like
phenacetin, quinin, strj-chnin, etc. All bromids were stopped at this time. Be-
tween January 22 and the date of her death, February 22, she had perhaps
six of the following peculiar spells : She would throw up her hands as if in
great pain or distress, but could not tell where, except that at the same time
she would say that the left side of her body felt numb or asleep or dead. She
was not unconscious, but the feelings and condition would last two or three
minutes. The morning before her death she had a severe spell. The morning
she died she lapsed into unconsciousness and never recovered.

Necropsy having been permitted. Dr. Samuel Leopold, of the clinical and
pathologic staffs of the neurologic service of the University of Pennsylvania,
made a postmortem examination and obtained the brain. The specimen was
placed in the hands of Dr. William G. Spillcr for investigation in the laboratory
of neuropathology of the University of Pennsylvania. The following report was
made by Dr. Spiller: "The tumor is on the left side of the brain. The left
cerebral hemisphere is much larger than the right. Li a transverse section at
the level of the upper part of the callosum the tumor fills the lateral ventricle
above the thalamus, extending to the roof of the ventricle and cutting off the
anterior part of the ventricle above the head of the caudate nucleus from the
posterior part of the ventricle. The tumor has the appearance of a glioma,
is infiltrating and gelatinous in places. The upper part of the thalamus is
entirely replaced by it. At a little lower level, where the anterior and posterior
limbs of the internal capsule are well formed, the tumor invades the thalamus,
fills the front part of the posterior horn of the lateral ventricle, implicates the
extreme anterior part of the median aspect of the occipital lobe, distorts the
outer wall of the posterior horn of the lateral ventricle without extending into
the temporal lobe, and involves the posterior part of the posterior limb of the
internal capsule (Fig. i) and a large part of the lenticula.

"At a level about one-fourth of an inch lower than the one above described
the tumor extends to the anterior median surface of the occipital lobe, involves
almost all of the lenticula, but does not extend into the white matter of the

8 mills: centers for taste and smell

temporal lobe nor into the head of the caudatum, nor into the foot of the
cerebral peduncle, which just below this level is beginning to form (Fig. 2).

" At a level about one- fourth of an inch still lower, where the cerebral peduncle
is well formed, the tumor fills all of the anterior and median portions of the
temporal lobe (uncinate and hippocampal convolutions) and the middle part of
the lobe, but leaves the occipital lobe intact except in its extreme anterior part,
and does not invade the lateral aspect of the temporal lobe or its subcortical
white matter (Fig. 3). The tumor extends as a pointed process of brain tissue
over the left cerebral peduncle (Fig. 4) this being the only portion of the tumor
appearing on the surface."

It will be noted in the description of the position and extent of the
lesion in this case, that it involved the uncinate, hippocampal, and the
fourth temporal convolutions and their subcortical white matter, but
did not extend further into the temporal lobe. It is probable that
the gustatory and olfactory discharges were due to the implication
of the cortex in these regions. Of course, it cannot be overlooked that
the tumor extended considerably beyond the lower temporal region,
invading the anterior part of the occipital lobe and destroying large
portions of the lenticula and the thalamus. But with our knowledge
of previous cases, and with the experimental evidence regarding the
cortical localizations of taste and smell, the findings in this case may
be regarded as confirmative of the view that the olfactory and gusta-
tory centers are located in the inferior portion of the temporal lobe.
In fact, little doubt exists as to olfactory cortical localization, but the
case is not the same with regard to the centers for taste. It may, I
think, eventually be demonstrated that the hippocampal convolution
is the chief cortical area for taste.

So far as the recorded notes show, this patient had no visual phe-
nomena, with the exception that when she looked in a certain direction
she felt as if an attack were coming on. No sensations of light, and
no hemianopsia appear to have been present. The sensory phenomena
may have been due to the involvement of the thalamus and the ex-
treme posterior portion of the internal capsule.

When this case was seen by me, I regarded it as probably one of
tumor in the early stage of development, situated in the uncinate region
and especially involving the gustatory area. The further history of
the case and the necropsy show that this diagnosis was probably cor-
rect, for although the glioma involved at the time of death, as has just
been detailed, a much larger area of the brain, both cortical and sub-
cortical, than is presumably connected with the representation of taste

mills: centers for taste and smell 9

and smell, it is probable that it developed from the inferior anterior
aspect of the temporal lobe, later implicating the occipital lobe, the
cornua of the ventricles, and the basal ganglia, as shown by the
sections made at different levels. A glioma so situated could not have
been reached by the surgeon.

To J. Hughlings Jackson- we owe the idea that visceral, and espe-
cially digestive sensations have their cortical representation in the
inner convolutions of the temporal lobe, that is, in the uncinate and
hippocampal gyri, and probably in the adjoining anterior portions of
the fourth temporal convolution. It would appear from the view
which this neurologist has expounded and which he has illustrated
by well studied cases, that discharging lesions of this portion of the
brain give rise to crude sensations of smell and of taste, to gastric and
gastrointestial sensations, to movements of the lips and nostrils, such
as smacking or clapping or sniffing, and movements connected with
mastication, deglutition and digestion, such as champing, swallowing,
and stomachic and intestinal twisting. In addition he found associated
with these sensations and movements, apparent alterations in the size
and distance of external objects, and above all a peculiar intellectual
aura which he designates as the " dreamy state.'"

Herpin^ in his writings on epilepsy, to which Jackson refers at
length in one of his articles, drew attention to these sensations of odors
and to the peculiar intellectual aura sometimes presented by such
patients. How frequently gastric sensations and chewing movements
are spoken of by patients, every experienced neurologist must recall.
He will remember also to his regret, when he seriously takes up the
subject in describing a case of peculiar interest, like the one here pre-
sented, how often he has neglected to give the consideration which they
deserve to these phenomena.

So few have been the recorded cases with necropsy, in which phe-
nomena of taste and smell have been present as the aura of the motor-
epileptic seizure, or with the addition of a dreamy or other peculiar
mental state as the sole or chief phenomena of the epileptic attack,
that the case given in this paper may be regarded as of considerable
value.

A discussion of this case brings before us Hughlings Jackson's

- Medical Times and Gazette, 1879, i; Brain, July, 1880, and July, 1888; Lancet,
Jan. 14, 1899, 79.

' On Epilepsy, 1852, p. 275.

10 mills: cexters for taste axd smell

uncinate group of fits. He has described these fits as occurring as
the result of a cortical discharge of the uncinate gyrus or its neighbor-
hood, the patients having, seizures with evidences of gustatory and
olfactor}' sensations and other phenomena such as those above de-
scribed. The cases with necropsy are so few that Purves Stewart*
was able in 1899 to collect only six. In his enumeration he omitted
the case of Worcester.^ which was given by me in my paper on " Cere-
bral Localization in Its Practical Relations." published in 1888, and
also in my book on " The Xervous System and Its Diseases."

This case was that of a farmer, aged 30, who had had epilepsy for
two years before he came under Worcester's notice. The case pre-
sented no special features until January 26. 1878, when after a severe
convulsion the man remained in a state of alarming collapse. He
remained for three days in stupid condition. Shortly after the attack
slight interference with innervation of the right side of the face was
observed when the patient was talking or smiling. On February 1 1
he regained his ordinary mental condition. Xo paralysis was dis-
covered except as above mentioned, and no impairment of sensibility
except a transient numbness of the hand at times. For several days
hallucinations of smell — at first constant, afterwards transitory — were
present. Once he imagined the room was full of smoke. He fancied
at times there was an odor like the vapor of alcohol passing quickly.
He thought this took the place of a convulsion. X'^o test was made
of his taste or smell. X'o marked changes occurred until his death
on February' 28, after a series of tonic convulsions, with opisthotonos.

At the necropsy inspection revealed a small red spot of softening
at the most prominent point of the left gyrus uncinatus. After the
brain had been hardened in alcohol a focus of softening existing in
the white matter of the anterior part of the left temporal lobe was
revealed. This extended to the surface externally, and internally in-
volved the pes hippocampi in the floor of the descending cornu of the
lateral ventricle. The portion of the hippocampus major not dis-
colored was swollen and softened. A very small focus of softening,
without discoloration, about the size of a large pea. was found in the
white matter of the frontal lobe on the same side. Xo other gross
lesions were discovered.

Two of the cases collected by Purves Stewart and recorded by

* Brain, 1899, xxii, 534.

"Amer. Jour. Insanity, July, 1887.

mills: centers for taste and smell H

Jackson and Stewart, those of Anderson" and Hamilton," were referred
to in the contributions already cited. These six cases, given mainly
in the language of Jackson and Stewart, were as follows :

Case i.— Drs. liughliiigs Jackson and Beevor' recorded a case of a sarcoma,
the size of a tangerine orange, situated at the most anterior extremity of the
right temporo-sphenoidal lobe. During life the patient had left hemiplegia
without hemianopia, hemianesthesia or afifection of smell, taste or hearing. She
also had numerous fits with an "intellectual aura" and a "horrid smell of dirty

burning stuff."

Case 2.— Drs. Hughlings Jackson and Colman" also recorded a case of a small
subcortical patch of softening in the left uncinate gyrus. The patient had been
subject to epileptic attacks accompanied by a "dreamy" state, with smacking
movements of tongue, lips and jaw, but without crude sensations of taste or

smell.

Case 3.— Dr. James Anderson" published a case of a large basal cystic
sarcoma arising from the pituitary body and spreading into the left temporo-
sphenoidal lobe. It had given'rise to attacks of peculiar sensation in the right
hand, preceded by a sensation of a bitter taste in the mouth, with occasionally
also a peculiar smell, and accompanied by the " dreamy state." There were never
any chewing or smacking movements of the lips. Smell was impaired on the
left side, and there was slight defect of taste on both sides, especially the left.
From afifection of the left optic nerve, optic chiasma and optic tract, the patient
had blindness of the left eye with temporal hemianopia in the right.

Case 4.— Mr. Nettleship" also recorded a case of tumor of the pituitary body
implicating the left optic nerve, chiasma and tract, as well as the left temporo-
sphenoidal lobe. In that case there had been paroxysmal sensations of suffoca-
tion in the nose and mouth.

Case 5.— Sanders'' recorded a case of a large glioma on the under surface of
the brain, involving the anterior part of the left temporo-sphenoidal lobe and
the lower convolutions of the left frontal lobe and destroying the left olfactory
tract. The patient had fits, preceded by a warning of a " dreadful disagreeable
smell," also chewing movements of the jaw and spitting of saliva. Later he
had convulsions affecting the face, but not the limbs. No dreamy state was
recorded in that case.

Case 6.— Dr. McLane Hamilton'' also published a case of localized chronic
pachymeningitis affecting the right uncinate gyrus and part of the adjaceni con-
volutions, but without involvement of the olfactory bulbs. The patient had been
subject to attacks, preceded by a peculiar disagreeable odor, either of smoke or
of fetid character.

'Brain, 1886, viii.

'New York Med. Jour., 1882, xxxv, 575

^ Brain, 1889, xii, 346-357-

"Brain. 1898, xxi, 580.

'» Ophth. Soc. Trans., iv, 285.

"Archiv f. Psychiat., 1874. iv. 234.

12 mills: centers for taste and smell

Linde^- has recorded a case of tumor of the left hippocampal gyrus
and uncus in which haUucinations of smell were present; also loss of
the right half of the visuaj field in the right eye and loss of the
pupillary reflex to light in the same eye. In a paper by Southard/^
his second case had among other manifestations, chewing movements.
The history of the case was a not unusual one of an epileptic becoming
insane, and the autopsy showed as the major lesions pachymeningitis,
especially of the vertex, but spreading in many directions.

The left temporal convolutions were distorted opposite the chiasm.
This latter process w^as merely incidental to the distortion of structures
consequent on an aneurism imbedded in tissue below the left uncus,
the substance of which appeared to have been largely destroyed by
the aneurism, but remains of it, together with a portion of the superior
temporal gyrus, could be made out in that portion of the brain sub-
stance which had been displaced outward and downward by the lesion.
The lenticula had been displaced outward and upward, but appeared
to have undergone no diminution in size.

A considerable number of clinical cases which bear out the sup-
position that the dreamy state with movements of the lips, tongue,
jaws and associated parts, are evidences of a cortical discharge, pecu-
liarly localized, have been put on record. Spiller^^ has recorded
three such cases. In the first of his cases the patient was a woman
27 years old, who had had epileptic attacks since the age of 3 years ;
and the aura of the paroxysms were a bright light in front of her,
objects appearing strange and out of place, sometimes large and
sometimes small, and having the taste of raw, unsalted beef in her
mouth. At this point consciousness was lost.

In the second case, in the attack vision was blurred and the patient
had a " gassy taste.' The attack was ushered in with a creeping
sensation in her left upper limb, which seemed to ascend the limb grad-
ually and to involve the left side of the tongue, so that the tongue
felt thick.

In the third case, the patient, a man 40 years old, for two years had
had at intervals what his wife called " swelling spells " in which he
became unconscious, made the noise and movements of swallowing,
and rubbed his fingers together. These attacks lasted a minute or

'^ Monatschr. f. Psychiat. u. Neurol, vii, No. i, p. 44; cited in Progressive
Medicine. September, 1900, 205.

" Amer. Jour. Insanity, April, 1908, Ixiv.
"American Medicine, March 19, 1904, 474.

mills: cexters for taste and smell 13

two. In this case the patient after a very severe convulsion was
paralyzed in his right upper extremity, which was the seat of great
pain. Power was gradually regained. As the paralysis disappeared
in the right limb, he became weak in the left, in which pain was felt
on movement. Interesting phenomena in this case were appearances
of subcutaneous hemorrhages in the conjunctiva, in the face and about
the right shoulder, and in a subsequent attack much like the first the
patient had severe hemorrhages in the right side of the neck, right
shoulder, and right upper limb. In a third severe attack other
hemorrhages occurred. Between his attacks or as a part of them
he had swallowing spells. Although this patient had the swallowing
spells, he had never had any phenomena indicating sensations of taste
or smell. Spiller regarded the case as belonging to the uncinate group
of fits.

Gowers,^^ out of 119 cases of epilepsy in which special sense aurje
were present, found 7 olfactory and i gustatory. In regard to cases
of epilepsy with a gustatory aura, Spratling^" cites a case in which the
patient stated that he almost always had light attacks following severe
ones at night, and following the light attacks he had a peculiar taste
and sensation in the mouth which he described as follows :

The first is a " sour taste " ; the second similar to that of " wheat bran in the
mouth " ; the third, a feeling of " stringiness," combined with " numbness in the
upper part of the mouth " ; the fourth, a " sickish, sweet taste " sufficietit to
produce extreme nausea ; the fifth a " filthy, nasty taste," which is extremely
disagreeable; the sixth and last being a feeling of " sliminess " in the mouth.
The morning following the patient feels " unusually well," much more so than
during any time more distant from his seizures. These disorders of taste may
continue for a day and gradually shade out from the sixth condition to that of
normal sensation. They have persisted for years and can not be ascribed to
stomachic indigestion.

Spratling in his record of cases which he regards as illustrations of
the psychic epileptic equivalent, gives the record of the case of a
man 40 years old, a commercial traveller, who kept a diary of his
movements for several months, this being interesting in various par-
ticulars, as for example, in the fact that he wrote down accounts of
the seizures which he had suffered, and also of other occurrences, for
which for one period of several weeks of the time in which he kept
this diary, he had no recollection or only for some of the events a dim

'^Epilepsy and Other Chronic Convulsive Diseases. William Wood & Co.,
1885.

" Epilepsy and Its 1 reatmcnt, W . B. Saunders & Co., 1904.

14 mills: centers for taste axd smell

recollection. Two of the attacks which were noted during this time
were accompanied by chewing movements, in one of which he chewed
a thermometer and spat ou,t the pieces of glass, of this seemingly
having had some faint recollection. In another, the record is made
of an " epileptic attack between 4 and 5 p. m. Chewing severe."

Spratling does not discuss in detail the views of Hughlings Jackson
as to the " dreamy state/' although he speaks of its occurrence and
records two or three instances taken from L. Pierce Clark.

William Aldren Turner^' in his monograph on epilepsy, discusses
certain dream states as distinct from the dreamy state described by
Hughlings Jackson as frequently showing itself as an aura in con-
nection with crude sensations of smell and taste. While these dream
states may occur more or less paroxysmally, they are especially
interval phenomena. They are to be regarded, as Janet has regarded
them, as psychasthenic phenomena. In them the patient suffers from
a sense of unreality of the things around him. and from a distressing
disturbance of his usual mental equilibrium. They are subjectively
so painful at times that the patients express a feeling of dissatisfaction
or worse, because they have replaced the paroxysms which, although
transiently more severe, leave a clearer and firmer mental atmosphere.
Such cases have been described by Crichton-Browne, Pick, Janet,
Turner, and others, and must not be confounded with cases showing
the dreamy state as described by Hughlings Jackson as one of the
aura in the uncinate group of fits. It is probable, however, that the
dream state just described, and the dreamy state of Hughlings Jackson,
have something pathogenetically in common, although the psychas-
thenic dream state may be more dependent on the deteriorative cerebral
condition of the patient than on the discharge of a more or less
localized lesion.

Turner in discussing the special sense auras, refers to sensations of
taste and smell, and epigastric sensations, with or without the dreamy
state, following Hughlings Jackson in his description of the uncinate
group of fits. He gives one case in whicli " there was described a
' smell of spring ' which occurred both as the representative of the
minor attacks and as the aura of the complete seizure. In the subse-
quent course of the case the aura was no longer present."

Spratling seems to take the ground that the epigastric aurc-e which
he enumerates and describes in fifty cases were in some way dependent

" Epilepsy, Study of the Idiopathic Disease, Macmillan & Co., 1907.

mills: centers for taste and smell J5

on conditions of indigestion or special disturbance in the gastro-
intestinal tract, in this regard differing from Sir William R. Gowers,
and also from Hughlings Jackson. This way of looking at the
subject has practical enticements. It helps to support the view that
the epileptic attacks can be prevented by close attention to the gastric
or gastrointestinal state. It is probably true that the condition of
the alimentary canal, especially the presence of fermentative disorders
in connection with constipation and imperfect or halted digestion,
should be corrected as far as possible with the hope of doing some-
thing toward the relief of the epilepsy and especially toward decreasing
the number of the seizures.

^ly own observation, however, inclines me to the view that the
epigastric aura is only a part or at least a manifestation of the localized
cerebral discharge, that it is due, in other words, to a discharge lesion
which affects the cortical centers concerned with the representation
or control of the sensory or motor or both sensory and motor side of
the epileptic syndrome. Many facts would tend to indicate the truth
of this view, among these being those which show the frequency of
epigastric with gustatory and olfactory phenomena in reported cases.
It is true that these gustatory and olfactory phenomena are often
absent. It is nevertheless probable that sensory and motor centers
concerned in some way with gastrointestinal interpretation have a more
or less separate existence, and are situated in the same region of the
brain as are the cortical centers for taste and smell.

In the study of cases illustrating the uncinate group of fits, especial
attention should always be given to the respiratory phenomena. In
the case of the patient whose history is recorded in this paper, some
peculiarities of respiration in the attacks were noted. At least the
patient's appearance was such as to indicate the occurrence of light
asphyxia or interference with respiration and cardiac action. Her
face would almost invariably turn of an ashen hue. In the severe
attacks her face not only Ijecame pale as in the light ones, but also
became congested.

Hughlings Jackson has especially called attention to states of slight
asphyxia which are sometimes observed in the uncinate seizure. In
one of the cases recorded by him the patient's face turned blue, or as
he quotes the paradoxical expression of some one who observed the
attack, " dark pale." He advanced the suggestion that this partial
asphyxia and change of color is due to spreading of the discharge

16 mills: centers for taste and smell

lesion in the uncinate g}'rus to Spencer's respiratory arrest center, and
from this center to the respiratory centers in the oblongata. He speaks
of the probable occurrence of writhing movements of the arm in
these uncinate respiratory attacks, saying that they are "' the conse-
quence of (the physical condition for) suffocation."

He cites from Herpin the account of a patient who had attacks with
epigastric sensations, champing movements, and slow irregular move-
ments of the arms.

He also illustrates his opinion by another case of his own. This
patient, a man of 52 years, had a " funny feeling " which was not
true vertigo; of this feeling he said "I can't tell what it is.'' This
feeling might or might not have been the dreamy state. His wife
knew when his attacks were coming on by a noise he made, smacking
his lips as if tasting to indicate what this noise was. When the noise
stopped the patient's lips turned blue : his eyes were half closed. His
arms dropped limp, not stiff'. He came round by drawing a long
breath, then breathed easily and seemed dazed and muddled. He had
a nasty taste in the mouth after the attacks. Says Jackson :

Smacking movements of the lips, nasty taste, dreamy state, if there was one,
and " turning blue " may seem at first glance to have no sort of relation to one
another. I submit that thej^ have an association in the sense that there is one
discharge-lesion of some cells of the uncinate g}'rus and that there is spreading
of the discharge from this focus. I believe, too, that the dreamy state (which
I have said often occurs in this group of fits, although perhaps not in the case
just narrated) is a consequence, a very "indirect consequence, of a discharge-
lesion so situated.

The observations made by W. G. Spencer,^^ to which Jackson refers,
were " on the effect produced on respiration by faradic excitation of
the cerebrum in the monkey,' dog, cat, and rabbit." This excitation
which produced arrest of respiration, was of a particular spot which
Spencer describes as " situated in all the animals examined to the outer
side of the olfactory tract, just in front of the junction of the tract
with the uncinate. And this arrest can be constantly obtained and the
experiment repeated again and again under certain conditions. . . .
This arrest in the monkey was nearly always in expiration, but only
rarely was any active respiration seen."

Some work remains to be done regarding the localization of func-
tions in the inferior portion of the temporal lobe and the orbital sur-

" Trans, of the Royal Society, vol. clxxxv, 1894, pp. 609 to 657.

mills: centers for taste and smell 17

face of the frontal lobe. Cases like the one here first recorded, and the
others to which reference has been made, are of value in the solution
of the physiologic problems concerned with these portions of the brain.
The indications are all in favor not only of the location in the uncinate
region of the centers for smell and taste, but also for the representation
in this region and its vicinity of the sensations produced by the ac-
tivities of the abdominal and thoracic viscera.

It will be seen that an epigastric sensation was a portion of the aura
in the case here reported. Similar sensations have been present not
only in a few recorded cases in which gross lesions have been found
at necropsy in the inferior temporal region, but also in many cases
of so-called indiopathic epilepsy. Other sensations, processes, or phe-
nomena, connected in some way with the functions of the great abdom-
inal and thoracic viscera have been recorded as occurring in patients
with lesions in the part of ^he brain under discussion. Voracicyjs
hunger and thirst, for example, have been noted in cases of abscess
and other lesions of the temporal lobe (Hughlings Jackson. Purves
Stewart, and Stephen Paget).

THE EPICOXUS SYMPTOM-CO^IPLEX IX Cl'lREBRO-
SPIXAL SYPHILIS^

By William G. Spiller, ]\I.D.

PROFESSOR OF NEUROPATHOLOGY, AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE

UNIVERSITY OF PENNSYLVANIA; NEUROLOGIST TO THE PHILADELPHIA

GENERAL HOSPITAL, PHILADELPHIA.

It is not often that the symptoms of an epiconus lesion are caused
by syphihs, and yet a case has been observed by the author in which
the diagnosis had to be made between syphiHtic multiple neuritis and
a lesion of the epiconus or its roots. The existence of the former
condition is questionable. Remak thinks it is not yet positively deter-
mined whether syphilis may cause polyneuritis, although it is probable.
Flatau refers to a case studied by Oppenheim and Siemerling in which
the saphenous major and the cruralis nerves showed a slight decrease
of nerve fibers and a slight increase of the endoneurium. The radial
and peroneal nerves were intact. This is the only case with necropsy
he mentions, but he states that pathologic anatomical findings in a case
of pure syphilitic polyneuritis have not been obtained. Implication
of the cranial nerves is common in syphilis of the brain and is caused
by the syphilitic meningitis, a similar involvement of the spinal roots
from syphilis of the spinal cord also occurs frequently.

Remak says that Ehrmann has observed cases of neuritis nodosa
on a syphilitic basis, and he (Remak) has seen painful swellings of
nerves especially of the ulnar, radial and peroneal nerves, and swellings
of the brachial plexus in cases of localized syphilitic neuritis. In a
case of brachial neuritis on a syphilitic basis studied with Westphal,
the nerves were hard to the touch, but Remak remarks that such
swellings occur in nonsyphilitic cases and can not always be attributed
to the syphilis. In cases in which the neuritis was supposed to be
produced by syphilis there were other signs or history of syphilis, or

^ From the Department of Neurology and the Laboratory of Neuropathology
of the University of Pennsylvania, and from the Philadelphia General Hospital.

Read at the General Meeting of the ^ledical Society of the State of Penn-
S}-lvania. Reading, September 23-26, 1907.

Published in the Pennsylvania Medical Journal, Jan., 1908, and in the Review
of Xeurologj- and Psychiatr}-, 1908.

1 18

spiller: the epiconus symptom-complex. 2

improvement from antisyphilitic treatment. Syphilitic neuritis is rare,
whereas other manifestations of syphilis are common. A few clinical
cases of syphilitic mononeuritis are referred to by Remak.

A brachial neuritis may be simulated by pressure on the plexus by
enlarged syphilitic glands. Bilateral symmetrical paralysis may be
caused by implication of the vertebr?e or meninges, as in a syphilitic
case observed by Remak, in which the circumflex nerve was paralyzed
on each side and the fifth and sixth cervical vertebrae were thickened.
Primary bilateral syphilitic brachial neuritis may occur, in Remak's
opinion, and he refers to a clinical case of Leyden's as an example.
He refers to clinical cases of syphilitic polyneuritis reported by Gross,
Oppenheim, lUizzard, Fordyce, Taylor, Schlossberger, Sorrentino,
Perrero, Brauer, Spillman and Etienne, Crocq, and Fry.

Some so-called cases of syphilitic neuritis may have been caused by
the mercurial treatment. In Brauer's case the treatment with mercury
had been employed, and Brauer was uncertain whether both the
mercury and syphilis together caused the polyneuritis, or whether
other causes existed. Mercury had been employed five weeks before
the symptoms of polyneuritis appeared. Neuritis was found by
microscopical examination and some of the cells of the anterior horn
were vacuolated.

Cestan, in 1900, reported two clinical cases of syphilitic polyneuritis
and collected eleven cases he found in the literature. In both his cases
the neuritis occurred very soon after the infection. Of the cases he
collected, only two were with necropsy (Brauer, Kahler). In regard
to mercury being the cause of the polyneuritis, he refers to the fact
that Lewin observed only once symptoms of neuritis in 8000 cases of
syphilis treated by injections of the bichlorid. Cestan's two cases
were without sensory involvement, and suggested very much the form
of neuritis seen in lead palsy, inasmuch as the symptoms were purely
motor, and in the first case the paralysis was confined to the upper
limbs and was most pronounced in the extensors of the hands. It
is possible, I think, that the symptoms in these cases were caused by
lesions of the spinal cord and not by peripheral neuritis.

Oppenheim in the fourth edition of his text-book, page 537, says
that Schultze, Buzzard and he (Oppenheim) have described cases of
syphilitic polyneuritis, and Cestan recently has also reported unc|ues-
tionable cases. He acknowledges its existence but speaks of it as a
very rare affection.

3 spiller: the epiconus symptom-complex.

In the discussion following the report of Fry's case of syphihtic
mukiple neuritis before the American Neurological Association, Dana,
Starr, T- I- Putnam and Leonard Weber said they had never seen a
case of syphilitic multiple neuritis. Starr in the first edition of his
text-book, published in 1903, expresses himself a little more guardedly,
and says there is a certain probability that some of the cases were of
syphilitic origin, but the condition is extremicly rare. At a discussion
of the New York Neurological Society it was found that no one had
seen a case of multiple neuritis undoubtedly syphilitic.

The case that forms the subject of this paper is as follows:

The patient, a colored man, admitted syphilitic infection. He denied alcohol-
ism except that he had occasionally taken a little beer. He entered the Phila-
delphia General Hospital, July 2-j, 1905. About three months previously pain
had been felt in the back on the left side low down near the os innominatum.
Numbness and pain were then felt in the left lower limb, especially severely in
the calf. When he entered the hospital he moved the left lower limb in walking
as in foot-drop. The power of extension of the left foot was impaired. No
tenderness was felt over the nerve trunks, and the patellar reflexes were pre-
served. The voluntary movement of all the limbs at this time was good,
except in the dorsal flexion of the foot. When the sole of the right foot was
irritated flexion of the toes was produced, but irritation of the sole of the left
foot caused no response. Ankle clonus was not obtained. The patellar tendon,
triceps and biceps tendon, cremasteric and epigastric reflexes were preserved and
equal on the two sides and about normal, indeed, the patellar reflexes seemed
a little prompter than normal. Achilles jerks were not obtained.

The pupils were unequal, the right being the larger. Reaction to light was
absent, but contraction in convergence was preserved. The extraocular muscles
were normal. The tongue was not affected. The functions of the bladder and
rectum were not disturbed. Sensation, objectively tested, was normal.

August 12, 1905 : An examination by Dr. William Pickett on this date showed
that the right pupil was myotic, but the light reflex was obtained, and in a
dark room the right pupil' became larger. Dorsal flexion of the feet was
performed only by the tibialis anticus muscles. Tactile anesthesia was present
on the dorsum, outside of the foot, and plantar surface on each side, and on the
lower and outer part of the legs; it was more pronounced in the left limb where
it extended nearly to the knee. The man complained of pain at night nearly
circling the body at the level of the iliac crests. An area of anesthesia was
found near the arm on the left side, about 10 cm. in breadth.

September 14, 1905: Difficulty in talking was observed on this date, and
speech was unintelligible. The man was weak and drowsy. He understood at
times what was said to him, but often failed to understand commands ; for
example, he raised his upper limb when told to put out his tongue. He had
more difficulty in moving his left lower limb. When he was aroused he opened
the right eye but kept the left eye closed, or opened it only slightly and with
effort. He was able to forcibly close the eyelids. The muscles of the facial

spiller: the epiconus symptom-complex. 4

nerve supply were not affected. The tongue was protruded straight. The left
eyeball was not rotated outward on voluntary movement and moved very little
in convergence, and slightly upward and downward.

The patellar reflex was exaggerated on the right side ; on the left side it was
not so prompt but not diminished. Achilles reflex was absent on each side.
The plantar reflexes were preserved.

October 22, 1905 : An examination was made on this date by Dr. J. William
McConnell in Dr. Mills' service. Ptosis of the left upper lid was complete.
The motor fifth and seventh nerve supplies were not affected. The left pupil
was dilated, the right moderately contracted. The light reflex was obtained on
the right side but not on the left side. The left internal rectus and superior
rectus muscles were completely paralyzed, the left inferior rectus and inferior
oblique muscles were weak. Looking far to the left caused lateral nystagmoid
movement.

The upper extremities were not affected as regards motion, sensation and the
reflexes. The extensors of the leg were normal and much stronger than the
flexors of the leg. The flexors and extensors of the thigh and the sartorius
muscle on each side were strong. In the leg the anterior tibial muscle on each
side was the only muscle contrat^ting in voluntary action, and the left was
weaker than the right. The patellar reflex on each side was exaggerated, the
Achilles and plantar reflexes were lost. Both lower extremities, especially
below the knees, were atrophied. Sensation was normal in the right lower limb,
in the right buttock and in the perineum. The man had control of bladder and
rectum. Squeezing the testicles seemed to produce pain.

December 16, 1905 : An examination was made on this date by Dr. J. William
McConnell, and showed that sensation was not affected in any form in the
right lower limb, but tactile sensation was lost in the left lower extremity over
the dorsum of the foot and plantar surface as far as the second toe. The
anesthesia extended on the outer surface of the left leg half way to the knee.
An area of anesthesia was found on the posterior surface of the thigh from
about four inches below to one inch above the gluteal femoral fold, and from
two inches from the perineum almost to the great trochanter. This was the
first time any anesthesia was found in the region of the buttock. In all these
areas of anesthesia sensations of temperature and pain also were lost.

An examination of the eyes made January i, 1906, gave the following results:
O.D.V., 5/5; O.S.V., 5/8; O.D., pupil, 3 mm.; O.S., pupil, 6 mm.; O.D., reaction
free to light and in convergence and accommodation ; O.S., no reaction in any
way; paresis of all ocular muscles excepting the external rectus; slight impair-
ment of levator palpebrie; O.D., media clear, fundus negative; O.S., media clear,
fundus negative.

March 11, 1906, notes were made 1)y me at the time the patient was in my
service. He was shown twice in lectures by me chiefly because of bilateral
peroneal palsy. He was weak in both lower limbs but the weakness was much
greater in the peroneal distribution on each side. He had a steppage gait, and
when sitting with his feet firmly on the floor could not raise the toes well when
the heels were on the ground. The case was striking because of the peroneal
palsy, occurring with cerebrospinal syphilis.

5 spiller: the epiconus symptom-complex.

Notes on condition, March ii, 1906: He is able to pull up his right lower
limb on command but it is impossible to get him to pull up the left lower limb.
Although his stupor is very great he pulls up the right lower limb and prob-
ably therefore has weakness of the left lower limb. When the left lower limb
is pricked with a pin he flexes it somewhat at hip and knee, the limb therefore
is probably weak but not paralyzed, and the test of movement is interfered with
by the stupor. He has marked bilateral foot-drop, and the lower limbs below
the knees are much wasted both in the muscles in the front and back of the
legs. The soles of the feet are also wasted. The lower limbs are abnormally
flaccid, especially the left. When either lower limb is stuck with a pin the
patient gives distinct evidence of discomfort and puts his hand at the place stuck.
The patellar reflex is present but not very prompt on either side, slightly more
so on the left. The Achilles jerk is lost on each side. Babinski's reflex is
absent on each side, the toes not moving in either direction. The cremasteric
reflex is not obtained on either side. The muscles of the calves and the
peroneal nerves are not tender to pressure. He can not be tested for tactile
sensation because of his mental condition. Upper limbs : He can raise the
right upper limb on command but can not raise the left upper limb. He is there-
fore hemiparetic. The biceps tendon reflex and triceps tendon reflex are
prompt on each side, more so on the left. He feels pin prick when stuck in
either upper limb but he can move the left very feebly. The upper limbs are
not wasted. The left side of the face is paralyzed and he does not close
the left eyelids as well as the right. It is impossible to get him to put his
tongue out or to test the movements of his eyes. There seems to be weakness of
the right external rectus but this is not positive.

March 17, 1906: The man was stuporous and breathing was difficult. Bub-
bling rales were heard over the chest. The heart was rapid and weak. He died
on this date.

The necropsy revealed pulmonary hypostasis and edema, chronic adhesive
pleurisy, cyanotic induration of spleen, follicular enteritis, hepatic congestion,
cyanotic kidney, purulent meningitis.

The results of my microscopical examination of the nervous tissue are as
follows :

While sections from the third lumbar region show the cells of the anterior
horns to be normal, with the exception of an occasional diseased cell, those from
the lowest lumbar and sacral regions show these cells intensely degenerated.
The nuclei are displaced to the periphery, some of the cells contain several
vacuoles, chromatolysis is intense, the dendritic processes in many of the cells
have disappeared, and the cell bodies are swollen. The round cell infiltration of
the pia although intense is not any greater at this region than elsewhere in the
cord. Both posterior columns are degenerated in the lower lumbar and upper
sacral regions, but the degeneration is much greater on the left side and is of
long standing, although recent degeneration in both posterior columns especially
the left, is also present, as shown by the Marchi method.

Sections from the mid-thoracic and lower cervical regions show intense
round cell infiltration of the pia and thickening of the pial vessels and degenera-
tion of the columns of Goll, much greater on the left side, and slight degenera-

spiller: the epicoxus symptom-complex. 6

tion of each crossed pyramidal tract. Perivascular round cell infiltration is
also found within the cord.

The round cell infiltration of the pia and the thickening of the arteries is
very intense over the medulla oblongata, cerebral peduncles, chiasm and optic
nerves. The optic nerves are partially degenerated.

The left third nerve is intensely degenerated and a small vessel accompany-
ing the nerve is almost occluded by proliferation of the intima.

The left seventh nerve and sensory part of the left fifth root are also much
degenerated; the motor portion of the left fifth root is only partially degenerated.
The right seventh and third nerves and the root of the right fifth nerve are
slightly degenerated. The contrast afforded by the condition of the two third
nerves is very striking.

Right and left peroneal nerves : Muscle attached to these nerves shows very
intense atrophy, the muscle fibers are small and the connective tissue is in-
creased in amount. The Weigert hematoxylin stain shows considerable de-
generation of the nerves.

Right and left plantar nerves : These are partially degenerated. The muscles
on these nerves are also much atrophied, and their connective tissue is much
increased.

A gumma was found in the right island of Reil.

Summary

A male, syphilitic, complained of pain in the lower part of the back
on the left side about April. 1905. This was followed soon by numb-
ness and pain in the left lower limb especially in the calf. He entered
the hospital July 27, 1905. At that time he presented foot-drop on
the left side. No tenderness was felt over the nerve trunks. The
voluntary movement was good everywhere except the dorsal flexion
of the foot. It is uncertain from the notes whether dorsal flexion
of the right foot was affected at this time. Irritation of the sole of
the right foot caused flexion of the toes ; irritation of the sole of the
left foot produced no movement of the toes. The patellar reflexes
were a little prompter than normal. Achilles reflexes were absent.
The bladder and rectum functionated normally. The pupils were un-
equal and the light reaction was lost. Objective sensation was normal.

On August 12, 1905, the tibialis anticus muscles alone contracted
on attempt at dorsal flexion of the feet, the right also being afifected.
Tactile anesthesia was present on the outer parts of the feet and legs
in the area of the first and second sacral roots ; more pronounced on
the left side. Pain encircled the lower part of the trunk.

On October 22, the extensors of the legs were normal, the flexors
of the legs were paretic. The flexors and extensors of the thighs were

7 SPILLER : THE EPICOXUS SYMPTOM-COMPLEX.

normal. The plantar reflexes were lost. Signs of cerebral syphilis
and left hemiparesis developed. The legs below the knees were much
wasted. The lower limbs were flaccid, the partellar reflexes later be-
came diminished.

The remarkable features of this case were the bilateral peroneal
palsy affecting the left side before the right, with the escape of the
tibialis anticus muscles, weakness of the flexors of the legs and ex-
tensors of the foot, disturbance of objective sensation in the distribu-
tion of the first and second sacral roots or peroneal supply, loss of
Achilles reflexes, later loss of plantar reflexes and preservation of
patellar reflexes and of the function of the bladder and rectum, in a
man clearly affected with syphilis of the nervous system, as shown
by the history and cerebral manifestations and pathological findings.

A bilateral peroneal palsy, such as this man presented, is most com-
monly caused by neuritis and especially neuritis from alcoholism.
The abence of tenderness to pressure over the peroneal nerves and
their muscle supply does not exclude the diagnosis of multiple neuritis,
as a purely motor neuritis may occur. The escape of the bladder and
rectum also is in favor of neuritis, but is a feature also of a lesion of
the epiconus, especially in connection with the escape of the tibialis
anticus muscles. These muscles probably have centers in the spinal
cord above those of the other muscles in the peroneal distribution,
and may escape in lesions of the spinal cord, as seen frequently in
anterior poliomyelitis. They may escape also in lead palsy when the
lower limbs are affected, just as the supinator longus muscles often
escape when the posterior interosseous distribution is affected from
lead causing wrist-drop ; but it is still undetermined whether lead palsy
is primarily due to disease of the nerve cells or of the peripheral
nerves. Weakness confined to nerve distribution is one of the most
diagnostic features of neuritis, but peroneal palsy may be caused by
a lesion of the spinal cord in the epiconus. The patient complained
of pain in at least the left lower limb, but pain in the limbs is common
in syphilitic meningomyelitis, and probably results from irritation of
the posterior roots; it by no means indicates necessarily peripheral
neuritis. A diagnosis in this case between multiple neuritis and a
lesion of the epiconus or of the roots pertaining to this region of the
spinal cord was difficult.

A lesion of the gray matter of the fifth lumbar, first and second
sacral segments of the spinal cord, a region to which Minor has given

SPILLER : THE EPICONUS SYMPTOM-COMPLEX. O

the name epiconus, gives a very definite clinical picture characterized
by the presence of certain symptoms, as well as by the absence of
others belonging to lesions of the conns ; the conns to be regarded as
beginning with the third sacral segment and extending to the end of
the cord. There is paralysis of motion and of sensation in the inner-
vation of the sacral plexus, especially in that of the peroneal nerves.
These muscles are most atrophied, and electrical reactions in these are
most affected. The gait is of the steppage type because of foot-drop.
When the lesion extends higher than the first and second sacral seg-
ments into the fifth lumbar segment, the flexors on the back of the
thighs and the gluteal muscles are weak, because of the im])lication
of the fifth lumbar segment. The Achilles tendon reflexes and the
plantar reflexes are lost. The negative signs are as important in the
diagnosis as the positive ; the sphincters of bladder and rectum and the
sexual functions are not afl^ected, because the conus in which the
centers for these muscles and functions are situated, and the white
columns above the conus are not implicated ; the patellar reflexes are
preserved and may be exaggerated because the lesions does not extend
into the fourth lumbar segment, the saddle-shaped area of anesthesia
over the buttocks is not present because of the integrity of the conus.
IMinor had no cases with necropsy when he wrote his first paper on
this subject, nor does he refer to any necropsy in the few cases of
epiconus lesions he quotes from the literature. These as well as his
own were all traumatic cases. Sensation may be affected in the feet
and outer part of the legs about half way to the knees, and possibly
also in a narrow strip extending up the back part of the thighs. In
his second paper published in June, 1906, Alinor reports two cases of
poliomyelitis of the epiconus confined to one side, also a traumatic
case, but all without necropsy. A necropsy was not obtained in
Bernhardt's case and this was not a traumatic case. The lesion was
supposed to be hemorrhage of myelitis of the epiconus. Cestan and
Babonneux's Case 4 in their paper is regarded by ]\Iinor as one of
epiconus lesion, caused by hematomyelia. It is not stated by ]\Iinor
whether or not necropsy was obtained. A case of Laignel-Lavastine
is not regarded by Minor as entirely typical.

A traumatic case of lesion of the epiconus was under my observation
a long time and was reported by Weisenburg. That also was without
necropsy.

These cases referred to by Elinor seem to be the only instances in

9 spiller: the epiconus symptom-complex.

literature of lesions of the epiconus, and by far the majority of these
cases are the result of trauma. Unless Cestan and Babonneux's case
was with necropsy all were, merely clinical cases.

In a diagnosis between lesions of the epiconus and the roots per-
taining to it or lesion confined to these roots, the following points are
recognized : In lesions of the epiconus the deformity of the vertebrae,
if one exists, is at the first lumbar vertebra, the symptoms develop
rapidly and rapidly extend, anesthesia is pronounced and the sensory
disturbances are of the dissociated type, signs of sensory irritation are
absent, and the disturbances are bilateral and symmetrical. In lesions
of the Cauda equina in the roots pertaining to the epiconus, the de-
formity of the vertebr?e if one exists is lower, the symptoms begin
more slowly and extend more slowly, pain is severe and lasts a long
time and precedes other symptoms, and the disturbances are asym-
metrical. Tenderness to pressure is common in the peripheral lesions,
but inasmuch as hypersensitiveness is common in meningitis probably
from irritation of the posterior roots, it does not imply neuritis of the
peripheral branches.

In my case, the report of which has just been given, the left leg
was afifected first, but the right was soon implicated; there was no
deformity as there was no trauma ; the symptoms developed rapidly,
soon reached their height, and remained stationary some time without
involving either upper limb until cerebral hemiparesis occurred. An-
esthesia was pronounced but dissociation of sensation was not present,
and the implication was bilateral and symmetrical. The symptoms
were therefore suggestive of a cord lesion. Even with the micro-
scopical study before us it is difficult to say whether the multiple
neuritis occurred first and the cellular changes in the lower lumbar and
sacral regions were secondary, in the form of a reaction at distance ;
or whether the roots of the peroneal nerves arising in the epiconus
were first affected, as they may have been by the meningomyelitis. No
greater intensity of the meningitis is present in the lower lumbar and
sacral regions to explain the implication of the roots of these regions
and the escape of roots from higher levels. It is possibly more rea-
sonable therefore to assume that the peroneal nerves were the first
affected, and that the case was one of syphilitic multiple neuritis oc-
curring with syphilitic meningo-myeloencephalitis. With this explana-
tion we can understand why the nerve cells of anterior horns of the

spiller: the epiconus symptom-complex. 10

upper lumbar region afforded such a striking contrast to those of the
lower lumbar and sacral regions.

Two other cases of the epiconus symptom-complex have come under
my observation :

Case 2. G. Iver, aged thirty-five years, a patient of Dr. Stengel, was ad-
mitted to the University Hospital, April 27, 1907. In the middle of February,
1907, he was taken ill with a high fever. When seen by a physician a few days
later he had an enlarged spleen and rose spots. The temperature went down to
normal and he was able to work at the end of a week. He worked about ten
days, when he again had fever, and the symptoms indicated incipient typhoid
fever, although the spleen was not enlarged and rose spots were absent. He
rapidly grew worse and developed meningeal symptoms, with positive Koenig's
sign and ankle clonus. He was stuporous for two weeks. This condition dis-
appeared and was followed rapidly by pneumonia of the lower left lung, which
resolved very slowly. The pneumonia occurred about March 20. Symptoms of
empyema on the same side followed the pneumonia. About three weeks ago
pus was obtained, by needle, and soon after this he coughed up large quantities
of mucopurulent material, and this he continued to do.

May 12, 1907: Examination by Dr. Spiller resulted as follows:

The lower limbs have good voluntary power except in the peroneal distribu-
tion on each side. Bilateral foot-drop is present, slight on tlic right side but
very pronounced on the left side. He is able to dorsally flex the right foot
even to a moderate degree of resistance, but in attempting to dorsally flex the
left foot contraction occurs only in the anterior tibial muscle. The muscles of
the legs below the knees are wasted. He has no fibrillary tremors. The patellar
tendon reflex is exaggerated on each side and patellar clonus is present on each
side, ankle clonus also, but the latter is soon exhausted. Ankle clonus with
pronounced foot-drop on tlie left side is very striking. Sensations of touch and
pain are normal in the lower limbs. Babinski's sign is not obtained on either
side in a cliaracteristic manner, but on the right side at times all the toes except
the big toe are extended. Bal)inski's reflex is not indicated on the left side by
extension of any of the toes. Cremasteric reflex is weak on the left side,
prompt on the right side. Sensations of touch and pain are normal about the
anus and in the perineum. He has no pain nor tenderness in the lower limbs.
Micturition and defecation are normal.

The grasp of the hands is good. The biceps tendon reflex and triceps tendon
reflex are exaggerated on each side. Sensations of pain and touch are normal
in the upper limbs. No wasting of hands or forearms is detected. Voluntary
power in the upper limbs is good.

He closes the eyelids, shows the teeth and draws up the corners of his
mouth very well. Pupils are equal and respond promptly to light and in con-
vergence. Extraocular muscles are normal. The tongue is normal. Speech is
that of a patient weak from sickness, not from organic nervous disease.

Diagnosis : Lesion of epiconus, poliomyelitic in character, following pneu-
monia.

Case 3. F. S., aged forty-two years, male, was injured November 6, 1905,

11 spiller: the epiconus symptom-complex.

by falling and striking his back in the lumbar region. At the present time,
October, 1907, sensations of pain and temperature are diminished but not lost
over the outside of each leg below the knee, and on the dorsum and sole of
each foot, especially on the right side; and are normal on the inner side of
each leg and back and front of each thigh. Tactile sensation is normal in the
lower limbs. The patellar reflex is present on each side but much diminished,
and is shown only by contraction of the quadriceps muscles. The Achilles ten-
don reflex is nearly normal on the right side but is very weak on the left side.
Complete foot-drop is present on each side. Babinski's sign is not present on
either side. The flexors on the back of the thighs are a little weak. The
functions of bladder and rectum and of the sexual organs are not impaired.
Sensation about the anus and down the back of each thigh is intact.

I call attention to the preservation or even exaggeration of the
Achilles tendon reflexes in certain cases presenting the epiconus
symptom-complex. It may indicate that the centers for this reflex
are at a higher level. Exaggeration of tendon reflexes from a lesion
below a reflex arc I have seen repeatedly.

BIBLIOGRAPHY.

Remak and Flatau. " Neuritis and Polyneuritis," Nothnagel's System.
Brauer. Neurol. Cent., 1896, p. 671.

Cestan. Nouvelle Iconographie (4e la Salpetriere, 1900, p. 153.
Dana and Others. Journal of Nervous and Mental Disease, Vol. XXV.,
1898, p. 598.

Starr. " Organic Nervous Diseases," Lea Bros, and Co., 1903, p. 160.
Minor. Deutsche Zeitschrift fiir Nervenheilkunde, Vol. XIX., p. 331 ; Vol.

XXX., p. 395.

Bernhardt. Salkowsky's Festschrift, cited by Minor.
Cestan and Babonneux. Case 4, cited by Minor.

2.S

TUMORS OF THE CAUDA EQUINA AND LOWER
VERTEBRA

A Report of Nine Cases: Seven with Necropsy, Three
WITH Operation ^

By William G. Spiller, M.D.
professor of neuropathology and associate professor of neurology in the

UNIVERSITY OF PENNSYLVANIA; NEUROLOGIST TO THE PHILADELPHIA
GENERAL HOSPITAL.

The sypmtoms of tumor of the canda equina resemble closely those
produced by certain other cdnditions. The diagnosis must be made
between hysteria, multiple neuritis confined to the lower limbs, intra-
pelvic tumor, tumor or caries of the lumbar vertebrae or sacrum, lesions
within the vertebral canal but external to the dura, tumor or other
lesion (hemorrhage) of the conus, and tumor of the cauda equina.

Hysteria. — One of the most important symptoms of tumor of the
Cauda equina is pain in the lower limbs, severe in type, usually appear-
ing first in one limb and later in the other, and more commonly simulat-
ing sciatica. Pain in the lower limbs may be distinctly hysterical. I
have recently observed two cases of this character in which organic
lesions as causes of the pain probably did not exist. A dilTerential
diagnosis usually is possible after careful study, but in certain cases
it may be very difficult. In addition to the pain, weakness of the lower
limbs may occur in hysteria.

Multiple Neuritis of the Lower Limbs. — Neuritis is very seldom
confined to both sciatic nerves and their branches, although it may be
limited to the lower limbs, and I cannot agree with Miiller- when he
says that the upper limbs are almost invariably (fast regelmassig) im-
plicated. I have seen neuritis confined to the lower limbs, but it is
undoubtedly uncommon. Such neuritis does not usually cause great
difficulty in diagnosis. The history of alcoholism or preceding in-

^ Read at the meeting of the American Neurological Association, May 7, 8,
and 9, 1907. From the Department of Neurology and the Laboratory of Neuro-
patholog>' of the University of Pennsylvania.

Published in the American Journal of the Medical Sciences, March, 1908.

" Deutsche Zeitschrift fiir Nervenheilkunde, 1899, xiv, i.
1 29

2 spiller: tumors of the cauda equixa

fectious disease, or even when these factors are absent, the intense
pain on pressure over the nerves and muscles, especially those of the
calves, is very diagnostic, and there may be impairment of objective
sensation and of motion ; but pain on pressure over the limbs may occur
in tumor of the cauda equina. The peroneal nerve supply is most
likely to be affected in neuritis, but we recognize fully that isolated
peroneal palsy occasionally occurs from intrapelvic lesions, and it is
believed by some that the fibers forming the peroneal nerves are dis-
tinct from the other fibers in the sciatic nerve to their origin in the
plexus of nerves in the pelvic cavity, and that they are more exposed
to trauma within the pelvis than are the other fibers of the sciatic
nerves. Bilateral peroneal palsy may occur from a lesion within the
dura confined to the fifth lumbar and first sacral roots.

Bilateral sciatica is usualy indicative of tumor. Xo diagnosis should
be made with more caution than this of bilateral sciatica, and the
condition is far too frequently regarded as merely ordinary sciatic in
each limb. The prognosis should always be given with great reserve.
Tumor of the pelvis, vertebrae, or cauda equina is often diagnosticated
as sciatica which at first may be unilateral and later bilateral, and the
condition in its commencement is the same as that of sciatica without
disturbance of objective sensation and without motor paralysis.

Sciatica or multiple neuritis, even when the latter is in the lower
limbs, is not usually associated with paralysis of the bladder and
rectum, but the statement of Miiller,^ that this paralysis " noch nie
beobachtet ist,"' is surely too strong. I should modify it by saying
that is is very uncommon. I have seen multiple neuritis with implica-
tion of the bladder, and have a case under my care at present.

\Miile tenderness of the muscles is usually indicative of peripheral
neuritis, it has been observed in cases of tumor of the vertebr?e and
Cauda equina, also in my cases II, R', and Y, and in the case reported
by Sailer. In Schmoll's case of tumor of the cauda equina both sciatic
nerves were exceedingly tender to touch. The condition is like the
pain of meningitis. The posterior roots are greatly irritated, and any
moderate peripheral stimulation is transmitted with unusual intensity.
At least, this seems to me a reasonable explanation of the hyperesthesia
of meningitis, and I do not regard the diagnosis of a complicating
neuritis as necessary. We can hardly assume that peripheral neuritis
occurs in every severe case of meningitis, as we must do if we attribute

^Loc. cit.

spiller: tumors of the cauda equina 3

the pain of meningitis to neuritis, as hyperesthesia is so common in
meningitis.

Neuritis of the lower hmbs is often confined to the territory of
certain nerves, although it may be general.

The diagnosis between neuritis confined to the lower limbs and
tumor of the cauda equina may, in the early stages of the disease, be
exceedingly difficult.

Intrapelvic Tumor. — A tumor of the pelvis may cause symptoms
much like those of lesions of the cauda equina. The nerves forming
the plexuses are more spread out in the pelvis than are the roots of the
cauda equina, consequently an intrapelvic tumor may cause only uni-
lateral symptoms, or at least symptoms confined to one side a long
time, or preponderatingly unilateral, but the symptoms may be bilateral
from the beginning if the tumor is medianly situated, or is multiple,
or if the lesion is fracture. Ttiere may be the same pain and weakness
either from tumor in the pelvis or in the cauda equina. Examination
by the rectum or vagina is often useless in the early stages of intra-
pelvic tumor, as the new growth may be higher than the finger can
reach, or may be too small or too flat to be detected. Later, when it
has attained considerable size, it may be more easily palpated by the
examining finger.

In a case seen by me in consultation, with Dr. David Riesman,
bilateral sciatica was for a long time the only symptom of intrapelvic
tumor, and rectal examination was at first of no assistance in the
diagnosis ; later, the tumor grew to a large size and the diagnosis
became easy.

The tenderness over the spine on pressure is not so likely to be
present in intrapelvic tumor, but this tenderness has seemed to me
rather an unreliable sign.

The motor and sensory symptoms usually occur more closely to-
gether in point of time in intrapelvic lesions than in caudal lesions,
as in the pelvis both motor and sensory fibers are combined in the
nerves, whereas in caudal lesions they are separate.

In pelvic lesions we expect symptoms indicative of implication of
groups of fibers belonging to the lumbar and sacral plexuses, and not
of one single nerve ; still, we know that the peroneal nerve alone may
be implicated in intrapelvic lesions. This is more likely to occur from
trauma than from tumor.

In all the groups of cases under consideration the paralysis is flaccid,

4 spiller: tumors of the cauda equixa

reaction of degeneration may be present, later atrophy may develop,
and the tendon reflexes are diminished or lost, although it is possible
to have some exaggeration of certain tendon reflexes when the nerves
concerned in these reflexes are not implicated, and the neuritis is con-
fined to other nerves even of the same limbs; or, in the early stage
of neuritis, when the condition is one of irritation rather than de-
generation.

. In all these groups of cases the lesions implicate the peripheral
segments (peripheral neurons), either the nerve fibers or nerve cells.
When the lesion is above the lumbar region the clinical picture is a
very diiterent one.

The anesthesia of lesions below the cord proper (in the cauda
equina, vertebrae, or pelvis) is not likely to be dissociated, as it may
be in lesions within the spinal cord, but occasionally it is.

In plexus lesions, when the bladder and rectum are paralyzed, the
power of erection and ejaculation is likewise usually impaired or
lost, and yet a case of pelvic tumor reported by Miiller* shows how
difficult the diagnosis as regards these symptoms may be.

The pain was confined to the right lower limb one year. Three-
quarters of a year after the beginning of the pain paralysis of the
bladder and rectum appeared, and after the first year pain was felt
in the left lower limb. The occurrence of vesical and rectal symptoms
three-quarters of a year after the pain began ^^luller regards as con-
trary to the diagnosis of intravertebral lesion, and yet in one of my
cases (Case II) of cauda equina tumor there can be little doubt that
had the patient lived paralysis of the bladder and rectum would
have occurred, even though it would have been months after the
symptoms began. ]\Iuch experience. IMiiller says, shows us that in
lesions of the lower part of the vertebrae causing diminution of space
the vesical and rectal nerves are first aft'ected. This Case II of my
series shows plainly that this statement is not always correct. Fibril-
lary tremors were present in ^kliiller's case of intrapelvic tumor, and
these are uncommon when the nerve cells are not involved. Although
the bladder and rectum were paralyzed, erection was preserved, but
ejaculation of semen was disturbed by paralysis of the ischiocaver-
nosus and bulbocavernosus muscles. The fibers for erection, there-
fore, seem to rise higher in the cord and to leave the cord at a dif-
ferent level from those of the bladder and rectum, but the dissociation

* Loc. cit.

spiller: tumors of the cauda equina 5

of these symptoms is certainly uncommon in lesions of the plexus. It
is well to remember that it may occur, as demonstrated by this im-
portant case of Midler's.

Extremely interesting is a fact brought out by Miiller, that the
sensation of the lateral part of the scrotum is not from the coccygeal
plexus, as is that of the median portion, but, like that of the testicles,
is from the lumbar portion of the cord. Very little attention has been
paid to this difference in origin of the fibers providing sensation to the
lateral and median portions of the scrotum, but I have been able to
confirm the correctness of the observation repeatedly.

Tumor of the \^ertebr.e. — Here, as in all the tumors under con-
sideration, pain is a very prominent symptom, and is likely to become
bilateral sooner than in intrapelvic tumor. The posterior roots are
caught in their passage through the intervertebral foramina, and the
pain is usually constant, with exacerbations. Pressure over the lower
vertebrae and sacrum may be exceedingly painful. It is impossible, in
my experience, to decide from the symptoms whether the tumor is
confined to the vertebrze or whether it extends also over the anterior
surface of the dura. Of differential value in diagnosis from tumor
of the Cauda equina is the fact that the symptoms are likely at first
to be confined to the distribution of a few nerve roots when the tumor
begins in a vertebra, but that this is not always a reliable sign is shown
by my Case II, in which the tumor was confined to the cauda equina,
therefore was within the dura, and yet the symptoms indicated impli-
cation of only a few nerve roots. It is desirable to find some reliable
means of clinical differentiation between tumor confined to the verte-
brae and tumor on the dura or inside the dura without implication of
the vertebrae. The former condition makes a radical operation im-
possible, although it does not forbid the attempt to cut posterior roots
and relieve pain. A comparison of my Case II with Case IV is most
instructive. In both pain was intense, with paralysis occurring after
many months, and with no involvement of the bladder and rectum, and
yet in the first the tumor was entirely within the dura, and in the
other it was entirely extradural, and also in the vertebrae.

Lesions of the Conus. — The nerve tracts in the conus occupy a
very small space, therefore when a lesion occurs in this part of the
cord it is likely to implicate most of the tracts at nearly the same time.
As a result paralysis of sensation and implication of the bladder and
rectum occur almost simultaneously. If the lesions are strictly

(') spiller: tumors of the cauda equina

limited to the conus, which, following the lead of Raymond, may be
recognized as beginning at the third sacral segment, we cannot expect
motoi paralysis except of the bladder and rectum, inasmuch as the
limbs receive their motor supply from the lumbar and upper sacral
segments. Pain is not usually a prominent symptom in conus lesions,
unless the meninges or posterior roots are likewise affected.

Anesthesia in certain territories innervated by the lower sacral
segments may occur in conus lesions and may be dissociated ; it may
even be bilateral from unilateral conus lesion, in that the buttocks
near the anus, the perineum, and the external genitalia may be anes-
thetic on both sides. This bilateral anesthesia, so limited, is caused
by involvement of the lowest posterior root fibers entering the conus
on the side of the lesion and by involvement of the corresponding
fibers from the opposite side after they have decussated in the conus.

When the lesion is confined to the conus it may by irritation of
the adjoining upper region cause not only a zone of hyperesthesia
above the anesthetic areas, but exaggeration of the patellar reflexes;
yet this exaggeration of the patellar reflxes may occur from tumor
of the Cauda equina, as in Volhard's case.

Chronic meningitis confined to the lower part of the cord and
cauda equina has often been described, according to Schultze, and
such a lesion causes severe pain, atrophy, and paralysis, not unlike
symptoms of tumor. Schultze^ excluded meningitis in one of his
cases (Case VI) because the symptoms were progressive and in sud-
den increases, and not afifected by antisyphilitic treatment.

MiJller'' thinks that in conus lesions the degenerative atrophy occurs
more rapidly and intensely than in cauda equina lesions, because in
the latter the nerve fibers are not so completely and so quickly impli-
cated. This is a reasonable supposition. I must agree with Muller
when he takes issue with Valentine regarding his statement that there is
no improvement in conus lesions. Muller reports spontaneous im-
provement in one of his cases of cauda equina lesions, and this surely
may also occur occasionally in conus lesions. Improvement in cauda
equina lesions after trauma is not uncommon. Indeed, Schultze^ has
shown that Valentine's statement is not correct for all cases, as in
three of his own cases im])rovement did occur, but the improvement

■"' Mitteilungen aus den Grenzgebieten der Medizin und Chirurgie, 1903, XIT,
163.

' Loc. cit.

'Deutsche Zeitschrift fiir Ncrvenheilkunde, 1894, V, 247.

spiller: tumors of the cauda equina 7

seems to have been in the regions innervated from the lumbar cord.
It is very true, 1 think, that improvement of the fimction of the
conus in conus lesions is usually unimportant, because the transverse
area of any part of the conus is so small that lesion in this part of the
cord is likely to destroy the conus in its entire diameter, but it is also
true that improvement is usually slight in cauda equina lesions so far
as the functions of the conus roots are concerned. Certain symptoms
may disappear, but the residual palsy is likely to be persistent.

Another of Miiller's cases is worthy of note because of certain un-
usual features. He believed the lesion to be traumatic myelitis of the
conus. The bladder and rectum were completely paralyzed, and yet
erection and ejaculation were possible (potentia coeundi et gerandi),
as the patient became the father of two children after the accident.

I have had a similar case as the result of injury, possibly to the
cauda equina, from muscular ^strain, although the lesions may have
been in the conus :

H. C, a male, aged thirty-one years, was injured eighteen months
previously by a bale of cotton falling against the abdomen. He was
unable to work about three weeks, but then returned to heavy work,
feeling not quite so well as formerly. About a month after return-
ing to work, while lifting a bale of cotton, he felt something give way
in the right inguinal region, and at the same time heard a tearing
sound. He immediately felt weak, and limped on the right lower
limb, but walked home, a distance of about two blocks, and went to
bed. After one day he got out of bed, but remained at home about
a week. He then returned to heavy work, but has not been quite so
strong as he was before the injury.

After the accident he lost control of the bladder, so that when he
coughed or exerted himself urine would flow. The condition grad-
ually became worse, until now he has no control of his bladder, and
has been wearing a bag about a year. The sexual desire is not weak-
ened, but he refrains from the sexual act because of the dribbling of
the urine. He had sexual intercourse successfully six months ago
without impairment of ejaculation and erection. During the past
two months he has noticed that the, rectal sphincter functionates
weakly, and that when there is a call to stool it is urgent.

Present Condition. — His gait and station are good. The lower
limbs are well developed, but the man thinks he is weaker than he
was before the accident. The left side of the scrotum, left side of the

8 spiller: tumors of the cauda equina

perineum, and the left buttock near the anus have fully normal sensa-
tion to touch and pinprick ;^ whereas the right side of the scrotum,
except the upper outer portion, the right buttock, in a small area near
the anus, and, to a less degree, the right side of the perineum, show
diminution of sensation to touch and pinprick. The right side of
the penis is less sensitive to touch and pinprick than the left side.
He has no control of the bladder, but can control his bowels, except
-when they are loose. The sensation of the testicles is normal. The
patellar reflexes are prompt. The Achilles reflexes are absent or slight.
Babinski's sign is not present. The upper portion of the body is not
affected.

Miiller refers to a case similar to his own reported by Rosenthal,
and also to a case reported by Bernhardt in which the bladder and
rectum were paralyzed, although the sexual powers were preserved,
except that the muscles for expelling the semen (the ischiocavernosus
and bulbocavernous) were jmralyzed, that is, the condition, as Miiller
puts it, was potentia and libido coeundi with impotentia generandi,
and he refers to a case reported by Schiff in which ischuria paradoxa
and paralysis of the sphincter ani existed, but power of erection was
retained. The loss of function of the bladder and rectum, with pre-
servation of erection and ejaculation, is in favor of a conus lesion,
but in my case the lesion probably was in the cauda equina, as the
symptoms indicated probably unilateral injury, although a lesion of
the conus cannot be excluded.

The conus may be injured, while the surounding roots escape, as
in the cases reported by Oppenheim, Schultze, and Sarbo.

Miiller says he was unable to find any cases, with necropsy, of conus
lesions without trauma.

Van Gehuchten,*' from a study of the literature and of a case of
lesion of the cauda equina and experimental work, believes that the
bladder, rectum, and genital organs may retain their functions to a
certain degree even when all the roots of the cauda equina, beginning
with the fifth lumbar, are affected. He believes in the existence of a
reflex center for micturition, defecation, and erection outside the cord,
but not for complete ejaculation of the semen. The restoration of
micturition, defecation, and erection after trauma does not indicate
that the conus is intact. The primary centers for these functions are

*A year later disturbance of sensation has been noticed on this side.
" Le Nevraxe, 1903, IV.

spiller: tumors of the cauda equina 9

in the hypogastric plexus of the sympathetic system ; only the pres-
ervation of the anal reflex and of ejaculation indicates anatomical
integrity of the conus and its roots. While primary centers for the
functions mentioned are in the sympathetic system, other centers for
them are in the conus, and are necessary for normal discharge of these
functions.

I have had the opportunity to study a case of lesion of the cauda
equina which bears out these statements. The disturbance of sensa-
tion is in the lowest sacral roots. By passing the urine and feces
every three or four hours the man exerts a certain control over the
bladder and rectum. The discharge is started by voluntary pressure
of the abdominal muscles, but the man has no sensation from the
flow of the urine, as the urethra is anesthetic. When the urine has
nearly ceased to flow he is unable to stop the stream or to expel the
last few drops. Erection oc-curs. There is, therefore, a certain
periodicity in this case, but in another under my observation, in which
sensory disturbances are also limited to the lower sacral roots, both
cases resulting from injury to the end of the spinal column, the
urine dribbles without the slightest control by the patient.

Warrington refers to the fact that Raymond has shown that a
spinal lesion may cause incontinence of urine without any anesthesia,
and Van Gehuchten, that in damage to the conus complete control
over the sphincters may be preserved with the urine voided in a
nearly natural manner while the mucous membrane of the urethra
and bladder are absolutely anesthetic. Erection of the penis was
preserved in Van Gehuchten's case, and this, Warrington thinks, is
difficult to explain, inasmuch as the penis was absolutely anesthetic,
and presumably the sacral center had been completely destroyed.
As he believes the preservation of control over the sphincters may
have been due to the activity of the lumbar center through its fibers
to the sympathetic, it should not be difficult to explain the erection
in the same way, as this is not always dependent on the sensation
of the organ. Ejaculation of semen and forcible expulsion of the
last few drops of urine depend on the contraction of the bulbocav-
ernous and ischiocavernosus muscles, and are always paralyzed if the
lowest sacral part of the cord or the corresponding roots are damaged,
according to Warrington. It would seem, from Warrington's state-
ments, that these function are not disturbed by a lesion of the lumbar
center. He refers to Van Gehuchten's statement that it is only with

10 spiller: tumors of the cauda equina •

unimpaired capacity for these actions and in the persistence of the
anal reflex that the diagnosis of integrity of the conus meduUaris
and of the corresponding roots can be made. The following points,
as Warrington^° concludes from the writings of Raymond and Sippy,
are in favor of a lesion of the cauda equina in distinction from one
of the conus.

1. An insidious onset, with general development and progression
•of symptoms. A lesion in the conus is likely to cause a more rapid
development of the symptoms.

2. Pain, violent in nature, spontaneous or excited by change of
position or by movement, is in favor of root lesion, though moderate
pain may be present in either condition.

3. Pain over the vertebral column below the level of the second
lumbar spine exaggerated by percussion and radiating toward the
lower limbs speaks for a root lesion. Warrington adds to this rigidity
of the lower part of the spine from irritation of the nerves of the dura.
Pain higher up, increased by pressure and not radiating very clearly
over the lower limbs, is in favor of a medullary lesion. Lasegue's
sign is indicative of a root lesion. Asymmetrical development is a
radicular symptom, and is, perhaps, especially suggestive of an extra-
dural lesion. My Case II shows, however, that one may be readily
deceived in this sign. Remission of symptoms, especially amelioration
in the condition of the bladder and rectum, and the slow appearance
of muscular wasting, of the reaction of degeneration, and of trophic
disturbance are, according to Warrington, in favor of an affection of
the cauda equina.

Tumor of the Cauda Equina. — The pain in cases of this type is
often unilateral at first, but, as a rule, soon becomes bilateral, and
yet it may remain unilateral many months. If the tumor develops at
the lower end of the cauda equina, it will implicate the nerve fibers
supplying the bladder and rectum, causing disturbance of function
in these viscera, and disturbance in sensation in the region of the anus
from the implication of the lowest sacral and coccygeal roots. This
symptom-complex does not necessarily imply that the tumor is so
low. because a new growtli within the filum terminale near its origin
from the cord may press at first upon those roots nearest to the filum,
that is, the coccygeal and lower sacral, and cause the same combina-

" Lancet, 1905, II, 749.

spiller: tumors of the cauda equina 11

tion of symptoms as when the tumor is considerably lower. This was
well shown in the cases of Lachmann and V^olhard.

In Lachmann's case of glioma of the upper part of the filum ter-
minale the diagnosis of carcinoma of the bladder was made. The
symptoms had existed two years, and began with incontinence of
urine, paradoxical ischuria, and retention of feces. Later, digestive
disturbances and emaciation developed. Hemorrhage into the bladder,
especially with sudden return of the power to empty the bladder,
strengthened the diagnosis of carcinoma, as in papilloma of the bladder
catheterization by removal of the occlusion of the orifice may cause
hemorrhage and return of the power to empty the bladder. The
necropsy revealed catarrh of the bladder, with swelling and injection
of its mucous membrane.

A case of Cruveilhier, reported in his atlas, to which Lachmann"
refers, shows, however, that a. tumor of the filum does not always
cause symptoms confined to the bladder and rectum. The symptoms
were paralysis of the lower extremities, with contracture, and paralysis
of the bladder and rectum. A tumor, which Lachmann thought the
picture showed to be in the filum, was found. It was diagnosed as
" encephaloide."

A tumor of the filum. however, may exist without causing symptoms
if it is not very large. P- reported a lipoma of the filum without any
symptom caused by the growth, as the tumor had not attained a size
sufficient to cause pressure.

Volhard's case is an exceedingly interesting one. The symptoms
developed slowly (during four years), and began with disturbance
in emptying the bladder and in sexual power, then dilatation of the
bladder occurred with pyelitis, and dyspeptic symptoms, dryness of
the skin and mucous membranes, and constipation. The muscular
supply of the left sciatic nerve later became weak, and slight hypes-
thesia was detected in this region. The patellar reflexes were lively
and the cremasteric reflex was present on both sides. The Achilles
tendon reflex was weak on the left side and absent on the right side.
Pain was not felt, and the only subjective sensory disturbance was a
feeling of pressure over the sacrum. The absence of pain in this
case, in which the symptoms persisted four years, was most extra-
ordinary, and no similar case of cauda compression was known to

"Archiv fiir Psychiatric, XIII, 50.

"Journal of Nervous and Mental Disease, 1899, P- 287.

;^9

12 spiller: tumors of the cauda eouixa

Volhard, nor have I been able to find any. although tumor elsewhere
on the spinal cord without pain has been observed. Operation w^as
not attempted, as the patient's condition became too grave. The
necropsy revealed a benign tumor of the cauda equina close to the
conus, pressing the roots forward, and thereby injuring the motor
roots most, as they were pressed against the bone, whereas the pos-
terior roots were next to the soft tumor. A tumor lower in the
cauda. A'olhard^^ thought, could not have caused the symptom-com-
plex. The w^eakness of the left lower limb seems to have been present
about two years or a little more, and yet in all this time the right lower
limb did not become weak : but a still slower development of the
symptoms was present in a case of tumor of the cauda equina observed
by Schultze.^*

In this case pain was felt in the distribution of both sciatic nerves in
1885. and paralysis in the distribution of the right peroneal nerve in
the end of 1886. but paralysis in the distribution of the left peroneal
nerve did not occur until 1891. five years later. A'esical symptoms
did not appear until seven years after the beginning of pain. Opera-
tion was attempted and a part of the tumor was removed, but the
bleeding was profuse, much cerebrospinal fluid escaped, and the patient
died on the eleventh day after the operation. The necropsy showed
a large tumor of the cauda equina, an angiosarcoma myxomatodes,
that had eaten its way through the bone.

A tumor may implicate both posterior and anterior roots, and for
a long time cause only pain, usually in the hips, over the coccyx, and
in the distribution of the sciatic nerve. The growth may be quite
large, but it will still find space without causing a paralyzing pressure
upon the nerve roots. A moderate degree of pressure is, however,
sufficient to irritate the nerve roots, and this irritation will usually be
shown first in the posterior roots, and therefore as pain, but there
may be spasmodic twitching of the muscles of the lower limbs. The
paralyses of motion and of sensation are usually later symptoms, and
indicate that the tumor has attained considerable size. The paralysis
may develop rapidly or within a few days, and in one of my cases
of rapid paralysis (Case II) a blood cyst was found at necropsy.
This cyst, with its well-defined wall, could not have formed within so

"Deutsche med. Wochenschrift, August 14. 1902. Xr. 33. p. 591.
" Mitteilungen aus den Grenzgebieten der Medizin und Chiriirgie. 1903. XII,
163.

spiller: tumors of the cauda equina 13

short a period before death, a few weeks. It is probable that a cyst
existed in association with the tumor, and that hemorrhage into the
former occurred.

Usually the development of symptoms in cauda equina lesions is
very gradual, the symptoms beginning with pain and loss of reflexes
in the lower limbs, followed often much later by paralysis and atrophy
and anesthesia. The anesthesia is to be sought for especially in the
perineum, external genitalia, and in a narrow zone about the anus, as
this region receives its sensory nerves from the lowest roots of the
cauda ; and, although the tumor may not be at the exit of these roots
through the sacrum and coccyx, it may exert pressure upon them near
their exit from the cord, notwithstanding they are centrally situated in
the Cauda equina ; but it is not true that these centrally situated roots are
always first affected, as shown by my Case II, although Miiller says it
has been demonstrated that in high lesions of the cauda equina the most
central nerve roots, those that arise lowest from the cauda, that is,
the roots innervating the bladder and rectum, usually are first affected,
and he refers to Lachmann in support of this statement; but this
combination of symptoms by no means invariably occurs in high lesions
of the cauda equina. In Lachmann's case the tumor was in the filum,
and therefore in immediate relation with the lower sacral and coccygeal
roots.

In determining the level for operation, the highest roots giving evi-
dence of implication should be exposed.

A very interesting case of cauda equina lesion, reported by jNIiiller,'^
shows how difficult the diagnosis between cauda equina and conus
lesions may be :

A man, aged twenty-two years, after carrying heavy weights, had
severe pain in the lumbar region lasting two days. He then worked
fourteen days, when the lumbar pains returned, were severe, and soon
radiated into the lower limbs. These limbs, as well as the muscles
of the trunk, rapidly became weak; the right lower limb, however, did
not become weak until a few days after the left lower limb had
become completely paralyzed. Then followed paralysis of the bladder
and rectum. Weakness was present in the legs below the knees, but
the feet were completely paralyzed. Reaction of degeneration was
obtained in the back of the thighs, and in the legs below the knees,
and ataxia in the heel-to-knee test was seen. Sensation was greatly

"Loc. cit

14 spili.er: tumors of the cauda eouixa

affected in the distribution of the sacral roots. The patellar tendon
reflexes and Achilles tendon reflexes, erection and ejaculation were
lost. The pain indicated a cauda equina lesion, but the rapidity of
the development of the symptoms, early cessation of pain, and the
complete paralysis of the aft'ected muscles indicated a conus lesion.
Dissociation of sensation was imperfect over the scrotum and penis
(impaired tactile sensation, loss of pain and temperature sensations),
and bedsores developed rapidly ; all this made the diagnosis of myelitis
probable, but the cord was found to be normal, while the cauda equina
was the seat of inflammation. This interesting case shows that at
least an imperfect dissociation of sensation may occur in lesions of
the cauda equina. ]\Iiiller was unable to find any case in the litera-
ture like this one of his.

One reason why tumor of the cauda equina is likely to cause first
sensory symptoms is that at the lower end of the cord the sensory
roots are more numerous and larger than the motor roots, as shown
by ^Miiller.

When trauma is the cause of cauda equina lesions the symptoms
may develop rapidly, as a fracture may implicate many nerve roots.

In the case reported by Laquer^*' pain, sharply localized to the middle
of the sacrum, had persisted two years with pain on pressure in the
same region. The rectus femoris muscle on both sides was weak and
slightly atrophied. There had been transitory vesical and rectal
paralysis. The tendon reflexes of the lower limbs were diminished.
The sexual functions were impaired. Lumbar kyphosis was present.
On account of the great pain by pressure over the sacrum the diagnosis
of tumor was made, not within the dural sac, but outside the dura or
within the sacrum. Every symptom of bone disease, swelling, dis-
placement of the sacrum, was absent, and disease of the pelvis was
considered improbable ; therefore the conclusion was reached that the
tumor was within the sacral canal. An intradural tumor implicating
the cauda equina was considered improbable, as extensive motor, sen-
sory, and trophic disturbances were absent notwithstanding the long
duration of the process, and the vesical and rectal paresis was of short
duration, except that slight ischuria remained. This case, however,
clinically has great resemblance to my Case II, in which the tumor
was within the dura.

The entire sacral canal was opened, and a tumor (lymphangioma

" Neurologisches Centralblatt, 1891, p. 193.

4: J

spiller: tumors of the cauda equina 15

cavernosnm ) was removed from about the middle of the sacrum. It
was about the size of the Httle finger, extradural aud not united with
the roots or dura, but pressing the dura and cauda equina forward.
The pain had. disappeared two weeks after the operation, and the
patient left the hospital cured four weeks after the operation. The
dura was not opened, and jiossibly the recovery was due to this fact.
The case seems to have been a most successful one.

In Laquer's case the pain in the sacrum was intensified by coughing,
sneezing, defecation, or by the dorsal position. The absence of pain
on pressure over the sciatic and crural nerves was one of the points
used in favor of the diagnosis of a tumor beneath the sacrum, but
some of my cases show that this pain on pressure over the nerves may
be present in cauda equina or vertebral tumors.

A very thorough report of a tumor upon the conus and infiltrating
the cauda equina is given by Sailer.^' A man had severe pain in his
lower limbs for some time. His gait had been ataxic six months.
The pain commenced in the feet, and in this the case is remarkable,
and extended upward, and was more severe in the left lower limb.
Diffuse tenderness was present in the lower limbs, and especially over
the nerve trunks. The sciatic nerve was more sensitive than the others.
Continuous pain and tenderness were felt in the lumbar region. The
feet were edematous. (This condition in Schmoll's case caused him
to suspect involvement of the vessels, but exploration of the abdomen
did not reveal any vascular obstruction.) Paresthesia was present
in the lower limbs, but objective sensation was probably not impaired.
The patellar reflexes were absent. The man was able to move his
lower limbs, but they were wasted. Later, frequent micturition ap-
peared. A melanotic sarcoma was found in the lumbar region at the
necropsy, about one inch in diameter, lying upon the left side of the
lumbar enlargement and cauda equina roots. The tumor extended
downward to the conus. It total length was 4 cm., its antero-posterior
diameter i cm., and its lateral diameter 7 cm. in the widst part. A
considerable mass of granular tissue infiltrated the cauda equina.
Sailer discusses the rarity of this variety of tumor within the vertebral
canal. The spinal cord in the region of the tumor was much enlarged.

In the case of tumor of the cauda equina studied by SchmolP*^

"Contributions from the William Pepper Laboratory of CHnical Medicine,
Vol. I, p. 129.

"Amer. Jour. Med. Sci., 1G06. CXXXT., 133.

16 spiller: tumors of the cauda eouixa

both sciatic nerves were excessively tender. The nerves became so
tender that the patient could not lie on the back or abdomen, and to
obtain relief was obliged ^to rest in a kneeling position. In order to
sleep he knelt on cushions and reposed his head on the bed. He had
rectal crises, with extremely painful tenesmus.

The bilateral sciatica was of three and one-half years' duration, and
the rectum was involved in the painful area without involvement of
the bladder. During this period the symptoms had not increased, and
were merely those of pain. This is a most extraordinary case in this
respect, and affords a rem.arkable contrast to Volhard's case, also of
long duration, in which pain was not felt. It resembles my Cases II
and IV. It is not strange that Schmoll could not believe that the
lesion w^as a tumor, and, as the man had pulmonary tuberculosis, he
concluded that a slowly progressing inflammation of tuberculous nature
was involving the nerves. An " intraspinal " gliosarcoma was found
beneath the sacrum, banana-like in form, about 6 cm. long and 2 cm.
wide. The statement is made that it was " beneath the dura mater,
which formed the floor of the opening " ; therefore, it was a tumor
of the Cauda equina. The patient died on the fourth day after the
operation with meningitis and pneumonia.

Schmoll thinks that the interval in his case between the beginning
of irritation of the posterior roots and of other signs of compression
is the longest on record. The cauda equina at necropsy showed no
signs of the long-standing compression. Motor symptoms appear to
have been entirely absent, but they did not develop until after many
months in my Cases II and IV.

In A'alentine's^'* case a prominence was observed over the third to
the fifth lumbar vertebrae. A small piece of tissue removed showed
the tumor to be a small round cell sarcoma, but neither operation nor
necropsy permitted a confirmation of the diagnosis. The two cases
of tumor of the cauda equina reported by Sibelius-'^ are interesting
chiefly from a pathological standpoint.

Schlessinger,-^ in his work on tumors of the spinal cord, gives pic-
tures of a glioma of the conus terminalis (p. 26 ) at the junction with
the filum ; of a small cell sarcoma attached to the nerve roots and
reaching nearly to the cauda (p. 34) ; and of an endothelioma of the

'® Zeitschrift f iir klin. Med.. 1893, p. 246.

^ Homen's Arbeiten, Band I, Heft i, 2.

"' Beitrage zur Klinik der Riickenmarks- uiid W'irbeltumoren, 1898.

spiller: tumors of tiik cauda equina 17

dura l\ing upon the cord, reaching to the beginning of the cauda
equina, and apparently well defined (p. 39).

The preservation of the sexual functions, even when the sensation
of the bladder and urethra is lost, is shown in a case reported by
Valentini,-- in which paralysis of the bladder, with ischuria para-
doxa and anesthesia of the penis existed, but the sexual functions were
almost intact, except that the ejaculation of the semen into the anes-
thetic urethra was not noted. Valentini concludes that the nerves
controlling the sexual functions must be distinct from the sensory
and motor nerves of the bladder and urethra, and other cases referred
to in this paper fully justify this opinion.

In Thorborn's'-" case, with symptoms of extensive lesion of the
cauda equina, only a very small tumor, a fibrosarcoma about the size
of a hcmi)sced, was found on one of the nerve roots of the cauda
equina, with no signs of diffuse inflammation. As Thorburn says,
some other lesion must have existed. Operation was not attempted.
In a case of tumor of the cauda equina reported very briefly by
Gowers,-'* operation was not attempted. Many nerve roots were
involved in the tumor, which was a fibrosarcoma, and from the picture
given by Gowers, it could hardly have been removed by operation.

As regards the level of the lesion, the important case of Schultze-^
must be borne in mind. He has shown that a fracture of the twelfth
thoracic and first lumbar vertebrse, causing a spicule of bone to pro-
ject into the vertebral canal in the median line, and thus involving
the lower part of the lumbar swelling of the cord, may give a symptom-
complex confined to the distribution of the sciatic nerves and lower
roots of the cord, providing the upper lumbar roots, situated laterally
at this level, are not implicated. In such a case Erb had made a diag-
nosis of lesion of the cauda ef|uina not higher than the fifth lumbar
vertebse. A lesion, therefore, at the level of the first lumbar vertebra,
if it injures the cord alone, may allow the function of the lumbar roots,
except, perhaps, of the fifth lumbar, to be preserved; and cause the
same symptoms as a lesion of the fifth lumbar vertebra ; while a com-
plete transverse lesion, as a tumor, at the level of the second lumbar
vertebra, may implicate all the lumbar roots, as well as the sacral, and

^Zeitschrift fiir klin. Med., 1893, XXII, 245.
^ Brain, Vol. X, p. 388.

"A Manual of Diseases of the N^ervous System, second edition. 1892, Vol. I,
P- 546.

"■■ Deutsche Zeitschrift fiir Nervenheilkunde, 1894, Vol. V, p. 247.

18 spiller: tumors of the cauda equina

cause complete motor and sensory paralysis of all the nerve distribu-
tions of the lower limbs.

Surgical Ixtervextion. — The question of operation is an exceed-
ingly important one in cases of tumor -of the cauda equina. Laquer's
case is the only one A'olhard could find in 1902 of cauda equina tumor
correctly diagnosed and successfully operated upon. He found only
six cases of tumor high in the cauda equina, and in those the correct
clinical diagnosis was not made.

In Laquer's case the tumor was on the outside of the dura, and the
latter was not opened. It is not improbable that the success of the
operation depended upon this fact. Selberg-'' reports (1904) a case
of spina bifida, complicated by a lipoma implicating both spinal cord
and cauda equina. The tumor was removed. Recovery from the
operation occurred.

In another case a sarcoma was found at operation compressing the
cord and cauda equina. The opening in the vertebral canal was 14 cm.
long. The portion of tumor removed measured almost 9 cm. : it was,
therefore, a very large tumor. Hemorrhage during the operation
was slight, but the pulse was feeble after the operation — 84. and death
occurred the same day in collapse. The symptoms were not propor-
tionate to the size of the tumor.

It is evident that the most thorough asepsis must be maintained in
operations upon the spinal cord, especially in the lower portion, and
this often is difficult, as decubitus and eczema occur, especially over
the sacrum, making the opening of the lower part of the spinal canal
especially dangerous. The escape of cerebrospinal fluid probably is
considerable in the first few days after the operation. Selberg sug-
gests that the patient, after the operation, should be kept with the
upper part of his body slightly raised, in order to lessen the danger
of pneumonia, and he should lie on one side to prevent the urine
running into the bandages. The abdominal position during the opera-
tion increases the danger of pneumonia. If this be true, it would be
possible to operate while the patient is lying on his side. Selberg says
we must acknowledge that only a small portion of spinal tumors are
operable, on account of their character and position. The operable
tumors are those of the membranes that have not implicated the cord,
and are lipomas, fibromas, psammomas, echinococci, and exostoses.

="Beitrage ziir klin. Chirurgie, 1904, Vol. XLIII, p. 197.

spiller: tumors of the cauda equina 19

In Sachs' two cases,-" reported in 1899, operation was performed.
In the first a tumor (alveolar sarcoma) the size of a small cherry was
found adherent to the dura and bone, and compressing the cauda
equina. As much as possible of the diseased tissue was removed.
In the second case laminectomy of the second and third lumbar verte-
brae was performed. A gelatinous mass (small cell sarcoma) was
exposed invading the body of the third lumbar vertebra. The tumor
was removed. It had compressed the cauda equina. Improvement
was pronounced in both these cases, but in neither was the dura opened.

In Starr's-^ case the spines and arches of the second, third, and
fourth lumbar vertebrae were removed. An endothelioma involving
both the soft and hard tissues was found. The dura seems to have
been left unopened. The patient was in a critical condition for two
days after the operation, but later improved. The case was reported
shortly after the operation.

In the case of tumor of the cauda eciuina, reported very briefly by
Fraenkel,-® operation was performed, and the patient reacted with diffi-
culty. The tumor was a fibrosarcoma. " Certain symptoms improved
after the operation." Death occurred two months later.

The case of tumor on the cauda equina and lower part of the spinal
cord reported by Putnam and Elliott^*' has some features of unusual
interest. The patient, a woman, had been operated upon for cancer
of the breast, and the diagnosis was metastasis to the spinal cord.
Removal of the spinal tumor was attempted. An incision ten inches
long was made over the lower thoracic and upper lumbar region, and
the laminae of the last thoracic and first two lumbar vertebrae were
removed. On opening the canal, a carcinoma was found imme-
diately beneath the bone. It was removed as far as possible, and
two nerve roots on each side at the level of the growth were divided
outside the dura but within the spinal canal. The dura was not
opened. Relief of pain from this operation was very marked, but
death occurred after a few months. The authors had entertained the
thought of dividing the spinal cord in order to relieve the pain.

In the case of tumor reported by Box,^^ the first, second, and third
lumbar spines and arches were removed. The dura was red, pulpy,

■'Journal of Nervous and Mental Disease, 1900, p. laS.
-'* Ibid., 1901, p. 156.
■"Ibid., 1903, p. lOi.
"" Ibid., p. 670.
^Lancet, 1903, II, 1566.

20 spiller: tumors of the cauda equina

and thickened. The cord appeared to be normal when the dura was
opened, but no tumor was found. A piece of dura examined micro-
scopically showed inflammatory changes, with small areas of calcifica-
tion. The operation wa's well borne and the wound healed well.
Later, a prominence appeared in the neighborhood of the laminectomy
scar. A necropsy was not obtained. The tumor was supposed to
have arisen in the meninges or the vertebras, and thereby to have caused
root symptoms, which were bilateral from the beginning.

Warrington's^- interesting case of tumor of the cauda equina was
typical in its symptomatology. Operation was performed and a soft-
looking tumor was found within the cauda equina. It bled very
freely wdien the attempt was made to remove it, and only a part could
be obtained. The tumor was an endothelioma, or hemorrhagic
angiosarcoma. Improvement occurred, but symptoms began to return
about the end of three months. When a second operation was at-
tempted the tumor was found to have grown enormously and was two
to three inches long. A number of posterior roots were divided. The
patient made a good surgical recovery after each operation, although
in each the dura was opened. The first operation was on September
2, the second about three months later, and death occurred in the
following May. At the necropsy the original growth was found to
be of very large size, and multiple growths were present in the pia along
the whole length of the spinal cord. A similar growth was present
in the angle formed by the cerebellum, pons, and medulla oblongata.

The pain in this case is worthy of special notice ; it was increased
by flexing the thigh on the pelvis (Lasegue's sign), but distinct pain on
pressure was not felt over the muscles of the thighs or legs or at the
points of Valleix.

Warrington, on referring to the literature on tumors of the cauda
equina, says Dufour (1896) collected 21 cases; in only one had an
operation been performed, and this was successful (Laquer's case, to
which I have already referred). At a meeting of the Neurological
Society, on October 2^, 1904, Warrington says Dr. Ferrier and Sir
Victor Horsley showed a patient from whom a fibromyxoma com-
pressing the conus and cauda equina had been removed with marked
success.

In one of the recent cases of spinal tumor reported by Batten^^ a

•^ Ibid., 1905, II, 749.
^^ Lancet, 1907, I, 139.

spiller: tumors of the cauda equina 21

sarcoma was fouiKl at operation outside the dura extending along the
left twelfth posterior thoracic root. It was removed. The dura
evidently was not opened. The condition of the patient following
the operation is not stated.

This review of the literature shows that operations on the lower
part of the vertebral column for tumor have been rather discouraging.
True, the number of operated cases is small. I leave out of considera-
tion here the successfully operated case of spina bifida, complicated
by lipoma, reported by Selberg, and others of this character are known.
The conditions in spina bifida are not the same as in tumor of the
cauda equina.

Death occurred following operation in the cases of Selberg, Schmoll,
Schultze, and cases of my series, I, II, and IV, although in the latter
the tumor was extradural. Operation was not attempted in the cases
of Cruveilhier, \^olhard, A'alcntiiii, Sailer, and in my Cases III, V,
\T. and VII. It was of benefit in the cases of Sachs, Starr, Fraenkel,
Putnam and Elliott, Ferrier and Horsley, and Warrington ; but War-
rington's seem to be the only case in which the attempt to remove a
tumor of the cauda equina did not result fatally, and the amount of
improvement in this case is questionable. Opening the dura where
a tumor of the cauda equina exists seems to be a serious undertaking.

We should like to accept the opinion of Miiller when, in discussing
tumors, he says that the prognosis from surgical intervention on the
sacrum and lumbar vertebrae is decidedly better than on the other
vertebras and cranium, but statistics are not yet sufficiently large to
warrant this statement. In my own cases, when the tumors could be
examined they were all of such a character that complete removal
would have been impossible, and this seems to have been true of most,
if not of all, recorded cases. The well-defined, almond-shaped fibromas
or fibrosarcomas occurring frequently at higher levels of the cord are
much less likely to develop in the region of the cauda equina. Volhard
regarded the tumor in his case as a glioma, although it was external
to the cord, but intradural. He expressed the opinion that the tumors
of this region that have been described under various names are all
of the same character, and possibly are congenital ; but, so far as I can
judge from my own experience, they are usually sarcomas, and very
often infiltrate about the roots of the cauda equina, or implicate the
surrounding bone. The tumor in my Case II was a fibrosarcoma, but

22 spiller: tumors of the cauda equina

not like the sharply defined tumor of this character often found at
higher level of the cord.

If the prognosis at present is very gloomy, in operating for tumor
at the lower part of the vertebral column, it may be possible at least
to cut posterior roots in order to relieve pain. It is not easy to deter-
mine the individual roots presenting in the opening formed by the
exposure of three vertebrae. When the dura is incised the roots
appear in contact with one another and they cannot be traced except
in a very imperfect manner to their point of origin in the cord, or to
their point of exit through the' dura. The uppermost roots presenting
in the opening are, of course, the sensory, and by observing whether
the roots pass toward the posterior or the anterior part of the cord
it may be possible to determine whether they are sensory or motor.
If the attempt is made to divide all the sensory fibers of any one root,
it is probable that some motor fibers will be included and may be cut,
but it is doubtless unnecessary to cut every sensory fiber of any one
root in order to relieve pain.

The roots of the cauda equina descend far within the dural sac, but
on leaving the dura they pass only a little way downward before enter-
ing the intravertebral foramina ; the sacral roots, however, have a
longer extradural course than the lumbar. Having determined the
lumbar vertebrae, each root at the lower border of a lumbar vertebra
has the same name as the vertebra immediately above it, and we may,
therefore, recognize it, and by tracing its course into the dural canal
the sensory fibers may be separated from the motor.

If the opening in the vertebrae is high enough to permit exposure
of the end of the conus, the second lumbar root may be determined
by finding the first root leaving the dura below the conus ; in the adult
this is the second lumbar root.

The sensory roots supplying the bladder and rectum are at the in-
terior of the cauda equina, near the conus, and it may be possible to
leave these uncut. In cases in which the bladder and rectum are not
paralyzed great care should be taken in cutting posterior roots to
avoid the roots to the bladder and rectum, as paralysis of these im-
portant organs is a most undesirable complication. A few cases in
the literature in which the posterior roots of the cauda equina have
been cut to relieve the pain of tumor are referred to in this paper.
It is permissible only when the tumor is irremovable and the pain is
intense.

spii.ler: tumors of the cauda equina

Original Observations.
Case I.— The first case of tumor of the cauda equina which I have
studied was in the service of Dr. Dejerine, at the Salpetriere.^* This
case was reported in 1895 by Dr. Dejerine and myself, more, however,
as an anatomical and pathological study. The tumor was a round
cell sarcoma. The patient had had pain and paralysis in her lower
limbs eighteen months, implicating chiefly the right side. The opera-
tion was performed by Chipault. The third, fourth, and fifth lumbar
and first sacral vertebrde were operated upon, and the tumor was found
within the dural sac implicating the roots of the cauda ecjuina. Hem-
orrhage was profuse, and the operation was abandoned. Death oc-
curred three hours later, and at the necropsy the lower vertebras were
found implicated by the tumor.

Case II. — Mrs. E., about fifty years of age, was first seen by me
August 7. 1906, in consultation 'with Dr. N. L. Knipe, at which time
the following notes were made : Pain began in the left hip one or,
posssibly, two years ago, but at first occurred only occasionally. In
February, 1906, the pain in the left lower limb began to be severe,
and Dr. Cleveland was called to see the patient. In about two weeks
after the pain had become severe in the left lower limb, it was felt
in the distribution of the right sciatic nerve, but here it lasted only a
few days. Dr. Cleveland, when he first saw the patient, regarded
the pain in the left limb as sciatica, which it resembled. The pain
has persisted until the present in the left limb. Mrs. E. has always
had a tendency to constipation, but this has been more pronounced
during the past year. She was catheterized during one or two months
while in the Presbyterian Hospital in the spring of 1906, but not since,
and the retention of urine was attributed to the patient being in bed.
Whether or not it was a transitory paralysis of the bladder is uncertain.
Pain has been felt since February, 1906, at the end of the spinal
column deep in the tissues and extending upward to about the lumbar
region, but not above this. Periods of improvement had occurred.
The pain came on in attacks and was very severe, and was chiefly
over the sacrum and coccyx, but she has had some severe pain down
the front of the left thigh. Pain was seldom in the right lower limb.
It was through the left hip, but not in the right hip.

Present Condition. — She has no motor weakness, no atrophy, no
objective sensory changes in the lower limbs, no points of tender-

^* Comptes-rendus des seances de la Societe de Biologic, seance du Juillet, 1895.

2-i spiller: tumors of the cauda equina

ness over the sacrosciatic notch, or over the sciatic nerves in the hmbs.
Lasegue's sign is not present, as no pain is produced by overflexing
either lower Hmb. The le-ft patellar reflex is diminished ; the right is
about normal. The Achilles-jerk is weak, but present on each side.
The patient has not been out of bed for months on account of the pain.
She can move either lower limb freely.

Tumor was the diagnosis made by me at this time, but the absence
of motor paralysis and the almost unilaterality of the symptoms made
the diagnosis a guarded one. ^lultiple neuritis was possible, but the
severe attacks of pain and the absence of tenderness to pressure over
the nerves were not in favor of this diagnosis. \^aginal and rectal
examination were advised.

Dr. Knipe informed me, on August 30, 1906, that the patient had
had none of the agonizing attacks of pain since she was seen by me,
but this was not unusual, as these attacks had in the past recurred
after long periods of comparative comfort. Dr. John Hirst made a
vaginal examination, but could discover no timior of the pelvis, no
enlargement of the sacral bone, and no other abnormal condition
except a deflection of the uterus to the left side and adhesions on that
side. He thought this might be sufficient to cause some pressure upon
the sacral roots. The patient had been complaining of great sore-
ness in the back, especially in both buttocks and thighs.

She was seen again by me October 23, 1906. in consultation with
her physician. Dr. Cleveland, when the following additional notes were
made : She has had severe pain in the right lower limb about two
months, but the pain in the left limb has become less severe during
the past few weeks ; she has had pain down the front of each lower
limb, as well as down the -back of the limbs. The pain has been severe
in each lower limb and like toothache. Both legs below the knees
feel numb, and she has a sensation as of a band about the right ankle.
Since last week she has become weak in her lower limbs, but was not
so previously. She has had some rise of temperature a long time, and
has cystitis with albumin and casts.

Present Condition. — Sensations of touch and pain are normal in
all parts of the lower limbs; sensation of touch is normal about the
anus and vulva and in the perineum. She can flex each thigh at the
hip very little, but has more power in the legs than in the thighs.
The voluntary power at the knees, ankles, and toes is better than at
the hips, but is not normal. The patellar reflex is lost on each side.

spiller: tumors of the cauda equina 25

She has no Babinski sign, and the plantar reflex on each side is un-
certain, if present. The lower part of the sacrum may be more tender
to pressure, but tenderness to pressure is present over the spine as
high or higher than the lumbar region. She has no girdle sensation.
The muscles of the calves are a little tender to pressure. As objective
sensation was normal and micturition and defecation were not much,
if at all, affected, it seemed to me that the tumor could not be within
the substance of the cord, nor could it have destroyed any of the roots.
It probably involved the lower lumbar and upper sacral roots chiefly.
She was examined by me again in association with Dr. Frazier,
November 9, 1906. The following notes were then made : The move-
ment of the toes is performed fairly well on each side, but not with
normal force. She can flex the right ankle very well, but has very
little power in the left ankle. She can raise the right knee from the
bed better than the left. The^ voluntary power at the knees is less
than that at the toes. She is very weak, also, at the hips. Sensations
of touch and pinprick are normal everywhere, even in the perineum
and around the anus. The patellar reflex and Achilles tendon reflex
are completely lost on each side, even with reinforcement. She has no
involvement of the bladder and rectum. Pressure over the lumbar
vertebrae gives more pain than anywhere else over the vertebrae. Move-
ment of the lower limbs causes no pain in her spinal column.

Urinalysis. — Cloudy, straw color, flocculent precipitate ; neural,
1008 ; albumin and sugar, negative. Under the microscope : a trace
of mucus, a few white blood cells and red blood cells, a few^ mixed
epithelial cells, few leukocytes, oxalates with triple phosphates.

Blood Exmnination. — Hemoglobin, 90 per cent. ; red blood cells,
5,440,000; white blood cells, 8960.

November 10, 1906, an operation was performed by Dr. C. H.
Frazier. A horseshoe incision was made in the midline, with the
base at the lower border of the first lumbar vertebra, exposing the
structures from the first to the fifth lumbar vertebrae. Laminae and
transverse processes of the fourth lumbar vertebra were removed,
then those of the third, and finally those of the second. There w^as
no sign of tumor outside the dura. Nothing abnormal w^as observed,
and no adhesions were found between the dura and the vertebrae. The
dura was slit in the length of the opening in the three vertebrae. A
blood clot was removed from within the right side of the dura, about
one inch long, one-quarter inch wide and flat, and having a red

26 spiller: tumors of the cauda eouixa

gelatinous appearance. A soft, friable mass was seen connected with
this clot, within the dura, more on the right side and opposite the body
of the third lumbar vertebra. It was about one-half inch in diameter
each way. The cord pulsated after the roots of the cauda equina were
separated. Only a small quantity of cerebrospinal fluid escaped. All
the tumor found was removed, but in pieces. There was no growth
outside the dura. The dura was closed with three or four interrupted
catgut sutures. The muscles were closed with continuous catgut
sutures and the skin was sutured with interrupted silkworm-gut
sutures. A cigarette drain was introduced down to the dura through
a stab wound in the flap of the skin.

A day or two following the operation the pain in the lower limbs
was intense, and weakness of these limbs was increased ; but by the
fifth day after the operation the pain was much less than before the
operation, and the patient could draw up the lower limbs as well as
before the operation, and move the left ankle and right lower limb
better than before the operation. She died on the morning of Novem-
ber 25. The spinal cord was removed in my presence a few hours
later. A cyst, almost empty, was found by the side of the lower part
of the conus ; it was about the shape and size of an almond and was
ruptured at the lower end. The clot of blood removed at the operation
probably came from this cyst. Some tissue was found within the cyst
that looked like tumor, and a small mass of tissue, looking like tumor,
about 2x4 mm. in size, was found on the roots where the tumor had
been removed, that is, about opposite the third lumbar vertebra. No
evidence of infection was detected.

The cyst (Fig. i) extends within the roots of the cauda equina a
half inch upward above the termination of the conus, and is one inch
long and half an inch wide. It does not implicate the roots of the
lower part of the conus, and, probably because of this, the bladder
and rectum escaped. These lowest roots are caught in the small mass
of sarcoma tissue still remaining. The tumor is a fibrosarcoma.

In this case the pain began in the left hip. Miiller says that the
outer and posterior parts of the pelvis are supplied by the iliac branch
of the iliohypogastric nerve and the lateral cutaneous nerve of the
thigh, from the lumbar plexus (second or third lumbar, according to
his chart). Later, the pain was felt in the course of the left sciatic
nerve (first and second sacral according to chart), and the right sciatic
was affected temporarily and later permanently. This meant extension

spiller: tumors of the cauda equina

to the sacral roots. Still later, pain was felt down the front of each
lower limb (fourth and fifth lumbar, according to chart). This part
is supplied from the lumbar ])lexus. The patient had a sensation of
numbness in both legs below the knees, therefore in the sciatic distribu-
tion (first and second sacral, according to chart). She also had pain
at the end of the spine. This part is supplied from the lowest sacral
and coccygeal (fourth and fifth sacral) roots. The weakness in the

Fig. I. Fibrosarcoma and blood cyst in the cauda equina. Vesical and rectal
symptoms were not present, although the tumor was in the centre of the cauda
equina.

lower limbs meant involvement of lumbar motor roots, and the greater
power of the legs, as compared with that of the thighs, meant less
involvement of the anterior roots forming the sacral plexus, than of
those forming the lumbar plexus. Loss of patellar reflexes meant
lumbar involvement also. Important was the escape of the nerves
for the bladder and rectum (fourth and fifth sacral, according to
chart), and absence of all objective disturbance of sensation. The
syinptoms pointed to a lesion beginning on the left side, then extending
to the right side, and involving the second, third, fourth, and fifth

28 spiller: tumors of the cauda equina

lumbar, and first and second sacral roots, the posterior more than the
anterior. Pain alone at the end of the coccyx suggested the involve-
ment of lower sacral nerves, but the escape of the bladder and rectum
showed that such involveme-nt if it had occurred must be slight. The
tenderness to pressure was not strictly localizing, as it was felt both
over the sacrum and the lumbar vertebrje. It did not seem probable
that the tumor was in the center of the cauda equina, as the bladder
and rectum escaped. It might be a tumor on the outside and upper
part of the cauda equina not affecting the innermost nerve roots (third,
fourth, and fifth sacral and coccygeal; escape of bladder and rectum) ;
this w^ould be indicated by the involvement of the second and third
lumbar, and later, of the first and second sacral, and fourth and
fifth lumbar. It must, if within the dura, be high enough to catch the
second and third lumbar roots, and if so it could hardly be a very long
tumor or it would extend downward far enough to catch the lower
sacral roots ; nor could it be a very large tumor, as, at the exit of the
second and third lumbar, it would press upon all the roots of lower
origin and cause paralysis of them; or, possibh', by extending upward,
might press upon the conus, in which case paralysis of the bladder
and rectum would be likely to occur. It might well be a tumor of
the low-er lumbar and upper one or two sacral vertebrae; this would
explain the escape of the bladder and rectum and the extensive area of
tenderness to pressure over the sacrum and lumbar vertebrae. The
second and third lumbar roots could hardly have been involved at
their exit from the cord, as the implication of the fourth and fifth
lumbar and first and second sacral would imply in that case a large
tumor, and this would probably cause pressure upon the cord sufficient
to produce more impairment of function. It was evident that none
of the sensory roots was completely destroyed, as even to the time of
operation no objective disturbance of sensation was found. For
localization in operation, the second, third, and fourth lumbar seemed
to be the proper vertebrae. The blood cyst probably caused the rather
rapid development of paralysis in the lower limbs.

Case III. — H. was seen by me July 31, 1905, in consultation with
Dr. MacLeod and Dr. Edward Martin. He had also been seen by
Dr. Alfred Stengel and Dr. Charles K. ]Mills. My notes, made July
31, 1905, are as follows: In the early part of 1905 he had a fall on
the ice and came down heavily on the buttocks. About three months
ago he began to have pain in the lumbar region and down the left

spiller: tumors of the cauda equina 29

lower limb, following the sciatic nerve. In about four or five weeks
the pain was felt in the course of the right sciatic nerve. The case
was supposed then to be one of double sciatica, and the patient was
sent to Hot Springs, Virginia. All venereal disease is positively
denied, and the wife has had no miscarriages. While at the Hot
Springs he found that the urine did not come promptly, and gradually
retention developed, and he had to be catheterizcd, and his bowels
became constipated. Then weakness of the lower limbs developed, and
gradually became a complete paralysis. He thinks he has had some
pain down the front of the thighs. Dr. MacLeod, of Bryn Mawr, who
has been attending him, says that the patellar reflexes at first were
very prompt. He has had no involvement of the upper limbs or of the
face, but has had tachycardia; pulse, 130. He has lost flesh, is very
pale and sickly looking. The paralysis of the lower limbs has de-
veloped within about three mqnths. He has been taking 105 grains
of potassium daily and as much as 6 ounces of mercurial ointment
daily, and has stood this treatment well.

Present Condition. — He has complete motor paralysis of the lower
limbs, and cannot move the toes or the limbs at the hips. The patellar
reflexes and Achilles reflexes are completely lost, even on reinforce-
ment. The Babinski sign is not present, and there is no movement
of the toes in either direction. The upper and lower limbs are
emaciated. Sensations of touch and pain are lost below the knees
and in the posterior part of the thighs, or, if not lost, are almost so;
occasionally he seems to have a little sensation in the soles of the feet.
Sensations of touch and pain are preserved in front of the left thigh,
but are diminished, especially in the front of the right thigh, and are
normal over the abdomen to the inguinal region, but are lost or nearly
lost over the buttocks about the anus. There is some tenderness over
the spine in the lumbar region, but no deformity of the spine. The
cremaster reflex is lost on each side. Grasp of each hand is good.
The biceps jerk on each side is prompt. The pupils are equal, and
respond promptly to light. The facial nerves are not afifected. The
tongue and muscles of mastication are normal. The man remained
in this condition, gradually losing strength, until his death, on Septem-
ber 30, 1905, I a.m. Necropsy was held by me September 30, 3 p.m.

The laminae of the lower lumbar vertebrse were so soft they could
be cut with a knife. The interior portion of the dura was adherent
to the bodies of these vertebrae, which also were soft. A mass of

spiller: tumors of the cauda equina

tumor tissue was removed, adherent to the anterior part of the dura.
Numerous hard, bony-hke masses were found in the roots of the
Cauda equina, and most of these were in the roots where they pene-
trate the dura; some were within roots in the dural cavity (Fig. 2).
These masses, though hard, could be cut easily with a knife. No

Fig. 2. Osteosarcomas (indicated by lines) in the cauda equina.

tumor was found within the cord or within the abdominal cavity.
The bedsore over the sacrum was nearly round and about four inches
in diameter, and extended to the sacrum. Sores also were over both
heels. The lower limbs were wasted.

The tumors both within and without the dura contain numerous
calcareous areas resembling imperfect bone formation; within them
are many round or oval cells, and in some places spindle-shaped cells

spillkr: tumors of the cauda equina

like those of a fibroma. They have the appearance of an osteosar-
coma. Calcareous deposits and sarcoma cells are found in one of the
tumors of the nerve roots within the dura examined microscopically.

Sections from the lumbar segments stained with thionin show much
degeneration of the cells of the anterior horns, chromatolysis, peri-
pheral displacement of the nucleus, and loss of dendritic processes
(Fig. 3.). The posterior columns in sections from about the twelfth
thoracic region show intense degeneration by the Marchi method.
Some diffuse degeneration is present in the anterolateral columns, and
the posterior roots from tb.is level are much degenerated.

Fig. 3. Osteosarcoma in one of the roots of the cauda eciuina. The dark
patches indicate calcareous deposits.

Case IV. — H. H.. aged fifty-five years, was seen by me in con-
sultation with Dr. L. Loeb, September 4, 1905, at which time I made
the following notes : He has been having pain in the right thigh about
one year, and, although he has occasionally had pain in the left thigh,
it has only been within the last two months that he has had severe
pain in this thigh. The pain now is over each sacrosciatic notch and is
excruciating and comes independently of any movement. Coughing

32 spiller: tumors of the cauda equina

or deep breathing increases the pain greatly. There is no imphcation
of the upper Hmbs or head. Sensations of touch and pain are normal
in the lower limbs. The patellar reflex is prompt on each side, and
is shown not so much by jerking of the leg as by contraction of the
quadriceps muscle, and it is prompter on the right side. Achilles reflex
is not present on either side. There is no Babinski reflex, and the toes
are not moved distinctly in either direction. There is no distinct
tenderness to pressure over the muscles of the calf, thigh, or anywhere
else ; nor over the nerves, except, possibly, at the sacrosciatic notch.
Some slight tenderness to pressure is felt over the lumbar region.
The spinal column is not deformed. He has control of the urine
and feces. There is no localized atrophy, although he has lost flesh
generally. The pain seems now to shoot down the legs, following
the sciatic nerves, and extends to the feet, and movements of the
patient cause pain. The lower limbs have good voluntary power, and
whatever apparent loss of power there may be is probably because of
the pain. The pain, by extending from the front of the thighs to the
back, and in being bilateral, suggests pelvic tumor.

Additional notes were obtained from Dr. W. W. Keen.

December 5, 1906. In the summer of 1903 he fell about four or
five feet while building a dam. His knees caught between some
rocks and stopped his fall forward, and he fell backward, but did not
strike the end of his spine or injure himself in any way, excepting that
his knees were bruised by the rocks.

He had drunk quite freely in past years. About eighteen months
to a year ago (a little longer than in the statement made to me) he
began to suffer pain in the right sciatic nerve. Some months later
in the left sciatic, later still, the pain involved the anterior crural,
first on the right side, and later on the left side. He has had also
some diffuse pain in both hips. The pain has been progressive, but
has been worse of late on the left side. He had had some obscure
sacral pain, but none in the lumbar region. About four or five weeks
ago he began to lose power in the lower limbs, first in the right leg,
then, about three weeks ago, in the left leg, after a severe attack of
pain. The bowels and bladder have never been involved in any way.
There is no local muscular wasting.

When Dr. Eshner first saw this patient about four weeks ago the
patellar reflex on the left side was absent ; on the right side it could
just be elicited; both of these tests being with reinforcement. There

spiller: tumors of the cauda equina 33

was no plantar reflex. Babinski's sign on the left side was doubtful.
Cremasteric and anal reflexes were normal. The man has never had
any fever excepting two months ago, when for three or four days his
temperature ran up to 103° from some temporary gastric disturbance.
Bowels are regular. An .r-ray photograph showed no alteration.
There has been no loss of sexual power, but inability to perform the
sexual act on account of pain.

Dr. Harvey Gushing, in examination of the pelvis, found nothing
wrong. Dr. Keen found that the cremasteric reflex was elicited only
on the inside of the thigh, but was quite well marked on both sides.
The patellar reflexes were absent, and there was no Babinski reflex.
The man could voluntarily draw up both legs well, the right more
forcibly and quickly than the left, which moved feebly and after re-
peated efforts. He could stretch out both legs, the right better than
the left. Examination of the back showed no tenderness in the
lower dorsal, lumbar, or sacral region either upon pressure or sharp
percussion with the fingers. IMercury and potassium iodide had been
used to the limit of tolerance.

On December 9, 1905, a consultation was held with Drs. Mills,
Eshner, Keen, and myself, at which time the following notes were
made : In the left leg foot-drop is almost complete. Whether this was
true four days ago Dr. Keen did not know. There is no Achilles-jerk
on either side. Voluntary flexion and extension of the toes and ankle
on the right side are present, but, except in very slight movement of
the toes, are absent on the left. The movements are very weak in the
whole left leg. On the right side they are impaired, but much less
so than on the left, and are worse below the knee than above. There
is no anesthesia anywhere. On the dorsum of the left foot there is
very slow appreciation, with slight diminution on the left thigh pos-
teriorly. Sensation on the scrotum is normal. On passively moving
the right leg considerable pain is felt in the sacral region. Moving
the left leg, the pain is much more acute in both legs. It is especially
elicited by flexing the leg on the thigh and the thigh on the pelvis with
the thigh in abduction. Grasping the muscles of the anterior part of
either thigh and compressing them elicits considerable pain on each
side, but more on the left. Squeezing the foot on either side is
especially painful, particularly so on the left. There is no pain or
muscular disability in the arms. The back is rigid. The man was
made to sit on the side of the bed, and this position produced marked

spiller: tumors of the cauda equina

pain in and about the right trochanter; in the joint, as he expressed it.
The pain also extended over the right buttock and sacrum. The con-
clusion was reached that the lesion was probably either a neoplasm in
the spinal canal, most likely in the cauda equina, or possibly a pelvic
sarcoma, which, in consequence of the patient's large form and great
stoutness, could not be detected. Laminectomy in the lumbar region
was decided upon.

^^1 i

^■k.

Figs. 4 and 5. Extradural librosarcoma over the roots of the cauda equina.
The stitcliing of the dura ends just above the tumor, shown as a mass of tissue
on the outside of the dura.

Operation December 10, 1905. Dr. Keen removed the laminse of
the first, second, and third luinbar vertebrae. The dura was divided
the length of the wound. The tumor was not found. The tem-
perature rose to 101° within a few hours after the operation, and
fluctuated between that and 102° until he died at 1.45 a.m. on the

SPILLER : TUMORS OF THE CAUDA EQUINA 35

1 2th, about forty hours after the operation. (Edema of the lungs
set in speedily after the operation, and the patient gradually lost con-
sciousness. His pulse rose to 156, and later became uncountable. The
necropsy was made by me.

A tumor was found on the anterior part of the spinal dura. It be-
gins just above the exit of the second lumbar roots from the dura and
extends to the exit of the third lumbar roots, and is more on the left
side in its upper part, but not in its lower part. The cord appears
somewhat swollen just below the portion exposed at the operation.
The dura was adherent to the bodies of the vertebrse just below the
part exposed at operation. The bodies of two or three vertebrae at
the level of the tumor were soft and could be partly penetrated by
the knife. The tumor seemed to have originated in the bodies of
these vertebrse. The stitching of the dura ended just above the upper
part of the tumor (Figs. 4 and'5).

The tumor contains round and oval cells and also spindle-shaped
cells, with considerable fibrous connective tissue, and has the ap-
pearance of a fibrosarcoma. The nerve cells of the anterior horns
in the lower lumbar and upper sacral region show intense degeneration,
peripheral displacement of the nucleus, chromatolysis, loss of dendritic
processes, and vacuolation. The alteration of the cells is equally
intense on both sides of the cord.

Sections from the fourth lumbar segment show intense degenera-
tion of the left posterior column by the Marchi method, and of the
posterior root fibers entering at this level, but only a slight degenera-
tion of the right posterior column. The degenerated fibers are found
also in the left anterior horn. The degeneration by the Marchi method
is very slight at the first sacral segment, but is more pronounced on
the left side. It is more pronounced at the third sacral segment, and
here also is greater on the left side, but is not nearly so great as at the
fourth lumbar segment.

Case V. — The notes and i)atliological material of this case are
obtained from Dr. Charles K. Mills. S. D. C, aged sixty-two years,
white, male, living at Kennett Square, Pennsylvania, was seen by
Dr. Mills in consultation with Dr. U. G. Gifford, March 15, 1902.
He was well until August, 1901, when he first had j)ain in the back
and along the distribution of the left crural nerve and the thigh. He
gradually lost power in the left leg, and before this loss was complete
the right leg also became aft'ected, the loss of power gradually in-

36 bPILLER: TUMORS OF THE CAUDA EQUINA

creasing until, by December, he was almost completely paralyzed in
both lower extremities. He had more or less diffuse pain in both
legs, but this was most marked in the anterior portion of the left
thigh. Early in the case there was tenderness as well as pain in the
distribution of the left crural nerve. In December, 1901, and Jan-
uary, 1902, he complained of much pain in all parts of the legs. This
pain was not only subjective, but he had much pain on movement
of the legs and trunk and also in spots on handling the legs. Taking
hold of the legs about the ankle, for instance, would cause him to cry
out with pain, and this without any movement of the limbs or body.
Later, the pain on handling became less, but he continued to suffer
with severe pain in the back, and also with pain which radiated from
the back and down the limbs. From about the middle of December
his bowels began to be inactive, and in a short time were completely
torpid or paralyzed. Later about the middle of February, incon-
tinence of the sphincter ani came on. and he had at time involuntary
evacuations. He never lost control of the sphincter of the bladder,
nor were the bladder walls paralyzed, although he occasionally had
some apparent difficulty in completely emptying the bladder. He
was catheterized by Dr. Gifford, who found no residual urine. Sensa-
tion was retained, except, perhaps, on the inner anterior aspect of the
thighs, where he was slightly hypesthetic. He had no girdle sensation.
When examined by Dr. Mills, March 15, 1902, the man was in bed
on his back and unable to move without assistance. The movements
of the thigh on the pelvis and extension of the leg on the thigh on the
left side were completely lost ; on the right side the conditions were
similar, but the loss of power was not so complete. The anterior
aspect of the left thigh showed marked atrophy, and no responses to
the faradic or galvanic current could be obtained in the quadriceps
and other muscles of this aspect of the limb. The same was true on
the right side. The movements of the foot were preserved, but im-
paired, and this was especially true on the left side, that is he could
dorsally flex, extend, adduct and abduct the foot and perform various
movements of the toes, but these were impaired and somewhat un-
certain, especially on the left. The muscles below the knee were
somewhat wasted, but not distinctly atrophied, and responded to the
electric current, but there was some quantitative diminution. Sensa-
tion was retained, except the slight partial loss over the anterior aspect
of the thigh, where the responses were somewhat uncertain. The

spiller: tumors of the cauda equina

bowels and bladder were as above noted. Knee-jerks and ciuadriceps
jerks were abolished; no clonus was obtained; he had the Babinski
reflex on the left side, and on the right side there was no reflex move-
ment of the toes. At this time the man still complained of much
pain in the back and at times of pains radiating to the groin and into
the limbs. He was not tender to pressure over the nerves of the
extremities, and pain was not caused by lateral squeezing or other
manipulations of the foot. The spine was rigid, all movements being
restricted. Jarring caused some pain. Percussion and deep pressure

Fig. 6. Endothelioma on tlic outer part of the dura over the cauda equina.
The tumor has been cut through.

caused pain over the position of about the second and third lumbar
vertebrae, where there was a slight defonnity. He had no symptoms
above the waist line.

The movements of the right foot and leg were as noted at the time
of this examination, but a few weeks previous to this the loss of power
had been greater, and was then somewhat more marked on the left
side. Under treatment he had regained in part the movements of the

38 spiller: tumors of the cauda eouixa

foot on the left side, but no improvement took place in the muscle
groups above the knee.

About the i6th or 17th of the month his temperature began to rise;
he had frequent and profuse sweating; he sank into a torpid and
eventually into a typhoid state ; at times he was delirious ; he grad-
ually became weaker, and died ]\Iarch 25.

The necropsy was held ten hours after death by Drs. Gifford,
Reynolds and Darlington. In opening the spine the left transverse
process over the third lumbar vertebra was seen to be necrosed. It
was loose and dropped out ; no pus was present. The posterior arches
were removed from the twelfth thoracic vertebra to the sacrum ; the
layer of fat and cellular tissue between the periosteum and dura was
normal, except at the first, second, and third lumbar vertebrae, where
it was replaced by what appeared to be a growth, at least, by a reddish,
fleshy-looking mass (Fig. 6). The dura was congested, and the spinal
fluid was turbid and bloody. Examining the bodies of the vertebrae
laterally and in front, they were found to be decidedly diseased, espe-
cially on the left side and in front. Pus, with broken-down bone,
could be seen and felt. Between the dura and the bone was a large
fleshy mass. This occupied the position in front and on, each side of
the cord. The cord itself was intact, although probably considerably
flattened. Permission was given to remove the cord and attached
mass, but not the bone.

^Microscopic examination of the extradural tumor showed it to be
an endothelioma.

Case VL — Probably tumor of the cauda equma. I^.Irs.E., aged fifty-
two years, was seen by me in consultation wath Dr. S. Stalberg in the
beginning of 1906, from whom the following history was obtained :
She was born in Russia. Family history negative. Occupation, ordi-
nary housework duties. She has had eleven children, of whom nine
are living, and has had no miscarriages. She has never been sick,
except many years ago, when she had hemoptysis during pregnancy.
Xo rheumatism or tuberculosis has occurred in the patient or family.
Present illness began about five months ago, with pain and tenderness
over the right sciatic nerve at its point of exit from the innominate
bone. This was followed in two months by the same condition in
the left side, though on the right side the pain was always more severe.
At the end of the third month of her illness she had quite profuse
uterine bleeding lasting three or four days, her menopause having

SPILLER: Tl'MORS OF THE CAUDA EQUINA 39

occurred two years previously. This bleeding was followed by another
in two weeks. A vaginal examination was made by Dr. Borland, who
attributed bleeding to an eroded cervix, but he was unable to find either
uterine cancer or any other pelvic condition causing the " sciatica."

Almost all of the antirheumatics and analgesics, nitroglycerin,
potassium iodide, mechanical means, such as splint (for a few days)
and compression, bloodless stretching, were tried, but without success.
The pain became intense, extending to the muscles of the thigh and
to the knee and back.

Notes dictated by me on February 6, 1906, are as follows : She is
in great pain. Passive extension of the lower limbs causes much pain.
The lower limbs are not greatly wasted. She can flex either leg at
to knee to about the normal extent but can flex either thigh at the
hip only partially, and she prefers to lie with thighs partly flexed, in
order to avoid stretching the sciatic nerves. The patellar reflex is pres-
ent, but diminished on each side ; she has no movement of either leg
on the thigh, and merel/ slight contraction in the (juadriceps muscle,
which is weaker on the left than on the right side. She cannot be
tested for tactile sensation on account of the difficulty to make her
understand. The Achilles reflex is present on each side, but dimin-
ished. The muscles of the calf and thigh seem to be somewhat tender
to pressure, but it is impossible to tell to what extent. She seems to
have voluntary movement of the feet. She complains of some pain
in the lower part of her abdomen, but the abdomen does not seem to
be at all tender to pressure. The muscles of the abdomen are so
flaccid that it is impossible to determine any abdominal reflex. There
seems to be no tenderness over the spinal column, and no deformity
of the vertebrse. She has no disturbance of the bladder and rectum.

February 27, 1906. — ^Extension of the right leg on the thigh, or
left leg on the thigh, causes pain as soon as the leg. is almost fully
extended. The patellar reflex is much diminished and apparently
lost on each side. Achilles-jerk on either side is much diminished.
iVIuscles of calves are very flabby, but pressure of them does not seem
to cause intense pain. She has great difficulty in flexing either thigh
on the abdomen. She can move either leg on the thigh much better
than she can move the thighs. She has no Babinski reflex. Pinprick
is perceived in both legs. There is no disturbance of sensation around
the buttocks or in either lower limb, objectively tested by a pin. She
says the spinal column is tender to pressure in the lower part, but

40 spiller: tumors of the cauda equina

this tenderness cannot be tested positively. It cannot be determined
whether there is any diminution of sensation or not. Any turning of
patient in bed causes great pain, and the sciatic notch is very tender.

March 26, 1906. — The lower limbs are weaker than they were
when she came to the hospital, especially the right. Sensations of
touch and pain are normal in the lower limbs. The patellar reflex
is greatly diminished on each side, or even lost. The Achilles reflex
is prompt on the left side, lost on the right. She still has perfect
control of the bladder and bowels. Aluscular atrophy is present in
each leg, especially the right. Babinski's sign cannot be determined,
because of resistance of patient. She has some tenderness to pressure
in the lumbar region, but no deformity.

The patient left the hospital, and Dr. Stalberg informs me that she
died about May, 1906, six weeks later. She was not under his care
at that time, but her symptoms during these last weeks of her life
were about the same as during the previous period.

Operation was not desired by the patient, and a necropsy was not
permitted.

C.\SE VII. — This patient, a woman about forty years of age. was
seen November i, 1906. She had had signs of pulmonary tubercu-
losis, and had had for some months pain in the right lower limb and
in the lumbar region. About October 24 she had become rapidly
paralyzed in both lower limbs.

The notes of her condition at my examination are as follows : The
lower limbs are completely paralyzed, with the exception of slight
movement at the hips. The muscles of the entire limbs are slightly
sensitive to pressure. The patellar reflex and Achilles tendon reflex
are completely lost on each side. Babinski's reflex is not obtained.
Pressure over the muscles of the calf causes no movement of the big
toe. There is no control of the bladder or bowel. Sensation of pin-
prick and touch seems to be about normal over the front of each
thigh; it is completely lost over the left buttock, to about four and
one-half inches from the anus, and the line of demarcation seems to
be sharp. It is lost to about the same extent over the right buttock,
luit the limitation of this side was not accurately determined, in order
to avoid disturbing the patient. The sensation of touch and pain is
lost in a narrow zone down the back of each thigh, to, or nearly to,
the popliteal space. It seems to be lost or greatly impaired down the
back of each thigh, and in the sole of each foot. In the rest of each

spiller: tumors of the cauda equina 41

leg below the knee sensation to touch and pain is greatly impaired,
as compared with that of the upper limbs. There is no tenderness
over the spine, sacral or coccygeal regions, nor has there been. The
paraplegia of the lower limbs occurred within twelve hours, about
October 23, 1906. She was probably getting weaker in the lower
limbs some time before this. The subjective pain in the left lower
limb has been present only about a week, and is about in the same
distribution as in the right lower limb, namely, in the outer portion of
each thigh. The pain in the right lower limb had been present several
months.

A necropsy was not obtained in this case.

In addition to these seven cases, I have observed, as a chance find-
ing at necropsy, in the service of Dr. Dejerine at the Salpetriere, a
lipoma of the filum termipale and a small osteoma of one of the roots
of the Cauda equina.

The following case is one in which a diagnosis has been very
difficult :

Case VIII. — H. H. P., aged thirty years, was first seen by me in
consultation with Dr. A. D. Whiting, February 12, 1903. He had
done heavy manual work. He was struck on the outer side of the
right knee about eight years ago. He had an extensive synovitis for
three months only. In February, 1902, he began to have pain in
the right knee. No cause was known for this, and he had not had
any injury. He had been working very hard during a strike and over
hours. He went out in all kinds of weather and had great exposure,
with little rest. Pain came at first in attacks, and the first attack
came after he had been out with the snow sweepers. He has never
had pain anywhere else than in the right thigh. At first the pain was
in the right knee only, and later he thought it extended from the knee
up the thigh. He has been treated only for knee trouble. He has
pain in the right hip now also, and thinks he has it in the outer and
inner sides of the right thigh. It is a steady aching pain, but at times
sharp and cutting. When he has the pain all the muscles of the right
thigh are sore to pressure, but no tenderness is felt except when he
has pain. It is worse at night and when he is in bed. He has been
to Hot Springs, Virginia. Atrophy seemed to increase when he was
in bed. He had gonorrhoea ten years ago, but never syphilis. An
^"-ray photograph shows nothing in the knee or pelvis, but the acetab-
ulum of the right side may be a little roughened. Dr. Willard says

42 spiller: tumors of the cauda eouixa

the patient has not bone or joint disease. Xo mahgnant history in
family, but there is tuberculous li^istory on the mother's side. A'ery
recently he has had a sensation like a tired muscle pain on the outer
side of the right leg below the knee. He had been riding horseback
before he had pain below the knee. Two other childrn are in the
family. A sister died of typhoid at the age of twenty-three years,
in ]May. 1900. She was fairly well until she had typhoid. An
older brother is living and has some bronchial trouble ( " fibrinous
bronchitis " ).

Present Condition. — February 12, 1903. The patellar reflex is dis-
tinctly exaggerated on each side, but more so on the right, where it is
much increased. Dr. Whiting says every reflex was normal six weeks
ago. Patellar clonus is obtained on the right side, not on the left.'
Slight indication of ankle clonus on each side. Xo Babinski is found on
either side, and the toes, including the big toes, are flexed. Achilles-
jerk is very prompt on each side, and a little exaggerated. Middle
of right thigh measures 15^ inches: left thigh. 16 inches. ^Middle
of right leg measures 12^ inches; left leg, I2f inches. The atrophy
in the right thigh, inner and outer aspects, is very visible ; atrophy of
the right leg is also very distinct, but the atrophy of the right thigh is
greater than that of the right leg.

Station and gait are nearly normal even with the eyes closed. He
has no Romberg sign and no bladder and no rectal symptoms. Tonicity
of lower limbs is about normal. Sensations of touch, pain, and tem-
perature are normal in the lower limbs and equal on the two sides.
A slight tendency for the right foot to turn inward is observed. Cre-
masteric reflex is present on each side, but not very prompt on either
side, and more distinct on the left side.

Upper limbs are not atrophied, but he has lost weight generally.
Biceps tendon, triceps tendon, and wrist reflexes are very prompt on
each side, but biceps tendon reflex is distinctly prompter on the right
side. \'oluntary power and sensation of touch, pain, and tempera-
ture are normal in the upper limbs. Resistance to passive movement
is normal in the upper limbs. The hands are not wasted.

Blood examination gives normal findings : hemoglobin, about 87
per cent ; over 4.000.000 red blood corpuscles.

Urine, normal ; a faint trace of albumin was found only once.

The head is normal. The tongue is protruded straight, not atrophied.
Jaw- jerk is not increased. ]^Iasseter muscles contract firmly. He

spiller: tumors of the cauda equina 43

has no involvement of muscles of the seventh nerve on either side.
The pupils are equal, irides react normally to light, in accommodation
and convergence. Movements of the eyeballs are normal. Vision is
good.

It is not known how long the atrophy has been present in the right
lower limb.

He has no pain on pressure over the spinal column, or on pressure
of the head and shoulders downward.

I saw this patient again ]\Iay 2, 1903. Pain is constant ; it has
never been less, and at times a little worse than when last seen.
The character of the pain has changed ; it is sharper, and when he
is walking it seems to be confined to the knee. He has no pain in the
left lower limb. When he is awakened at night the pain seems more
in the hip, and at times in the back. He has no symptoms on the
part of the bowels or bladder. His right foot gets cold at times.
Pain is still along the front of the thigh, not along the back of the
thigh, and not usually below the knee, except occasionally it extends
a little below the knee on the front of the leg.

Resistance to passive movement is a little less in the right lower
limb. Right thigh (a little below the middle) measures 15 inches; left,
I5:J inches. Right leg measures 13 inches; left, 13 inches. The
right lower limb does not appear so much atrophied as it did pre-
viously. The right patellar reflex is normal. There is no patellar
clonus on either side. Achilles reflex on each side in Babinski posi-
tion is about normal ; if anything, the right is a trifle weaker than
the left. Sensations of touch, pain, and temperature in lower limbs
are normal, even in front of the right thigh. Gait is normal, except
when he has pain. He had some pain just above the pelvis in back
during past three weeks ; since, he has had passive movement of limb.
The pain seems to be confined to the distribution of the right external
cutaneous nerve.

The eyes were examined by Dr. Shoemaker. His report is as
follows : " The results of my investigations into the ocular condition
of the patient are negative. His vision, accommodation, fundus,
pupils, ocular movements, muscle balance, and visual fields are normal."

I saw him again November 14, 1903. He had been in California
for five months riding horseback and exercising in the open air, and
had had Swedish movements, hot sulphur and hot mud baths. The
pain has continued as severe as ever, and is in the same place. Some-

44 spiller: tumors of the cauda equina

times he has pain above the crest of the ihiim. and it may extend as
high as the lumbar region. He thinks the pain is a httle more severe
at times. He has never had pain in the back of the thigh nor on the
inner side of the thigh. On rare occasions it may extend on the
outer side of the right leg half way below the knee. Never has he had
any pain in the left lower limb, and never had disturbance of bowels
or bladder. He has been .r-rayed repeatedly, and nothing abnormal
has been found. Pain is worse about 2 inches above the patella
on the outer side of the thigh. Middle of right thigh measures 16^
inches; of the left thigh. 17 inches. Aliddle of right leg measures
12^ inches ; of left leg, I2| inches. The right thigh is distinctly
atrophied, especially in its inner portion. He weighs now 130.
Normal weight is 150. The whole right lower limb is distinctly
wasted, but more in the thigh. The gluteal muscles on the right side
are also wasted.

The patellar reflexes are prompt, probably more so than normal,
but not excessively so. Patellar clonus is not present on either side,
and he has no ankle clonus on either side. Achilles-jerk is about
normal on each side. Sensations for touch and pain are normal in
all parts of the lower limbs. He walks now with a little limp in the
right lower limb. No points of tenderness are found in the lower
limb. The patellar reflex on the right side may not be quite so
prompt as at the former examination.

He was seen by me again April 30, 1904. Since he w^as last seen
Dr. Whiting cut the external cutaneous nerve, December, 1903 ; he
did not suture it or bring the ends together. In February, 1904,
Dr. Whiting made an exploratory operation in the lower part of the
right thigh, and nothing abnormal was found. General condition has
been good, with, at times, mental depression. Pain is unaltered. It
still centers about the knee, but extends now more below the knee than
into the thigh. He limps, but limping seems to depend upon the pain
and varies with the intensity of the pain. The electrical reactions
are normal in quality, but diminished quantitatively. The right lower
limb, both thigh and leg, is distinctly wasted, and the wasting of the
leg is greater than it was at my last examination. The patellar reflex
on the right side at times is weak, the leg is thrown forcibly out, but
the movement is sluggish. The patellar reflex on the left side is about
normal. The Achilles-jerk on the right side, Babinski position, is
not quite so prompt as on the left side. The pain is usually on the

spiller: tumors of the cauda equina 45

outer side of the leg, below the knee, seldom on the inner side of the
leg, and is of the same character — " sharp, like a toothache or head-
ache— a steady grind, never darts down the limb." Resistance to
passive movement is not so good in the right lower limb (flexion and
extension of leg or foot) as in the left lower limb. He is analgesic
and anesthetic in the outer part of the thigh in the distribution of the
external cutaneous nerve, but he has no paresthesia in this area. He
has no Babinski sign on either side. Gait is not ataxic, even with the
eyes closed. No vesical or rectal disturbance.

At the present time, February, 1907, his condition is about the same
as at my last examination. ?Ie has been seen by Drs. Osier, Erb,
Gowers and other neurologists in this country and in Europe. A
small non-malignant tumor, such as the small osteoma I have described
above, implicating a few nerve roots, might explain the symptoms in
this puzzling case.

I am indebted to Dr. Alfred Reginald Allen for most of the
photographs.

From the Department of Neurolog>' of the University of Pennsylvania.

A NEW DIAGNOSTIC SIGN IN RECURRENT LARYNGEAL

PARALYSIS ^

By Alfred Reginald Allen, M.D.
instructor in neurology and in neuropathology in the university of

PENNSYLVANIA, ASSISTANT NEUROLOGIST TO THE PHILADELPHIA
GENERAL HOSPITAL

I make the report of this sign, not with the expectation and hope of
its becoming one of the great diagnostic methods, but rather because it
seems to me of interest in that it permits of a quantitative estimation
of the contractile abihty remaining in a vocal cord in cases of recurrent
laryngeal paralysis.

I have noticed in these cases where the lesion is monolateral that
there is a very material difference in the upward excursion of pitch
when the vocal apparatus is stimulated electrically during the singing
of a tone.

To demonstrate this it is necessary to bare the neck completely and
by careful palpation determine the angle on each side of the larynx
formed by the cricoid and the thyroid cartilages. A small button
electrode with a thumb circuit breaker is placed in this angle over the
cricothyroid membrane, pressing back slightly the sterno-hyoid muscle,
and then the patient is instructed to sing the note C. For a man this

should be -^ -=i ^ and for a woman m — J hi- On the normal

side there will be a rise in pitch equal to from seven to fourteen half-
tones, or in other words the excursion will be an interval of from a
perfect fifth to a major ninth or over. On the paralyzed side, however,
the tone will only be raised from two to three half-tones — a major
second or a minor third.

As the amplitude of this excursion varies in the normal individual
according to the pitch of the note from which the test is made, it is
desirable to employ in all cases the same note. For this reason I have

^ Read at the thirty-third annual meeting of the American Neurological Asso-
ciation, May 7, 8 and 9. 1907.

This paper was pul:)lishcd in the Journal of Nervous and Mental Disease, 1908.

1 74

ALLEN : dl\(';nostic sign in laryngeal paralysis 2

suggested the C indicated, as it is found in every voice no matter of
what register. To illustrate how dififerent the findings might be if
a different note were taken, suppose that a man were told to sing E,

"^ -^ , and let us suppose that the voluntary range of his vocal

scale only extended to the G — a minor tliird above this — it would be
manifestly impossible to get an excursion of more than three half-
tones, although his vocal cords were in perfect condition. The C indi-
cated gives a note in every voice which is free from muscular strain,
and for this reason offers the best starting point for the test. The
exact point at which to apply the electrode although easily located
from the superficial standpoint, is at times hard to find and frequently
trials with gentle pressure at dift'erent angles are necessary to elicit
the phenomenon. But when once found the reaction is certain and
admitting of no doubt. The strength of current used has never to
be such as to cause pain.

Unless the physician is able to place the tone C without aid — which
is by no means common — he must have recourse to a tuning fork or
some musical instrument. With a little practice one should become
fairly familiar with the musical intervals if not cortically deficient
in this respect. I should like to call attention to the possibility of
confusing perfect fourths with perfect fifths, and vice versa. This is
easily explained when we remember that the inversion of a perfect
fourth gives a perfect fifth, and of a perfect fifth gives a perfect
fourth. The ear hearing the interval of a fifth at times refers the
upper note down one octave or the lower note up one octave, in either
case producing mentally a fourth. By the same mental process a fifth
can be perceived where a fourth is sounded. The intervals of the
octave, the second, major or minor, or of the seventh, major or minor,
will cause no trouble in detection. The interval of the diminished
fifth, or, what is the same thing, augmented fourth or tritone will cause
confusion to the unmusical.

Thirds and sixths are of easy detection, but it is not always possible
for the uninitiated to differentiate between the major and minor thirds
and sixths. This is possibly explained by the fact that an inversion
of a major interval produces a minor and vice versa. I do not think
it advisable to more than touch on this subject of intervals because
any one so inclined can get a full description of them in any text-book

3 ALLEN : DIAGNOSTIC SIGN IN LARYNGEAL PARALYSIS

on harmony or thorough bass. For this purpose I most highly recom-
mend the first chapter in Hugh A. Clarke's " A System of Harmony,"
Theodore Presser Philadelphia, publisher.

The points to which I have called attention are most necessary to
observe if anything like a quantitative test of vocal cord power is to
be made.

From the Department of Neurology in the University of Pennsylvania.

THE RELATIONSHIP BETWEEN THE SPINAL CORD, THE

SYMPATHETIC SYSTEM, AND THERAPEUTIC

MEASURES ^

Bv S. D. LuDLUM, ALD.

INSTRUCTOR IX NEUROLOGY AND NEUROPATHOLOGY, UNIVERSITY OF PENNSYLVANIA,

PHILADELPHIA

In 1834 there was published a book by William and Daniel Grififin,
brothers — one a physician of Edinburgh, the other a surgeon of
London — in which they say: "We should like to learn why pressure
on a particular vertebra increases, or excites, the disease about which
we are consulted, why it at one time excites headache or croup, or
sickness of the stomach " ; and so on ; and again, " Why in some in-
stances any of these complaints may be called up at will by touching
a corresponding point of the spinal chain " ; " Why that point should
always be sore to the touch in such attacks, ..." and so on.

These observers analyzed 148 cases of various disorders and grouped
them according to regions of spinal tenderness, and the complaints
arranged themselves in groups as the following table- shows :

Cases. Prominent Symptoms.

Twenty-eight cases of cer- Headache, nausea or vomiting, faceache, fits

vical tenderness, 8 men ; of insensibility, affections of the upper extremi-
8 married, 12 unmarried. ties. In 2 cases only, pain of stomach ; in 5,

nausea and vomiting.
Forty-six cases of cervical In addition to the foregoing symptoms, pain of

and dorsal tenderness, 7, stomach and sides, pyrosis, palpitation, oppres-
15 married, 24 unmarried. sion. In 34 cases, pain of stomach. In 10 cases,

nausea or vomiting.
Twenty-three cases of dor- Pain in stomach and sides, cough, oppression,

sal tenderness. 4, o . . . fits of syncope, hicccough, eructations. In one
6 married, 16 unmarried. case only, nausea and vomiting. In almost all,

pain of stomach.

^ Read in the Section on Nervous and Mental Diseases of the American Medi-
cal Association, the Fifty-eighth Annual Session, held at Atlantic City, June,
1907. Published in the Journal of the American Medical Association, 1908.

' This table is taken from the original publication, and the figures are copied
as given.

1 77

2 LUDLUM : SPINAL CORD AND SYMPATHETIC SYSTEM

Fifteen cases of dorsal and Pain in abdomen, loins, hips, lower extremities,

lumbar; i man; ii mar- dysury, ischnry in addition to the symptoms at-
ried, 3 unmarried. tendant on tenderness of the dorsal. In i case

only, nausea.
Thirteen cases of lumbar Pains in lower part of abdomen, dysury,

tenderness. ischury, pains in testes or lower extremities, or

disposition to paralysis. In i case only, spasms
of stomach and retching.
Twenty-three cases, all of Combines the symptoms of all the foregoing

the spine; 4, 0 ... 4 cases.
married, 15 unmarried.
Five cases; no tenderness Cases resembling the foregoing,

of spine.

These early writers thotight that a goodly number of disorders
originated in irritation in the region of the cord, as do certain -writers
of to-day. They state, moreover, that actite inflammations were not
evidenced in the back, but disorders, such as fevers, etc., showed symp-
toms of tenderness, and noted in cases of metastases a corresponding
migration in spinal tenderness. The book is so interesting that one
is tempted to write more about facts and inferences, etc., and to speak
of their remedies of blisters and leeches apphed over the tender areas,
along with their results, but this would become too lengthy, so the
reader is referred to the book.

Shortly after this Marshall Hall (1841) published his work estab-
lishing the status of a spinal reflex, thus opening the way for an
vmderstanding of why there is pain in the vertebrae, referred to by the
Griffin brothers, and physiologic and anatomic research has accumu-
lated from that time to this. Methods of therapy since 1834 have
fluctuated from the pole to the antipodes, leaving leeches and blisters
in the background, and have included massage, hydrotherapy, elec-
tricity, mechanotherapy, osteopathy, Christian Science, and what not.
Many of the therapeutic methods have accomplished excellent results.

It is the purpose of this paper to bring together enough evidence to
show that the many procedures of external therapy have been based on
the principle of the spinal reflex demonstrated first by Hall, and
evidence of which was clinically shown by the Griffin brothers, and
that the diseased part, when it is not in a quiescent stage, shows itself
reflexly by means of tenderness in the para-vertebral tissues.

Dana, in his clinical study of neuralgias, in 1888, divided the surface
of the body into sensory areas in which were associated pains from
various organs. He noted the sensory nerves which originated in

78 .

LUDLUM : SPINAL CORD AXO SYM PA'ITIKTIC SYSTEM

these areas and correlated them with the sympathetic gangha supplying
these regions.

Head, in 1893, demonstrated still more accurately the cutaneous
areas and maximal points of pain related to the viscera and their
corresponding spinal segments. The accompanying figures show his
results. It can be observed that the tender areas noted by both investi-
gators agree substantially with the regions of the tender vertebrae
recorded by the Griffin brothers seventy-three years ago.

Henrik Kellgren,in Sw^eden.in the early days of his practice observed
that inflamed or irritative conditions of various internal organs, etc.,
are in most cases accompanied by tenderness in various spinal nerves,
which is especially marked over the sites of the communicating cords
to the sympathetic ganglia ; moreover, it has been established clinically
that friction over tender areas can lessen the morbid conditions of the
parts specified.

P. H. Ling (1834) noted tenderness in heart aifections when fric-
tions were practiced over the fourth or fifth dorsal nerves of the left
side.

The Swedish gymnasts have established areas of tenderness for
various organs, over which they apply frictions, vibrations and mas-
sage. These do not in every case correspond to the tender skin areas
of preferred pain, as determined by Head, but they do not vary any
more than observations by dififerent individuals are apt to. In many
cases the tenderness can only be elicited by deep pressure, not merely
by touching the skin. The Swedish school regards the amelioration
which takes place in morbid conditions of various organs, consequent
on nerve manipulations, as being possibly analogous to that occurring
in the case of muscles, stimulation of the sensory nerves over the
muscles causing increased growth and activity. They also consider
that a vasomotor element may be present. They find in morbid
conditions of the stomach, in many cases, tenderness of the sixth,
seventh and eighth dorsal nerves on the left side ; friction on these
nerves may cause eructations. In morbid conditions of the pylorus
the same nerves on the right side are often involved. A book by
Edgar F. Cryax, p. 164 " Kellgren's Manual of Treatment," gives
in specific detail the parts regarded by the gymnasts as being related
reflexly with internal organs.

Lauder Brunton"* has graphically described the action of a mustard

' Principles of Pharmacology.

4 LUDLUM : SPINAL CORD AND SYMPATHETIC SYSTEM

plaster. The efficacy of blisters is well known. They act as an
irritant, and hence cause dilation of the peripheral vessels and con-
traction of the deeper channels. Manual movements and massage
reflexly produce the same phenomena.

It has long been known physiologically — that repeated light, mechan-
ical pressure to the skin calls into activity the vasoconstrictors, and
that steady pressure evokes vasodilation. Likewise hydrotherapists
have shown that long continued applications of cold, and brief appli-
cations of heat, produce sedative effects or dilation of vessels, while
prolonged heat or brief exposure to cold produce exciting effects or
contractions of surface vessels. It requires but the interpretation

Fig. I. Anterior cutaneous areas and maximal points of pain related to the cord

segments (Head).

of a number of hydrotherapeutic and electrotherapeutic cases to
demonstrate that there is a marked parallelism between the effects of
hydriatic applications and the effects produced by electricity, if ap-
plied wth scientific precision.

The researches of Brown-Sequard, Charcot, Winternitz. Benjamin
Barac, Fleming, Simon Baruch, Chapman and other hydriatists have

LUDLUM : SPINAL CORD AXl) SYMPATHETIC SYSTEM

established distinct relationships between the following named external
and internal regions, respectively :

I. Scalp and skin covering neck, upper part of back and face, with
the brain. 2. The precordial region, with the heart. 3. The skin
covering the chest, with the lungs. 4. The middle dorsal region and

Fig. 2. Posterior cutaneous areas and maximal points of pain related to the
cord segments. Relation of cutaneous areas to viscera according to Head. 1-3
D, heart; 1-5 D, lungs; 6-12 D, stomach and intestines; 1-4 S, bladder; 10
D-4 S, genitalia.

epigastric region, with the stomach. 5. The lower third of the
sternum and lumbar region, with the kidneys. 6. The skin overlying
the liver and spleen, wnth these organs. 7. The umbilical region,
with the intestines. 8. The epigastric, the lower lumbar and sacral
regions, inner surface of thighs and the feet, with the uterus and
ovaries.

In general, the skin overlying an organ is reflexly associated with
it, which is the reason why applications of electricity over an organ
usually influence it, and not altogether because the current is passed
through the organ. When these areas are studied comparatively, it

6 . LUDLUM : SPINAL CORD AND SYMPATHETIC SYSTEM

is noted that they are practically the same as those regions pointed
out as showing reflex pain, which would suggest a nervous path from
the organ to the skin and from the skin to the organ, the terminations
of which are in the same visceral and cutaneous fields.

Clinically, the evidence is conclusive that we get reflex pain in
disease from the heart in the precordial region. Head has demon-
strated pain in areas marked i, 2, 3 dorsal, in his diagram, which
include the precordial region, all of which is embraced in the sensory
distribution of the i, 2, 3 and 4 dorsal segments, and which is the
area described by Brown-Sequard and the above mentioned investi-
gators as reflexly afifecting the heart.

I am aware that the statements I shall make in this paper may
require some amendment, but the main outlines will be found to be
true. The skin areas of dorsal segments i, 2, 3 and 4, which include
the precordial region and encircle the body at the level of dorsal
vertebrae i to 4, as shown by Thornburn, Sherrington and others
(these would include Head's areas) would be in direct relation to the
accelerators of the heart, which are located in the first, second, third
and fourth segments of the cord.'* It is well known that prolonged
application of ice to the heart area inhibits active accelerations,
whereas short applications accelerate.

Inhibitory fibers to the heart come by way of the vagus from the
spinal accessory nucleus. They constitute an outflow of the auto-
nomic system, as this nucleus is in close relationship with the auto-
nomic system described by Langley and shown embryologically by
Streeter. They unite with fibers from the inferior sympathetic
ganglion to form the cardiac plexus (also autonomic fibers). Inhibi-
tion is obtained by stimulation of the vagus, and also by prolonged
applications of cold over accelerations in dorsal segments i, 2, 3 and
4, as shown by brief applications of ice to tire heart region hastening
the movement, while prolonged cold to the same region produces
inhibition. Reflex inhibition of the heart is familiar to every labo-
ratory worker who has seen slowing of the heart by stimulation of
the control end of the sensory nerves.

Howell says: "Few subjects in physiology are of more practical
importance than that of vasomotor regulation ; it plays such a large
and constant part in the normal activity of the various organs." The
impetus to study vasomotor phenomena was given by Claude Bernard

* Howell's Text-book of Physiology, 1906, p. 531.

LUDLUM : SPINAL CORD AND SYMPATHETIC SYSTEM 7

when he discovered that by cutting the sympathetics in the neck of a
rabbit, the blood vessels in the ear on the corresponding side became
very much dilated, and that stimulation of the peripheral end caused
the ear to become blanched. Streeter's (Johnstone, " Anatomy of
Vertebrates") drawing of nerves in a six weeks' embryo shows the
close relationship the spinal accessory and vagus bear to the sympa-
thetic system and to the spinal system. While the vagus distribution
spreads over a good deal of visceral territory, yet facts from anatomy
in lower vertebrates would indicate that the viscero-sensory tract was
spinal.

In fishes and other lower vertebrates there exist visceral afferent
fibers taking impulses from the viscera to the central nervous system.
The visceral afferent fibers form a component part of each of the
dorsal nerves of the trunk and head, with the exception in most
vertebrates of the trigeminus and ophthalmic profundus nerve. In
the trunk the fibers have their ganglion cells in the spinal ganglia
and pass by way of the white rami communicantes through one of the
ganglia of the sympathetic nerves to certain of the organs of the
viscera. ""^

In the spinal cord these fibers have their central endings in a part
of the gray matter lying at the base of the dorsal horn, known as
Clarke's column, and perhaps with other cells which lie near the
median plane dorsal to the central canal. This column of cells and
its central relations have recently been proved to be the central ending
of sympathetic sensory fibers in certain organisms. The cells in
Clarke's column pass lateralward to the surface of the cord, and
thence cephalward, forming the direct cerebellar tract (column of
Flechsig) which enters the cerebellum, ending in the vermis. This
fact suggests the possibility that the cerebellum is useful in coordina-
tion of visceral function.

In lower vertebrates the efferent viscero-motor fibers go directly
to the smooth muscles, glands, etc. In higher forms the sympathetic
system is interposed. The visceral efferent nuclei in the cord occupy
a position dorsal to the ventral horn, between it and the visceral
afferent column. In human anatomy this has recently been em-
phasized by Alexander Bruce.'' The fibers from these cells pass, some

^Johnstone's Anatomy of Vertebrates.
"Alex. Bruce: Rev. Neurol, and Psychiat., 1907, i.

S'S

LUDLUM : SPIXAL CORD AND SYMPATHETIC SYSTEM

ludlum: spinal cord and svmi'atiiktic system 9

out of the dorsal root and some out of the ventral root of the eord,
dependhig on the height in the development scale of the ammal.

Tracts from higher brain centers bring iinpulses to both somat.c
and visceral motor nuclei, but much remains to be done to expla.n the
mechanisms by which somatic and visceral activ.ties are correla <h
Collaterals from afferent visceral fibers directly to the visceral efferent
nuclei are probably present in mammals. The short vscero.notor con-
"ections form a two-linked chain between the --ero-sensory and
viscero-motor apparatus. It is reasonable to assume that n, fishes t, ac s
from the cerebellum, or mesencephalic nucle,, brnrg mipulses to the
visceral n,otor nuclei, especially for the coordination of somat.c an
visceral muscles in the act of seizing food. Th,s ts m accord wth
the conception that the cerebellum exercises a coordmatmg action on

*Rli" ning by analogy front lower mamn.als an<l work on human
anatomy by Langley, Onuf, Gaskell and others, ,t seems proper to
describe the anatomy as has been done above, and notmg that n, each
segment of the cord we may have a viscero-sensory ending, a viscero-
motor center, connecting collaterals to each other and cephalward ;
also from this same segment are sympathetic fibers and sensory fibers
from the skin. We can locate these segments by changes in sensation
as did Head, or by cutting roots, as did Sherrington. Each segment
may be afTected bv altered conditions in the viscero-sensory tract
The tonus of the segment is altered perhaps, consequently the afferent
sensory fiber from the skin is hypertonic an.l a sensation of pain is
felt ' By applving heat or cold or other stimuli to the segment of skin
whose endings are in a segment in which arise viscero-motor vaso-
motor or other activities, we can reflexly afl^eet the organ supplied by

these tracts. ^ ^c p,-^,vn

That this can be done has been shown by the researches o Biown
Sequard and others already mentioned, chiefly through the vaso-
moors. The change may be observed by heat and cold, impact o
water hand pressure, steady or alternated electricity, meehamcal
stimulation or other means, yet the un.lerlying principle remains the

In' the digestive tract the vasomotor centers for small intestme and
stomach ha;e been located in the cord in dorsal segments 6 to 12.

Dana's areas of reflex neuralgia for stomach come m the region of
the skin segments supplied by fibers from cord segments hokhng vaso-

10 LUDLUM : SPIXAL CORD AXD SYMPATHETIC SYSTEM

motors to the stomach and small intestine. We find in the stomach
and intestines, according to the skin areas of reflexed pain, that the
segments involved are the sixth to ninth dorsal inclusive.

]\Iy own observations in cases of affection of the heart would indi-
cate that when the heart is not in a state of compensation or is not in a
quiescent condition, tenderness is found in one or more of the first four
dorsal vertebrae and often exhibited in the contiguous erector spinae
muscles when firm pressure is exerted over these parts ; in addition
there may be tenderness in the skin, in any part of the skin distribu-
tion of these segments of the cord, as shown by Head.

In ten cases where the heart was markedly distended, firm pressure
over the spinous processes provoked a feeling sometimes of acute pain,
sometimes of tenderness. The sensations, as shown in Figure 3. A,
were mostly confined to the first four vertebrae and the contiguous
erector spinae muscles. When vertebrae below the fourth showed pain
there were other factors entering into the case, such as marked dyspnea
and constipation or other diseased condition.

In twenty-nine cases of tuberculosis of the lungs twenty-five of them
showed well demarcated tenderness in vertebrae ranging from three to
six inclusive. Where there were marked digestive derangements the
tenderness was continued down the spine to the ninth dorsal vertebrae.
Conditions of asthma and pneumonia showed tenderness in the same
vertebral regions as did tubercular cases (Fig. 3, B).

Those abdominal conditions which I examined gave evidence of ten-
derness on pressure from the fourth dorsal vertebrae down as far as
the second lumbar, and in some cases there was tenderness over the
sacrum, especially in the distribution of the twelfth segment (Fig. 3,
C D E F). These areas correspond quite accurately with the vaso-
motor centers for the digestive tract. The cases were of diseases in
the digestive tract, gastritis, typhoid fever, dysentery, etc. This same
fact is true of the observations on heart affections, that the vertebral
tenderness corresponded with the spinal vasomotor centers. It is also
true of the pulmonary conditions that the spinal tenderness was in the
same region as the location of the origin of sympathetic fibers going to
the inferior cervical ganglia, which is in close relation to the lungs and
heart. This ganglion receives fibers from the upper six dorsal seg-
ments. In the cord from the sixth dorsal to the second lumbar are the
vasomotor cells for the digestive viscera. While they arise as high as
the sixth they do not go to the celiac ganglia until they descend in

LUDLUM : SPINAL CORD AND SYMPATHETIC SYSTEM 11

the cord to the level of the eighth thoracic segment and then emerge
in the splanchnic nerves.

However, putting aside the peripheral anatomy, we find clinically
that our combined areas of tenderness, first thoracic to the second
lumbar, correspond with the location in the cord of the column of
Clarke and the intermedio-lateral column of cells. Onuf, Collins and
others, and lately Alexander Bruce, have called attention to these
columns of cells as being the spinal centers of vasomotor phenomena.
The intermedio-lateral tract in the lower vertebrates sends fibers out of
the cord in both the ventral and dorsal roots. These fibers enter the
sympathetic system and form an efiferent track. They may be excito-
glandular, pilo-motor or vasomotor. Between the column of Clarke
and the intermedio-lateral tract we have collateral fibers. Hence, in a
segment of the cord, we have an afiferent viscero-sensory ending and an
efiferent sympathetic nucleus, with collaterals connecting them. In this
same segment we also have sensory nerve endings from the skin.

Dogiel and Onuf found that the axis cylinder processes of certain
cells of sympathetic ganglia terminate in a spinal ganglion around
cells of a spinal type, thus establishing the existence of a sensory sym-
pathetic nerve element. KoUiker, on the other hand, claims that there
are no specific sympathetic sensory fibers, but that the visceral sensory
fibers are the peripheral branches of the T dividing fibers of the spinal
ganglion cells. Onuf and Collins have shown that apparently both
views are incorrect if adhered to exclusively.

A reconciliation of these contradictory observations can be made
if the view is taken that the sympathetic sensory fiber ends in Clarke's
column, but that during its passage through the spinal ganglion it
gives ofif collaterals. Such a collateral connection would enable us
to localize visceral sensory impressions on the skin surface. Hence,
irritation of the visceral sensory nerves will coexcite the neurones for
the skin, and the superficial tenderness will locate the visceral disorder.
Or it may be, as pointed out by Donaldson, that the peripheral branch
of a sensory nerve splits, and that a portion of the same neurone may
end both in an organ and in the skin, in which case visceral irritation
would so alter the condition of the cell that the skin portion, when
touched, would give a sensation of pain.

However that may be. the observations cited in this paper would
seem to indicate that the combined vertebral tenderness would coincide
with the position of Clarke's column and the intermedio-lateral column,

12 LUDLL'M : SPIXAL CORD AXD SYMPATHETIC SYSTEM

and that the spinal tenderness noted in pathologic conditions of the
heart, lungs and digestive tract would coincide with the location of the
vasomotor centers in the cord.

By applying heat or cold, or other stimuli to the segment of skin
whose endings are in a segment in which arise viscero-motor, vaso-
motor or other activities, we can reflexly affect the organ supplied by
these tracts. That this can be done has been shown by Brown-
Sequard, and others already mentioned, chiefly through the vasomotor
system.

Recently Meltzer and Auer" made an important observation showing
the possibilities of visceral changes taking place as a result of
peripheral agencies. They have shown that caecal peristalsis of the
rabbit is inhibited by dissecting the skin over the abdomen. This is
reflex inhibition, for it does not occur if the spinal cord is previously
destroyed. On the other hand, previous destruction of the cord does
not prevent the direct inhibition of peristalsis observed when the ab-
domen is opened. These observations are in accord with others in
demonstrating what can be done to viscera through reflex action from
the skin.

Bayliss and Starling^ conclude from investigations on animals that
the nervous motor mechanism of the small intestine is by way of the
vagus nerve, and that inhibitory action is conducted by the splanchnics.
They affirm that in the large intestine nervous impulses can arise from
the local nerve plexuses, and that the sympathetic supply, by way of
the splanchnics, has an inhibitory action, and that the pelvic visceral
nerve is motor to both coats. There are, apparently, in the cord
centers, vasomotor and motor and inhibitory libers, all of which it may
be possible to aft"ect by impulses going from the skin into the segments
of the cord containing these centers.

Professor Lennander, of Upsala,^ states that the pain of intestinal
conditions does not emanate from the organ itself, but from the base
of the mesentery and from the parietal peritoneum, which is well sup-
plied with sensory nerves. This being so, the reflex pain area in the
skin might in all cases not be the correct area to aft"ect in order to
cause a change in the visceral area diseased.

Cannon and Murphy, working in the Harvard Physiologic labora-
tory, point out the fact that inhibition of the intestines, when of central
origin, is caused by impulses coming by way of the splanchnic nerves.

' Proceedings of Society for Experimental Biology and ^ledicine, 1906.
*Jour. of Physiology, vol. xxvi, 1900, 1901.
*The Journal A. M. A., Sept. 7, 1907.

LUDLUM : SPINAL CORD AND SYMPATHETIC SYSTEM 13

Hence, any agent that will check the inhibitory impulses from the cord
to the intestinal canal will permit the canal to resume its normal
functioning-, and we might also expect that any agent that would
stimulate the inhibitory influences emerging from the cord would coun-
teract an overactive peristalsis.

I am of the opinion that by therapy, thermal or manual, applied to
these areas of skin whose sensory nerves end in that segment of the
cord whence also originate vasomotor and viscero-motor fibers to the
organs, we can produce a change in the functional status of those
organs. The application of these physical forms of therapy must be
made more and more accurately to get the best effect. In lung
affections, manipulations or cold applications should be made over the
fourth, fifth and sixth segments and not include the region of the first,
second and third, because the^e last are more closely connected with the
heart, and we should avoid producing the same effect on the heart, as
we do not wish this effect to complicate that of the lung condition.
This same principle is true of abdominal affections. If an ice bag is
placed in contact with the whole length of the spine the same effect on
the heart and lungs is produced as that which is desired on the intes-
tines, and the whole process is negative, whereas if limited anteriorly
to the sixth segment the effect is localized on the abdominal viscera.

If we wish to create a dilatation of the abdominal vessels we do not
want also a dilatation of the thoracic vessels, else the abdominal viscera
are not flooded with blood, as desired, because the thoracic organs are
dilated by the same stimuli. This also occurs if the application over-
laps the thoracic segments.

This accuracy of application to specific segments is the underlying
principle of the successful carrying out of a number of different forms
of physical therapy. An ice bag applied to the spine from the sixth
vertebra on down will influence diarrhea; if applied above the sixth
vertebra the heart and lung vessels are also affected, and a large part
of the effect is lost. If a warm application is made over the first to the
sixth thoracic vertebrae the combined applications then work together
by contracting thoracic vessels and dilating the abdominal, which is
just the effect desired. Electrical applications should be given, using
similar principles.

This is true of massage to the back ; a relaxing effect can be given
from the sixth vertebra on down, and a quick contracting effect from
the first to the sixth. By this means the abdomen is flooded with blood
under a good pressure and certain conditions are cured.

Reprinted from the Journal of Nervous and Mental Disease, April, 1908.

ICXAGGERATION OF THE PATELLAR TENDON REFLEXES IN
ACUTE ANTERIOR POLIOMYELITIS^

By William G. Spiller, M.D.

It is taught that tendon reflexes in the affected limbs are lost in acute
anterior poliomyelitis. This is usually the case, but occasionally exaggeration
of these reflexes occurs. When the patellar tendon reflex is exaggerated, the
wasting is chiefly in the leg below the knee, or in the leg of the opposite side.
A case of poliomyelitis with exaggeration of the tendon reflexes was presented
before this society by F. Savary Pearce some years ago. Another has recently
been exhibited by C. E. Atwood before the New York Neurological Society
(Journal of Nervous and Mental Disease, 1907, p. 600). Two cases are
recorded by Minor (Deutsche Zeitschrift fiir Nervenheilkunde, vol. 30, p. 398).
All of these were clinical cases, and no explanation is afforded by them for the
exaggeration of the reflexes.

In Brain, 1903, Dr. Spiller published the report of a case of acute anterior
poliomyelitis with necropsy, following variola. The degeneration of the lumbar
region was typical of this disease, and was confined to the anterior horns. In
preparations made by the Weigert hematoxylin method the anterior horns con-
trasted strongly by their pale coloration with the white columns. A photo-
graph of one of the sections from the lumbar region appears in the article men-
tioned. Implication of the lateral columns occurred in a limited area in the
thoracic region. This case offers an explanation for the exaggeration of reflexes
seen in certain cases of acute poliomyelitis. If the degeneration of the lower
part of the cord does not implicate the cells innervating the thigh muscles, but
is confined to those supplying the muscles of the leg below the knee, as is so
aften the case ; and if the lateral column of one or both sides is implicated in
the thoracic region, exaggeration of the patellar tendon reflex may occur.
Poliomyelitis is not a process absolutely confined to the gray matter of the cord.

The following two cases are examples of acute anterior poliomyelitis with
exaggeration of the patellar tendon reflex.

Case I. R. G. J. Aged 29 years, male, white, was examined February 27,
1904. He had anterior poliomyelitis when about fourteen months old, and since
that time the right lower limb, especially below the knee, was not developed
properly. The right thigh is not much smaller than the left. Sensation in the
right lower limb has not been impaired.

The right lower limb is much smaller than the left lower limb in all parts
below the knee, where there is scarcely any muscular development. The right
foot is in the position of marked talipes equinus, but the deformity can easily
be overcome by passive movement. The man can flex and move the right toes

' From the Department of Neurology and the Laboratory of Neuropathology
of the L^niversity of Pennsylvania.

1 90

SPILLER : EXAGGERATION OF PATELLAR REFLEXES 2

inward, but has no power of extension of the toes. All movement of the right
ankle is lost. The Achilles jerk is lost on the right side and is prompter than
normal on the left side, but the patellar reflex is much exaggerated on the right
side, and is also somewhat exaggerated on the left side.

The upper limbs appear to be normal.

Case 2. B. Colored, aged 14, male, came to the dispensary of the University
Hospital, February 23, 1907. Three years previously he had some disease with
fever and became paralyzed in the right upper and lower limbs simultaneously.
He recovered the use of the right upper limb, so that the grasp of the right
hand is as good as that of the left, and the biceps tendon reflex is al)out the
same on both sides, but not very prompt. The whole right upper limb is dis-
tinctly smaller than the left. The atrophy in the right lower limb below the
knee is pronounced. The right foot is everted and contractured so that the
patient walks on the inner side of the foot. He has steppage gait on the right
side from foot-drop. The patellar reflex is exaggerated on the right side and
about normal on the left side. Sensations of touch and pain are normal in the
right lower limb. The electrical e5camination was made by Dr. C. S. Potts. In
the right leg feeble faradic response is obtained in the soleus, gastrocnemius,
flexor longus pollicis, flexor longus digitorum, and extensor longus digitorum
muscles. The faradic response is lost in the tibialis anticus, extensor proprius
pollicis and peronei. Galvanic reaction could not be determined carefully
because of the patient's resistance, but there was evidently diminished galvanic
contractility in the muscles of the distribution of the peroneal and internal
popliteal nerves. The other muscles responded normally.

Reprinted from the University of Pennsylvania Medical Bulletin, April, ic

INJURIES OF THE SPINAL CORD ^
By Alfred Reginald Allen, M.D.

INSTRLXTOR IN NEUROLOGY AND NEUROPATHOLOGY IN THE UNIVERSITY OF

PENNSYLVANIA; ASSISTANT NEUROLOGIST TO THE PHILADELPHIA

GENERAL HOSPITAL.

(From the Department of Neurology and Laboratory of Neuropathology of the

University of Pennsylvania. Published in the Journal of the American

Medical Association, March 21, 1908.)

It is my purpose to deal with injuries of the spinal cord secondary
to external violence such as gunshot wound, fracture-dislocation of the
spinal column with involvement of the cord, and involvement of the
cord in spinal concussion without spinal lesion. At this time I shall
not discuss injuries of the cord from stab wounds.

There are a few points concerning the anatomy of the spinal column
to which I wish to call attention before discussing any cases of fracture-
dislocation.

From our standpoint the spinal column consists of twenty-four true
vertebrae. The sacrum, which is morphologically five vertebrae, we con-
sider in the light of a single bone. Of the coccyx I shall say very little,
as it is interesting chiefly on account of a possibly faulty ankylosis in
cases of fracture or contusion. The ligaments of the spinal column,
next to the bony conformation of the vertebrae themselves, play the
largest part in the prevention of displacement. The following ligaments
are those concerned in the binding together of the integral parts of the
spinal column : The intervertebral disks, the anterior common ligament,
the posterior common ligament, the interspinous ligaments, the supra-
spinous ligaments, the intertransverse ligaments, the capsular liga-
ments, and the ligamenti subflavae. The last named are composed
chiefly of yellow elastic tissue, and serve rather to close in the vertebral
canal than to give protection from injury or prevent overfiexion.

The articulation between a superior articular process and its con-
tiguous inferior process, is a true diarthrosis of the arthrodial type.
On examining a ligamentous preparation of the spinal column, one is

'Read before the meeting of the Neurological Section of the American Medi-
cal Association, Atlantic Cily, June, 1907.

1 92

ALLEN: INJURIES OF THE SPINAL CORD 2

Struck with the fact that there is very httle movement between any
two vertebrae, with the single exception of the atlas upon the axis.
Taken as a whole, however, there is a very great latitude of movement.
This is most conspicuous in the cervical region where extension is
possible, to a surprising extent ; likewise flexion, to some extent. In
the lumbar region flexion is chiefly predominant. In the thoracic
region, on account of the thinness of the intervertebral disks, as also
on account of the tile-like arrangement of the lamin?e through the
middle part of the thoracic spine, there is very little motion. At the
two ends of the thoracic spine, however, this tile-like arrangement of
the laminre and the obliquity of the spinous processes are much less,
and motion is consequently greater in these regions. Another condi-
tion which limits motion in this region is the articulation of the thorax
and the very heavy binding ^which the heads and tubercles of the
various ribs possess in their stellate ligaments. According to Wagner
and Stolper the greatest capability of extension and flexion of the
spinal column is in the cervical region from the third to seventh
vertebra, and also from the eleventh thoracic to the second lumbar
vertebra.

A vertebral dislocation presupposes in the first place a greater or
less disturbance of the integrity of the intervertebral disks between
bodies of the two vertebr?e in question. One finds numerous cases
cited in literature, where, upon postmortem examination, the inter'
vertebral disk has been pronounced uninjured. I consider this an
absolute impossibility. A more careful histological study would have
determined a torn condition of the disk.

Fractures and dislocations in the thoracic region above the tenth
thoracic vertebra are not so frequent as those in the cervical region,
or as those below the tenth thoracic vertebra. Also, it can be said
that fracture and dislocation more frequently involve the lumbar
vertebrae than the junction between the twelfth thoracic and the first
lumbar. ]\Iore than half of all the fractures of the spinal column are,
according to Pearce Bailey,^ located below the tenth thoracic vertebra.
This statement is hardly borne out by statistics. Burrell,- in a collec-
tion of 244 cases of fracture-dislocation, gives 86 in the cervical
region, 43 in the upper thoracic, 75 in the lower thoracic, and 40 in
the lumbar. When thoracic fracture-dislocation takes place it is on
account of terrific violence, deformity is usually pronounced and the
cord much injured, but, as will be seen further in this discussion,

3 ALLEN : INJURIES OF THE SPINAL CORD

there must be fracture in this region to produce a crushing of the
cord, the possibiHty of a thoracic dislocation without fracture having
been denied by Treves.^

The articular processes of the cervical vertebrae are set well out
from the body, to such an extent, in fact, that any force which would
tend to push a vertebra forward, providing it were great enough to
overcome the ligamentous protection common to all vertebrae, would
not find the bony obstruction, which, lower down the anterior aspect
of the inferior intervertebral notch, ofifers to the posterior aspect of
the superior intervertebral notch of the vertebra immediately below
it. In fact the articular surfaces are so far separated in the cervical
region that with very little longitudinal separation a complete luxation
backward can obtain, the body of the upper vertebra resting upon the
spinal foramen of the vertebra below. As a matter of fact, however,
this luxation is rarely seen, the opposite being usually the case ; that
is to say, the upper or luxated vertebra passes forward. To my mind
there are several reasons for this. The first is that usually the direc-
tion of force which brings about the fracture-dislocation is such that
the spine is flexed instead of extended, and in a flexion the line of
least resistance is certainly forward, on account of the fact that the
anterior superior lip of the vertebral body is rounded off and inclines
downward in the cervical region, while in the thoracic region the
bodies of the vertebrae are a trifle thicker posteriorly than anteriorly.

This inclination forward and downward invites a slipping forward
of the luxated vertebra, which carries with it the intervertebral disk
attached to its inferior surface. This intervertebral disk, in the vast
majority of cases, succeeds in tearing away the anterior superior lip
of the body of the vertebra next below. The obliquity of a fracture
of this nature, that is to say, from above posteriorly in a direction
down and anterior, makes a sliding displacement of the upper frag-
ment very easy, and the deformity is often great. It is this deformity
which most frequently nips the spinal cord in two, or, if it do not
actually sever it, so crushes it as to make all function or restoration
of function an impossibility. This cutting or crushing of the cord
takes place between the posterior upper border of the vertebra next
below the luxated one and the arch of the dislocated vertebra, as illus-
trated in Case VII (Fig. 2).

Another variety of fracture-dislocation is illustrated in Case IV
(Fig. 3) in which the continguous surfaces of two vertel^ral bodies

ALLEN: INJURIES OF THE SPINAL CORD

have crumbled under the forced flexion. The greatest pressure being
exerted anteriorly, the anterior lips, superior and inferior, of the two
vertebrae in question, are chiefly concerned in the crumbling. A second
result of the disposition of the pressure is the forcing backward of the
intervertebral disk. This disk, together with the bony detritus, presses
against the anterior aspect of the cord.

With forced flexion of the cervical spine, the head being pushed
forward and down the chest, the upper or luxated vertebra can slip
forward, the posterior lips of the anterior articulating surfaces riding
over the anterior lips of the superior articulating surfaces of the
vertebra immediately below and becoming locked, as it were, in its
superior intervertebral notches. The attenuation of the spinal foramen
in this case, though pronounced, is not so complete as in backward
luxation, unless the deformity be further accentuated by a crushing
of one or both vertebral bodies, as in the case reported by Carson.*
When we reach the lumbar region, what with the increased thickness
of the intervertebral disks and the great mobility of the vertebrae,
particularly in flexion, we find that the bony obstacles to dislocation
present in the thoracic region do not here obtain, and an antero-
posterior displacement is not only possible but is rendered probable
if there be forced flexion of the lumbar spine at the time of, or con-
sequent to, the injury. Although from a mechanical standpoint it is
theoretically possible to have a pure dislocation in the lumbar region
uncomplicated by fracture, the lower vertebra slipping forward or
backward, yet there are many who think that fracture almost always
plays an important part in these injuries.

In the cervical and lower thoracic regions self-reducing dislocation
without fracture frequently takes place. We are all familiar with the
picture of a case of this kind. A man receives an injury to his spinal
column; careful examination fails to reveal any deformity, crepitus,
ecchymosis, or other signs of fracture. Rut there is a partial or com-
plete paralysis present of segmental nature. If this case come to
necropsy there is found no bony lesion ; there may even be a fair
amount of firmness in the ligamentous binding, but the spinal cord
shows signs of compression opposite an intervertebral disk. At times
this compression is so great that the cord appears as if a string had
been tied tightly around it.

There have been many who have advanced the theory that the
spinal musculature was a preventive factor in dislocation. This I

5 ALLEN : INJURIES OF THE SPINAL CORD

consider is erroneous. As far as prevention of dislocaton or fracture-
dislocation is concerned, the muscles play absolutely no part whatever.
As an argument to uphold this statement, I would call attention to
the fact that the most frequent point for fracture dislocation is between
the fifth and sixth cervical vertebrae. Let us turn to the anatomy of
the muscles in this region. The muscles whose attachments lie directly
over the junction of the fifth and sixth cervical vertebrse are the
multifidus spinge, the semispinalis colli, the complexus. the trachelo-
mastoid, the transversalis cervicis, the cervicalis ascendens, the scalenus
posticus, the scalenus medius. the scalenus anticus, the rectus capitis
anticus major, and the longus colli. Here, then, we have eleven
muscular attachments covering this articulaton on each side, twenty-
two in all. and yet it seems to be the point of election for fracture-
dislocation in the cervical region.

We find the spinous processes more frequently broken from the
fifth cervical to the end of the thoracic vertebra. Curlt^ says that in
the cervical region in less than one-quarter of all cases, and in the
thoracic in more than one-half of all cases, are the spinous processes
broken. In the thoracic region, on account of the peculiar tile-like
arrangement of the laminae, above referred to. when one lamina is
fractured usually several are fractured with it. This, of course, does
not refer to gunshot fractures. The spinous processes in the lumbar
region, according to Gurlt. are fractured in less than one-eighth of all
cases. As a rule transverse and oblique fractures of the vertebral
bodies are nearer the upper than the lower surface. Isolated fractures
of the articular processes are extremely rare.

Although, as stated above, the muscles of the spinal column play no
part in its protection from fracture, yet there are a number of cases
on record where fracture has been caused wholly by muscular action.
One of these cases reported by Gurlt happened in this peculiar man-
ner: A sailor, going in bathing from the deck of a ship, dived and
while his body was shooting downward he realized that there was not
enough depth of water for him to escape an accident. For this reason
and in order to save himself, he threw back his head with all his force
as he struck the water, and sustained a fracture in the cervical region.
Another case, likewise reported by Gurlt. was that of a man violently
insane, who, for purposes of restraint, had to be tied in an arm chair.
In this position he made strenuous nodding movements of the head,

Vi6

ALLEN: INJURIES OF THE SPINAL CORD 6

endeavoring to loosen his bonds. He also sustained a fracture in the
cervical region.

Philipeaux,® in his fracture experiments upon the cadaver, found
that the most frequent point of fracture was between the eleventh and
twelfth thoracic vertebrns, the fracture being the usual variety from
posteriorly and above to anteriorly and below. Experiments of this
nature upon the cadaver fail so completely to reproduce the conditions
present in a spinal fracture accident that the finding of Philipeaux's
is not of great importance as a comparison to the statistics of spinal
fracture-dislocation in the living subject.

Gurlt classifies fractures of the spine as follows : ( i ) Fracture of
the first and second cervical vertebrae; (2) fracture between the third
cervical and the second thoracic vertebrae; (3) fracture between the
third thoracic and the second lumbar vertebrae; (4) those below the
second lumbar vertebrae. He does not think that fractures of the
first class are necessarily immediately fatal, unless there be a pro-
nounced dislocation. He cites the case of a patient who lived eight
days. These patients die because of making some sudden voluntary
or involuntary movement which displaces the vertebrae or fragments,
and causes pressure on the upper cord or the medulla oblongata.
Gurlt calls attention to the fact that in none of the cases he cites,
in which there was a fracture of the odontoid process, was there also
a tearing of the ligamentum transversum dentis, although in several
cases there was considerable displacement. He cites an interesting
case quoted by Sir Astley Cooper,^ in which a syphilitic woman under
mercurial treatment, suddenly, while eating breakfast in bed. had her
head fall forward on her chest and was dead. A fracture of the
odontoid process was found. Another patient of Cline's- had a
fracture of the atlas, and for one year aided all neck movements with
his hands in order to prevent injury to the medulla oblongata. In
these two cases, and in five others, making seven in all, there was frac-
ture of the first or second cervical vertebrae or both. One patient died
instantly. Another patient lived two months. Two patients lived five
months. Still another patient lived eleven months. Another lived
twelve months, and still another twenty-eight months. In only one
of these cases was there any disturbance of the integrity of the joint
between the atlas and the occiput. This joint is tremendously strong,
as anyone can testify who has endeavored to remove the head at the
occipito-atloid articulation.

7 ALLEN : INJURIES OF THE SPINAL CORD

The dislocation of the atlas on the axis occurs in death by hanging.
I present, herewith, a photogroph of such a dislocation involving both
articular processes. The specimen is in the ]\Iiitter IMuseum of the
College of Physicians. The case was one of suicide by hanging. The
spinal cord was found crushed, although the spinal canal was not
reduced in area at the point of luxation more than about one-third
(Fig. i). In the cases enumerated by Gurlt, where the fractured
odontoid process injured the medulla, it did so by backward pressure
of its lower fractured surface.

When fracture occurs involving the phrenic nerve, death is usually
very sudden from paralysis of respiration. There are cases where
the patient lives a longer or shorter time with only a partial incon-
venience in breathing. This may point to a partial lesion lower down
in the cervical region, but some untoward movement will convert a
possible simple fracture to a fracture-dislocation with involvement of
the phrenic nerves, and the patient drops dead.

In injuries to the spinal column the spinal cord can be injured in a
number of ways. I have already spoken of the possibility of its being
transversely squeezed or even cut in two. There are also cases on
record where the plane of injury is rather oblique than transverse.
In one case, reported by Gurlt, the spinal cord seemed to be wedged
into a longitudinal cleft in the posterior aspect of the body of the
fifth cervical vertebra.

I herewith give the histories of nine cases of injury to the spinal
cord : Six of these were consequent to fracture dislocation ; one was
caused by a gunshot wound; one was a pure self-reducing dislocation
without fracture ; and one was consequent to spinal concussion without
spinal lesion.

For the pathological material of these cases I am indebted to Dr.
William G. Spiller, who has most kindly put at my disposal the material
bearing on this subject in his laboratory. One of these cases was
reported by Dr. C. S. Potts, another by Dr. Spellissy, and a third by
Drs. Spiller and Martin.

In the clinical histories of these cases there will be noticed many
statements that seem vague, indefinite, and careless. There will fre-
quently be apparent glaring omissions of data which, for the scientific
consideration of the case in question, are most important. These
shortcoming will be easily understood and condoned by those who
realize how very faulty is the system of history taking in many of our

ALLEN: INJURIES OF THE SPINAL CORD 8

large institutions where the resident physicians are constantly changing
and rarely ever interested.

Case I. — ]\Iale, aged twenty-five years, laborer, born in Italy, was
admitted to the Polyclinic Hospital, November 17, 1899, with the
history that a bank of earth had fallen on his back. On examination
there was discovered a paralysis of both lower limbs and loss of sensa-
tion up to a " short distance " above the umbilicus. There was like-
wise a prominence of " several of the lower dorsal vertebne."

At II o'clock that night Dr. Roberts performed a laminectomy under
ether. The tissue surrounding the seat of injury was ecchymosed,
and when the patient was anesthetized the prominence of the injured
vertebrae was increased. Owing to the injury of the spinous processes
of several of the vertebras as well as the dislocation forward of one
vertebral body, the spinal canal was entered almost at the first incision.
The spinous processes and laminae of two vertebrae were removed.
The bodies of two of the vertebrae were found fractured, and parts
of these had to be removed and the dislocation reduced. In hope
that the reposition might be permanent, two of the vertebrae were
wired around their laminae. The dislocated vertebra seemed to cause
the chief pressure on the cord, the examination of which showed some
swelling below the point of injury. There was, however, no proof
that the cord had been destroyed. A drainage tube was inserted, the
wound closed, and a plaster cast applied to the chest. Catherization
was performed every eight hours. There was no record of suspension
of function of the bladder and rectum, although, from what follows,
I presume this condition obtained.

An examination on November 19 (the second day after the opera-
tion) showed that sensation had returned "to one inch below the
umbilicus " across to the anteriosuperior spine of the ilium on each
side. On the following day the return of sensation had spread down
to the pubes and Poupart's ligaments.

The cast was removed several days after, the wound found in good
condition, the drainage tube taken out, and the part dressed with
acetanilide and gauze. On December 4 it is noted that the motor and
sensory condition remaned unchanged, and that the wound had healed ;
also that a slight cystitis and urethritis had developed during the past
week, for which urotropin, gr. v, t. i. d., was prescribed. Micro-
scopic examination of the urine showed erythrocytes.

On December 12 he had a severe chill about midnight, with a tem-

9 ALLEN : INJURIES OF THE SPINAL CORD

peratnre of 104° F. This condition was ascribed to urethral fever.
He became dehrious. and later perspired profusely. For several days
he complained of pain in his kidneys. On the 13th he had another
chill, and after this his temperature began to fall until the 17th, when
it was slightly subnormal. On the i8th he began passing urine in-
voluntarily. On the 19th it is recorded : " His general condition has
changed quite a great deal for the worse during the last week." On
the 28th it is recorded that there had been no improvement since the
19th, and that he was losing sensation in the lower part of the
abdomen, " showing that there is apparently ascending degeneration
of the cord." On January 2 it is noted that a bedsore had gradually
developed over the sacrum.

The next note of interest is on March 12, when the history records:
" The line between the sensitive and non-sensitive points seems to
be about the level of the superior spinous processes. There his tactile
sense is more quick to recognize a dull than a sharp instrument."
Aleaning, I presume, that the tactile sense is better preserved in this
region than the pain sense. On May 14 it is recorded: " He complains
of a great deal of abdominal pain and of pains in his limbs." Occasional
attacks of depression and also of delirium are reported. On August
12 it is recored: " Patient looks and eats well. Flis general condition
is good." On October 2 the patient was transferred to the Phila-
delphia Hospital, it being recorded on his chart, '' discharged, unim-
proved."

In the records of the Philadelphia Hospital it is noted in his initial
examination that the patient could not walk; there was wasting of
both lower limbs ; the u^ine dribbled all the time, and feces passed
involuntarily. There was noted a diffuse tenderness of the abdomen
which was greatest about the umbilicus. On October 5 the extent
of motor and sensory paralysis was defined as " about two inches
below the umbilicus anteriorly and the level of the second lumbar
vertebra posteriorly." There was also noted under October 5 : " The
extremities are very much atrophied. The toes of the foot are be-
ginning to be contracted, muscles flaccid. Reflexes totally absent."

Notes by Dr. Weisenburg, dated June 13, 1903, state specifically:
" The reflexes, patellar and Achilles-jerks and ankle clonus are all
absent. Plantar irritation produces no movement of toes. Sensation
of touch, pain, and temperature lost in both lower extremities, and

100

ALLEN: INJURIES OF THE SPINAL CORD 10

lower abdomen to a line drawn one inch below the umbilicus. Com-
plains of a girdle sensation."

Patient died July 2"], 1903. No notes on his immediate antemortem
condition can be found. The full postmortem record does not par-
ticularly bear on this study, therefore I shall not quote it.

This case had skilful surgical treatment at once. The kyphosis was
reduced and all refinements of technique were used, and yet here,
as in so many other similar cases, the element of uncertainty is present
as to just how much of the future difficulty was due to the fracture-
dislocation, and how much to an unavoidable manipulation of the
spinal cord on the part of the surgeon.

The microscopic examination of the spinal cord by the Weigert
hematoxylin method showed complete disintegration in the level of
the sacral region. Opposite" the seat of compression, the tenth
thoracic vertebra, there is great destruction and distortion of the
white and gray matter. There are large irregular areas of degenera-
tion, a small amount of normal white matter being left, chiefly in
juxtaposition to the gray matter. This is particularly so in the middle
root zone on one side (Fig. 5).

There is also, on one side, a preservation of many normal fibers
situated between the posterior horn of gray matter and the periphery
of the cord, in the area of the direct cerebellar tract. A segment
above the tenth showed an intense degeneration in the columns of
Goll, in the direct cerebellar tract, and in Cowers' tract. Marchi
preparations show no system degenerations, but some black dots still
remain along the bloodvessels.

Case II. — jVIale, aged thirty-eight years, colored, married, born in
Maryland, was brought to the Philadelphia Hospital from the Jeffer-
son Hospital where he had been for three weeks following an accident
to his spinal column. With the exception of the facts that he had
been a heavy drinker and that he denied venereal disease, his past
history is not relevant.

On June 7, 1901, while at work, he fell from a ladder, a distance
of thirty feet, striking on his back. He was taken to the Jefferson
Hospital unconscious. He regained his consciousness late on the
same day, but was delirious and had to be restrained by straps for
three or four days. When he eventually became rational it was
found that there was complete paralysis, both sensory and motor, of
the lower extremities upward as far as the upper border of the pubes

101

11 ALLEN: INJURIES OF THE SPINAL CORD

and encircling the body. Incontinence of urine and feces came on
about one week after the accident, both micturition and defecation
having been unimpaired up to this time. There was a very tender
protrusion over the lower dorsal and lumbar vertebrae which was the
seat of severe pain on motion. There was a bedsore over the lower
part of the vertebral column.

Dr. Spiller's notes, dated July i, 1901, state that there was complete
paralysis in the lower limbs, the patient being unable to move a muscle,
" even with the greatest exertion." The lower limbs were flaccid
and without contractures. The knee-jerk, Achilles tendon-jerk and
Babinski reflex were all absent on each side. There was no priapism.
Irritation of the sole of the foot produced no toe movement, and the
cremasteric reflex was not obtained.

Owing to his having to be restrained with strapping during his
delirium, there was numbness and paresis of the left upper extremity,
most likely from strap pressure, as his violence had caused skin
abrasions. The dynamometer (lower scale) recorded: " Right hand
10; left hand, o." He moved the right upper limb freely and with
normal power.

Dr. Spiller's notes concerning the left upper limb read as follows:
" He raises the left upper limb at the shoulder and bends it at the
elbow fairly well. He is unable to straighten his fingers of the left
hand. He is able to make a fist with either hand, and the flexor
power in both hands is normal, even in the left hand it is difficult to
open his fist. The weakness is most apparent in the left upper limb
and the extensor muscles of the fingers. The weakness in the left
upper extremity is probably in large part due to muscular injury."

The line limiting the anesthesia was very sharply defined and
passed directly through the umbilicus. On the left side this line was
absolutely horizontal, but on the right side it rose about one inch
above the horizontal. In the middle of the back the line was about
one-half inch above the level of the umbilicus.

Dr. Spiller's diagnosis was : " The man has signs of complete inter-
ruption of the spinal cord in the lower thoracic region. The probable
cause of this interruption is fracture of the lower thoracic vertebrae."

On July II, dulness, singultus, and free perspiration were reported.
On the 1 2th it is recorded that the hiccough had been present almost
constantly, that there was pain in the umbilical region, and that upon

1012

ALLEN: INJURIES OF THE SPINAL CORD 12

irrigation of the bladder much dark-brown fluid and detritus returned
through the catheter.

The patient died at 8.45 p.m., July 14, hiccough having been present
constantly, except for short intervals, three days before death. There
was much albumin reported in the urine.

I shall omit the general necropsy notes with the exception of a
quotation on the kidneys, ureters, and bladder, to wit : '* Ureters
dilated, especially on the left side, where ureter reaches size of index
finger. Kidney enlarged, capsule adherent, and adhesions to sur-
rounding tissue. On removal of the capsule numerous abscesses in
substance of kidney. Pelvis dilated and both pelvis and ureter con-
tain creamy pus. The right kidney was enlarged, less than the left ;
capsule peels readily ; no abscess in cortex. Bladder is distended ;
contains thick, yellowish pu^." From Dr. Spiller's dictation at the
autopsy I quote the following: "The scar of an old injury 4.8 cm.
long, cicatrix of which is directly over the spine of the eleventh
thoracic vertebra, extends on to the tenth and twelfth vertebrse. The
spinous process of the tenth vertebra is driven downward. The
surface edge shows an irregular loss of substance, affecting also the
entire superior surface. The interval between the tenth and eleventh
spinous processes is increased. Beginning 5 mm. from the superior
surface of the spine there is an irregular fracture line extending
laterally on each side and passing through the corresponding laminae.
The right lamina is prominent and shows the chief injury. The
fracture on this side extends downw^ard and forward througli the
entire diameter of the lamina. The tissues about the lamina are
edematous and slightly hemorrhagic. On the left side the tissues are
involved to a slight extent, but the remains of the hemorrhage are
found in the fascia and spinal muscles. The fracture extends through
the upper portion of the body of the tenth thoracic vertebra so that
the two portions of the vertebra move freely on one another. The
spinal cord is apparently completely compressed at the line of fracture,
and is softened for a distance of 4 cm. above this line. The softening
at the line of fracture may have been produced at the autopsy. The
cervical region shows no gross lesions. The dura below the line of
fracture is much injected, much more so than above." Spiller founds
his paper, " The Sensory Segmental Area of the Umbilicus," on this
case.^

I wish to call attention to the urinary condition in this case. In

103

13 ALLEX : INJURIES OF THE SPINAL CORD

many cases of fracture-dislocation, involving the lower thoracic region
of the spinal column, hematuria is a common symptom. This is at
times caused by trauma to the kidney substance, but there are likewise
many of these cases in which the kidney is uninjured, and yet in which
hematuria comes on the second, third, or fourth day, or even later.
Wagner and Stolper^" in speaking of the temporary suppression of
.urine often seen immediately following spinal fracture, advance the
theory that this suppression is due to the fact that consequent to the
attraction of blood to the lower limbs on account of the vasomotor
palsy of these parts there is produced in the kidney substance a
traumatic anemia. This anemia causes a great lowering in vitality
in the kidney parenchyma which proceeds in many cases to the abso-
lute death of the epithelium lining the tubules. These investigators
found this condition of afifairs two hours after the accident.

It seems to me that the hematuria, above referred to, may well be
explained on the same hypothesis : the vascular equilibrium being re-
stored the kidney finds itself supplied with blood, which, on account
of a badly damaged parenchyma, it cannot handle, and hence the
hematuria. I have recently seen this picture in a dog, a portion of
whose spinal cord I exsected in the mid-thoracic region. In this
animal the hematuria set in on the fourth day after the operation.

Case III. — Male, aged fifty-six years, carpet weaver, born in Eng-
land, was brought to the Philadelphia Hospital thirty-six hours after
falling down stairs. He denied venereal history. He stated that
while passing from one room to another he fell down a flight of stairs.
It was Saturday night and he had been drinking ale. He was rendered
unconscious and did not regain consciousness until next morning,
when he found he had no power in his legs and was unable to walk.

On his initial examination, at the hospital, it is recorded that he
had " no feelings whatever " in his lower limbs, and was suffering
from retention of urine and feces. Pupils reacted normally to light
and accommodation and convergence, and were equal. The abdomen
was distended and there was dulness above the pubes due to retention
of urine. The arms offered poor resistance to passive movements
of flexion and extension. The biceps tendon-jerk was present and
increased, but the triceps tendon and wrist-jerks were absent. There
was present hypalgesia and hypesthesia from " about the shoulders "
downward. The legs offered no resistance to passive movements,
paralysis being complete. Total loss of pain and tactile sensation

104

ALLEN: INJURIES OF THE SPINAL CORD 14

upward upon chest to a line one and one-half inches above the nipples.
Regarding the decubitus, I quote as follows : " Patient, well-nourished
man, lies with fingers flexed, forearm flexed on the arm, the arms
elevated about 15 degrees from body." A slight power of flexion and
extension at elbow was preserved, and he could elevate the upper
extremities to right angles with the trunk. The act of pronation and
supination of the forearms seemed to be accomplished by the biceps
muscle as there was no contraction of the supinator longus when this
motion was executed. There was no paralysis in either upper or lower
extremities. The plantar reflexes were slight, the big toes flexing.
The knee-jerks, Achilles tendon-jerks, cremasteric, epigastric, and ab-
dominal reflexes were all absent. The ciliospinal reflex was present.
There was a marked retraction of abdominal muscles in respiration.
At times his penis became partially erect. The patient complained of
pain in the back of the neck. There was a tenderness extending over
the seventh cervical and first dorsal vertebrae. A bed sore had begun
over the right trochanter and one also over the scapulae. The stick
of a pin was followed by a persistent hyperemia of the skin. The
patient could slightly rotate his arms outward.

On September 16, Dr. Potts has noted that when the soles of the
feet were irritated the toes were neither flexed nor extended ; also,
there is noted under this date that the right biceps-jerk was " prob-
ably " absent, but that the left biceps-jerk was plus. At times the
patient was delirious. The urine had albumin present, also hyaline
and granular casts.

Dr. Weisenburg has made the following notes : " Patient's face blue
and cyanotic, difference in color between his face and hands being
more marked; left pupil is dilated slightly. The left palpebral fissure
is narrower than the right. Right biceps-jerk is better marked to-day
than it was yesterday."

On September 19 the patient became cyanosed, tem]:ierature elevated,
pulse rapid and weak, and the respiration became labored. Both
pupils were contracted and the reaction to light could not be obtained.
On this date the patient died.

At the postmortem examination a fracture of the skull was dis-
covered over the left parietal and temporal bone, beneath which was
an extradural blood clot 8 cm. in diameter and having a maximum
thickness of 1.5 cm., situated mainly over the motor area. There was
no subdural hemorrhage, and the ventricles were normal. The spinal

105

15 ALLEN : INJURIES OF THE SPINAL CORD

cord exhibited a depression which corresponded to the body of the
fourth cervical vertebra which had been shghtly displaced backward.
The depression was so deep that the cord appeared as if entirely
severed. Dr. Spiller's notes read : " Compression seems to be be-
tween the sixth and seventh cervical segments. There were sensory
disturbances in the distribution of the fifth cervical segment."

This case is interesting for a number of reasons. In the first place
the mechanics of the fracture-dislocation was the backward displace-
ment of the cervical vertebra to which I referred in my general
considerations. The case was made the subject of a paper by Dr.
Charles S. Potts,^^ whose patient he was, discussing the location in
the fifth cervical segment of the biceps tendon reflex. Now bearing
in mind that there was complete solution of continuity of the cord at
the fifth cervical segment, there was retention of normal plantar reflex
and abolition of all the other reflexes of the lower limbs ; also, he had
partial erection of the penis at times.

The peculiar position assumed by the arms in this case coincides
with the position depicted by Thorburn^- in a fracture-dislocation in-
volving the same segment of the cord. The extreme crushing of the
cord in this case is owing to the remarable attenuation which the cross-
section of the vertebral canal suffers in these cases where the displace-
ment is backward in the cervical region.

The question of the preservation or loss of the plantar reflex in
cases of complete lesion of the spinal cord is at present a mooted one.
J. J. Thomas^" say that in complete transverse lesion of the spinal cord
the plantar reflex is often retained.

Unquestionably the majority of cases of complete transverse lesion
reported have abolition of the plantar reflex. There is probably some
other equation in the preservation or loss of this reflex other than
complete solution of continuity.

The question of erection of the penis, in cases of spinal-cord injury,
is possibly more unsettled than it was a few years ago when it was
generally accepted that the center of erection and ejaculation, as well
as for the detrusor of the bladder and the rectal mechanism, were
all located within the cord. Lately, evidence points to the fact that
these centers are largely if not wholly extraspinal, as exploited by
Miiller/* being possibly in the hypogastric and hemorrhoidal plexuses
of the sympathetic. There are, nevertheless, many who defend the
intraspinal location of the these centers (E. Reichert in a personal

106.

ALLEN: INJURIES OF THE SPiNAL CORD 16

communication). Wagner and Stolper speak of priapism, in cases
of injury to the spinal cord, as of purely vasomotor origin when the
lesion is above the erectile center. This center they locate in the
second sacral segment. They differentiate between erection in the
sensual sense and a soft, semiflaccid turgescence. The former depends
upon a stimulation of the motor neurons for the ischiocavernosus,
transversus perinei, and bulbocavernous muscles. The soft turges-
cence, with our incomplete knowledge, only point to a spinal lesion.
In other words we are unable to say, the one symptom points to com-
plete transverse lesion, the other to incomplete.

Case YV. — IMale, age, nativity, etc, unknown, was admitted to the
Philadelphia Hospital, Februaiy 13, 1903, with the history that about
ten months prior to this date he was crushed, an injury to his spine
resulting. Since this time any motion involving the spinal column
caused pain. His knee-jerks were exaggerated, ankle clonus was pres-
ent, and the Rabinski reflex was obtained on the right side.

On February 20 the statement is noted on the chart that the patient
lay supine and could not lift his right leg from the bed, but could
elevate the left leg to an angle of 45 degrees. The upper extremities
showed no atrophy, the dynamometer registered equally on both sides,
and the reflexes were normal. There was a well-marked kyphosis at
the tenth thoracic vertebra together with some induration laterally,
and tenderness on percussion. The abdominal and epigastric reflexes
were well marked, but the cremasteric reflex did not seem to be present.
There was a slight hypesthesia below the kyphosis and to the right of
the spinal column. It exact distribution is not indicated, and it can
only be surmised in that it is stated it " extends down as far as the
buttocks " and was not present on the left side. The history also
records that there was no sensory change on the anterior aspect of
the thigh.

With a few exceptions there is a gap of almost two months in the
history. On April 10 it is entered on the chart : " Patient operated
upon at 1.30 P.M. An incision over dorsal vertebrae was made about
eight inches long and a laminectomy done." Just why this patient
was operated upon is not indicated. There is no history, recorded or
unrecorded, that he was getting worse prior to the operation. A
vertebra was found displaced and rotated to which the cord was bound
on the left side of the canal by dense adhesions. The cord was found
to be softened at the site of the luxated vertebra.

107

17 ALLEN : INJURIES OF THE SPINAL CORD

On April ii (the day after the operation) is is noted that the patient
on examination showed a loss of sensation in left leg and lower third
of thigh, also in the right leg to the knee. Also. " patient is unable to
move toes of either foot." On the i6th it is noted that the patient
seemed unimproved. It was necessary to catheterize him since the
operation, and his bowels would not move without an enema. It
was impossible to keep the skin on the patient's back in a healthv con-
dition. On the 19th the patient was still unable to void urine volun-
tarily, and this retention obtained until October 25, when it is noted
that incontinence had occurred. On May 2 it is recorded that he
" cannot move lower extremities at all."

Dr. Spiller came on duty on June i, and I quote his notes of that
date : " Lower limbs much wasted and equally so. Exceedinglv flaccid
in all parts. Patellar reflex is entirely lost on each side, even by rein-
forcement, and there is a trace of an Achilles- jerk on the right side,
but none of the left. Babinski reflex distinct on right, as also on
left, movement quick, big toe and adjoining toe being moved promptly
on each side. Sensation for touch and pain exceedingly impaired in
lower limbs, but impossible to determine the degree of loss because
of inability of patient to understand. Sensation over the abdomen and
back of trunk, for both touch and pain, seems to be preserved. Sensa-
tion for touch and pain is much impaired in scrotum and penis, but
pressure on testicles is perceived at once. Symptoms are those of
almost complete lesion of the lumbar and sacral cord."

Notes by Dr. Weisenburg, under same date, record that the patient
was unable to move his legs at the hip-joint. The sensation was
" irregularly obtained on the anterior surface of the thighs."

Here occur great gaps in the history, with a few unimportant ob-
servations. I quote Dr. Spiller's notes of July 23, 1904, ten days
before the patient's death : " Lower limbs are extremely wasted. The
only movement possible is a slight drawing upward of the limbs on
either side, but there is no flexion of either limb. He cannot move
the toes of either foot. Involuntary jerking movements occur from
time to time in the lower limbs, especially the left. The patellar reflex
is much exaggerated on each side and equally so. Ankle clonus is
persistent on each side. The Babinski reflex is very distinct on each
side. Sensation for touch and pain is lost in each lower limb below
the knee, but is present for touch and pain in each thigh, back and
front above the knee. Sensation for pinprick is lost in each buttock,

108

ALLEN: INJURIES OF THE SPINAL CORD 18

three or four inches away from the anus. Pinjirick is not felt directly
back of either thigh. There is deformity in the region of the sacrum.
The lower limbs are very spastic."

In a long postmortem record there is noted " necrotic abscess forma-
tion of seventh, eighth, and ninth dorsal vertebrae."

The microscopic examination of the spinal cord shows, by the
Weigert hematoxylin method, well-marked degeneration in the columns
of Goll, in Gowers' columns, and in the direct cerebellar tract (Fig. 7).
The method of Alarchi shows degeneration in Coil's column, and also
a few black dots in Gowers' column on the right side. Numerous fat
crystals were found in the columns of Goll above the lesion in the
IMarchi preparation which I shall discuss elsewhere.

It appears evident that on admission to the hospital the patient had
not an absolute solution of continuity in the spinal i:ord, in fact there
must have been a goodly 'amount of normally functionating axons
bridging the seat of injury to account for the amount of motor and
sensory function still intact. The fact that this condition of affairs
obtained ten months after the accident, should be enough to warrant
the prognosis that the acute degenerative changes had extended as
far as they were going to extend, and barring future trauma and the
very slow sclerotic changes, the probabilities were that he would have
remained in statu quo. The operation in this case can, therefore, not
be defended by even the most radical advocates of the knife.

The injury the surgeon is able to do to the spinal cord in these cases
is here beautifully demonstrated. From a chronic condition at rest
the case was converted again into an acute condition, the results of
the operation being loss of bladder and rectum control, loss of motor
and sensory function, and a tendency to bedsore function.

Case \\ — .Male, aged fifty-nine years, colored, born in Delaware,
was admitted to the Philadelphia Hospital January 2, 1905, with the
history that he had fallen from a wagon and had struck on his head.
After the accident he was able to walk with assistance for about one
hour, when his lower limbs became completely paralyzed. He had
a loss of tactile and temperature sensation in the lower limbs and on
the trunk extending up to above the third rib. Above this there was
a band of hyperesthesia. He was able to move his arms, but was
unable to flex his fingers or move his thumbs. His third rib on the
left side was " dislocated " at its sternal junction. He answered
questions coherently.

109

19 ALLEX : INJURIES OF THE SPINAL CORD

He had, on admission, retention of urine and feces, and complainea
of pain in his neck. There are no further notes on the case during
hfe. He died on January 3. The following is quoted from the post-
mortem record : " Fracture of sixth cervical vertebra, body. Fracture
of the laminae of the fifth and sixth cervical vertebrae. Ligamentous
rupture between sixth and seventh spinous processes of the cervical ver-
tebrae, also between second and third dorsal vertebrae. Transverse frac-
ture of sternum. The musculature over the sixth, seventh, and eighth
cervical vertebrae infiltrated with blood. The part of the cord over-
lying the fracture is slightly compressed and softer in consistency than
portions either above or below." Here follows a rather involved de-
scription of a fracture of the skull, the extent and localization of which
is not evident, but which is obviously considerable in extent from this
quotation: " Subdural hemorrhage in the left anterior fossa, left middle
fossa, and right posterior fossa. There is an irregular macerated area
involving the inferior frontal and middle convolutions of the left side."

Section were made from the second or third, the sixth, seventh, and
eighth cervical regions, and also from the first and second thoracic
regions. They were stained by the hemalum-acid fuchsin and Wei-
gert's hematoxylin methods. The sections from the highest block of
tissue, either second or third cervical, appeared normal. At the level
of the sixth cervical segment there appears a hemorrhage in the gray
matter on both sides of the cord, and also in the anterior commissure.
These hemorrhagic foci are all distinct and separate. There is great
injection of the anterior central branches of the anterior spinal artery,
and in the eighth cervical segment this pressure has been so great that
there appear minute areas where the red blood corpuscles have broken
through the wall of the bloodvessel and are lying free in the anterior
fissure. In the upper part of the seventh cervical segment the hemor-
rhages found in the segment above are not present. There exists
here simply an overdistention of the central branch of the anterior
spinal artery, with a leakage from this vessel of red blood cells, into the
margins of the anterior columns of the cord. In the lower part of the
seventh cervical segment there is a rupture of an artery in the anterior
gray commissure, the hemorrhage extending directly backward into the
posterior columns for about one-fifth their depth. There is also in
this level a very minute hemorrhage in the center of the gray matter
of one anterior horn, and the central branch of the anterior spinal
artery is greatly distended. In the eighth cervical segment the entire

110

ALLEN: INJURIES OF THE SPINAL CORD 2U

gray matter is hemorrhagic, the extravasations extending into the
contiguous white matter. The first thoracic segment is the same as the
eighth cervical, except much more pronounced, and here there seem
to be hemorrhagic processes in the white matter of the lateral and
posterior columns (Fig. 8). In the second thoracic segment the hemor-
rhage is not found in the white matter, and, except for one minute
focus in one anterior horn, is confined altogether to the other anterior
horn.

This case brings up the very interesting subject of traumatic hema-
tomyelia. This condition is frequently found in cases of fracture-
dislocation where the spinal cord has been pressed upon ; it is also
found in cases of severe spinal concussion where there has been no
fracture and no crushing violence to the spinal cord. Much specula-
tion has existed as to the cause of traumatic hematomyelia, and why
the gray matter is so preponderatingly chosen as the seat and limit of
the hemorrhage. As a rule the hemorrhage is not multiple, and although
found in dififerent parts of the gray matter in different levels of the
cord, still, if examined in serial sections, a continuity will often be
demonstrated among the several seemingly separate foci. Pearce
Bailey-^ has published a classical case of multiple hemorrhages into the
cord.

The consensus of opinion seems to be that the hemorrhage is caused
by the commotion in the cord substance, and is located in the gray
matter on account of its weaker and softer consistence. That the
gray matter has less cohesive strength is axiomatic, but I take it that
the hemorrhage in traumatic hematomyelia is of purely hydraulic
origin. There is usually, in these cases, a marked flexion of the
spinal column, and conse(|uent forcing of blood into the anterior fissure
of the spinal cord. As this causes an hydraulic expansive force in the
central arteries from the anterior spinal artery, and as the distribution
of these arteries is practically wholly within the gray matter, the reason
for usual limitation of the hemorrhage to the gray matter is obvious.

My explanation for those cases of traumatic hematomyelia, where
there has been simply concussion of the spinal column without hyper-
flexion or fracture, is likewise on the hydraulic hypothesis. That
homely and painful practical joke, in which we have indulged in youth,
of knocking together two stones under water when another boy is
completely submerged demonstrates upon our tympanum the absolute
incompressibility of liquids and the terrific force which can be brought

111

21 ALLEN : INJURIES OF THE SPINAL CORD

to bear by a seemingly insignificant means. Although the blow usually
comes on the posterior aspect of the spinal column, yet the greatest
hydraulic action is felt in the anterior spinal artery because of its
being a more direct and straight blood channel than any found on the
posterior surface of the spinal cord. The difiference between the
posterior and anterior blood supply to the spinal cord I have referred
to elsewhere. ^^

It seems to me that the views of Kadyi,^*' that the small arteries qf
the anterior fissure pass to one side or the other, rarely bifurcating,
explain why it is that one finds, in serial section study of a case of
traumatic hematomyelia, the so frequent shifting of the seat of
hemorrhage from one side to the other. Without doubt the hydraulic
pressure extends over a distance sufficiently great to take in at least
two of these median branches from the anterior spinal artery.

Case VI. — Ma.\e, aged thirty-two years a minor, born in Italy, was
brought to the Philadelphia Hospital, December 22, 1905. sufifering
from a paralytic condition consequent to a gunshot wound of the
spinal column received two months prior to admission. His past
history is negative. He denied venereal infections, but admitted hav-
ing used a great deal of alcohol without ever becoming " very intoxi-
cated." The point of entrance of the bullet was just above the in-
sertion of the deltoid muscle in the left arm. The humerus was
fractured. Upon receipt of this injury he fell, having no power in
his lower limbs. One month after his wound he began to have shoot-
ing pains in his lower limbs and a bedsore began to develop over his
sacrum.

On his initial examination he was found much emaciated. On the
left side there was marked atrophy of both pectoral muscles. Over
the lumbosacral region there was a bedsore measuring 8 x 14 cm. with
undermined edges. In places the bone was exposed. Sloughs were
present in several areas, and the lesions discharged profusely. There
was a dark, dry slough the size of a man's hand over the right hip.
This was surounded by an inflammatory zone.

The left arm was emaciated, and the grip was very weak. A few
bony projections, two inches above the deltoid insertion, showed the
faulty result of a united fracture. A small scar two inches above this,
the patient said, was due to the bullet wound. Motion of the elbow-
joint, wrist, and in the hand, normal. A partial ankylosis of the
shoulder-joint combined with muscular weakness limited the move-

112

ALLEX : IXjL'RlKS OF THE SPINAL CORD 22

ment of this articulation. Attem])ts at motion caused pain at the seat
of fracture. The right arm was sHghtly wasted, but the patient had
good control over it.

Emaciation in the lower limbs was very marked, but especially so
in the left. He was unable to move his legs and complained of a
shooting pain which began in the hips and shot down into his feet.
This was worse in the left than in the right leg. The reflexes of the
upper limbs were normal on the right side and diminished on the left.
As to the reflexes and sensation of the lower limbs I shall quote from
Dr. Spiller's notes made several weeks after the patient's admission:
" The lower limbs are much wasted. The skin over the soles of the
feet is much thickened, presenting the appearance of trophic disturb-
ance. \Miile lying quietly in bed he gives no expression of pain.
He lies with legs flexed on thighs and thighs semiflexed on abdomen.
Any passive movements of lower limbs cause much pain. There is
not the slightest movement in the lower limbs, which are spastic and
present contractures at all the joints. Slight Achilles-jerk on right
side, more distinct on the left with ankle clonus on left. Irritation
of sole of right foot produces slight downward movement of small
toes, also of great toe. Movement of toes on left side is indistinct.
Patellar tendon reflex exaggerated on left side, shown only by prompt
contraction of quadriceps muscle. Cremasteric reflex not present on
either side. Sensation for touch and pain is lost except over back of
each thigh, where it is still partly retained. Occasionally he seems
to feel a pinprick elsewhere in his lower limbs. Sensation for touch
and pain is greatly impaired and almost lost over the trunk below the
level of four inches above the umbilicus. The upper limbs are not
paralyzed for motion or sensation, nor is the face. The wasting of
the left upper limb is distinct, and is probably a result of the injury
to the left humerus and an arthritis of the left shoulder-joint. There
is an area of slight tenderness to percussion over the third or fourth
thoracic vertebra. The lesion seems to be at about the fifth thoracic
segment of the cord, and is not complete but is almost so. Sensation
for touch and pain is felt everywhere in trunk and lower limbs,
although it is impaired, but not intensely in lower limbs and lower
part of trunk. It is more impaired over the front of the thighs and
legs than over the back of these parts."

On January 2, after an .r-ray examination in which the bullet was
located, the patient was operated upon, an exploratory laminectomy

113

23 ALLEN : INJURIES OF THE SPINAL CORD

being done. The fourth, fifth, and sixth thoracic spines were removed,
but the bullet was not found. The patient lived until February 22.
During this time he gradually grew weaker. The wound proved very
refractory, and there remained several sinuses leading to the spinal
cord.

The history records, after the operation, an incontinence of urine
and feces, but whether this condition obtained before the operation
is not indicated. There is also noted, on February 10, that there is a
" large bedsore over the right hip exposing the trochanter and eight
inches of the shaft of the femur."

The microscopic examination shows at the level of the lower part
of the pyramidal decussation an intense degeneration by Marchi's
method in Goll's columns, in the direct cerebellar columns, and in
Gowers' columns. In the eighth cervical segment there is a degen-
eration of some of the anterior cornual cells (Fig. 12). There is also
seen at this level, in Weigert's preparations, an intense degeneration
in Goll's columns as well as some degenerative change in the direct
cerebellar and Gowers' tracts (Fig. 9). Both Weigert's hematoxylin
and Marchi's preparations show degeneration in both crossed pyramidal
tracts in the lumbar region. Sections from the seat of compression
show complete disintegration of the spinal cord, only a few irregularly
scattered groups of axis cylinders remaining normal and intact, and
these mostly at the periphery of the spinal cord. The degeneration,
when followed up into the medulla by the Marchi method, shows very
beautifully the parting in different directions between the direct cere-
bellar tract as it swings back into the restiform body and Gowers' tract
continuing upward.

This case introduces an important point which is frequently brought
up in arguments pro and con laminectomy in cases of spinal fracture-
dislocation. One of the chief reasons advanced by the surgeon, or
surgically inclined neurologist, for operating in these cases is as Burrell
says, " that if pressure on the cord is allowed to remain for many
hours, irreparable damage to the cord may take place." This state-
ment would infer that the immediate removal of pressure would tend
to prevent the " irreparable damage to the cord," which idea is wholly
fallacious. The s])inal cord, in this case, had barely been grazed by the
bullet, the dura mater being intact and without abrasion (Fig. 4 ). The
bullet had lodged in the vertebra and was exerting no pressure what-
soever upon the spinal cord. Therefore, whatever injury obtained

114

ALLEN: INJURIES OF THE SPINAL CORD 24

resulted from the instantaneous pressure of the bullet in passing, a
pressure indenting possibly the dura, but not enough to abrade or tear
it, and then absolutely removed. And yet, note the wholesale de-
struction of cord tissue in this case (Fig. 6). It seems to me that in
weighing your data pro and con operation in these cases the pressure
of fragments should not be the reason for a rapid operation. If a
fragment has exerted pressure sufficient to cause degeneration that
degeneration will take place, and be as immutable one second after the
reception of the injury as it would be one week after. When the dura
was opened, in this case, the cord was found completely softened.

Case VII. — Male, aged thirty-eight years, an ironworker, born in
America, was brought to St. Joseph's Hospital, October 14, 1906.
Dr. Spellissy, whose patient he was, has kindly furnished me with the
following brief facts : The family history was negative as was also his
past history. On the date of his admission he was working on a
scaffold when it gave way andJie fell a distance of thirty feet. What
part of his body struck the ground is not known. He did not lose
consciousness, but was unable to get up. He complained of pain in
his arms, and upper part of back. This pain he described as like red-
hot irons going through his arms.

He was a well-nourished and well-built man. There was loss of
sensation up to the second rib, the limiting line of anesthesia circling
the body horizontally. He had retention of urine and incontinence
of feces. There was a slight laceration of the scalp. The eyes were
normal. The loss of the sense of pain extended above the loss of
tactile sensation. Knee-jerks and plantar reflexes were absent. There
was a loss of the power of the flexors of the fingers. The patient
gradually lost ground, and died on October 31.

I received the pathological material, in the shape of a portion of
the spinal column, including two vcrtebr?e below the point of fracture-
dislocation and six vertebrae above (Fig. 2). Judging from the topog-
raphy of the gray and white matter of the highest segment, the lesion
involves the upper part of the thoracic cord. For a distance of about
4 cm. the cord is absolutely softened, having been nipped between the
posterior superior edge of the vertebral body next below the luxated
vertebra and the arch of the dislocated vertebra. From a point 1.5
cm. below the point of constriction to fully 5 cm. distal there was an
area of softening occupying the posterior part of the left anterior

115

25 ALLEX: INJURIES OF THE SPINAL CORD

horn and extending backward into the posterior horn to within a short
distance of the periphery.

The 3o cm. of cord were divided into five pieces and mounted in
celloidin and cut. They were numbered from below upward. Block
number five shows an area of degeneration in the white matter at the
periphery on the opposite side to the cerebral softening. This area is
small and is situated at what might be the junction of the direct cere-
bellar and Gowers' tracts. In the fourth block (Fig. lo) there is in
-addition to this same area a similarly situated area of degeneration
on the other side. These areas are true degenerations, showing swollen
axis cylinders. They were probably caused by the injury inflicted
to the cord bv stretching it over the posterior surface of the body of
the vertebra next below the vertebra luxated. In the third block this
degeneration is not present.

The photomicrograph shows the area of central softening together
with the degenerations as found in the fourth block.

Had it been possible to cut and stain an entire unbroken serial set
of sections throughout this part of the cord, doubtless one would have
found occluded or damaged bloodvessels to account for the central
softening.

Case \'III. — ]\Iale. a young student, was brought to the University
Hospital. ^larch 15. 1906, with the following history: He had been
wrestling in a gymnasium and his opponent having gotten his head
on the mat was endeavoring to force the shoulders down also. \\'ith-
out any sudden wrench the patient collapsed, and was in a perfectly
limp condition. Afterward, he stated that he had experienced a sensa-
tion as if something had broken. There was diaphragmatic breathing,
motor and sensory paralysis, the area of anesthesia extending well
above the umbilicus, and there was also priapism and loss of reflexes.
The grasp of each hand was weak, but the motion of the arms at the
shoulders was normal. There was tenderness on pressure over the
sixth cervical vertebra, but no deformity.

The following notes, dictated by Dr. Spiller, were made on the day
of admission: "The upper limbs were very weak. The grasp of the
right hand was nil. The grasp of the left hand was better, but very
weak. The extensors of the carpus on each side were unimpaired.
He could not extend his fingers at all on the right side, and but very
slightly on the left. The resistance to passive movement at elbows
was good. Sensation for touch and pain was much diminished along

116

ALLEN: INJURIES OF THE SPINAL CORD 26

the whole anterior part of each upper Hmb and seemed a trifle more
preserved on the outer side of the left than on the outer side of the
right upper limb. The biceps tendon reflex was possibly a little exagger-
ated on the right side, although very nearly normal. Biceps-jerk was
normal on the left side. The triceps-jerks were not obtained on either
side. The upper limbs could be raised easily. The right pupil was a
trifle larger than the left. Each pupil reacted promptly to light,
accommodation, and convergence. Anesthesia extended up to the
level of the umbilicus, but there was a zone of hyperesthesia midway
between the umbilical and nipple lines. The lower limbs were com-
pletely paralyzed. The knee-jerks, Achilles- jerks, and Babinski reflex
were all absent on each side.. Sensation for touch and pain entirely
lost in lower limbs. There was marked priapism attended by ejacu-
lation."

Dr. Spiller's notes of March i6 record the following facts : " The
sensation for touch and pain seemed to be lost up to the junction of
the third rib with the sternum. There was partial priapism. Sensa-
tion for touch and pain was lost in the area of distribution of the
first thoracic roots along the ulnar side of each forearm. The weak-
ness of both hands was very pronounced, especially of the right hand."

Dr. Spiller's notes of March 17 record: " The disturbance of sensa-
tion in the forearms was the same as the day previous. There was
slight sensation in sole of each foot for touch. The priapism was very
slight. The sensation in the trunk remained the same."

Dr. Spiller's examination of March 19 states : " The patient's
answers, in regard to the tactile and pain sensation in the soles of the
feet were ' entirely unreliable.' "

The patient died on March 30 from " pulmonary edema and general
exhaustion."

Operation was not resorted to in this case owing to the hopeless
outcome, which was indicated. In a letter to Dr. Spiller, Dr. Edward
Martin, who saw the patient in consultation, said : " I have not accentu-
ated a feeling that I had then and still have, that it is a rather good
thing to go into these absolutely hopeless cases on general principles
with the idea of finding, perhaps, the one in a thousand who can be
helped."

The autopsy discovered a luxation of the seventh cervical vertebra
which had been spontaneously reduced. There was no fracture. The
cord had been completely crushed. Dr. Spiller's notes were as

117

27 ALLEX : IXTURIES OF THE SPIXAL CORP

follows : " There was an adhesion between the posterior part of the
dura and the lower part of the seventh cervical vertebra. The seventh
cervical vertebra was a little displaced upon the first thoracic. There
was a slight protrusion backward of the body of the seventh cervical
.vertebra, which was visible when the cord was removed. The body
of the seventh cervical vertebra was very movable. No hemorrhage
anywhere. The cord was swollen above and below^ the seat of com-
pression. The compression is very marked and is at the extreme
upper part of the eighth cervical segment."

Sections for microscopic examination were made immediately above
the level of the compression. There were areas of softening in the
lateral, posterior, and anterior columns on both sides, together with a
great comminution of the white matter. Only the part of the cord
imediately involved at the seat of injury was allowed to be taken at
necropsy. This case was presented by Dr. Spiller and Dr. Martin
before the Philadelphia Academy of Surgery.

This case can be classified among the pure dislocations. As stated,
it was self-reducing. The injury was completed in the twinkling of
an eye, and yet note from the picture the obvious cord destruction.
This appearance is quite typical of this class of injury, the cord look-
ing as though it had been constricted by having a string tied around
it. The terrible destruction of tissue, above and below the seat of
injury, is shown in the accompanying illustration (Fig. ii). Although
the history records priapism as a pronounced symptom. I am told by
Dr. Spiller that semiflaccid turgescence would more accurately describe
the condition.

The following case was reported by Dr. Spiller^' before the Ameri-
can Neurological Association in 1902.

Case IX. — A man, aged forty-six years, fell eight feet out of a
window and struck on his face. He remained on the ground uncon-
scious for several hours after his fall before being found. His ex-
amination revealed that he had no control over his bladder and rectum.
He could draw up his lower limbs, but the movement was much im-
paired. The knee-jerks were present, but not exaggerated. The
Babinski reflex was uncertain. The voluntary movement of the upper
limbs was much impaired. He could not move the fingers of either
hand, but could move his arms at the shoulder and elbow-joint im-
perfectly. Sensation for touch was normal all over the body. The
sensation for pain and temperature was much impaired in the lower

118

ALLEN: INJURIES OF THE SPINAL CORD 28

limbs and over the trunk anteriorly and posteriorly as high as the
base of the neck. There was also some impairment of pain and tem-
perature sensation. in the upper limbs, but not so much as in the lower.

The seventh day after his accident the patellar tendon reflex on the
left side was absent, and on the right was feeble. The Babinski reflex
was present on both sides. The fourteenth day after the accident, it
is recorded that the restoration of power to the lower limbs was re-
markable. On the thirteenth day after the accident he could make
feeble extensor and flexor movements of the fingers of each hand.
He died thirty-eight days after accident.

In this case were found two distinct foci of disease, (i) A trau-
matic myelitis, which Spiller defines as a degeneration afl"ecting the
nerve cell body and the nerve fiber, together with a round-celled in-
filtration, proliferation of neuroglia, congestion of bloodvessels, and
miliary hemorrhage. This affected the fourth, fifth, and sixth cervical
segments. (2) A degenerative change in the cell bodies of the anterior
horns, which was much greater in the eighth cervical segment than
elsewhere. The hemorrhages were too minute to be detected with
the unaided eye.

On account of the findings in this case Spiller says he considers it
impossible to make a differential diagnosis in life between hemato-
myelia and traumatic myelitis.

The limitation of time and space prevent me discussing spinal local-
ization, the reflexes and motor and sensory paralysis.

The treatment of a case of spinal fracture-dislocation is often
rendered much more difficult than it otherwise would be by reason
of the ignorant ministrations of the well-meaning laity present at
the time of the accident. When a man receives an injury of the back
which renders him unable to rise, he should be carefully placed on
a flat stretcher — perferably one extemporized by boards and not soft
and yielding. Care should be taken to preserve, as nearly as possible,
the position of the body as originally found after the accident unless
the position threaten life. This can only be done by bolstering up
certain parts with pillows, or, better still, extemporized sandbags or
mounds of earth. Absolutely no attempt should be made to straighten
out his body until his clothes have been cut from him, and then only
by an experienced physician. After the patient has been duly ex-
amined it is well to pass a catheter as soon as possible, under strict
aseptic precautions, and empty the bladder. In this way one is able

119

29 ALLEN : INJURIES OF THE SPINAL CORD

to more accurately judge of the functional ability of the kidneys dur-
ing the first twenty-four hours. If the patient be delirious morphine
is indicated to counteract a restlessness which might displace a frag-
ment of bone and so wound the cord. If given hypodermically care
must be taken not to give it in an area which shows a circulatory stasis
or edema, otherwise there will ensue very slow and imperfect absorp-
tion, and trophic changes might occur.

The question of operation will always be much discussed. I wish
to note a few of the justifications which have been advanced for
operation in these cases: Tubby^^ says operation is indicated if there
be " pressure from fractured laminse, or from a process driven inward,
or a spicule of bone perforating the theca and cord, hemorrhage, and
when the cauda equina and lumbar sacral plexus of nerves are impli-
cated." McCosh^^ says: (i) "The risk of the operation of laminec-
tomy is slight. (2) Early operation is of the greatest importance.
Operate before the onset of degenerative changes. (3) Operate
rapidly. (4) Employ but a few artery forceps or ligatures. (5)
Support of the spinal column after operation is generally unnecessary."
Burrell says: "An open operation gives definite information as to the
condition of the cord and, above all, allows pressure to be removed."
Haynes-" says: "All patients who survive the shock of the injury to
the spinal cord and its complications should, in gunshot cases, be
operated on." The following are the conditions which he thinks
demand laminectomy: (i) "To remove the bullet, or spiculse of bone
or particles of clothing; (2) to remove blood clots; (3) to arrest
hemorrhage; (4) to allow oozing in traumatic edema of the cord; (5)
to prevent pressure and sepsis by drainage ; (6) in advancing paralysis ;
(7) to suture a severed cord." Oliver-^ says to operate where there
is " some acute function of the spinal cord. In the other cases, those
in which there is no such evidence, it is highly probable that nothing
is lost by waiting two or three days." ]Munro-- suggests a union of
the roots above and below the injury in cases where there is division
of the cord. Horsley-^ advises waiting a few days and then operating
to remove fractured laminae or projecting portions of the vertebral
bodies, or at times intervertebral disks.

Tubby's argument as to pressure I have answered above (Case YD.
To AlcCosh's advice, relative to the slight risk of laminectomy in spinal
fracture dislocation cases, I can only say that literature teems with
cases whose future course after operation is the strongest denial of

120

ALLF.X : INJURIES OF THE SPINAL LORD 30

that statement. Xo matter what good you are going to do meclian-
ically, there is no denying that you are converting a simple into a com-
pound fracture as soon as you operate, and the risk of such a measure
can never be spoken of as sHght, no matter how skilful or aseptic the
operation. To Burrell's statement anent the definite information as
to the condition of the cord which we gain by the open operation, it
is really difficult to take that seriously. If there is one thing that an
operation frequently fails to do it is. give much or at times any clue
to the condition of the cord (Case VI).

Spiller-* answers Haynes' argument in favor of operation by calling
attention to the fact that some authorities consider the symptoms of
compression due in reality to- secondary degeneration. He thinks,
moreover, that the removal of an external clot on the cord is of doubt-
ful value, and he questions the good influence of an operation on an
edema of the cord following fracture or injury of the spine. The
seventh point taken up in Haynes' argument, /. c. " to suture the sev-
ered cord," need not be discussed except to say that the entire weight
of all careful observation and evidence is against the possibility of
the cut ends uniting, so that there will ever be functional continutiy
between the two ends. This takes into account the several alleged
cases of spinal-cord suture with partial return of function afterward.
M. Allen Starr"-^ denies that repair of the spinal cord ever occurs.
He says that scar tissue may form, but that " restoration of continuity
of the nerve fibers " is impossible. He gives the weight of his opinion
against operating, saying that the results are " uniformly disappoining."

Oliver's advice to operate where there is " some acute function of
the spinal cord " remaining, seems to me diametrically opposed to
reason. If one be fortunate enough to have an incomplete lesion of
the spinal cord following a fracture-dislocation, why run the risk of
converting it into a complete lesion as in one of my cases quoted above
(Case IV) ?

Munro's suggestion of suturing the nerve roots immediately above
the lesion to those immediately below is fantastic onlv because of our
lack of experimental data in this direction. He evidently advances
that means, accepting the doctrine that axon regeneration is dependent
on the nuclei of the neurilemma and can never take place in a cord
suture, the cord lacking neurilemma.

The work of Kilvington-" has lent additional weight to ]\Iunro's
suggestion. This investigator (Kilvington) sutured, in a dog, the

121

31 ALLEX : INJURIES OF THE SPIXAL CORD

central ends of spinal-nerve roots leading to the hind limbs to the peri-
pheral ends of the nerves to the rectum and bladder with successful
results. He thinks that nerve crossing would not be feasible in the
cervical region in man, but that in the dorsolumbar region it would
be possible. He found from dissection that the second and third
sacral roots could be joined to the first lumbar root, provided that the
latter be divided as low as possible (where it pierces the dura).

Horslev's views have been so adequately answered by Spiller in his
reply to Haynes, that I need not further dilate on them.

When are we to recommend operation in cases of fracture-disloca-
tion of the spinal column? In answering this question we must not
base our advice on the isolated cases reported in which brilliant results
have come after radical operative procedure. These cases are often
will-o'-the-wisps leading to disaster, because we are as yet too ignorant
of the processes of regeneration and spinal surgery to be able to see
what factor in a particular case made for success, and in our ignor-
ance we are prone to ascribe it to superlative surgical technique,
promptness in operating, etc.. factors any one of which is easily within
our power, and which, if they were the true crux of the question, would
forever set aside the dispute on the usbject.

A compound fracture of the spinal column, whether caused by gun-
shot or other violence, is often a suitable case for operation. Here
there is not the conversion of a simple into a compovmd fracture.
Do as little as possible in the operation in the way of removal or
reposition of fragments. Of course, should you find a lamina driven
into the spinal cord it would be indicated to remove it. But rather
than clear the premises of all existing debris, trust to the natural proc-
esses of organization and ankylosis with the aid of immobilization to
bind the fragments, which you might otherwise tediously dissect away,
out of reach of the spinal cord. \\'hen the spinal canal is opened the
greatest care should be exercised in sponging, so that the spinal cord
be not forcibly pressed on. A great deal of injury can thus be done.
I think it better to never let the gauze pad even so much as touch the
cord. Clots of blood can be removed by a stream of warm sterile
physiological salt solution after sufficient time has been given for the
oozing in the small vessels to cease spontaneously.

Scudder,-' in an attempt to arrive at some conclusions as to the
advisability of operating in cases of .complete transverse lesion of the
cord, does not render the subject more lucid by the following two sen-

122

ALLKX : IXJIRIKS OF THE SPIXAL CORD 32

tences, which, in contlict, seem to be past reconcihation : (a) "These
specimens demonstrate the utter futihty of operative interference in
cases of crush of the cord with signs of complete transverse lesion " ;
and (b) '' All fractures showing complete transverse lesion of the cord
should be terated by immediate operation."

There seems to be much testimony in favor of operating in fracture-
dislocation involving the cauda equina. Thorburn.'- in his classical
work, comes to these conclusions : " The operation of trephining the
spine for traumatic lesions, as compared with the condition which it
is intended to relieve, does not present any great dangers, and appears
unlikely to increase the gravity of the prognosis, but that as both,
apriori argument and the results of published cases show that it is
unlikely to be of service, it should be abandoned, except in cases of
injury to the cauda ecjuina, and that in the latter, on the other hand,
it will probably prove to be an eminently justifiable and serviceable
procedure." He says that if improvement have not started in six
weeks, in cases of injury of the cauda equina, he recommends opera-
tion. Scudder speaking on the same theme says: " If at the end of
six weeks evidences of beginning recovery do not appear or if re-
covery once begun has ceased, it will be wise to operate upon injuries
to the cauda equina."

The treatment of the bladder difficulties and trophic disorders must
be carried out along the lines of general medicine and surgery. Often
in cases of fracture-dislocation of the thoracic region of the spine the
patient will present, in due time, troublesome meteorismus. This is
probably owing to an irritation of the splanchnic inhibitory nerves
from the thoracic region of the cord.

BIBLIOGRAPHY.

1. Pearce Bailey. Diseases of tlic Nervous System Resulting from Accident
and Injury, 1906.

2. Herbert L. Burrell. A Summary of all the Cases of Fracture of the Spine
(244) which were treated at the Boston City Hospital from 1864 to 1905.
Trans. Amer. Surg. Assoc, 1905, vol. xxiii, p. 66.

3. Frederick Treves. Surgical Applied Anatomy, 1894, P- 546-

4. Carson. Trans. Amer. Surg. Assoc, 1905.

5. Gurlt. Handbuch der Lehre von der Knochen. Hamm, 1862.

6. Philipeaux. Revue medico, chir., 1852, Tome xi, p. 178.

7. Sir Astley Cooper. A Treatise on Dislocations and on Fractures of the
Joints, new edition by Bransby Cooper, London, 1842, p. 536.

8. Ibid., p. 533.

123

33 ALLEN : INJIKJES OF J HE SPINAL CORL

9. Wm. G. Spillcr. The Sensory Segmental Area of the Umbilicus, Phila.
I\Ied. Jour., February 8, 1902.

10. Wagner unci Stolper. Die Verletzungen der Wirbelsaiile und des Riicken-
marks, Deutsche Chirurgie, 1898, Lief, 40.

11. Chas. S. Potts. A Case of Traumatic Cervical Hematomyelia and Com-
'plete Division of the Cord, with Probable Dislocation of the Fifth Cervical
Vertebra. Remarks on the Location of the Centre for the Biceps Reflex, Jour.
Nerv. and Ment. Dis., 1905, vol. xxxii, p. 359.

12. Wm. Thornburn. A Contribution to the Surgery of the Spinal Cord,

1889.

13. J. J. Thomas. Five Cases of Lijury of the Cord, Resulting from Frac-
ture of the Spine, Med. and Surg. Reports of the Boston City Hospital, 1900,
Series xi, p. i.

14. L. R. Miiller. Klinische und Experimentelle Studien iiber die Innervation
der Blase, des Mastdarms und des Genitalapparates, Deutsche Zeitschrift fiir
Nervenheilkunde, 1901, Band xxi, Seite 86.

15. Alfred Reginald Allen. Combined Pseudosystemic Disease, with Special
Reference to Annular Degeneration, Univ. of Penna. Med. Bull., January, 1905.

16. Kadyi. Ueber die Bliitgefasse des Menschlichen Riickenmarks, Lemberg,
1899.

17. Wm. G. Spiller. Traumatic Lesions of the Spinal Cord without Fracture
of the Vertebra, Univ. of Penna. Med. Bull., February, 1903.

18. A. H. Tubby. Injuries to the Spinal Column and Spinal Cord, Clinical
Jour., 1904, vol. xxiv. No. 25, p. 385.

19. Andrew J. McCosh. Remarks on Spinal Surgery with Illustrative Cases,
Jour. Amer. Med. Assoc, vol. xxxvii, Nos. 9 and 10.

20. Irving S. Haynes. Gunshot Wound of the Spinal Cord. A Plea for
Early Myelorrhaphy, with Report of a Case of Bullet Wound through the Liver,
Spinal Column, and Cord; Laparotomy; Recovery, New York Med. Jour., Sep-
tember 22 and 29, 1906.

21. John C. Oliver. Injuries to the Spine and Their Treatment, Cincinnati
Lancet-Clinic, 1903, p. 489.

22. J. C. Munro. Laminectomy, Jour. Amer. Med. Assoc, January 6, 1900,
vol. xxxiv, p. 12.

23. V. A. H. Horsely. Clififord Allbut's System of Medicine, vol. vii, p. 871.

24. Wm. G. Spiller. Keen's System of Surgery, 1907, vol. ii.

25. M. Allen Starr. Organic Nervous Diseases, 1903, p. 381.

26. Basil Kilvington. An Investigation on the Regeneration of Nerves with
Regard to Surgical Treatment of Certain Paralyses, Brit. Med. Jour., April 27,
1907, p. 988.

27. C. L. Scudder. The Treatment of Fractures, 1905, 5th edition.

28. Pearce Bailey. Traumatic Hemorrhage into the Spinal Cord, Medical
Record, April 7, 1900.

124

/^'

ml ^

1 i ■

^^^y^lr^i^ll^i^

3^2

^gjy

Fig. 5

Fk;. 6

Fig. 7

iMC. 8

Fig. 9

Fig. 10

Fk;. 1 1

U-i

From the Department of Neurology and the Laboratory of Neuro-Pathology
the University of Pennsylvania, and from the Philadelphia Home for In-

of
curables.

SYRINGOMYELIA WITH SYRINGOBULBIA ^

By John H. W. Riiein, AI.D.
neurologist to the howard hospital, etc.

While syringobulbia is by no means rare, the following case presents
a number' of interesting features. These consist, clinically, of the
presence of paralysis of the superior oblique muscle of the right eye,
transient ptosis on one side, contracted fields of vision, hemiatrophy
of the tongue, hemiplegia, and ataxia ; and pathologically, the presence
of cavity in one anterior jiyramid of the medulla oblongata, with as-
cending degeneration in the anterior pyramid on the same side, the
presence of intense lepto-meningitis, the predominance of the cavity
on one side; and the probable syphilitic nature of the lesion of the
spinal cord and meninges. Furthermore, the pathological findings
have an important bearing upon the explanation of the bulbar symp-
toms in cases of syringomyelia and syringobulbia which are not due
to the cavity formation.

The patient, a married woman fifty-nine years old, was admitted to the
Philadelphia Home for Incurables Feb. i, 1904.

F. H.— Her father died of phthisis, and her mother of heart disease. One
brother was drowned, and one was killed. One sister died of asthma.

p_ H. There is no history of previous serious illness. She suffered from

intermittent fever some years ago. She was married at the age of thirty-hve
years, never bore children, nor had any miscarriages.

In 1891 she began to stagger toward the left without vertigo. About the
same time she frequently burned herself without recognizing it until afterward.
She also had severe transient attacks of tic douloureux, associated with doul)le
vision.

Shortly after the onset of these symptoms, in December, 1891, she con-
sulted Dr. John T. Carpenter, to whom she gave a history of having had head-
aches. I am indebted to Dr. Carpenter for the report of the examination of the
eyes at this time. He stated that the entire nerve was altered in appearance,
showing large globular hyalin masses, or so-called drusen tumors of the optic
nerve. The nerve was grayer than it sliould be, but there was no contraction
of the retinal vessels.

In November, 1892, she was again examined by Dr. Carpenter, who elicited
a history of an attack of unconsciousness, occurring shortly before her visit to

^Received for publication Jan. 14, 1908.
1 125

2 RHEIN : SYRINGOMYELIA WITH SYRINGOBULBIA

him, and also of intense pain in the branches of the left trifacial nerve, with
fleeting double vision and unsteady gait.

In February, 1896 (five years after the onset of the disease), all the symptoms
were more marked, and she could not walk without a cane. At this time there
was considerable ataxia, and with her feet together and the eyes closed, she
would have fallen if not supported. The knee jerks were increased.

On July 17, 1896, there were some sensory changes in the left arm, and sharp
lancinating pains in the distribution of the cervical nerves added to the symptoms
already described. There was at this time, also, a paresis of the superior
oblique muscle.

In August, 1896, she complained of numbness in the left arm and leg,
and pain in the left arm. She also complained of a subjective sensation as of
boiling water running down the bone in the left arm. The ataxia had increased,
and the left arm and hand had begun to show signs of weakness.

The field of vision showed a peripheral concentric contraction for form and
colors, more marked on the left side.

On July 21, 1902, all the symptoms had progressed markedly. The field of
vision in the left eye was contracted almost to fixation point ; in the right eye,
to twelve inches. The field was mapped out as follows : temporal side, seven
inches ; up, seven inches ; in, four inches, down, two inches. There was a deep
pallor of the optic nerve.

When examined, shortly after her admission to the Home in February, 1904,
she was found to be generally well preserved, excepting in the regions exhibit-
ing local atrophy.

Atrophy. — The tlienar and hypothenar eminences of the hands, especially on
the left side, were markedly atrophied. The muscles of the forearm also ap-
peared to be somewhat wasted on both sides, especially the flexors. There was
apparently no wasting elsewhere in the upper or lower limbs. There was
a marked right hemiatrophy of the tongue.

The station was very unsteady, and with the eyes closed the patient would
have fallen to the ground if unsupported.

The gait was a curious one, being at the same time that of ataxia and of a
left hemiplegia.

There was a marked ataxia in the arms, more apparent in the right tlian in
the left, which, however, may have been due to the fact that the left arm was
weak and somewhat spastic.

Reflexes. — The knee jerk on the right side was much increased; on the
left side it could not be elicited on account of the rigidity present. The ankle
jerk on the right was present, but on the left it could not be obtained on account
of the spasticity. There was no clonus on either side. Babinski's reflex was
readily developed on each side. The arm jerks could not be elicited on either
side. The left arm and leg were distinctly rigid, while the right arm and leg
were not at all spastic.

Sensation. — There was a subjective sensation of cold in the left hand, and of
burning pain in the left forearm.

In the left hand there was almost complete loss of the thermal sense, and
the pain sense was almost wholly lost over an area including the entire left arm,

126

RHEIN : SYRINGOMYELIA WITH SYRINGOBULBIA S

left anterior trunk to the median line, left side of the face, and the left
anterior cervical region on both sides, extending only as far as the clavicle on
the right, and posteriorly to about the tenth dorsal on the left, involving the
entire scalp posteriorly.

There was loss of the thermal sense in the right hand, although this was not
as pronounced as in the left hand.

From the elbow to the shoulder on the right side the patient confused heat
and cold, and on the right side the thermal sense was lost as far down as the
tenth dorsal spine.

Dr. W. C. Posey examined her eyes with the following result : The palpebral
fissures were equal on both sides. There were no pathological lid signs present.
Fixing in the median line caused slight rotary nystagmic movements, which
became more marked at the limit of the excursion of the eyes in all directions.
Tests showed a paralysis of the superior oblique muscle of the right eye. The
pupils responded to light and in accommodation. There was simple atrophy of
the optic nerve in both eyes, with persistence of the hyaloid changes already
noted by Dr. Carpenter.

Examination of the eyes made in August, 1904, by Dr. Van Epps, showed the
presence of cataractous changes in each lens. At this examination ptosis of the
right upper lid was observed. Ljiter, however, this symptom did not persist.

At the examination made in June, 1905, the paralysis of the left arm and leg
had progressed so that she could not walk, and she was unable to use the left
arm at all. At this examination the sensory disturbances had extended to the
legs. The pain sense was lost in the right leg and thigh, but sensations for
touch, heat, and cold were preserved. The temperature and pain senses were
lost in the entire left leg.

There were marked flexor contractures of the left forearm muscles, so that
the fingers of the hand were tightly flexed on the palm, and this contracture
could not be overcome by the examiner. There was also some contraction of
the forearm on the arm, and the left leg was spastic and totally paralyzed.

The atrophy of the hand muscles had progressed moderately.

The patient died in February, 1907.

At the autopsy the hrain and s])inal cord were removed and placed
in formalin sohition for two weeks, and afterwards in Miiller's fltiid.

The pia of the medulla oblongata and pons was much thickened, and
the medulla oblongata was twisted out of shape. The change in the
pia extended well over to the cerebellum on each side, the whole an-
terior surface in this region, including the basilar artery, presenting a
matted appearance (see illustration). The basilar artery was closed
by a thrombus which had undergone calcareous degeneration.

The brain showed a moderate degree of hydrocephalus, involving all
the horns of the lateral ventricles, especially the posterior horns. The
fissure of Sylvius was not dilated.

Sections from various levels of the cord and medulla were stained

J27

4 RHETN : SYRINGOMYELIA WITH SYRINGOBULBIA

by the Weigert hematoxylin method, and with the hematoxyHn, eosin,
and thionin stains.

The spinal cord, from the end of the conns, to about the third lumbar
segment, was divided into ten portions, as it was impossible to count
the roots.

In sections from Block No. 2, from the lower part of the conus, there
was a linear cavity in the left posterior horn, extending almost to the
posterior periphery of the cord. The pia was thickened in this region,
and infiltrated with round cells.

At Block No. 3, both lateral columns were degenerated, that on the
left side more intensely. The cavity occupied the same position, but
was a trifle larger.

At No. 4, the cavity became very slight, scarcely visible, and at No.

5 it was only visible under a high power.

At No. 7 the cavity extended into the anterior horn on the left side,
which was partly destroyed. It expanded very much at this level, and
invaded the posterior horn. The anterior roots on the left side were
only slightly degenerated. There was slight degeneration of the
posterior column on the left side.

At No. 8 the cavity occupied the same position, although the pos-
terior horn was invaded slightly more than in the level below. The
anterior and posterior roots, especially the latter, stained less deeply.
The degeneration of the posterior white columns on the left side was
more marked.

At No. 9 the cavity extended forward almost to the central canal,
and backward to the posterior border of the posterior horn. At this
level the anterior horn was intact. There was slight degeneration of
the crossed pyramidal tract on each side, more intense on the side of
the cavity. Degeneration of the left posterior column was still present.

At No. 10 the cavity was linear in shape, and extended almost to the
central canal in front, and to the posterior border of the posterior horn
behind. The degeneration in the crossed pyramidal tracts was very
marked on both sides. The degeneration of the left posterior column
still persisted.

At the third lumbar segment the anterior limb of the cavity extended
toward the central canal.

At the second lumbar segment the cavity was smaller, the degenera-
tion of the crossed pyramidal tracts was more intense, and the posterior
column on the left side was still slightly degenerated.

128

RHEIX : 5YRIXGOMYELL\ WITH S\TlIXGOBULBIA 5

At the eleventh thoracic segment the cord was much distorted. The
cavity, which was Hnear in shape, extended toward the central gray
matter on the right, and was less extensive in the posterior horn than
in the segment below. The degeneration in the left crossed p3-ramidal
column was intense, and more so than on the right side. The right
direct pyramidal tract was also degenerated, and the degeneration
was less marked in the posterior columns than in the second lumbar
segments. The posterior roots showed traces of degeneration on the
left side.

At the tenth thoracic segment the cavity involved the anterior horns
slightly, and was more extensive in the posterior horn, the latter being
almost entireh' destroyed. It also invaded slightly the posterior
columns on the left side. The degeneration in the crossed p}Tamidal
tracts was still quite marked.

At the seventh thoracic segment the cavity destroyed the left an-
terior and posterior horns in large part, extended to the posterior
border of the posterior horn. The pyramidal tract on the right was
much degenerated, while the degeneration in the posterior column had
disappeared.

In the fourth thoracic segment the anterior horn on the left side
was destroyed in large part. The anterior portion of both posterior
columns was involved, and the cavity extended forward into the lateral
column on the left.

In the second thoracic segment the cavity was larger and involved
the left anterior and posterior horns, and extended into tlie right
posterior horn. The right direct pyramidal tract and both crossed
pyramidal tracts were much degenerated. The roots stained fairlv
well.

At the eighth cervical segment the cavity occupied a large part of
the right posterior horn, involving the intermediate grav matter of the
right side extensively, and destroying the left anterior and posterior
horns. The left crossed pyramidal tract was degenerated intenselv;
the right, slightly. Most of the fibers of the posterior columns were
well preserved. There was degeneration of the border zone in both
anterolateral columns, which was more intense on the right side. The
leptomeningitis at this place was very intense, especially posteriorlv.

At the sixth cervical segment the appearance was the same, ex-
cepting that the degeneration in the border zones was much more
intense. In the ground fibers of the lateral columns there was an area

129

6 RHEIN : SYRINGOMYELIA WITH SYRINGOBULBIA

of degeneration on the right side, situated in about the middle portion.
The roots stained well.

The medulla oblongata at its lowest level was degenerated in large
part. The cavity destroyed the nuclei of the posterior columns on the
right side, and there was also a cavity present in the right pyramid.
The leptomeningitis was intense, the pia being much thickened, and
the blood vessels altered. The direct cerebellar tract was degenerated
on the left side.

At the level above this the cavity was much smaller and was situated in
the posterior portion of the medulla, occupying the position of the
nuclei of the posterior columns on the right side. The cavity in the
right pyramid was still present.

At a still higher level there was a large cavity which destroyed the
nucleus of the right hypoglossus nerve, and extended forward and
inward, lying in the position of the ninth and tenth nerve fibers, and
reaching nearly to the periphery of the medulla oblongata, destroying
the right olive, and fibers of the right twelfth nerve, the internal arcu-
ate fibers of the same side, and causing degeneration of the lemniscus
of the opposite side. There was also a small cavity in the right
pyramid, slit-like in character. The external arcuate fibers on the left
side were degenerated and the cerebellar olivary fibers on this side
stained poorly.

At a still higher level the cavity was situated posterior to the olive,
cutting the internal arciform fibers, and extending forward and inward,
opening into the central canal. The cavity could still be seen in the
right anterior pyramid.

The degeneration of the lemniscus on the opposite side was marked,
and was more intense in the anterior half.

A little higher still at the superior limit of the olive, the cavity had
disappeared. There was at this level degeneration of the pyramid on
the right side, and degeneration of the contralateral lemniscus. The
nerve fibers of the ninth and tenth nerves were normal on both sides.

At the next level, at which the acoustic fibers made their exit from
the medulla oblongata, the degeneration of the left lemniscus was still
present. There was also a slight degeneration of the right pyramid,
occupying the outer and posterior portions especially.

At the level of the sixth nucleus the degeneration of the left lem-
niscus was still visible. The nerve fibers of the facial nerve, and

]30

rhein: syringomyelia with syringobulbia 7

the nuclei of the sixth nerve, were intact. The degeneration of the
right pyramid at this point had disappeared.

At the level of the fourth nucleus there was still partial degenera-
tion of the left lemniscus, but it was much less than at the level below.
The- lateral fillets stained well. There was no degeneration in the
pyramidal tracts, although the fibers on both sides of the raphe stained
less intensely than the remainder of the pyramidal fibers. The decus-
sation of the fourth nerve showed no degeneration. A small area of
softening was present at this level, situated in the central portion of
the pons, cutting the raphe.

The optic chiasm showed some degeneration, especially on the right
side anteriorly. It was also present on the left side, but was not so
extensive and was situated centrally.

The right optic nerve was degenerated at the periphery, leaving
healthy fibers in the middle. The left optic nerve was degenerated,
but less so than the right.

The right third nerve showed no degeneration. In the left third
nerve there was only one small l)un(lle of degeneration observed, the
nerve in large part being normal.

Both right and left fifth nerves were slightly degenerated.

The right seventh nerve contained a few degenerated fibers. The
left seventh nerve was normal.

Both right and left eighth nerves were slightly degenerated.

The right ninth and tenth nerves stained very poorly, and contained
but few unchanged fibers. The left ninth and tenth nerves were
normal.

Sections from the lumbar region, stained by tliionin, showed some,
although not extensive, degeneration. Some of the nerve cells had
lost their processes. In many there was much yellow pigment, and in
one cell the yellow pigment almost filled the entire cell. In a few the
nuclei were eccentric, though many were still centrally situated. The
chromatic elements were granular, and arranged around the nucleus.
Most of the cells were more or less swollen. There were, however,
some normal cells present.

In the cervical region the pigment was granular in many of the cells,
and in some there was distinct atrophy of the chromatic bodies. Some
of the cells were very well preserved; others were distinctly swollen,
staining poorly.

In sections from the paracentral region on both sides the perivascular

181

8 rhein: syringomyelia with syringobulbia

spaces were distended. The pia was not diseased. A few of the nerve
cells contained a large amount of yellow pigment, and in some the
chromatic substance was atrophied.

The pia of the entire cord was the seat of a round-cell infiltration,
and was much thickened. This inflammatory process extended up to
the superior border of the pons, and became more intense at the second
thoracic segment, increasing in intensity in an upward direction, so that
the medulla, pons, and cerebellum were matted with the pia, and the
medulla oblongata was twisted out of shape.

In the lumbar region numerous blood vessels with thickened walls
were seen clumped together in the cavity and associated with round-
cell infiltration. This was also seen in the cervical region, to a less
degree. In one section the round-cell infiltration could be traced from
the pia through the cord as far as the wall of the cavity.

The blood vessels of the pia, in the region of the cervical cord,
medulla, and pons, were altered. In some the walls were thickened,
the lumen narrowed, and there was also some perivascular round-cell
infiltration.

Summary. — A woman fifty-nine years of age, developed, sixteen
years before her death, typical symptoms of syringomyelia with bulbar
involvement. There was left hemiplegia, ataxia of both arms and legs,
and atrophy of the hand muscles and of the right half of the tongue.
There were paralysis of the superior oblique of the right eyeball,
transient slight ptosis of the right eyelid, and nystagmus. Character-
istic sensory disturbances were observed.

A cavity was found in the spinal cord extending from the conus
medullaris into the medulla oblongata to the superior limit of the
olive, involving chiefly the left anterior and posterior horns of the
cord, and from the second thoracic segment upward to the right pos-
terior horn also.

In the medulla oblongata the cavity was on the right side, and de-
stroyed the nucleus of the right twelfth nerve, the nuclei of the right
posterior columns, the right olive in its lower levels, the right arcuate
fibers, and the right anterior pyramid. The left ninth, and tenth nerves,
the optic nerves, the left third, and both fifth and eighth nerves were
more or less degenerated. Ascending degeneration of the left lem-
niscus and the right pyramid in the medulla oblongata, and degenera-
tion of the direct and crossed pyramidal tracts and the left posterior
column in the lower part of the cord were present. There was a lepto-

132

rhein: syringomyelia with syringobulbia 9

meningitis extending from the conus medullaris to the upper limit of
the pons, and was most intense from the second thoracic region to its
upper limit.

The clinical symptoms of this case which deserve special mention
are the paralysis of the right superior oblique muscle of the eyeball;
the nystagmus; the contraction of the visual fields; the ptosis of the
right eyeball; the involvement of the fifth nerve; the ataxia; the pre-
dominance of the motor symptoms on the left side; the apoplectic onset,
and hemiatrophy of the tongue.

The paralysis of the superior oblique muscle is perhaps unique.
While Schlesinger has found paralysis of the external ocular muscles
in syringomyelia mentioned thirty-three times in the literature, as oc-
curring in thirty-one patients, no mention is made of palsy of the
fourth nerve. Diplopia was present in eleven per cent, of three hun-
dred cases, but was probably due to involvement of the sixth nerve,
the one usually attacked in syringomyelia.

Very rarely is affection of the oculomotor nerve observed; although
ptosis, which, however, was transient in character in my case, has been
described.

Nystagmus, which was present in my case, is a frequent symptom.
The cause of this is not yet clear. Some authorities, according to
Schlesinger, view nystagmus as an anomaly of the muscle tone, or as
an asthenic symptom of the eye muscles. He quotes Landois who
stated that injury to the corpus restiformis in animals causes nystagmus,
and also Hollopeau, in whose case the cavity reached as high as the
corpus restiformis. In Schlesinger's case, with pronounced nystagmus,
the corpus restiformis was injured. In my case the corpus restiformis
was apparently normal. In throe hundred and thirty cases in the
literature studied by Schlesinger there were fifty-one examples of
nystagmus and nystagmus-like movements.

Contraction of the visual fields, which was so marked in my case,
was present in one hundred and thirty cases in the literature studied
by Schlesinger. He believed that in the majority of cases of syringo-
myelia the visual field is normal. When the visual fields are con-
tracted it is, in most instances, due to concomitant hysteria. There
are, however, a few cases in which the visual fields — especially for
colors — are contracted, and in which there is no hysteria present. ^ly
case belongs to the latter classification beyond a doubt, and was due
to the atrophy of the optic nerve, which was present on both sides.

10 RHEIX : SYRINGOMYELIA WITH SYRINGOBULBIA

Involvement of the fifth nerve has been frequently described.
Schlesinger in two hundred cases in the literature, found this symptom
present seventeen times, and quotes Lamacq, who found it twenty-
eight times in three hundred cases.

It is interesting to note the cause of the ataxia present in my case.
The degeneration of the left posterior column, the posterior roots, as
well as the interference with the transmission of sensory sensations by
reason of the destruction of the nuclei of the posterior columns on
the right side of the medulla, and degeneration of the right direct
cerebellar tract, offer ample explanation for the presence of this
symptom.

The hemiplegia, associated with ataxia, which was present in my
case, was also described by Raymond-Cartaz."-

The points of special interest, on the part of the pathological find-
ings in this case, are the presence of the pronounced meningitis, its
probable syphihtic nature, the presence of ascending degeneration in
the right pyramid of the medulla oblongata, and the bearing which
the pathological findings have upon the cause, or causes, of cranial
nerve involvement in syringomyelia and syringobulbia.

Disease of the meninges of the cord, medulla, and pons, which was
present in my case, has been noted in a number of cases of syringo-
myelia and syringobulbia in the literature. The occurrence of pachy-
meningitis has been so frequent as to warrant the description of a
" pachymeningitic type " of syringomyelia. It is characterized clin-
ically by the presence of sharp pains in the distribution of the nerves
of the cranium and spine, and other subjective sensory phenomena,
symptoms which develop early in a number of cases* of syringomyelia
and syringobulbia.

Reference has been made to thirty-five cases in the literature of
cavity formation in the cord or medulla oblongata, in which, at the
autopsy, the meninges were found to be diseased.

Vulpian,^ in 1861, described cavity formation in the anterior horns
of the spinal cord, associated with chronic meningitis, and Simon,^ in
1874, reported four cases in which the spinal cord was the seat of a
cavity which was closely related, anatomically, to a co-existing specific
leptomeningitis.

Zenoni,'* described a case in which leptomeningitis was present in
a case of syringomyelia occurring in a child of three years. The
cerebral meninges were opaque and thickened, and there was a fibrous

134

rhein: svkixc.omyelia with syringobulbia 11

leptomeningitis of the cord. Arteritis and periarteritis of the vessels
of the spinal meninges and the cord itself were present. He stated
that it was evident that tlie meningitis and the vascular lesions were
of tuberculous origin.

In four cases reported by Rosenl)lat]r'' there was pachymeningitis
cervicalis hypertrophica associated with syringomyelia. He looked
upon the pachymeningitis as having some etiological significance.

Achard and Joffroy" reported a case in which the cavity was closely
related to the pachymeningitis. They believed that the syringomyelia
in their case was the result of an inflammatory process having its origin
in vascular disease and meningitic change.

In a case of syringomyelia reported by Jegorow there was meningitis
of syphilitic origin in the sacral region.

In Hoffmann's^ case, with bulbar symptoms, a chronic pachy-
meningitis cervicalis fibrosa was found, the cavity extending into the
medulla.

Miiller and Meder** described a case of cavity formation, associated
with pachymeningitis in which the pia and arachnoid were fused to-
gether in the cervical region.

Oppenheim^" described a case of gliosis of the spine, extending from
the cervical to the lower lumbar region, in which there were pachy-
meningitis and leptomeningitis.

In a case reported by A. Westphal'^ there was intense hemorrhagic
pachymeningitis and leptomeningitis of the brain and spinal cord.
The cavity extended from the third cervical to the third lumbar seg-
ment, and the medulla oblongata was the seat of numerous spaces or
crevices in the pyramidal tract. He described two other cases of
syringomyelia associated with pachymeningitis interna hemorrhagica,
in one of which a general miliary tuberculosis was present. In the
second case the spinal pia was thickened throughout its entire extent,
as far as the medulla oblongata.

Saxer^" quotes Charcot who was the first to describe pachymeningitis
cervicalis hypertrophica. Charcot reported two cases, in one of which
there was cavity formation.

In eleven cases with autopsy, reported by Phillip and Obcrthur,^^
there was pachymeningitis. In some cases the arachnoid and pia were
also involved. The pachymeningitis was slight in the region of the
cord which was least aft'ected by the syringomyelia, namely, the dorsal
and lumbar regions. The pachymeningitis cervicalis hypertrophica

i;35

12 RHEIN': SYRINGOMYELIA WITH SVRlNllOUL'L lUA

was more intense at the levels of the cervical enlargement and the
medulla oblongata, regions which presented the greatest amount of
syringomyelia.

In a case reported by Kaiser and Kiikenmeister/* the pia in the
upper cervical region, and in the medulla oblongata, was very much
thickened, and contained numerous dilated blood vessels with thick-
ened walls.

Schwarz^^ cited a case of cavity formation in the anterior horns of
the lower cervical region, and extending irregularly from the fourth
dorsal segment to the thoracic region, associated with meningomyelitis
syphilitica.

Jappha^® described a case of cavity formation, which he looked upon
as being due to syphilis. He designated his case as one of meningo-
myelitis syphilitica. Pachymeningitis spinalis gummosa and lepto-
meningitis were present.

Koehler^' described a case of extensive leptomeningitis with cavity
formation extending from the upper cervical to the middle dorsal re-
gion. There was central syringomyelia and widespread leptomeningitis.

H. S. Hutchinson^^ recently reported two cases of syringomyelia
in which the meninges were involved. In one of his cases there was
a history of syphilis, and the lesions in the vessels and meninges were
explained on this basis.

In a case observed by Schultze^^ there was diffuse leptomeningitis
spinalis, with transverse myelitis and cavity formation in the anterior
part of the posterior column.

Wieting^" described a case in which intense inflammatory thickening
of the meninges and cavity formation in the cervical swelling were
observed. He believed that the process had begun in the meninges.
Intense pachymeningitis was also present in the case reported by
Dercum and Spiller.

Whether the meningitis is the cause of the cavity formation in these
cases, or whether both processes are the result of one cause, and
whether that cause is syphilis or not, is still conjectural to a certain
extent.

Phillip and Oberthur, as a result of their investigation of eleven
autopsies, believed that the cavity formation and the pachymeningitis
were the result of a common cause, and developed parallelly without
being dependent one on the other. Adamkiewicz held to the same view.
On the other hand Rosenblath considered that the pachymeningitis in

136

rhein: syringomyelia with syringobulbia 13

his four cases was primary, and the syringomyeha the resuU of a
disintegrating process (" einschmehzungsprocesse ").

Schwarz admits tliat spinal meningitis is an etiological factor in the
formation of cavities in the cord which does not differ in the strict
sense from the cavity formation of syringomyelia.

In my case the character of the disease of the vessels of the cord
and the medulla oblongata, the occurrence of round cell infiltration in
the pia and around the blood vessels, and the presence of diseased
blood vessels and round cell infiltration in the wall of the cavity at
various levels, suggested the probability that these lesions were of
syphilitic origin. Instances of this nature are not rare in the litera-
ture, and a number of cases have been reported in which cavity forma-
tion had occurred during the course of a specific meningomyelitis.
Of such was the case of Schwarz, in which there was cavity formation
in the anterior horns on one side, situated in the cervical, dorsal, and
sacral regions. He cites a case, described by Lamy, of syphilitic
myelitis with cavity formation which seemed to be related to the dis-
tribution of the vessels, and also Schultze's case, in which cavity
formation was found associated with diffuse leptomeningitis spinalis
and transverse myelitis.

Jegorow reported a case of syringomyelia associated with meningitis
and syphilitic vascular change, and in Simon's four cases of syphilis
there was cavity formation and spinal meningitis. The cases of
Jappha, Nebelthur,-- and Hutchinson must be mentioned in this con-
nection. Jappha believed that the cavity formation in his case was
the result of a syphilitic meningomyelitis, and Wieting concluded that
syphilis is the cause in a great number of cases of pachymeningitis
cervicalis hypertrophica with secondary involvement of the cord and
cavity formation.

These observations warrant the conclusion that the presence of
syphilitic disease of the cord and meninges in syringomyelia is prob-
ably more than a coincidence.

The presence of disease of the blood vessels in syringomyelia has
been well recognized for a long time. There is usually an increase
in the number of blood vessels, especially in the neighborhood of the
proliferation of the ])ia, and in some cases numerous blood vessels are
found clumped together in a small area, as seen in the lumbar region
of our case. In Westphal's case (quoted by Schlesinger) there were
as many as ten vessels in one group. They are usually more numerous

187

14 RHEIN : SYRINGOMYELIA WITH SYRINGOBULBIA

in the peripheral portion of the proHferated gha. The blood vessels
are frequently distended and filled with blood.

Schlesinger quoted Simon and ^leinert, who described an unusual
delicacy (" zartheit "^ of the wall of the vessels, also cases in which
the lumen of the vessels were contracted, and sometimes obliterated.
as in the cases of Joffroy and Achard. Fiirstner and Zachner.-'' Ray-
mond.-* Redlich,-^ Leyden.-" Chiari.-' and others. Schlesinger has
never observed proliferation of the intima. although this has been de-
scribed by Rosenblath, and was present in my case. In uncomplicated
cases Schlesinger claims that round-cell infiltration of tlie walls of the
vessels is not regularly present, but lymph spaces are often distended.

The presence of thrombosis in the arteries, which was present in the
basilar arterv in my case, has been observed (though not frequently)
by Joffroy. Achard. and others (quoted by Schlesinger).

In the opinion of Muller and ^leder the degeneration of the vessels
was responsible for the necrotic process present in their case, and
Kolpen emphasized the fact that in his case the vascular conditions
were closely associated with the gliosis. Many hemorrhagic necrotic
foci were present. In Steudner's-* case, with cavity formation, the
vessels had undergone colloid degeneration. Jofifroy and Achard
believed that the vascular lesions are important factors in the forma-
tion of syringomyelic cavities.

Phillip and Oberthur found a great variety of vascular changes in
their eleven cases of syringomyelia and pachymeningitis cervicalis
hypertrophica, and in Jappha's case of syphilitic meningomyelitis,
already referred to, the blood vessel alterations were striking. Syphi-
litic change in the vessels was present in Jegorow's case of syringo-
myelia associated with meningitis, and Joffroy and Achard looked upon
the development of syringomyelia as the result of an inflammatory
process, having its origin in disease of the vessels and the meninges.

In my case the findings have some bearing upon the explanation of
bulbar symptoms in cases of syringomyelia and syringobulbia. It
is well known that the lesion in syringobulbia does not extend beyond
the lower part of the pons, except in the isolated case of Spiller,-^ in
which the cavity was found as high as the posterior limb of the internal
capsule just below the floor of the lateral ventricle. Involvement of
the cranial nerves above the nucleus of the facial nerve is generally
considered to be the result of some complication. In my case there
was an involvement of the third nerve, causing ptosis; of the fourth

138

RHEIN: SYRIXGOMYELIA WITH SYRINGOBULBIA 15

nerve on one side, causing jiaralysis of the right superior oblique
muscle; involvement of the fifth nerves, explaining the anesthesia of
the left side of the face and on the right side of the scalp; and of the
nucleus of the right twelfth nerve, causing right hemiatrophy of the
tongue. There was also degeneration of the right ninth and tenth
nerves, both eighth nerves the right seventh and both optic nerves. The
degeneration, however, was of peripheral origin in all instances (except
the right twelfth nerve) as the fibers of these nerves within the pons
and medulla were apparently normal. In all probability the degen-
eration of the nerves described was due to pressure from the intense
leptomeningitis to which they were subjected as they passed from the
medulla oblongata and pons to their peripheral distribution. It seems
probable, if this be true, that in some cases the symptoms in the dis-
tribution or nerves, above the seventh especially, may be due to de-
generation of the nerves, the result of pressure upon them by a co-
existing leptomeningitis. As far as I have been able to learn, this
observation has not hitherto been made, although Kolpen^" explained
in this way the atrophy and paralysis in the upper extremity in his case
of pachymeningitis cervicalis hypertrophica.

The presence of a cavity in the pyramids has not been frequently
described. It was present on one side in my case, in a case of Spiller's,
and in the right pyramid in a case observed by Raymond and Phillip
(quoted by Wilson). It was found in both pyramids in Rosenblath's
case, and in Wilson's^- case the cavity, involving both olives, destroyed
also partly the pyramids. In a case reported by A. Westphal.
numerous spaces, or small cavities, were found in the region of the
pyramidal tracts in the medulla oblongata, partly destroying the crossed
pyramidal fibers.

Brief reference should be made to the distribution of the cavity in
my case. It was limited to the left side of the cord, from the sacral
to the second thoracic, at which level it involved also the right anterior
horn, and this condition persisted to the fifth cervical segment, at
which point, to the lower levels of the medulla oblongata, the cord was,
unfortunately, lost at the autopsy.

The unilateral localization of the cavity is uncommon. It was
present in the case reported by Kolpen. and in a case of Dercum and
Spiller, who quoted Oppenheim as stating that only two cases of
unilateral syringomyelia with autopsy are on record: those of Rosso-
limo, and of Dejerine and Sottas. They also refer to a case of

130

16 RHEIX : SYRINGOMYELIA WITH SYRINGOBULBIA

Hatschek's which presented predominance of the lesion on one side.

The retrograde degeneration of the right pyramid in the medulla
oblongata above the cavity in my case is deserving of some special
mention. The presence of degeneration of the pyramidal tracts in the
cord and medulla in an ascending direction is, however, not an un-
common observation. References in the literature to this condition,
eijiher in the cord or medulla, or both, not all, however, in cases of
syringomyelia, have been made by twenty-four observers.

Michaud, in 1871, believed that it was not rare to see sclerosis of the
lateral tracts above the spinal lesion. With the exception of C. West-
phal,^* who described a case in which there was degeneration of one
of the pyramids of the medulla oblongata above the lesion, most of the
cases were reported in the last fourteen years.

In the cases of Goumbault and Phillip, Raymond, Sottas, ^^ Alurto,^^
Hutchinson, Dercum and Spiller,^' Williamson,^*' and in one of
Spiller's, the degeneration did not extend into the medulla oblongata.

In the cases reported by Hatschek,"'' Schultze, Miura,'*'' Schlesinger,
C. Westphal, Fiirstner, Zachner, A. Westphal Rossolimo,*^ Weiting,
Petren,*- Spiller-'' (in one of his cases), Wilson, and Lloyd,'*" the
ascending degeneration was situated in the pyramids of the medulla
oblongata.

The extent of the degeneration usually is limited to short distances,
but, on the contrary, it may extend from the lower levels of the cord
into the medulla oblongata and pons. In Petren's case the, degenera-
tion extended from the eighth cervical segment into the pons. In
Hunt's** case the degeneration was found as high as the crura, and the
degeneration extended from the lumbar region to the lower portion
of the medulla oblongata in a case reported by Goumbault and Phillip.
In Rossolimo's case of syringomyelia the degeneration extended from
the dorsal region into the pyramids of the medulla, and in Fiirstner
and Zachner's case it extended from the lower lumbar region into the
medulla.

Several theories have been advanced to explain the presence of
ascending degeneration in the pyramidal columns, and in the pyramids
of the medulla oblongata.

Schlesinger and Hatschek beheved that the ascending degeneration
of the pyramids was due to degeneration of the fibers connecting the
cerebrum with the bulljar nerves. A. Westphal, however, could not
reconcile the extensive degeneration in his case to this theorv. He

140

KIIEIN : SYRINGOMYELIA WITH SYRINGOBULBIA 17

thought that the nerve fibers of the me(Uilla oblongata during fetal life,
before they were mcdullated, were subjected to some depressing in-
fluence which lowered their resistance to irritating inlUicnces later in
life. Schlesinger later agreed that perhaps this, as well as his own
theory, explained the degeneration.

A third explanation offered by Schlesinger was that, ]^ossibly,
sensory fibers that were cut by the cavity normally had a cerebral
course for some distance after decussating with the pyramidal fibers.
This, he claimed, would explain contralateral degeneration in the
pyramids. He acknowledged that this hypothesis had no anatomical
foundation.

Fiirstner and Zachner regarded degeneration of the pyramids in
their case as an unusual form of degeneration, especially as it was
associated with ascending degeneration of the lemniscus. Hunt con-
sidered the ascending " atrophy " in his case a retrograde process.

Obersteiner''^ viewed retrograde degeneration of the pyramidal fibers
as a process very similar to the Wallerian degeneration, although in
the former the axis cylinders remained intact longer than in the latter,
an opinion shared by Klippel and Durante,*" and Goumbault and
Phillip, who stated that it differed from the Wallerian degeneration
in the tardy development of the destruction of the myelin substance
which occurred before that of the axis cylinders.

Sottas believed that it was in consequence of the destruction of
certain cells in the gray substance which formed the axis cylinders,
of which some ascend and some descend. He quotes Gudden, Farel
and von INIonakow, who have shown experimentally that the nerve
fibers when separated from their centers degenerate in two directions,
centrifugally and centripetally, but that the retrograde degeneration is
only manifested in young animals, and appeared only after the lapse
of a considerable time.

Von Bechterew and Ziehen (quoted by Petren) have demonstrated
the presence of scattered fibers in the pyramidal tracts which do not
belong to this system of fibers, and von Bechterew (quoted by Petren)
believed that they consist of descending fibers originating in the cere-
bellum.

Von Bechterew*^ elsewhere described fibers which are situated in
the posterior part of the ground bundle, between the pyramidal tracts
and the gray substance, which he called the " mediales Seitenstrang-
bundel." It is made up of short fibers, and is related to the gray

141

18 RHEIX : SYRINGOMYELIA WITH SYRINGOBULBIA

substance of the cord. In the guinea-pig these fibers degenerate up-
ward, but this has never been demonstrated in man. It is possible
that in some cases the ascending degeneration found in the lateral
columns is due in part to degeneration of these fibers.

According to Egger (quoted by Spiller) retrograde degeneration
attacks the short fibers in the pyramidal tracts, and the resulting
sclerosis sets up a degeneration of the long fibers.

The ascending degeneration of the left lemniscus in my case, which
was traced as high as the level of the nucleus of the fourth nerve, is
really explained by the character of the lesion in the right side of the
medulla. The cavity extended from a point a short distance to one
side of the central canal in a forward and outward direction, de-
stroving the right olive, and cutting the internal arcuate fibers, besides
the fibers of the twelfth nerve and the cerebellar olivary fibers.

Degeneration of the lemniscus has been described in a number of
cases, in fact, according to Schlesinger, the degeneration of the contra-
lateral lemniscus is a typical finding in the lateral cavity formation of
the medula oblongata. It has been observed by thirteen observers, viz. :
Wieting. }^Iiiller. ]\Ie(ler, Kolpen, Hoffmann, Hatschek, Schlesinger,
Wilson, Westphal, Rossolimo. ]\Iiura, and PhilHp and Oberthiir.

This degeneration is probably, in some instances at least, partially
the result of destruction of the nuclei of the posterior columns, as in
the case of Hatschek, Schultz, Hoffmann. :\Iiura. and Schlesinger,
and my own.

Phillip and Oberthiir (quoted by Wilson), who described degenera-
tion of the lemniscus, stated that, in their opinion, it is a constant
finding in advanced syringomyelia, basing their statement upon four
examinations in which there was a gliosis beginning in the posterior
horns.

RHEIN : SYRINGUM VliLlA UUll .-.^ K 1 .\ .ulii i i;i A 19

LITERATURE.

1. Schlesinger. Die Syringomyelic, 1902.

2. Vulpian. In Schwarz.

3. Simon. Arch. f. Psychiatric u. Xcrvenkrank., No. 5, 1874, 108.

4. Zcnoni. Revue Xeurologiquc, i8yo, 924.

5. Rosenblath. Deut. Arch. f. klin. mcd., 1893, 210.

6. Achard and Joffroy. Neurologische Centralblatt, 1891.

7. Jegorow. Neurologische Centralbl., 1891.

8. Hoffman. Deut. Zeit. f. Nervenheilk., 1892-93, 17.

9. ^Miiller and !Meder. Deut. Arch. f. klin. Med., 1895, 117.

10. Oppenheim. Neurologische Centralbl., 1892, 759.

11. A. Westphal. Deut. Arch. f. klin. Med., 1899, 355, and 1896.

12. Saxer. Centralbl. f. allgm. Path. u. Path. Anat., 1898, 59; and Ziegler's
Beitrage, 1896, 332.

13. Philippe and Oberthiir. Revue Neurologique, 1899, 907.

14. Kaiser and Kiikenmeister. Arch. f. Psychiatric, xxx, 250.

15. Schwarz. Zeitschr. f. kHn. Med., No. 34, 1898, 469.

16. Jappha. Deut. Med. Woch., 1899, No. 25, 299.

17. Koehler. In Schwarz.

18. Hutchinson. University of Pa. 'Sled. Bull., ]March, 1906.

19. Schultze. Virchow's Archiv., 1882, 510.

20. Wieting. Ziegler's Beitrage, No. 19, 207; ibid., xiii, 411.

21. Adamkiewicz. Pachymeningitis Hypertrophica, Wien., 1890.

22. Nebelthur. Deut. Zeit. f. Nervenheilk., 1900, No. 10.

23. Fiirstner and Zacher. Arch. f. Psychiatric u. Nervenkrank., 1883, 422.

24. Raymond. Arch, f . Neurologic, 1894 ! and in Schlesinger.

25. Redlich. In Schlesinger.

26. Leyden. In Schlesinger.

27. Chiari. In Schlesinger.

28. Steudner. In Schw-arz.

29. Spiller. Brit. Med. Journal, October, 1906, 1077.

30. Kolpen. Arch. f. Psychiatric, 1895, 3I9. and 1906, 286.

31. Goumbault and Phillip. Arch, de Med. Experiment de I'Anat. Path., 1894.

32. Wilson. Revue de Med., 1904, 685.

SS. Michaud. Theses Sur La Meningitis Et. La Myelitis Dans Le I\Ial Verte-
brale, 1871.

34. C. Westphal. Arch. f. Psych, u. Nervenkrank., 1874, v, 90.

35. Sottas. Compte rend, de la Soc. Biol., 1893, 925.

36. Murto. In Durante.

S/. Dercum and Spiller. American Jour, of Mcd. Sciences, 1896, 672.

38. Williamson. Brit. Med. Jour., 1893, 946.

39. Hatschek. Wiener Med. Woch., 1895, 1027.

40. ^liura. In Hatschek.

41. Rossolimo. Arch. f. Psj-ch. and Nervenheilk., 1899, tSgo, 897.

42. Petren. Nordish Medicinsk. Arch., 1901, No. 14, i.

43. Lloyd, J. H. University of Pa. Med. Mag., 1892, v, 293.

44. Hunt. Jour, of Nervous and Mental Dis., 1904, 504.

143

20 RHEIX : SYRINGOMYELIA WITH SYRINGOBULBIA

45. Obersteiner. Nervosen Central Organe.

46. Klippel and Durante. Revue de ]\Ied., 1895, No. 15.

47. Durante. Revue Xeurologique, 1894, 390.

48. Von Bechterew. Neurologisches Centralb., 1897, 6, and Die Leitungs-
Bahnen in Gehirn und Riickenmark.

49. Spiller. Johns Hopkins Bull., 1898, 125.

50. Raymond-Cartaz. Gazette des Hopiteaux, 1895, Nos. 21 and 34.

DESCRIPTION OF PLATES II. AND IIL

Fig. I. — Showing intense leptomeningitis and distorted medulla oblongata.

Fig. 2. Cavity in posterior portion of the medulla oblongata. Degeneration

of the right pyramid above the cavity in the pyramid, and of the left lemniscus
and cerebellar-olivary fibers.

Fig. 3. Cavity in right pyramid, and a second cavity destroymg the nuclei

of the posterior columns on the same side, the fibers of the twelfth nerve and
the internal arcuate fibers. The left median lemniscus is degenerated and also
the left cerebellar-olivary fibers.

Fig. 4. — Degenerated optic nerve.

Fig. 5. — Shows degeneration of the lemniscus on one side.

Fig. 6.— Cervical region. Cavity on the right side. Intense leptomeningitis.
Grounded fibers degenerated on the right side.

Fig. 7.— Thoracic region. Cavity in the left anterior and posterior horns.
Degeneration of both crossed pyramidal tracts and right direct pyramidal tract.

Fig. 8.— Lumbar region. Cavity involving the anterior and posterior horns on
the left side.

The Journal of Medical Research, Vol. XVIIL, No. i, ]\Iarch, 1908.

144

Journal of Medical Research.

Vol. XVIII, Plate

J.D7.C h5.se-

FlG. I.

Fig. 2.

Syringomyelia with Syringobulbia

Journal of Medical Research.

Vol. XVIII. Plate

Fig. 3.

.♦J.^-'

rl»^

Fig. 4.

Ki.;. 5-

Fig. 7.

Fig. 6.

Fig. 8.

Syringomyelia with Syringobulbia

Reprinted from the University of Pennsylvania Medical Bulletin, January,
1909.

THE OPERATn'E TREATMENT OF PAPILLEDEMA

(CHOKED DISK), WITH SPECIAL REFERENCE

TO DECOMPRESSING TREPHINING^

By G. E. de Sciiwkixitz, I\I.D.

PROFESSOR OF OPHTHALMOLOGY IN THE UNIVERSITY OF PENNSYLVANIA,

AND

T. B. HOLLOVVAY, ^I.D.
INSTRUCTOR IN OPHTHALMOI.OrA' IN THE UNIVERSITY OF PENNSYLVANIA.

In recent years the treatment by operatitni of those alterations which
take place at the intra-ocular end of the optic nerve as the result of
increased intracranial tension or pressure has attracted widespread
interest. Concerning this matter, Sir \^ictor Horsley writes : " That
the release of intracranial tension arrests and cures optic neuritis was
first published by myself about twenty years ago, and this has been
confirmed by very many surgeons and physicians during that period. "-
Our own attention was first called to this subject by Dr. William G.
Spiller at a meeting of the Neurological Society, in I90i,'' and in
America we are much indebted for advances along these lines to Drs.
W. W^ Keen, AL Allen Starr, C. K. I\Iills, W. G. Spiller, F. X. Dercum,
C. H. Frazier, W. J. Taylor, E. Martin, Harvey Gushing, and a
number of other surgeons and neurologists whose work has been
reviewed by Spiller in the LTniversity of Pennsylvania Medical
Bulletin, in September, 1906, and in the TriiJisactious of the Ophthal-
mic Section of the American Medical Association, June, 1908. Im-
portant recent foreign communications are those by Leslie Paton,*

^ Read before the College of Physicians. November 4, igoS.

" The Ophthalmoscope, 1908, vi, 658.

'Journal of Nervous and Mental Disease, ]\Iay, 1901. Writing in 1896 (New
York Medical Record, February i, i8g6), M. Allen Starr called attention to the
relief of optic neuritis and other brain tumor symptoms by removal of a portion
of the skull, as advocated by Horsley, and describes an operation of this charac-
ter on a patient with tinnor of the corpora quadrigemina. Headache was re-
lieved, but there was no effect on the optic neuritis.

* Transactions of the Ophthalmological Society of the United Kingdom,
1905, XXV, 129; and Ibid., 1908, xxvii, 112.

1 145

2 DE SCHWEINITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

von Kriidener/ and E. von Hippel,- but, as this paper is intended to
record simply a personal experience, based upon a series of cases
which are hereafter quoted, we have not thought it necessary to
analyze any of the previous writings upon this subject, but have
contented ourselves with the brief references that have already been
made.^

First a word as to the pathogenesis of the intra-ocular changes
which occur under the influence of increased intracranial pressure.
Numerous theories have been propounded, and a review of them at
the present time would be unprofitable, because much modern investi-
gation, so greatly helped by the results of cerebral surgery, indicates,
as Herbert Parsons-^ has said, that " all those who have had oppor-
tunities of watching the extraordinary effect of the relief of intra-
cranial pressure upon a choked disk must agree that no theory which
leaves this element out of account requires any further considera-
tion " ; or, as Sir Victor Horsley^ maintains, " I would point out, first,
that optic neuritis is produced by a combination of factors of which
the only one we know to be certainly present is a rise of intracranial
tension or pressure." It would, therefore, seem that under most
circumstances the views of Schmidt-Rimpler and Manz are correct,
namely, that the marked edema of the nerve-head, which is so con-
spicuous a symptom in the condition under consideration, is primarily
due to a distention of the sheath of the optic nerve, caused by the in-
creased subarachnoid fluid being forced into this situation under the
influence of elevated intracranial pressure, and that, as Bordley and
Gushing have written, " since there is an almost uniform subsidence
of what we consider to be choked disk (barring certain cases com-
plicated by destructive hydrocephalus), after decompressing opera-
tions, whether conducted for the pressure of a tumor, cerebral edema
of one sort or another, infections or intracranial hemorrhages, a

' Archiv. f. Ophth., 1907, vol. Ixv.

" Bericht iiber die XXXV Versammlung der Dentschen Ophth. Gesellschaft
in Heidelberg, August, 1908.

^ We are indebted to Ward Holden for the following extremely interesting
quotation: "When the sight fails in a person in good health, one must cut down
upon the bone at the vertex, draw aside the skin, trephine the skull, and permit
the escape of the exuded liquid. In this way the sight of the blind is restored."
(Hippocratic Writings, C. 8.) Hirschberg gives the original Greek in Graefe
und Saemisch, Handbuch des Gesamten Augenheilkunde, Teil II, Band xii,
Kap. xxiii, p. 94.

° Pathology of the Eye, 1908, iv, 1363.

" Ibid., p. 658.

146

DE SCHWEIXITZ AND IIOLLOWAY : TREATMENT OF PAPILLEDEMA o

mechanical rather than a toxic process must play the chief role in the
causation of this well-recognized lesion."^

If these views are correct, the old terms " optic neuritis," " descend-
ing neuritis," and " papillitis," inasmuch as they give rise to erroneous
impressions, should be abandoned. Nor is " tumor papillitis," sug-
gested by ]\lr. ]\Iarcus Gunn, an improvement. Perhaps " choked
disk " (stauungspapille) more nearly describes the process which
actually exists than any other term except the one introduced by
Elschnig, which is also recommended by Herbert Parsons, namely,
" papilloedema," and this term, we believe, at present gives the greatest
satisfaction. It has been adopted, provided it is recognized as in-
cluding all stages of the process, by Bordley and Gushing in their
most recent communication on this subject. -

i. goncerning the ophthalmoscopic appearances of the
Nerve-head which Indicate Operative Interference.

Systematic writers have divided papilledema, or choked disk, into
various stages, and for the purposes of the present discussion we may
in part quote, with some modification, the description of Mr. Alarcus
Gunn.^

(a) Increased redness of the disk, loss of definition of its edges,
slight prominence of its surface, beginning filling in of the porus
opticus represent the first stage.

(b) Edema of the nerve-head, disappearance of the porus opticus,
complete obscuration of the disk margins, moderate haze of the
surrounding retina, and uneven distention and darkening of the retinal
veins represent the second stage.

(c) Decided increase of the edema, elevation and size of the nerve-
head, striae of edema, in the form of lines in the swollen retina between
the disk and macula, marked distention of the retinal veins, and
retinal hemorrhages represent the third stage.

(d) Increase in the prominence of the disk which assumes a mound-
shape and begins to lose its reddish color and to become opacjue, exuda-
tion in and on the swollen disk and surrounding retina, elaboration

' Since writing this para.yraph the authors, as the result of experimental
work, are convinced that while increased intracranial pressure is an imjiortant
factor in the production of choked disc it is certainly not the only one.

' Ibid., p. 510.

^ British Medical Journal, October 26, 1907.

147

4: DE SCHWEINITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

of the retinal hemorrhages in size and number represent the fourth
stage.

(e) Decided subsidence of the vascularity of the papillo-edema and
increasing pallor, with or without sinking of its prominence, shrinking
of the retinal arteries and thickening of their perivascular lymph
sheaths, spots of degeneration in the retina, especially in the macula,
represent the fifth stage, which soon passes into the final stage of so-
called postpapillitic atrophy.

Vision, other things being equal, is usually good and capable of being
preserved or improved by operation during the first, second, and
third stages. Vision rapidly declines during the fourth stage, is
very poor, and sometimes obliterated in the fifth stage, and in either
of these stages the prognosis quoad visum is most unfavorable, although
not always, at least in the fourth stage, entirely hopeless.

Because so much of the success of operation in its relation to the
preservation of sight depends upon its early performance, as wall
presently be shown, it is important to discuss for a moment whether
(a) there are any sure signs of beginning papilledema before the
symptoms appear which are present in the first stage, and (b) whether
an early period of disk edema may be masked by other conditions.

Antedating the symptoms which indicate the first stage of papille-
dema, there may be the so-called imminence of neuritis or edema,
in which the retinal vessels, often only one division of them, are
darker, more distended, and more unevenly tortuous than is normal.
Such a condition of affairs may possibly be due to pressure upon the
vein in the intervaginal space which is already being dilated, and
which is the place where, at the height of choked disk, this flattening
of the vessel, as Dupuy-Dutemps has shown, reaches its maximum.
It is not safe to make a decision at this stage by ophthalmoscopic ex-
amination alone. Whether carefully conducted photometric examina-
tions, which usually reveal disturbances of the light sense at this
period, would be of practical avail or not we are unable to say. Thus
far they do not seem to have been very satisfactory. Of importance,
however, is the field of vision — not the general field alone, but the
effect of these early changes on the relation of the color lines one
to the other. In this connection the investigation at present being
made by Bordley and Gushing promises interesting results, and they
intend to record them in the near future. The change in the size of
the blind spot, particularly in the vertical direction, which has been

148

DE SCHWEINITZ AND IIOLLOWAY : TREATMENT OF PAPILLEDEMA O

found by Ramsay and Sutherland to be an early sign of congestion
of the disk in sympathetic ophthalmia, and which depends upon
turgescence of the superior and inferior branches of the retinal vessels,
may possibly be a sign of early disk edema, due to increased intra-
cranial pressure. Concerning this symptom we hope to report at a
future meeting of the College.

That an early stage of papilledema may be masked by a corre-
sponding general retinal edema, is an observation of great interest
which has been made by Bordley and Cushing. In one of their cases
both disk and retina were uniformly edematous, although the struc-
tures remained clear, and it was only after palliative exploration with
decompression that the undetected retinal edema subsided in such a
manner that the disks at once became prominent, which in their turn
subsequently also regained their normal appearance. This observation
we can confirm, as follows":

Case I. — A boy, aged nine years, with a bullet in his brain, was
admitted to the surgical ward in the service of Dr. Frazier in the Uni-
versity Hospital, April 6, 1907, and an ophthalmoscopic examination
made three days later records the absence of papilledema, although
there appeared to be an ill-defined edema of the retina. On the 13th
of the same month a decompressing trephining with exploration was
performed by Dr. Frazier, and within a week, with the disappearance
of the retinal edema, a marked papilledema, as much as 5 D., was
evident. Following a second operation, in which the bone was
curetted for better drainage, this choked disk, much more marked in
the right eye, began to disappear, and a month later had decidedly
lessened, and we vmderstand at the present time has disappeared, with
the preservation of normal vision.

It would therefore seem, as Bordley and Cushing remark, that some-
times, without decompression, an incipient choked disk is extremely
hard to distinguish from a simple hyperemia.

It is also difficult to differentiate a true congestion with edema, or
beginning papilledema, from a spurious optic neuritis, or so-called
hyperopic disk, as it occurs in association with refractive error. There
is, however, in the beginning of papilledema, that is to say, wdicn
it has reached the first stage, a certain ([uality which pseudoneuritis
does not possess. The blurring of the disk edges is more complete,
there is evident contraction or filling in of the physiological pit, and
even with the indirect method of examination the obscured margins

149

6 DE SCHWEIXITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

are with difficulty seen, not to mention the uneven tortuosity of the
retinal veins, which has already been referred to. Here, again, a
careful investigation of the size of the blind spot and of the light sense
will probably lend aid in diagnosis.

II. Concerning the Date of Occurrence and Character of the
Nerve-head Changes, Together with the Vision.

It is not possible to predict how soon after a tumor develops changes
in the nerve-head will begin to appear. Sometimes, as Bruns has
pointed out, the whole process from the beginning of papilledema
to the height of its swelling is complete in a few weeks ; sometimes
months may elapse. In one patient with whose clinical history we are
acquainted, the choked disk was postponed until within a week of the
patient's death, although all symptoms of brain tumor had been present
for more than a year. In general terms it may be stated that, with
certain notable exceptions, the process must have existed for some
time and the increased intracranial tension have lasted for a definite
period before the engorgement edema develops.

While it is true that tumors of the cerebellum are prone to give
rise quickly to disk edema this is not always the case, and one record
shows that a cerebellopontile tumor was present for eight years
before papilledema developed (Bordley and Cushing).

In the experimental work of Bordley and Cushing, particularly the
introduction of fluid into the subdural space, disk edema occurred
very rapidly, indeed while the injection was taking place.

It is not possible, however, to determine from the stage of the
retinal process what the duration of the cerebral lesion is, nor can
we predict in the case of tumor in the absence of choked disk when
this is likely to occur. We do know that disk edema may arise with
great suddenness, and under such circumstances it indicates an in-
crease in the intracranial pressure, either as the result of an elabora-
tion of the growth itself, or on account of hemorrhage. To quote
again from Bordley and Cushing, " the whole question resolves itself
into a study of the character of the intracranial pressure and its efifect
upon the cerebrospinal circulation, regardless of the histology, the
situation, or the duration of the neoplasm."

It is almost useless to point out again that choked disk caused by
intracranial growths is perfectly compatible with good acuteness of
vision, and that this may exist for long periods of time, although there

150

DE SCHWEINITZ AXD IIOLLOW.W : TREATMENT OF PAPILLEDEMA 7

is well-marked engorgement of the nerve-head and all the signs of
papilledema. Even where the visual acnteness ai)pears not to be
normal, but is reduced, for example, to one-half, or one-third, or even
one-tenth of normal, it may sometimes be raised nearly or quite to the
normal standard if any existing optical defect is corrected and, there-
fore, visual acuteness recorded without note of the refractive error
and the effect of neutralizing lenses does not give a true indication of
the real sharpness of sight. The important point is that good vision
must not be permitted to stay the hand in operative interference, be-
cause what is good vision to-day may in a few days be poor vision,
and already those processes may have started which, if unchecked, lead
to the degenerations of the nerve, which ultimately end in blindness
and atrophy.

Whether the character of the refractive errors bears any relation-
ship to the rate of rapidity of the development of papilledema is
somewhat in dispute. Touching this point, Mr. Alarcus Gunn says:
" Tumor papillitis is most commonly associated with hypermetropia,
even in countries where this form of eyeball is not so common as in
England, and papillitis is relatively rare in myopia, both here and
abroad. It would appear as if in myopia an increased pressure within
the sheath space was less likely to press directly upon the nerve and
more likely to be relieved by filtration or absorption of the contained
excess of fluid." This matter is also referred to by Paton in his well-
known analyses of the effects of decompressing trephining on the
preservation of vision, but Parsons, apparently quoting him, says
" there is no reason to suppose that myopia has any deterrent effect
upon the development of i)apillo-edema, as has been thought"; and
Bordley and Gushing are evidently of the same opinion. We are not
prepared, on account of insufficient experience, to express an author-
itative opinion, but as far as it has gone it tends to show that if the
patient's eye is myopic there is more apt to be a late development of
papilledema than if the patient's eye is hyperopic, and in this sense
our observations coincide with those of Mr. Gunn.

With these preliminary observations, we pass directly to the prac-
tical side of the present communication. For this purpose we have
analyzed 21 cases of cerebellar tumor or cyst, 19 of which were
operated on by Dr. Frazier in the University Hospital, and 2 of which
were operated on in the Orthopedic Hospital, the one by Dr. William
J. Taylor, and the other by Dr. G. G. Davis, both of these patients

151

8 DE SCHWEINITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

being in the service of Dr. Sinkler; and 22 cases of cerebral tumor,
cyst, or abscess, 20 of which were operated on by Dr. Frazier in the
University Hospital, and 2 in the Orthopedic Hospital by Dr. William
J. Taylor, in the service of Drs. Lewis and Sinkler. Inasmuch as
these cases have been utilized in other analyses from the surgical and
neurological standpoints, we omit detailed description and statistics
and submit certain propositions and illustrative cases.

i'. The effect of decompressing trepliining, or of radical operation,
on the preservation or restoration of eyesight: (a) zvhen the z'ision is
good in both eyes prior to the operation; (b) zvhen the z'ision is good
in one eye but practically lost in the other eye prior to operation: and
{c) zvhen the vision is defective^ that is, belozu one-half of normal in
both eyes.

Case H. — A girl, aged fourteen years, was admitted to the service
of Drs. Spiller and Frazier in the University Hospital on June 24, 1Q08,
with a diagnosis of encephalitis, or possibly of brain tumor, and with
double beginning papilledema, the swelling of the disks being i^ D. ;
vision with test types normal in each eye. Craniotomy, with opening
in the dura, was performed on July 13, 1908, by Dr. Frazier. Six
days later, vision still being normal, there was only slight edema, and
seventeen days after operation the pupils were normal ; there was no
swelling of the disks, and only the slightest blurring of the nasal
margins, vision being practically normal ; that is to say, fully %..->. and
probably better, as the patient was a little difficult to examine. So
far as we are aware, the good result remains at the present time.

Case HI. — A man, aged twenty years, referred to the University
Hospital, October 25, 1905, by Dr. Weisenburg, with cerebellar tumor,
had papilledema of the right, eye of 11 D., and a vision of %, and
subsiding papilledema of the left eye, with a vision of only shadows.
Simple decompression was performed by Dr. Frazier without opening
of the dura, and the first subsidence in the swelling of the edematous
disk of the right eye was noticeable twelve days after operation. At
the expiration of two months the papilledema had disappeared and
the vision was % in the right eye, while the vision of the left eye
had risen to counting figures. In this eye there was temporarily an
increase of i D. in the swelling of the disk, which later slowly subsided.
Five months later a second decompressing trephining upon the opposite
side of the skull was performed by Dr. Frazier because of some
increase in the patient's general symptoms, the vision of the right eye

152

DE SCHWEIXITZ AND 1 lOl.l.OWA ^• : I UI:A IMENT OF PAPILLEDEMA 9

remaining- % as before, but the left, which showed increasing atrophy
of the disk, had dropped again to shadows. The patient has remained
well, and at the last report, two years after operation, he was perform-
ing his duties as a working man each day.

It cannot be doubted that had the decompressing trephining not been
performed, the right disk would have met the same fate which befell
the left one.

Case IV. — A man, aged twenty-three years, w^as admitted to the
University Hospital, December 12, 1903, with cerebellar tumor and a
papilledema of the right eye of 4 D., and in the left eye slightly less ;
he had very poor vision, w-hich unfortunately was not recorded with
test types at the time of his admission, but which is reported to have
been only the ability to see large objects. A decompressing operation
was performed by Dr. Frazier, with removal of a portion of the
cerebellar lobe, and at the expiration of seven days the vision had risen
to %5 in the right eye and %.5 in the left, and the edema of the disk
had dropped i D. on the right side and 2 D. on the left. At the expira-
tion of eleven \veeks after the operation the vision of the right eye had
risen to %, and of the left to %. In the right eye there was only a
slight surface edema, and the left eye was normal. Four years after
operation both disks were normal, and the vision of the right eye was
•%.-,, and the left %.

The two cases which follow illustrate the good results along these
lines of radical operation :

Case V. — A woman, aged forty-five years, with cerebral cyst, had,
just prior to the operation, a pa])illedema of 5 D. in the right eye and
3^ D. in the left, and a vision of %2 i" the right and %.5 in the left.
The patient was admitted to the University Hospital, February 5, 1908,
in the service of Drs. Mills and Frazier and a large cerebral cyst
removed, with complete disappearance of the edema and restoration of
full visual acuity at the end of two months. Four months later the
edema began again to appear, although vision continued to be normal.
When the height of the swelling had reached 5 D. the brain was re-
opened and a large quantity of growth removed from the site of the
original operation, followed, in the course of two months, by complete
second disappearance of the papilledema, w^ith a vision of % of
normal in the right eye, and %.5 in the left, on which side there were
some indications of pallor in the deeper layers or the disk. This vision

153

10 DE SCHWEIXITZ AXD HOLLOWAY : TREATMENT OF PAPILLEDEMA

continues to the present time, the last test having been made a few-
days ago.

Case Vl. — A woman, aged twenty-three years, a patient of Dr.
Morris Lewis, in the Orthopedic Hospital, with cerebral tumor, had a
double-choked disk of sV^ D. in each eye, and vision of %. A radical
operation was performed by Dr. William J. Taylor over the fissure of
Rolando, and the tumor removed. Rapid subsidence of the disk edema
began on the second day and was nearly complete at the end of the
seventeenth day, and the patient is now able to walk around and attend
to her afifairs : her vision is normal and the disk entirely recovered,
nearly a year after operation.

All of these patients were operated on in the first or not later than
the second stage of papilledema except one in whom the stage of the
edema in the one eye had passed to the third period, and in the left
eye was well advanced in the fourth or beginning of the fifth period,
and the result was. as before noted, a preservation of the sight of the
better eye. It is unnecessary to consume more time by the recitation
of other cases. They serve to illustrate the first of the propositions
presented this evening, and confirm Marcus Gunn's statement, as well
as the experience of many operators, that if the intracranial tension is
relieved in the first, second, or third stages of papillo-edema, the prog-
nosis as to vision is decidedly favorable.

2. To illustrate the danger of delay in resorting to operative inter-
ference, and the rapidity zcith which vision may deteriorate in cases of
papilledema, zchiclj deterioration is clieckcd by a suitable operation.

Case \TI. — A male, aged twenty-five years, came to the University
Hospital. January 29, 1906. with all of the symptoms of brain tumor
and double papilledema, on ihe right side the elevation being 5 D.,
and on the left 4 D. The vision of the right eye was %..-. and of the
left %. Operation was urged and declined, and it was not until two
months after his first examination that surgical interference could be
undertaken. He was under constant medical treatment, but during the
first twenty-six days of his treatment his vision fell to %.-, in each eye
without any material increase in the size of the choked disk or change
in the ophthalmoscopic appearances. Decompressing trephining was
now performed by Dr. Frazier, with opening of the dura. \\'ithin five
days the disk edema began to subside, first in the right eye. It liad
practically disappeared by the end of the second month, and at the last
examination, eight months after the operation, the vision of the right

154

DE SCHVVEINITZ AND HOLLOWAV : TR1:ATMEXT OF PAPILLEDEMA 11

eye prior to operation was maintained at %5, while that of the left
had improved from %-, to %-, in spite of some evidence of beginning-
atrophy. So far as we know this vision is maintained at the present
time, now more than two years after the operation.

It cannot be doubted that had it been possible to perform the opera-
tion when the patient first entered the hospital, that is to say, when his
vision was %.5 and %, this acuteness of sight would have been pre-
served, becaue the depreciation of vision which so rapidly manifested
itself was immediately checked by the trephining, and ultimately, in so
far as the left eye is concerned, improvement in visual acuteness to the
extent already recorded was evident.

The outcome, however, is not so forunate as this in all cases if the
patient declines operative interference.

Case Ylll. — A woman, aged twenty years, was first examined bv
one of us. November 22, 1906, with all of the symptoms of brain tumor
and double papilledema of 5 D. in the second stage of this process,
but with a normal visual acuteness, that is to say, % in each eye. In
spite of frequent urging to submit to operation, she went on with
the usual treatment of iodide and mercury. Exactly when the vision
began to fail we cannot state, as she passed from our own observation
and we did not see her again until she became a patient in the service
of Drs. Mills and Frazier in the University Hospital, January 9, 1908,
or thirteen months after the original examination. Papilledema in
the late fourth or the beginning fifth stage was present, and vision was
reduced to the perception of very large letters held quite close to the
eye. A decompressing trephining was performed, with relief of the
general symptoms in so far as we are aware, but without any efifect in
restoring or preserving the declining vision.

This experience could be emphasized by the recitation of a number
of cases, which would only unnecessarily consume time. Inasmuch as
the operation was an entire success from the surgical standpoint at the
time it was performed, it cannot be doubted that an equal success would
have occurred had the trephining been possible when the vision was
still %, at which standard it doubtless would have been maintained.

3. To illustrate the preservation of, or even slight improvement in,
very poor vision existing prior to the operation by a decompressing
trephining.

A question of importance to decide in so far as vision is concerned is
this: Shall the surgeon operate even though the papilledema has

155

12 DE SCHWEINITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

reached the fourth, or even the fifth stage? Certainly even in the
fourth stage the prognosis quoad visum after operation is unfavorable,
and so high an authority as Mr. Gunn states that if the fifth stage is
reached there is no reason, so far as sight is concerned, for performing
any operation ; but he is willing that the operation shall be performed
in the fourth stage, because he has known useful vision to be obtained
after operation even at this period.

Case IX. — M. B., a woman, aged twenty-nine years, with cerebral
tumor, had papilledema in the fourth stage of 3 D. in the right eye
and 4 D. in the left, in which, indeed, the signs of the fifth stage were
already beginning to be manifest. The vision of the right eye was
^/loo ; the left eye was totally blind. A decompressing trephining
was performed by Dr. Frazier, and at the expiration of ten months
after the operation, at which time the last ocular examination was
made, the vision of the right eye had improved from >4/i.-,o to >^/oo;
all swelling of the disks had long since disappeared.

Case X. — A male, aged fifteen years, with cerebellar tumor, blind in
the right eye and with a vision of ^00 in the left, had double papille-
dema in the late fourth or beginning fifth stage of 6 D. in the right
eye, and 4 D. in the left. Decompressing trephining, with opening of
the dura, was performed by Dr. Frazier, and the first signs of sub-
sidence of the choked disk were manifest on the nineteenth day; at the
expiration of two months almost all of the swelling had disappeared,
and the vision of the left, or only eye which had any sight prior to the
operation, was still jM-eserved, in fact was slightly better, being %,, as
compared with ^o,,.

Certainly these two cases are good illustrations of our contention,
and also the contention of Mr. Gunn, that even though the visual
prognosis is unfavorable, and a late stage of the papilledema has been
reached, blindness may still be averted and a decompressing trephining
should be tried.

Unfortunately this is not always the case, as is illustrated by the
following record :

Case XI. — A girl, aged seventeen years, with cerebellar tumor, had a
papillo-edema of 9 D. in each eye in the late fourth or beginning fifth
stage, with a vision in the right eye of %o> the left being totally blind.
Decompressing trephining, with opening of the dura, was performed
by Dr. Frazier, and already, on the fouth day, there was a subsidence
of 3 D., and at the expiration of a month a practical disappearance

15G

DE SCHWEINITZ AND IIOLLOWAV : TREATMENT OF PAPILEI.DEMA 13

of the papillo-edema, without, however, preserving the slight vision
which had existed prior to the operation, inasmuch as the %„ at that
time was reduced to hand movements, and a short time afterward dis-
appeared entirely, the blindness being absolute. There could not be a
better illustration of the misfortune of delayed operation.

All who have to do with the blind will realize how much it means to
the sufferer if he has only light perception, and how he will cling to
that small blessing with almost the same tenacity with which we would
cling to the preservation of good vision. Therefore it would seem that
even from this standpoint it is fair to give the patient a chance bv a
decompressing trephining, although there is no hope of restoring
vision, but only hope of preserving this faint light perception. The
following is a good illustration :

Case XII. — A girl, aged fourteen years, witli double ])ai)ille(lema
of 5 D. in a late state of its development and vision reduced to faint
light perception, had a decompressing trephining with opening of the
dura performed by Dr. Frazier. Already at the end of the first day
there was distinct subsidence of the swelling and one month later it had
absolutely disappeared, fortunately leaving the patient with the light
percention that existed prior to the operation, but without improve-
ment.

It cannot be doubted that had the operation not been performed the
small measure of sight which this patient possessed would have been
speedily totally lost. Whether what she now has will later disappear
cannot be stated, but at least for a period of time she has had this
satisfaction, and, as we have said, to a blind person it is not a small one.

On the other hand, in a certain number of cases of brain tumor,
with very little vision prior to the trephining, for example, a vision
reduced to hand movements or even light perception only, this sight
may rapidly disappear after the trephining, especially if there has been
much hemorrhage. For example, three patients, the one with a tumor
of the lobe of the cerebellum, another with a growth of the cerebello-
pontile angle, and the third with a cyst of the left lobe of the cerebellum
and with much reduced vision prior to the operation, namely, hand
movements in two and the ability to distinguish very large letters held
close to the eye in the third, were completely blind, even to the aboli-
tion of light perception within the first week after operation, although
in each intance the surgical success was a brilliant one. We do not
think, however, that experiences of this character should be recorded

14 DE SCHWEIXITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

as contra-indications for the operation, because these patients with very
much vision are doomed to speedy bhndness without operation, and
with it they have a small chance of the preservation of such sight as
they have.

We come now to take up certain complications and certain disap-
pointments connected with the operation which is under discussion.

I.. The occasional delay in the- subsidence of the papilledema and
the restoration of vision, hut with an ultimate good result.

Case XIII. — A woman, aged fifty-one years, with a tumor of the
cerebellopontile angle, had a papilledema of 4 D. in the right eye
and 3 D. in the left, and a vision of % in the right, and % in the left.
After decompressing, with opening of the dura, there was absolutely no
change in the appearance of the disks at the expiration of several
weeks. After a second operation performed by Dr. Frazier, in which
the dura was opened and some blood evacuated and the dura left un-
sewed, there was again no change, for nearly a month, but on the
twentv-third day the disk edema began to subside, and five weeks later
the swelling of the disks had been reduced to 2 D. in the one and to
i^ D. in the other eye, and vision had improved in the right eye from
% to %, and was practically maintained in the left at %.-,.

This case illustrates a point to which Mr. Gunn also makes refer-
ence when he says : " A'isual improvement after operation, although
long delayed, may ultimately be very satisfactory."

2. Increase in papilledema and visual disturbance following decom-
pressing trephining.

Everyone interested in this matter must have noticed in a certain
number of cases a temporary depreciation of vision within the first
week after operation probably due to shock, perhaps to hemorrhage,
and which is apparently of no importance in the subsecjuent restoration
or preservation of vision, provided the primary vision has been good
and the disk edema of comparatively short duration. So, too, in other
cases during the first day or two after trephining, there may be a slight
increase in edema associated with fresh hemorrhage. This is appar-
ently of no importance, as the added edema and fresh hemorrhage
disappear in the subsequent general subsidence of the swelling. For
example :

C.\SE XV. — A woman, aged seventeen years, with sarcoma of the
brain and double papilledema of 5 D. each, had a decompressing
trephining performed by Dr. Frazier, with opening of the dura. At

158

DE SCHWEINITZ AND IIULLUWAY : TREATMENT OF PAPILLEDEMA 15

the end of eleven days vision had decreased and the papilledema in-
creased 2 D. on the rig-ht side and i D. on the left; but one month later
there was gradual improvement followed by the usual subsidence.

This temporary increase of edema is probably due, as Bordly and
Gushing point out, to manipulation of the brain at the time of opera-
tion, or sometimes depends upon hemorrhage which has gathered
beneath the dura and which must be let out at a subsequent operation.
The point is that if it occurs the prognosis should not be too unfavor-
able, and it should lead to a careful examination of the field of opera-
tion to ascertain whether some such complication as has been referred
to has taken place.

3. Preservation of the vision of one eye, hut failure to preserve the
sight in the opposite eye by a decompressing trephining.

Case XVI. — A woman, aged twenty-one years, with cerebral tumor,
had a vision of % in each eye and a papilledema in the third stage of
7 D. in each eye. A decompressing trephining, with opening of the
dura, was performed by Dr. Frazier, and at the expiration of nine
days there was marked subsidence of the swelling, and after one
month it had been reduced just 50 per cent., with complete preserva-
tion of the vision of the right eye, but with a reduction of the vision of
the left eye from % to counting fingers.

It is possible that under these circumstances, in the eye which fails to
regain or preserve its vision after the trephining, atrophy of the nerve
has already begun, but has not yet become opthalmoscopically manifest,
and is usually the eye on the same side as that on which the lesion
occurs. In other words, the degree of the swelling of the papilla is not
always an accurate indication of the visual prognosis, nor is the
ophthalmoscopic examination always a complete guide as to what
process has already started deeper in the nerve.

4. Loss of vision zvhich has been good prior to the operation and
zvith the papilledema still in an early stage.

We have already seen that the prognosis as to vision after decom-
pressing trephining is unfavorable when the disk edema is at a late
stage of its development, and that though occasionally successful in
preserving what vision exists it is sometimes also followed by rapid
disappearance of the trifling vision which the patient has. Unfortu-
nately also, occasionally after technically correct operations, when one
would expect good results, there is rapid loss of vision.

Case XVII. — A boy, aged ten years, a patient of Dr. Sinkler's, in

159

16 DE SCHWEINITZ AXD HOLLOWAY : TREATMENT OF PAPILLEDEMA

the Orthopedic Hospital, with cerebellar cyst and double papilledema
of 8 D. in the right eye and 7 D. in the left, had a vision of %o in
the right eye and % in the left. Trephining was performed by Dr.
William J. Taylor, and the operation suspended on account of severe
hemorrhage. At the exph-ation of thirteen days there was not the
slightest change in the swelling of the disks. Twenty days after the
first -operation a second one was performed, the dura opened, and the
cyst ruptured during the manipulation, with the result that at the end
of three days the swollen disks had been reduced respectively to 3 and
5 D., and the expiration of a little more than a month their swelling
had entirely disappeared ; but in the meantime rapid atrophy had
ensued, and the right eye had become totally blind and the vision of
the left reduced to %o with difficulty. The last examination, made a
few days ago, indicates slight visual improvement, namely, %(,.

Referring to these cases, and there are not a few of them on record,
one of us have written as follows : " Occasionally there is a rapid loss
of sight after trephining when one would expect just the contrary
result. A possible explanation of this unfortunate sequel is great loss
of blood at the time of operation. The hemorrhage may have caused
some change in the ganglion cells of the retina, exactly as such changes
may be produced by free loss of blood after hemorrhage from the
stomach or bowels. If so, the mechanism of the blindness under these
circumstances is the same as that which occurs in quinine and methyl-
alcohol poisoning."

Concerning these cases Dr. Harvey Gushing^ writes as follows : " I
recall very much the same experience that you speak of on one or two
occasions, and it is possible that there may have been others. It is
my impression, however, that they have all been patients in whom
just before the time of operation there had been rather a rapid loss of
visual acuity. I think I may possibly be able to explain the loss of
vision which occurs in some cases of subtentorial lesion, even though
the swelling may subside and even though vision may have been fairly
good at the time of operation. Total blindness, however, with total
deafness, occurred in two of my patients shortly after suboccipital ex-
plorations, which disclosed no lesion. Both of these patients had a
growth involving the corpora quadrigemina, and the blindness and
deafness were attributed to implication of the geniculate bodies. Is
it possible that a similar condition could have been instrumental in the

^ Personal communication.

160

DE SCHWEINITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA 17

loss of vision in the patient with cerebellar cyst whom you mention ? "
He also discusses the relationship of acute obstructive hydrops ventri-
culorum which is present in subtentorial lesions, and which as we know,
leads to a hio^h grade of papillo-edema and subsequent atrophy. In
these cases of cerebellar cyst he says that there may be a gradual shut-
ting-down upon the nerve of the new- formed connective tissue during
the process of subsidence of the swelling.

In conclusion, we desire to call attention to certain practical points
connected with the ophthalmoscopic examination of patients suffering
from intracranial tumor, both with and without decompressing
trephining.

I. The earliest date at zcJiich subsidence of the szvoUen disk is
noticeable.

In eleven cerebellar tumors recently observed, the first distinct
lessening in the disk edema after operation ; that is to say, at least i D.
was noticed as early as the end of the first day in one case and deferred
as late as the twenty-third day in another case, the average being a
little over nine days. A similar analysis of nine cerebral tumors
showed that the first decrease occurred in one case as early as the end
of the second day, and in another case, in which there was a primary
increase of the edema, it was delayed to the thirtieth day, the average
being a little over ten days. A very common result, even in those
cases in which the disk edema itself does not appreciably diminish for
the first few days, is a lessening of the general vascularity of the eye-
ground, which is ofen quite noticeable within a few hours after the
trephining, although we have not been able to observe it while watch-
ing the eyeground during the progress of the operation. This, how-
ever, is rather difficult to do, and doubtless future investigations along
these lines may yield other results. In general terms, it may be stated
that while the disk edema may subside as much as i D. within the
first twenty-four or forty-eight hours, and (juite commonly begins to
subside from the third to the fourth day, an average of a number of
cases usually indicates that the first distinct improvement occurs from
the ninth to the tenth day. This observation is in accord with the
statistics already published by Mr. Paton, who states that generally
with a week or a fortnight after the second operation (all of his cases
were submitted to two operations ; or, rather, to two stages of opera-
tion, the first consisting in removal of the bone and sometimes incision
of the dura, and the second, if possible, the removal of the tumor

Itil

18 DE SCHWEIXITZ AND HOLLOWAY : TREATMENT OF PAPILLEDEMA

itself) there was distinct subsidence of the sweUing. but between the
two operations there was httle measurable alteration in the disks.

Accordino- to IMr. Gunn, the date of the first occurrence of the
subsidence of disk swelling and the rapidity with which it proceeds
vary considerably, but are earlier and more rapid in cases where the
operation has been performed not later than the third stage of disk
edema. We haye not found in our obseryations that there was any
material adyantage. in so far as the preseryation or restoration of
yision is concerned, in sudden subsidence of disk edema, or, more
accurately, in rapid subsidence, with perhaps one notable exception,
operated on at the Orthopedic Hospital by Dr. Taylor. Indeed we
haye come to belieye that the more gradual subsidence, beginning at
about the tenth day. yielded more fayorable results than when the disk
eleyation subsided more rapidly. This point, howeyer, is one which
would require further study before it could be presented in a dogmatic
statement.

2. The indications zvhich the disk appearances give as to the sise
and situation of the grozi'th; that is to say, zL'hether it is on the right
or left side of the brain or cerebellum.

As we haye already stated, and as has been pointed out many times
by other observers, opthalmoscopic examination yields no information
in regard to the size or structure of a tumor, because it is perfectly
well known that small growths may cause an elevation of intracranial
pressure greater than large and infiltrating ones, depending upon the
situation of each. If the growth has caused an increase in intracranial
tension through what has been called the intermediation of an internal
hydrocephalus, as Bordley and Gushing point out. the disk edema is
apt to be equal in the two eyes, and it is probable, as these authors
further maintain, that if every brain tumor exerted its pressure
equally in all directions the swelling in the two disks would always be
likewise equal. This we know is not always the case ; and, moreover,
under certain circumstances there is a greater pressure of fluid in the
optic nerve sheath on one side than the other, and, therefore, a greater
swelling of the optic disk in one eye than the other. \\'hether this
greater swelling on one side is a sufficient indication that the growth
is also upon the same side of the intracranial contents is a matter of
dispute. Thus, Sir \'ictor Horsley holds that the disk edema, or
neuritis as he calls it. occurs first of all. or is more intense, on the
side of the tumor, while Mr. Leslie Paton's observations indicate that

162

DE SCnWEINITZ AND HOLLOW AY : TREATMENT OF PAPILLEDEMA 19

no reliance can be placed on this sign, for although the preponderance
was somewhat in favor of the more severe neuritis being on the
affected side, yet in some cases it was more marked on the opposite
side. On the other hand, Sir William Gowers, and apparently Dr.
Beevor, agree with Horsley in regard to the homolaterality of the disk
sWelHng and the tumor. Touching this point, Bordley and Gushing
state that in 70 per cent, of their cases the papalla on the side of the
tumor was the most involved, and in 80 per cent, of the cases seen be-
fore the choked disk made its appearance, the first swelling ultimately
occurred in the eye homolateral to the tumor, and in 72 per cent, the
ultimate damage to the nerve was greatest on this side.

So far as we are able to judge from our own experience, we would
say that in the majority of cases the greater swelling is on the same
side as the tumor, but we would agree with Horsley, Gushing, and
others, that it is not so much to the swelling of the disk that atten-
tion should be paid, as to the indications as to which disk has been
longest afifected by finding the evidences of beginning atrophy, by
attention to which side of the disk has first been affected, the nasal or
the inferior quadrant, and by the information which is given by a test
of the visual acuteness, the light sense, and the field of vision. As
Bordley and Gushing point out, a tumor may, for instance, be present
on one side of the brain, and by the character of its growth impinge
on the opposite side and cause an unequal forcing of cerebrospinal
fluid into the intravaginal sheath of the optic nerve, and therefore
a greater swelling of the disk on the opposite side of the tumor ; or
it may happen that the examination is made when the disk which was
originally the largest in size already begun to decline, and this would
throw an element of doubt on the examination. Therefore, the mere
swelling alone does not furnish the most important information, but
the other symptoms to which we have called attention. So sure of
this is Sir Victor Horsley that he states : *' Within my own knowledge
the early failure of visual acuity indicates beyond doubt the situation
of the tumor." The essential point, according to him, is the estimation
of the age of the neuritis, and tliis means minute examination of the
interstitial tissue of the disk.

3. Delay in the development of the optic disk changes.

We have already pointed out that there is some reason to believe
that a study of the refraction of the eye is of some importance in the
determination of this point, and that although all observers are not in

108

20 DE SCHWEIXITZ AXD HOLLOW AY : TREATMENT OF PAPILLEDEMA

entire accord, there is a good deal of evidence to show that in the
presence of myopia the disk changes do not develop as quickly as when
the other type of refraction is present.

A still more interesting question in this connection is the age of the
patient, and if Singer's^ tables are correct, the absence of optic neuritis,
or disk edema, in intracranial tumors, exclusive of those occurring in
the pons, is rare in cases under forty years of age and becomes in-
creasingly more frequent after that period of life. Thus in an analysis
of 88 cases of cerebral and cerebellar tumors, he found in 35 cases
under thirty years of age well-marked choked disk in 97.1 per cent,
of the cases, and its late or slight development in only 2.9 per cent.
of the cases, it being absent in none of them. Between thirty and
fortv the figures read thus: 18 cases, well-marked neuritis, 76.6 per
cent.: late, or slight neuritis, 2.2 per cent.; absent, i.i per cent.: be-
tween forty and fifty the neuritis was well marked in 61.9 per cent,
of the cases, 21 in all, and absent in 19; while over sixty years of age
in 7 cases it was not well marked in any of them, late or slight in 14.3
per cent, of them, and absent in 57.1 per cent.

If these figures are correct, and we shall return to more elaborate
analyses at some future time, the search for the earliest stages of
papilledema, according to the directions already given in cases of
suspected brain tumor after the thirtieth year of life, becomes in-
creasingly important.

In final conclusion we would say :

1. The most satisfactory treatment for the purpose of preserving
vision in any case of choked disk or papilledema not due to a toxic
process or constitutional disease ("infectious optic neuritis"), but
depending upon increased intracranial tension, is decompressing
trephining, with the removal of the growth if it is accessible.

2. This operation should be performed early, and if it can be done
during the first, second, or even third stage of papilledema, the
prognosis as to sight is most favorable.

3. If for any reason the operation is postponed until the develop-
ment of the fourth and fifth stages of papilledema, already associated
with marked depreciation of vision, the prognosis as to sight is un-
favorable ; but, even under these circumstances the operation should
be performed, because it sometimes preserves such vision as still
remains, and if it should happen to be followed by a rapid depreciation

^Lancet. 1902.

164

DE SCHWEIXITZ AXD HOLLOWAY : TREATMENT OF PAPILLEDEMA 21

of vision, as, unfortunately, is apt to occur, it at least gives the patient
a chance, because without it they are doomed to blindness.

4, The investigation of the eyes under these circumstances must
include not only an ophthalmoscopic examination, but one which in-
cludes a careful investigation of the visual field, the color perception,
light sense, size of the blind spot, etc.

5. Patients afflicted with papilledema dependent upon increased
intracranial tension should have the case fairly stated to them, and
the operation should be urged in spite of occasional unfavorable re-
sults, because in its absence ultimate blindness is almost sure to result.

I60

Reprint from the Journal of Nervous and Mental Disease, May, 1908.

HEMIANESTHESIA TO PAIX AND TEMPERATURE AND LOSS OF

EMOTIONAL EXPRESSION ON THE RIGHT SIDE, WITH

ATAXIA OF THE UPPER LIMB ON THE LEFT. THE

. SYMPTOMS PROBABLY DUE TO A LESION OF

THE THALAMUS OR SUPERIOR PEDUNCLES^

By Charles K. ^lills, M.D.

Professor of Neurology in the University of Pennsylvania.

The following case shows such an unusual combination of symptoms as
to make it worthy of being presented to the society and permanently recorded.
It is difficult to fix the site of a single lesion capable of producing a symptom
complex which includes hemianesthesia and loss of emotional expression on one
side of the body and ataxia of the upper extremity on the other, but it is prob-
able that such lesion is situated in the thalamus of one side, and perhaps extends
into the superior peduncles of both sides.

G. S., thirty-four years old, barber, was admitted to the men's nervous wards
of the Philadelphia General Hospital, November 29, 1907, complaining chiefly of
weakness on the left side of his body and numbness on the right. The patient's
mother died at the age of 39 from " typhoid pneumonia." His father is living
and well. He has two brothers and one sister who enjoy good health. There
is no history of renal, cardiac, tubercular or malignant disease in the family.
The patient's history as to nervous or mental disorders is negative. He has
alwajs been an excessive drinker, principally using whiskey. He averaged about
one-half pint of whiskey daily. He has had two attacks of gonorrhea and he
had a sore on his penis, but no secondaries.

Ten weeks ago he was suddenly seized with vertigo, accompanied with nausea
and vomiting. He was confined to his bed for a week and was under the care
of a doctor. When he tried to resume his work as a barber, he found that his
left side was somewhat weaker and the left arm more awkward than before
his attack. He also found, while cutting a boy's hair, that this arm was tremu-
lous, and he was not so proficient in his trade as heretofore. In addition he
experienced a sense of numbness in the entire right side. His friends have
noticed that since his illness, when laughing he does so only with the left half
of his face.

Examination of the patient shows that the pupils are moderately dilated,
equal and regular. They react promptly to light, accommodation and con-
vergence. Extraocular movements can be well performed. Associated move-
ments are normal. There is no ptosis, nystagmus, exophthalmus or hemianopsia.
When asked to look upw-ards, he does so and his forehead w-rinkles equally well
on both sides. The nasolabial folds are as well marked on one side as on the

^ From the Department of Neurology- of the L'niversity of Pennsylvania.
1 166

l^'

Fig. I. — r>ilateral nin\cments in \(i'mntarv cti'nrt to show tlic Icflli.

Fig. 2. — Unilateral movement in laughing.

MILLS: IIEML\XESTIIESL\. TO PAIN 2

other. The palpebral fissures are about the same on the two sides. He can
purse his mouth fairly well, as in whistling, and he can move the two sides of
his face equally well. In spite of the fact that this man can perform all move-
ments of the face voluntarily, without any difference between the two sides, on
involuntary laughter he only smiles with the left side of his face, the opposite
side being perfectly passive. He protrudes his tongue in the midline and can
move it from side to side. There is no atrophy nor tremor. The jaw drops in
the median line. The actions of the temporals and masseters are well preserved.

The arms are of good development. Power, by the grip test, is somewhat
diminished in the left side. Resistance to passive movement is equally good on
both sides. He can perform all the normal movements well. In the finger to
nose test there is a marked ataxia on the left side, almost amounting to an
intention tremor. In the right extremity no ataxia is apparent. When he
extends his hands there is a coarse tremor of both hands, that in the left being
much more marked than that in the right. The oscillations are upwards and
downwards. There is no tenderness over nerve trunks. Atrophy is absent.
Biceps and triceps jerks are^exaggerated, and equally on each side. The legs
present a good musculature. All normal movements can be well performed.
In the heel to knee test no ataxia is demonstrable in either extremity. No
atrophy or tenderness is present over nerve trunks. Knee jerks and ankle jerks
are exaggerated on both sides. Plantar stimulation causes plantar flexion of
the toes on both sides. Ankle clonus is absent. The patient shows no altera-
tion of station with eyes open or closed. He also exhibits no abnormality of
gait with eyes open, but when closed incoordination is present to a slight
extent.

In the right half of the body to the median line absolute loss of sensation to
pain and temperature is present. In testing he calls the pin prick dull, except
on the right side of the forehead, where he says he feels the sharp touch.
Although he feels here, the sensation on the opposite side is much more acute.
Touch and muscle sense seem to be well retained. There is no astereognosis.
The sphincters are apparently normal. No defects of speech are noted. The
mentality of the patient is good, no delusions nor hallucinations being present.

The chest of the patient is well formed and he has good expansion on both
sides. Pulmonary resonance is good throughout. On auscultation normal
breath sounds are heard. No adventitious sounds present. Examination of the
heart shows that the apex beat is not observed on inspection. On palpation it is
found to be diffuse, although not forcible in the sixth interspace. Cardiac
dulness is slightly enlarged. The first apex beat is weak. No murmurs can be
detected. The spleen and liver arc not enlarged, and no ascites is apparent.

167

THE SYMPTOM-COMPLEX OF OCCLUSION OF THE

POSTERIOR INFERIOR CEREBELLAR ARTERY:

TWO CASES WITH NECROPSY^

By William G. Spiller, M.D,

PROFESSOR OF NEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN

THE UNIVERSITY OF PENNSYLVANIA ; NEUROLOGIST TO THE

PHILADELPHIA GENERAL HOSPITAL

The symptoms of occlusion of the posterior inferior cerebellar
artery are so pronounced and usually so sharply defined, that the
clinical diagnosis should be easy, although it may be difificult to
exclude implication of the vertebral artery. Three American
investigators have, made a careful study of obstruction of this
artery, but with the exception of these papers by Hun and Van
Gieson, and H. M. Thomas, I have been unable to find any thor-
ough consideration of the subject in the American or English
literature. It is possible I may have overlooked some paper con-
taining investigation in tliis line.

The symptom-coiTiplex of occlusion of the posterior inferior
cerebellar artery is as follows: Sudden onset, usually without
disturbance of consciousness. Motor power in the limbs and
fifth nerve distribution not affected, or at most temporary and
slight weakness of the limbs on the side opposite the lesion.
Diminution or loss of pain and temperature sensations in the
limbs of the side opposite the lesion, and in the fifth nerve distri-
bution on the side of the lesion, or also in the side of the face
opposite the lesion ; the disturbance of sensation in the face may
be only in the first, or first and second branches, or in the whole
distribution of the nerve, depending on the upper level of the le-
sion. Spontaneous pain, or some form of paresthesia in the area
of disturbed objective sensation. Tactile sensation usually intact
in all parts. Sense of position usually intact but sometimes
affected. Occasionally a zone of intact sensation in the neck
between the zone of disturbed sensation in the limbs of one side
and the face of the opposite side. Ataxia in the limbs on the

'■ From the Department of Neurology and the Laboratory of Neuro-
pathology in the tJniversity of Pennsylvania, and from the Philadelphia
General Hospital.

i83 WILLIAM G. SPILLER

side of the lesion (involvement of restiform body, cerebello-
olivary fibers), indicating that the fibers of coordination do not
decussate below the medulla oblongata. Tendency to fall to-
ward the side of the lesion ; tendency for the head to drop toward
the side of the lesion. Nystagmus bilateral, more intense when
the eyes are directed toward the side of the lesion (Deiters'
nucleus (?)). Meniere's symptom-complex; vertigo, revolving
vertigo, auditory hallucinations, vomiting (Deiters' nucleus, ves-
tibular nerve and nucleus, cochlear and vagus nerves). Intense
headache occasionally. Disturbance of micturition occasionally.
Paralysis of the muscles of deglutition on the side of the lesion
(vagus) but causing complete inability to swallow, with impaired
sensation of the pharynx. Paralysis of the soft palate on the
side of the lesion. Paralysis of the larynx on the side of the
lesion, voice hoarse, and speaking is in a whisper (loss of phona-
tion with 'preservation of articulation). Tongue possibly a little
weak on the side of the lesion, but the weakness usually not per-
sistent, as the hypoglossus nerve and nucleus usually escape.
Taste lost in anterior two thirds of tongue occasionally. Paresis
of the sixth nerve on the side of the lesion occasionally. Paresis
of the facial nerve on the side of the lesion occasionally. The
paresis of the sixth and seventh nerves is often absent in a lesion
confined to the posterior inferior cerebellar artery, and possibly
it may be caused by further extension of the area of softening.
Sympathetic disturbance ; smallness of pupil, narrowing of palpe-
bral fissure, retraction of eyeball, on the side of the lesion. Loss
of sweating in the face on the side of lesion occasionally, or
increase of sweating in the face on the side opposite the lesion,
occasionally. Obstinate hiccough. Hemiasynergy on the side
of the lesion (Babinski and Nageotte). Pulse may be rapid from
paralysis of the vagus. Loss of tendon reflexes, including the
patellar tendon reflex, or in other cases exaggeration of the ten-
don reflexes. Disturbance of hearing.

With few exceptions I have confined my citation of cases in
the literature to those in which a necropsy was obtained.

Dumenil's^ case 8 reads like one of occlusion of the posterior
inferior cerebellar artery, but from the symptoms he describes it
is hard to understand his statement that the area of softening
certainly was confined to the restiform body.

Leyden's^ case i might have resulted from thrombosis of the

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 184

posterior inferior cerebellar artery, but he supposed the cause to
be embolism of a small artery, and yet the embolus was not found.
The lesion was a small myelitic focus in the right half of the
medulla oblongata. Objective disturbance of sensation was not
detected. The patient had pain in the forehead on the side of
the lesion. Leyden's case 2 presented a myelitic focus nearly in
the middle of the medulla oblongata. It was supposed to be from
senile softening.

Senator's^ patient had vertigo, but was too ataxic to stand or
walk, though he could move all his limbs, and was obliged to go
on " all fours," and had a tendency to fall to the left. He had
a sensation of cold in the left side of the face, and speech was
disturbed as if by some obstruction in the throat or larynx. The
left eye was smaller than the right. Every attempt to move the
patient caused vomiting. He had temporary diplopia, also diffi-
culty in swallowing. The pulse was 120. Sensation was much
disturbed in the left side of the face and in the right limbs and
right side of the trunk. Tactile sensation also was involved.
The patellar reflex was lost on each side.

The necropsy showed thrombosis of the left vertebral and
posterior inferior cerebellar arteries, and softening in the outer
and posterior lower part of the left half of the medulla oblongata.

Eisenlohr* reports a case which had much the appearance
clinically and .pathologically of thrombosis of the posterior infe-
rior cerebellar artery, as the lesion corresponded to that caused
by occlusion of this artery, and yet he says no large vessel was
occluded, and he regarded the lesion as subacute encephalitis.

In Senator's^ second case the lesion was left-sided and ex-
tended from the lower end of the abducens nucleus not quite to
the lower end of the hypoglossus nucleus. The left vertebral
artery was thrombotic, and the thrombosis extended 2 to 3 mm.
on the pons in the basilar artery. The attack began with vertigo.
The patient had paresthesia in the right limbs and the left side
of the face. Pain sensation was lost in the face only in the dis-
tribution of the second branch of the fifth nerve. Associated
ocular movements to the left were lost, and sensation for touch
pain, temperature, position and passive movement, and motor
power, disappeared in the right extremities after a second attack.
The case was therefore a complicated one from partial thrombo-
sis of the basilar arterv.

1 85 WILLIAM G. SPILLER

In Van Oordt's® case the lesion was on the left side and at
the level of the middle third of the lower olive, and did not extend
into the pons. Hemianesthesia was present on the entire right
side of the body, limbs and head, but was not complete every-
where. Tactile sensation on the right side was only slightly
affected, and sense of location was intact. Sensation was normal
on the. left side. The lesion was thrombosis of the posterior in-
ferior cerebellar artery.

Reinhold,'^ case 2 : A man had suddenly some disturbance of
sensation in the left upper limb, a few hours later complete hemi-
plegia of the left limbs developed without any disturbance of
consciousness. The face was not paralyzed. Nystagmus was
present in looking to either side, but more intense in looking to
the right. Sensation was much diminished in the left side of the
body. The movements of the right upper limb were ataxic.
There was complete inability to swallow and right-sided laryngeal
palsy. The right vertebral artery was thrombotic from the point
of union with the left vertebral artery to a distance of about 3
cm. The basilar artery was not thrombotic. The lesion ex-
tended from about the lower end of the inferior olive to the upper
part of the hypoglossal nucleus, and almost the entire right half
of the medulla oblongata was implicated, including the right pyra-
mid. Much mental excitement was present from the beginning
of the paralysis, and the patient was unable to sleep even with
narcotics. Reinhold believed this could be explained by inter-
ference with the circulation of the anterior part of the brain by
the thrombosis of the vertebral artery. Inasmuch as the patient
was a physician, and doubtless realized fully the gravity of his
condition, it may be that the explanation given is not necessary.
Although swallowing was impossible, only one side of the pharynx
was paralyzed.

In Wallenberg's® case the lesion was on the left side, and was
occlusion of the left posterior inferior cerebellar artery and
frontal part of the left vertebral artery. Vertigo, and pain in
the left eye occurring in the beginning of the symptom-complex,
and pain later implicating the entire left side of the face, are
explained by irritation of the restiform body, vestibular nucleus,
Deiters' nucleus, and the spinal root of the fifth nerve. Slowing
of pulse was observed. Diminution of pain and temperature
sensations was noted in the right half of the face, and there was

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 186

also disturbance of the sensations of pain and temperature in the
first and second branches of the fifth nerve in the left side of the
face. The tactile sensation was not affected. Complete paraly-
sis of deglutition was present. Herpes developed in the areas of
disturbed sensation.

The manner in which the symptom-complex of occlusion of
the posterior inferior cerebellar artery develops is remarkable.
One might well suppose that the destruction of so large an area
in the medulla oblongata would be attended by a general disturb-
ance of function. Hun's" patient is an example of the manner
in which the symptom-complex is likely to develop. The man
went to bed feeling fairly well. He awoke during the night with
a feeling of a lump in his throat, as if he had swallowed some-
thing. In the morning he remained in bed by his wife's advice
and not from any feeling of inability to rise. He found he was
unable to swallow his breakfast. His voice was hoarse, pulse 80,
temperature 99-5°.

In Hun's case there were three areas of softening, but the one
which interests us in this connection was in the left side of the
medulla oblongata, in the region affected by occlusion of the pos-
terior inferior cerebellar artery. The pathological study was
made by Van Gieson. Analgesia and thermic anesthesia were
present in the left side of the face and right side of the body.
The man was ataxic in the left upper and lower limbs, not in the
right. The " muscular sense " was lost only in these limbs. The
man showed a tendency to fall toward the left and for the head
to fall on the left shoulder, i. e., toward the side of the lesion.
This was explained by a lesion of the left restiform body. There
was a decided increase in the secretion of sweat on the right side
of the face and on the right hand. The fillet was not degener-
ated, " muscular sense " was lost on the left side, but returned
after the first month. Hun attributed the lesion to occlusion of
the posterior inferior cerebellar artery.

Ransohoff's^° case is unsatisfactory, as the clinical history is
very deficient. The lesion was on the right side of the medulla
oblongata.

In Breuer and Marburg's^^ cases the symptoms were:

Case I. Right side: hypalgesia on the trunk and limbs. Left
side: hypalgesia of face, falling toward the left, ataxia of the
upper and lower limbs, disturbance of sense of position in the

i87 WILLIAM G. SPILLER

upper limb, loss of triceps reflex, and sympathetic ophthalmo-
plegia. Bilateral symptoms were: paralysis of soft palate, pa-
ralysis of deglutition.

The left vertebral artery was thrombotic, both posterior infe-
rior cerebellar arteries were intact; therefore the symptoms must
be attributed to the occlusion of the vertebral artery and not to
occlusion of the posterior inferior cerebellar artery.

Case 2. Right side : mild disturbance of pain sensation on the
trunk and limbs (?). Left side: falling toward the left; slight
diminution of temperature sensation for cold in the fifth nerve
distribution ; slight facial paresis ; disturbance of hearing ; slight
weakness of tongue ; sympathetic ophthalmoplegia (myosis, pto-
sis) ; vasomotor disturbance in the face, and ataxia of limbs.
Bilateral symptoms : paralysis of soft palate ; paralysis of deglu-
tition ; disturbance of senses of position and of passive movement
in the upper limbs, slight impairment of lateral movement of the
eyes; diplopia, and disturbance of vision.

Thrombosis of the left vertebral and basilar arteries was
found following embolism of the vertebral artery.

In the first case, the disturbance of pain sensation was in all
three branches of the fifth nerve, but was more intense in the
third branch. Temperature sensation was unreliable.

The case of Babinski and Nageotte^- with necropsy was a
complicated one. The focus they designate as F.i corresponds
to the area of degeneration caused by occlusion of the posterior
inferior cerebellar artery, but three other minute foci were found
in the medulla oblongata on the same side. The basilar and left
vertebral arteries were thrombotic.

In Mai's^^ case, without necropsy, pain and cold sensations were
lost on the left side from the second intercostal space and spine
of the scapula downward, and in the right side of the head in the
distribution of the first and second divisions of the fifth nerve.
The muscles of the throat and larynx were weak, although it
seems uncertain whether the larynx was affected. The right pal-
pebral fissure was smaller than the left, and right enophthalmus
was present. Sensations of touch, pressure and location were
normal everywhere. Sensation of heat was increased in the area
of altered sensation. Herpes of the right upper lip was observed.
In the affected areas a sensation of warmth was felt.

H. M. Thomas" observed two cases of occlusion of the pos-

OCCLUSION OF INFERIOR CEREBELLAR ARTERY i88

terior inferior cerebellar artery. In his first case the symptoms
were sudden vertigo, pain in the right side of the face, tendency
to fall toward the right, vomiting, slight ptosis of right eye, nar-
rowing of right pupil, loss of sweating on the right side of the
face, transient lateral nystagmus, some difficulty in swallowing,
disturbance of pain and temperature sensations on the right side
of the face and in the left limbs and left side of trunk, and ataxia
of the right upper limb. The lesion was thrombosis of the right
vertebral and posterior inferior cerebellar arteries. There were
no marked microscopical lesions. In his second case the symp-
toms were: recurrent attacks of numbness in the left side of the
face, vertigo, difficulty in speech, tendency to fall to the left, dis-
sociated sensory disturbances in the left side- of face and right
limbs and right side of trunk, paralysis of left vocal cord, loss
of sweating on left side of face, diminution of conjunctival, nasal
and pharyngeal reflexes on the left side, and slight ataxia in the
left upper limb, more marked ataxia in the left lower limb.

It seems strange that in Thomas's first case, in which a ne-
cropsy was obtained, no softening was found in the medulla
oblongata, and he explains its absence by the rich collateral circu-
lation, but his seems to be the only case in literature of positive
occlusion of this artery without softening in the medulla oblon-
gata.

In Miiller's^^ first case the symptom-complex developed sud-
denly during the night. The patient was awakened with vertigo
and a feeling that his bed was moving to the right, and with head-
ache soon followed by vomiting. He had paresthesia (sensation
of warmth) in the entire left side, including the left side of the
face. The cool bed covering felt hot to the left hand, but warm
water was cold to the left hand and left side of the face. In
urinating he was obliged to wait ten minutes, and he had con-
stipation, and frequent and painful erection during the first few
days. He had also dysphagia, and paralysis of the soft palate
and larynx on the right side. Sympathetic paralysis was present
on the right side of the face ; the palpebral fissure and pupil were
smaller than those on the left side. The man had a somewhat
hoarse and nasal speech. He was not ataxic, but was weak at
first in the left side, and after three days the right lower limb
became weak for a time from the nearness of the lesion to the
pyramidal decussation. The triceps and patellar reflexes were

i89 WILLIAM G. SPILLER

more active on the left side, and there was an indication of the
Babinski reflex on this side. Pain and temperature sensations
were lost on the left side (diminished in the left side of the neck),
but tactile sensation and senses of position and of movement were
perfectly normal everywhere on the left side. The sensation in
the distribution of the right trigeminus was intact, except that in
the distribution of the first branch tactile sensation and pressure
sensation were diminished. The pulse was a little rapid.

Miiller's second patient awoke one morning with 'the symp-
toms, having been well the night before. He was probably
svphilitic. The symptom-complex was very similar to that of
the first case. Anidrosis was present with the ocular signs of
svmpathetic paralysis. The facial and hypoglossal nerves were
not involved in either case. The pain and temperature sensa-
tions in the second case were diminished in the distribution of
the first branch of the fifth nerve on the same side as the disturb-
ance of those sensations in the limbs and trunk, but tactile and
pressure sensations were intact. The trigeminal nerve of the
opposite side was not aitected. The corneal reflex was dimin-
ished in both cases on the side opposite the disturbance of pain
and temperature sensations in the face. In botli cases the dis-
tribution of the upper branch of the trigeminus was more af-
fected than that of the second and third branches. The mucous
membranes of the head in both cases seemed to show no disturb-
ance of sensation. The motor power of the limbs was not af-
fected in the second case, but the patellar and Achilles reflexes
were prompter on the side of the sensory disturbances. Ataxia
was not present.

The symptom-complex in these two cases was : Dysphagia,
paralysis on the side of the lesion of the soft palate, larynx and
sympathetic supply, and weakness of the corneal reflex; on the
opposite side dissociated sensation involving the entire side, and
especially of the first branch of the fifth nerve in the face, and
perverse temperature sensation. The absence of ataxia in these
cases is noteworthy. Death did not occur in either case. Hic-
cough is not mentioned. The lesion in one case was evidently
left-sided; in the other, right-sided. In the second case glyco-
suria and albuminuria were present a short time. The intense
headache was attributed to the involvement of the fifth nerve.
The vesical symptoms in both cases were regarded as bulbar, they

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 190

were forcible and continued pressing before the stream could be
started after the necessity of urination was felt. The painful
priapism in one case was noteworthy (both patients were males).

Duret is said to have been the first to state that usually the
left vertebral artery is the seat of obstruction, although he had
only embolism in mind, but Senator in 1881 expressed the opin-
ion that the left vertebral artery seems more liable to thrombosis,
possibly because its course is more in the direction of the sub-
clavian artery, and also because it has a greater blood pressure.

Wallenberg^® traced the course of the injected posterior in-
ferior cerebellar artery. He found that frequently there is only
one posterior inferior cerebellar artery, and when that occurs it
is usually the left that is present. Several smaller arteries,
branches of the vertebral, take the place of the missing cerebellar
artery, but only for the medulla oblongata ; and a larger anterior
inferior cerebellar artery replaces the missing posterior cerebellar
artery. The absence of the right posterior inferior cerebellar
artery I have repeatedly noticed.

Wallenberg sums up Buret's investigations on the supply of
the vertebral arteries. Each of these arteries, the left the larger,
gives origin about 2 cm. below their union to the posterior infe-
rior cerebellar artery, and higher to the anterior spinal artery.
Branches from the latter artery enter the raphe and nourish the
interolivary bundles, posterior longitudinal bundles, hypoglossus
nuclei and other nuclei below the floor of the fourth ventricle.
The pyramids are nourished by the anterior spinal arteries and
frequently by the vertebral arteries. [It is understandable there-
fore that when the anterior spinal arteries are not occluded the
central and anterior parts of the medulla oblongata are not
affected.] Branches from the posterior inferior cerebellar or
vertebral artery supply the lateral portion of the medulla oblon-
gata. The posterior inferior cerebellar artery supplies the resti-
form body. These are all terminal arteries. According to Wal-
lenberg the posterior inferior cerebellar artery is given off 12 to
20 mm. below the union of the vertebral arteries. The free anas-
tomosis of the terminal portion of the posterior inferior cerebel-
lar artery explains the escape of the cerebellum in occlusion of
this artery. I have, however, observed an abscess in the terminal

191 WILLIAM G. SPILLER

distribution of this artery in the cerebellum, without any evidence
of implication of the medulla oblongata.

The study of Breuer and Marburg^' shows that clinically the
diagnosis as to whether the vertebral artery or the posterior infe-
rior cerebellar artery is occluded cannot be made with certainty,
as the anterior branches of the vertebral artery may supply much
the same area (lateral and posterior part of the medulla oblon-
gata) as does the posterior inferior cerebellar artery. Great
variations occur in the distribution of the blood vessels. Thus
in Breuer and Marburg's first case the vertebral artery was oc-
cluded and the posterior inferior cerebellar artery escaped, and
yet the lesion occupied about the same region as in those cases
in which the latter artery was thrombotic. Breuer and Marburg
state therefore that in the majority of cases the diagnosis can-
not be made more exactly than that of a lesion of the vertebral
artery. When symptoms indicative of pontile lesion occur, the
vertebral artery is probably occluded rather than the posterior
inferior cerebellar artery. Thrombosis of the vertebral artery,
however, may give a dififerent clinical picture from that of throm-
bosis of the posterior inferior cerebellar artery, in that it presents
certain additional symptoms. Especially is this true when the
thrombus extends into the basilar artery.

Eisenlohr^^ reported two cases, nos. 3 and 4, in 1879; in one
(case 3) the symptoms were suddenly developing right hemi-
plegia, without loss of consciousness, with disturbance of speech
and swallowing, complete paralysis of the tongue eight days later,
of the pharynx, complete loss of speech, paresis of the left facial
nerve, and partial paralysis of the larynx. In the other (case 4)
the symptoms were : Sudden paralysis of the right arm, paresis
of the right leg, disturbance of articulation and swallowing, com-
plete loss of speech a few days later, inability to swallow, and
paralysis of the larynx.

Eisenlohr mentions that Lichtheim regarded as diagnostic of
occlusion of a vertebral artery sudden loss of speech and of the
movements of the lips and tongue, paralysis of the larynx and
paralysis of the limbs of one side. In Reinhold's case of throm-
bosis of the vertebral artery hemiplegia occurred.

In my cases of thrombosis of the posterior inferior cerebellar
artery, and in most other cases with this lesion, hemiplegia, com-

• OCCLUSION OF INFERIOR CEREBELLAR ARTERY 192

plete loss of speech, and paralysis of the tongue were not symp-
toms.

In 1888 Eisenlohr reported the findings in his two cases. In
case 3 the vertebral arteries were narrowed and obstructed and
numerous branches of the basilar artery entering the pons were
obliterated. It seems uncertain whether either vertebral artery
was completely thrombotic, but it is so implied. In case 4 the
important lesion was old thrombosis of the left vertebral artery
with several small foci in the lower half of the pons.

In some of the papers it is distinctly stated that prodromal
symptoms preceded the apoplectic attack. Thus Senator's patient
felt badly for a few days before the apoplectic insult, and this
probably was caused by the beginning thrombosis, or at least
disturbance of the circulation. In the clearly marked clinical
picture weakness is- not great, but inability to walk may exist
because of the marked ataxia (Senator).

The ataxia may be caused by the lesion of the inferior cere-
bellar peduncle, or of the olivary cerebellar fibers, or of the lower
olive, or of the fibers of the vestibular nerve and its nucleus, or
of Deiters' nucleus. In some cases ataxia is very striking, thus
Senator's patient was obliged to go on all fours, as he could not
stand ; in my first case also standing at first was impossible, prob-
ably largely because of ataxia. The ataxia is usually on the side
of the lesion, and may be nearly confined to this side (Reinhold,
Hun, Breuer and Marburg, Thomas).

The thrombosis was found on the left side in the cases of
Senator (two cases), van Oordt, Wallenberg, Hun, Breuer and
Marburg (two cases) and Babinski and Nageotte ; and on the
right side in the cases of Reinhold, Ransohoff, and Thomas.

The diplopia mentioned in some of the cases is usually tem-
porary, and probably is caused by an interference with the func-
tion of the sixth nucleus. My second case shows that at least
the lower part of this nucleus may be implicated in the softening.
In Senator's second case the lesion extended to the lower part
of the nucleus. The abducens nerve was weak on the side of
the lesion in Henschen's clinical case.

Weakness of the face occurred in the cases of Breuer and
Marburg (case 2), and in my case 2 Degeneration of fibers
in the facial nerve explained the facial weakness in the second
case of Breuer and Marburg.

■)■;

193 WILLIAM G. S FILLER

Weakness of the tongue was observed in Breuer and ^lar-
burg's case 2.

Difficulty in swallowing probably exists in every case, and
probably is caused by implication of the nucleus ambiguus. The
paralysis is probably unilateral, but the loss of function is com-
plete (Reinhold, van Oordt, Wallenberg, Spiller, two cases). It
was complete in a case of unilateral fracture of the base of the
skull with paralysis of the vagus on only one side reported by
me.^^ The patient was entirely unable to swallow.

Vomiting in some cases is very distressing. Every attempt
to move Senator's patient produced it, and in my case i it was
very intense and could not be controlled.

Slowing of the pulse was observed by Wallenberg, and was
attributed by him to irritation of the vagus nucleus.

I have been unable to find any satisfactory explanation for
the loss of the tendon reflexes in some cases, and the exaggera-
tion in others ; but this variation in these reflexes occurs also in
cases of cerebellar tumor. The patellar reflexes were lost in the
cases of Senator, van Oordt (except feebly present on reinforce-
ment) and Spiller. In Hun's case the patellar reflex was normal
on the right side and a little exaggerated on the left side. Rosso-
limo-° mentions diminution of the patellar reflex and of other
tendon reflexes, unilateral or bilateral, in five cases of lesions of
the brain stem.

The disturbance of the sympathetic fibers in the face on the
side of the lesion is very common, and consists of narrowing of
the palpebral fissure, narrowing of the pupil, and retraction of
the eyeball. Disturbance of sweat secretion probably belongs to
this symptom group. All these symptoms are not present in
every case. They occur usually on the side of the lesion, and
therefore demonstrate that the sympathetic fibers entering the
lower part of the cervical cord do not decussate below the upper
part of the medulla oblongata. In Hun's case sweating was
increased in the face and hand opposite to the lesion. Vasomotor
symptoms in the face on the side of the lesion were observed by
Breuer and Marburg in their second case. Ptosis also has been
observed.

In both cases of Breuer and Marburg the left side of the soft
palate was more affected, but both sides were weak. In Hun's
case and in van Oordt's the paralysis of the soft palate was bilat-

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 194

eral, and this is explained by Breuer and Marburg as the result
of bilateral innervation from each nucleus. The nucleus ambig-
uus, they believe, controls the muscles of deglutition, the larynx
and the soft palate.

Disturbance of speech has occurred in most of the cases. It
is explained by the implication of the nucleus ambiguus, as this
nucleus usually forms the center of the lesion.

The nucleus ambiguus is probably the center for the laryngeal
muscles. Van Gehuchten and de Beule believed that it had no
connection with the larynx, inasmuch as they found degeneration
of the dorsal nucleus after section of the laryngeal nerves.
Kohnstamm and Wolfstein-^ have cut the recurrent laryngeal
nerve on one or both sides in rabbits and dogs, and have always
found the dorsal nucleus intact, but the nucleus ambiguus on the
side of the division was degenerated. They therefore regard
Van Gehuchten's views as incorrect.

Vertigo is not uncommon (Senator, van Oordt, Thomas,
Miiller). In Henschen's case the symptoms were those of
Meniere's disease — revolving vertigo, hallucinations of hearing,
and vomiting — and were explained as a result of lesion of the
vestibular nerve or of Deiters' nucleus, cochlear nerve, and vagus.

Subjective sensory disturbances are common. Senator's first
patient had a sensation of cold in the face on the side of the
lesion ; his second patient complained of paresthesia in the limbs
opposite to the lesion, and in the face on the side of the lesion.
Wallenberg's patient had pain in the eye and entire side of the
face, on the side of the lesion. In Hun's patient tingling was
felt spontaneously in the face on the side of the lesion, and in
the limbs opposite to the lesion following slight friction, i. e., in
the analgesic regions, and was evidently a symptom of irritation.
Pain was complained of in the face on the side of the lesion in
one of Thomas's two cases.

Deep sensation was lost in Hun's case on the side of the lesion,
about one month and then returned. The fillet was not found
degenerated at necropsy, but the transitory disturbance of the
deep sensation may have been due to transitory implication of
the fibers forming the fillet.

Sense of position was disturbed in the upper limb on the side
of the lesion in one case of Breuer and Marburg, and this they
explain by the involvement of the fibers from the nuclei of the

195 WILLIAM G. S FILLER

posterior columns, especially those from Burdach's nucleus, as
these are more exterior.

Tactile sensation usually escapes, but temperature and pain
sensations are likely to be diminished, or lost, usually in the face
on the side of the lesion, and in the limbs and trunk opposite to
the lesion (Senator, Hun, Breuer and Marburg). Tactile sensa-
tion may in some instances be impaired, and was so in the cases
of Senator. It is possible that the explanation for tactile anes-
thesia is to be found in implication of the fillet, as some investi-
gators believe that this fasciculus contains tactile fibers.

Hemianesthesia, partial in places, was present in the entire
side, including the face, opposite to the lesion, in van Oordt's
case; the tactile sensation was only slightly affected. The state-
ment he makes is " Hemianesthesia vom Scheitel bis zur Sohle."

In Wallenberg's case the diminution of pain and temperature
sensations in the face were on the side opposite to the lesion, but
only for a few days, and this was attributed by him to implication
of the central tract of the fifth nerve after the fibers had decus-
sated. In this case pain and temperature sensations were dimin-
ished also in the face on the side of the lesion in the first and
second branches of the fifth nerve, and this was explained by the
involvement of the spinal root of the fifth nerve on the side of
the lesion.

The central tract of the fifth nerve, according to Wallenberg,
is very near the median fillet in the medulla oblongata, and in
front of the hypoglossus nucleus. A lesion to involve this tract
must extend nearly to the raphe. The tract passes from the sub-
stantia gelatinosa dorso-medially, ventral to the hypoglossus nu-
cleus, across the raphe to the opposite side.

The pathology of the spinal cord has shown with certainty,
according to Mai, that a lesion of the spinal root causes complete
loss of sensation, whereas a lesion of the central tract causes dis-
sociated sensation. This statement, I think, is questionable.

According to Wallenberg and Schlesinger the first branch of
the fifth nerve descends lowest in the spinal root. A lesion cor-
responding to the lowest and middle thirds of the spinal root of
this nerve would give disturbance of sensation of the first and
second branches of the nerve.

Henschen," in reporting a clinical case with symptoms such
as are observed following occlusion of the posterior inferior

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 196

cerebellar artery, remarks that tactile fibers ascend in the poste-
rior columns and are next the raphe, therefore they usually escape
in these bulbar lesions. He says that he has demonstrated by
several cases that destruction of the fillet causes a loss of tactile
as well as of pain and temperature sensations, therefore the lat-
ter fibers enter the fillet more proximally than the medulla oblon-
gata. If ataxia is on one side and disturbance of pain and tem-
perature sensations is on the other, the lesion is more caudal than
when the disturbances are on the same side.

Henschen's patient had normal taste on the posterior part of
the tongue, but taste was much affected in the anterior two thirds
of the tongue. The entire distribution of the left sensory fifth
nerve was affected in his case, so that pain and temperature sen-
sations were lost in this area while tactile sensation was preserved.

Analgesia and thermanesthesia in the trunk and limbs of the
side opposite the lesion is explained by Mtiller by the crossing of
the spinal tracts for pain and temperature soon after their en-
trance into the posterior horns, and the ascent of these fibers in
the tractus spinotectalis and tractus spinothalamicus.

From his two cases Miiller concludes that the substantia gela-
tinosa must be regarded as a continuation anatomically of the
cervical posterior horn, its disturbance must give sensory symp-
toms of the posterior horn type, i. e., implication of temperature
and pain sensations. The central fibers of this trigeminal nucleus
must decussate soon, and the crossed tract would be implicated
in the lesions of Miiller's two cases. His second case showed the
Brown-Sequard symptom-complex in the distribution of the fifth
nerve ; deep sensation and tactile sensation were diminished in the
territory of the first branch on the side of the lesion, and pain
and temperature sensations were diminished on the opposite side.
This indicates, he thinks, that the fibers of deep sensation and
tactile sensation in the spinal root of the fifth nerve have their
central tract uncrossed in the medulla oblongata. The first
branch has been shown to be connected with the distal end of the
terminal nucleus, and this Miiller explains by the fact that in the
lower vertebrates the first branch (the forehead) does not extend
forward as far as the second and third branches. In the conus
the lowest roots supply the anal region, which in animals is pos-
terior to the limbs.

197

WILLIAM G. SPILLER

In case i of Kutner and Kramer the sensation of the neck was
not disturbed, and this escape is explained by the gradual cross-
ing of the sensory fibers from this region, so that they were not
all caught in the lesion. In occlusion of the posterior inferior
cerebellar artery there may therefore be a sensory zone intact
between the disturbed area of the face and that of the opposite
side of the body.

My two cases are as follows :

Case I. Douglas, male, was admitted to the Philadelphia

Fig. I. Case I. Showing the lowest level of the lesion A.

General Hospital, to my service, March 6, 1907. He died March
12, 1907.

Pathological Diagnosis. — Catarrhal and ulcerative enterocoli-
tis, hemorrhagic fibrinous peritonitis ; localized chronic adhesive
pleurisy, emphysema and edema of lungs ; dilatation of right
heart; chronic perisplenitis; fatty metamorphosis of liver; cloudy
swelling of kidneys ; chronic interstitial cystitis.

Notes taken on March 6 are as follows: Chief complaint:
Inability to swallow, weakness in left upper and lower limbs. He
had a chancre fifteen years ago. Alcohol and tobacco have been
used to excess. He has not felt well for three weeks, and
stopped work about a week ago, but returned to it again and

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 198

worked several davs. The bowels had not moved for a week;
he began last Sunday to take salts after having had his usual
cathartic dose on Saturday, and took large doses all day Sunday
and during the night. His bowels did not move until Monday
morning, when they acted repeatedly, and he had a severe chill.
At nine o'clock Monday morning he drank a glass of hot milk
and this is the last time' he remembers swallowing anything.

At eleven o'clock he tried to get out of bed, but found that
he was unable to stand or walk. He slid downstairs to the second
floor and called to the family and was carried back to bed. He

Fig. 2. Case x. Showing tlic middle level of the lesion A.

then found he was unable to drink the milk which was brought
him ; although he states that he noticed that he was unable t(?
swallow saliva when he first tried to get out of bed. Before he
got out of bed he felt dizzy, things swam around, he saw specks
before his eyes and then couldn't see at all, although he did not
lose consciousness. He had a spasm of the right arm followed
by numbness. At present he is unable to swallow anything. He
has numbness and some loss of power on the left side. He com-
plains of seeing double and has persistent hiccough. There is no
involvement of the bladder or rectum. He is not able to speak
above a whisper, although he articulates distinctly. The facial
muscles seem to be normal.

199 WILLIAM G. S FILLER

My examination was made ]\Iarch 8, 1907, and gave the fol-
lowing results : Ptosis of each eyelid is slight and equal on the
two sides. He wrinkles the forehead well on each side, closes
his eyelids, shows his teeth, draws up well either corner of the
mouth separately. The right pupil is a little larger than the left.
Reaction to light is present in both eyes but slow. Reaction is
prompt in convergence and accommodation. Movements of ex-
traocular muscles are normal. No double vision is noticed at
present. The masseter muscle contracts well on each side. In
opening the mouth the jaw does not deviate toward the right

jm/SB^Bk. >''-■' '^3^KbI^ -..j

^^^kV ' ^^^.^^IH

s^KSwv^ '-*jff^"

^sSS^P '^ ^^IH^^^I

^"^^BKHk, -'''^

nBH

H»

iB8c'¥^' ' - «-'^i^?%?''

'jmSQ9|

Hk^\

>,^^B»».'' " s. "I'lisiKfiSSL.

^^i^^HB

"tl

'^w — v^m^KIh

l^^mSk^'

im-^ '^nj^HIHS

K^flP^Bk

wit^mSBM.

IPI^^

\ IJM^^miJS^^Bm

w^iHyp^i

^^nMR/>^i

IPP- '

Fig. 3. Case i. Showing the highest level of the lesion A.

until the mouth is quite widely open, when the lower jaw goes
suddenly to the right, as though there were some defect in the
articulation of the lower jaw on the left side. The tongue may
deviate slightly to the right, but this is questionable.

Sensations of pin prick and touch are normal on the two
sides of the face. The soft palate when the man says " ah " is
drawn up more on the right side than on the left, this is distinct.
He is entirely unable to swallow. The nasal tube is passed with-
out difficulty to a distance of 62 cm. and there seems to be no
obstruction to it. He speaks distinctly, but merely in a whisper
and expectorates constantly. There is no deafness to the voice
and he hears a low ticking watch at a distance of six inches in

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 200

each ear. The ton^^ue while in the mouth deviates toward the
right, more to the right while in the month than when it is pro-
truded. The hiccoughing, while constantly present during the
observation, has a tendency to occur in attacks of increased sever-
ity. The grasp of each hand is good. Biceps tendon reflex and
triceps tendon reflex are present, but not very distinct on either
side. The movements of the upper limb are free on each side.
Sensations of touch and pain are normal in both upper limbs
and there seems to be no involvement of either upper limb. Re-
sistance to passive motion is equally good in either upper limb.
There is no wasting of the upper limbs. He has little or no tend-

FiG. 4. Case 2. Showing the lesion A. The section is reversed in the

photograph.

ency to cyanosis of the fingers at present, although Dr. Evans, the
resident physician, said the cyanosis was pronounced yesterday
morning.

There is no cardiac- murmur and no accentuation of the sec-
ond pulmonic sound. No distinct weakness of the lower limbs is
detected. It is very doubtful whether the right lower limb is
weaker than the left. The patellar tendon reflex is lost on each
side, even on reinforcement. Heel to knee test shows some ataxia
on each side. Achilles jerk is lost on each side. Sensations of
touch and pain are probably normal in each lower limb, although

201 WILLIAM G. S FILLER

sensation of pin prick may be somewhat diminished over the right
sole. The gait and station are somewhat ataxic. The ataxia is
especially pronounced when he is standing with his feet together
and eyes closed. He has never had any severe pains in his legs.
He says he did not stagger when he w^alked on the street.

I was unable to see the larynx on account of the epiglottis.
Dr. Grayson later made an examination, but the paralysis of the
epiglottis prevented him from seeing the vocal cords. He in-
tended to bring suitable instruments and make another attempt,
but did not succeed in doing so before the death of the patient.

Fig.

Case 2. Showing the lesion A.

Pulse is lOO at present. Unfortunately the temperature sen-
sation was not tested. Dr. Knipe reported on March 9, 1907:
O. D., pupil oval, 4 to 5 mm. ; O. S., pupil, 3 mm. ; both pupils
react faintly to light and convergence; O. D.. media clear, fundus
negative ; 6. S., media clear, disc has slightly pale yellowish cast,
but no haziness ; normal-sized vessels ; tension normal.

March 11, 1907: Pulse is 124. Hands and feet are very
cyanotic. The vomiting continued last night- and this morning ;
he is weaker. The radial pulse is weak on the right side and
is scarcelv detectable on the left side. The throat is not very sen-

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 202

sitive to manipulation. 11.30 P. M., pulse is imperceptible. He
died 1.45 A. M.. March 12, 1907. His pulse while he was in the
hospital ranged from 80 to 160, and frequently was as high as
120 to 130. Respiration 20 to 30 or 35; temperature on several
occasions reached loi or 102.

The softening with hemorrhagic infiltration is on the left side
of the medulla oblongata and extends to the descending root of
the glossopharyngeus. It implicates a large part of the restiform
body, the anterior part of the descending root of the eighth ne-ve,
a part of the spinal root of the fifth nerve, and the posterior part
of the lower olive. It does not extend quite to the periphery of
the medulla oblongata, nor does it implicate the lemniscus or
nucleus of the hypoglossus. The area of softening in its upper
part almost disappears at the level where the restiform body be-
gins to pass into the cerebellum, and in its lower part it is very
small at the upper part of the hypoglossus nucleus. The escape
of a portion of the sensory root of the fifth nerve, of the fillet,
and of the periphery of the medulla oblongata explains the pres-
ervation of sensation. The ease with which the nasal tube was
passed suggests anesthesia of the pharynx from implication of
the vagus and glossopharyngeal nerves.

The diseased area stains a light brown by the Weigert hema-
toxvlin method and contains here and there small recent hemor-
rhages. The intramedullary portion of the hypoglossus nerve is
intact. The nucleus ambiguus is in the degenerated area, and
some cells in the position of this nucleus are swollen and greatly
degenerated. The anterior pyramids are intact. The nucleus of
the hypoglossus on the affected side shows a few degenerated
cells, the hypoglossus nucleus of the opposite side is intact. A
slight perivascular round cell infiltration is seen here and there in
the medulla oblongata. The vessels in front of the pyramids are
not notably thickened, but probably are a little so. The pia shows
a slight round cell infiltration. Many of the axis cylinders in the
necrotic area are much swollen, and this area contains many fatty
granular cells.

The left ninth and tenth nerves do not show any degeneration
by the Marchi method. No change is detected in a piece of
muscle from the left vocal cord, even by the Marchi stain, nor
can any change be detected in muscle from the left side of the
soft palate by the Marchi stain. It is probable that the duration
of life after the lesion occurred was not sufficient for degenera-
tion to be detected even by the Marchi method.

The left vertebral artery is occluded by a thrombus and dis-
tended just below where it joins the basilar. The left posterior
inferior cerebellar artery shows a partly organized clot.

Case II. Dawson. Male, age 30 years, bartender, was ad-
mitted to the Philadelphia General Hospital, to my service, March
27, 1907. He died the same day.

203 WILLIAM G. SPILLER

Pathological Diagnosis. — Chronic catarrhal gastritis ; active
hyperemia of kidneys; edema and congestion of lungs; chronic
fibrous pleurisy; right omental hernia.

A history was not obtained from the patient, as he was un-
able to talk sufficiently, and lived but a few hours after he came
to the hospital. He came with an ambulance history of facial
paralysis. Duration of illness was one week.

March 27, 1907. Last Monday, three days previous to his
admission, just after the patient had arisen in the morning, he
felt weak and dizzy. As this feeling passed he found that he
was unable to swallow anything, even saliva, and that the left
side of the face was very weak ; that when he opened his mouth
it was drawn strongly to the right. Since then he has been un-
able to eat or drink and has taken no nourishment for three days.

He has ptosis of the left upper lid. When he shows his teeth
his mouth is drawn strongly to the right side. His tongue is well
protruded, but deviates apparently to the right, although this may
be due to his mouth being drawn so far to the right. His
pharynx is anesthetic, as is shown by passing the nasal tube with-
out causing him any annoyance. He is unable to swallow, the
fluid passes to a certain distance and then is coughed out. He is
able to speak only in a whisper and with much difficulty. His
upper and lower limbs on each side are apparently unaffected, as
are also the sphincters of the bladder and rectum. His mind is
clear, and he is a man of fair intelligence.

The lungs seem clear. He expectorates a considerable
amount of grayish mucus. Heart sounds are fairly good. Pulse
is 120. Respiration, 40; temperature, 97.

The area of degeneration extends from the level of the upper
part of the twelfth nucleus to that of the lower part of the sixth
nucleus, and implicates the posterior half of the fillet. A section
made at the lower part of the pons where it joins the medulla
oblongata shows that a large part of the posterior part of the
left side of the section is in recent degeneration. The sixth
nucleus is slightly implicated at its lowest portion. The necrotic
area stains faintly by the Weigert hematoxylin method. It ex-
tends to, but not beyond, the raphe in the pons, and almost to
the floor of the fourth ventricle. The restiform body is not im-
plicated, and the spinal root of the fifth nerve only slightly so.
The area extends to, but does not invade the lower olive ; but
implicates the nucleus of the facial nerve. The degenerated
area contains numerous small hemorrhages, and is like that seen
in the former case, although it is a little higher. The necrotic
area contains numerous swollen axis cylinders. The perivascu-
lar round cell infiltration is ver\' intense within the medulla oblon-
gata, as is also the round cell infiltration of the pia. The sections
present the appearance of syphilis. The basilar and left verte-
bral arteries are thrombotic, and both are partly occluded by pro-

OCCLUSION OF INFERIOR CEREBELLAR ARTERY 204

liferation of the iiitima. The left vertebral artery is much larger
than the right. It is doubtful whether the clot in the basilar
artery existed during life. The left posterior inferior cerebellar
artery is filled with red blood corpuscles which do not form a
distinct thrombus.

The head of the right caudate nucleus is in intense acute
degeneration and is filled with fatty granular cells.

BIBLIOGRAPHY.

1. Dumenil. Archives generales de Medecine, 1875, Vol. XXV, p. 392.

2. Leyden. Archiv fiir Psychiatric, Vol. VII, 1877, p. 44.

3. Senator. Archiv fiir Psychiatric, Vol. XI, 1881, p. 713.

4. Eisenlohr. Archiv fiir Psychiatric, Vol. IX, 1879, p. i, and Vol. XIX,

1888, p. 314.

5. Senator. Archiv fiir Psychiatric, Vol. XIV, 1883, p. 643.

6. Van Oordt. Deutsche Zeitschrift fiir Nervenheilkunde, Vol. VIII,

p. 183.

7. Reinhold. Deutsche Zeitschrift fiir Nervenheilkunde, Vol. V, 1894,

p. 351.

8. Wallenberg. Archiv fiir Psychiatric, Vol. XXXIV, 1901, p. 923, also

Vol. XXVII, 1895, p. 504.

9. Hun. New York Med. Journal. 1897. Vol. I.

10. Ransohoff. Deutsche Zeitschrift fiir Nervenheilkunde, Vol. XIV,

1899, p. 72.

11. Breuer and Marburg. Obersteiner's Arbeiten, Vol. IX, 1902, p. 181.

12. Babinski and Nageotte. Nouvelle Iconographie de la Salpetriere, 1902,

p. 492.

13. Mai. Archiv fiir Psychiatric, Vol. XXXVIII, No. i, p. 182.

14. Thomas. Journal of Nervous and Mental Disease, 1907, p. 48.

15. Miiller, Deutsche Zeitschrift fiir Nervenheilkunde, Vol. XXXI, Nos.

5 and 6, p. 452.

16. Wallenberg. Archiv fiir Psychiatric, Vol. XXVII, p. 504.

17. Breuer and Marburg. Obersteiner's Arbeiten, Vol. IX, 1902, p. 181.

18. Eisenlohr. Archiv fiir Psychiatric, Vol. XII.

19. Spiller. University of Penna. Med. Bull, March, 1903.

20. Rossolimo. Deutsche Zeitschrift fiir Nervenheilkunde, Vol. 23, p. 243.

21. Kohnstamm and Wolfstein. Journal fiir Psychologic und Neurologic,

1907, Vol. VIII.

22. Henschen. Neurologisches Ccntralblatt, June i. 1907, p. 502.

23. Kutner and Kramer. Archiv fiir Psychiatric, Vol. XLII, 1907, No.

3, p. 1002.

Reprinted from the University of Pennsylvania Medical Bulletin, May. 1908.

(From the Department of Neurology and the Laboratory of Neuropathology

of the University of Pennsylvania, and from the Ayer Clinical

Laboratory of the Pennsylvania Hospital.)

POLIENCEPHALITIS SUPERIOR, WITH REPORT OF A
CASE WITH AUTOPSY 1

By E. B. Krumbhaar, A.B.

CLASS OF 1908

In 1881 Wernicke- described a more or less clearly defined disease
of the central nervous system imder the name of poliencephalitis acuta
hsemorrhagica superior. As this name implies, it is an acute hemor-
rhagic condition attacking the gray matter of the crura cerebri or
mesencephalon. The adjective " superior " is used to designate the
form that attacks the region of the oculomotor nuclei in the floor of
the aqueduct of Sylvius, giving an external ophthalmoplegia or paral-
ysis of the external muscles of the eye. Poliencephalitis " mferior "
is reserved for a similar condition in the pons and medulla involving
the lower cranial nerves, while the poliencephalitis of Striimpell and
Leichtenstern attacks the cerebral hemispheres. In cases where the
spinal cord as well as the pons or crura is involved, the term " Polien-
cephalomyelitis " is used.

In 1902 Batten''' unfortunately proposed another nomenclature, in
which poliencephalitis superior referred to the cerebral form ; polien-
cephalitis inferior to both Wernccke's and the old inferior form com-
bined, that is, to any case in which the nuclei of the cranial nerves were
affected ; and poliomyelitis anterior were the anterior cornua below
the medulla were involved. In view of the facts that the old names
have become well recognized in the literature, and that the old polien-
cephalitis superior and inferior are clinically very distinct types, this
change in nomenclature seems ill-advised.

Wernicke's description was based on three cases of his own with
autopsies, and one previous case of Gayet's* with autopsy, which he
had called diffuse encephalitis. He pictured a disease, fatal in ten
to fourteen days, usually caused by excessive use of alcohol, although

^ The pathological study of this case was begun by the late Dr. H. S. Hutchin-
son and about half a dozen slides were prepared before his death last spring.
" See synopsis of cases.
^ Lancet, December 20, 1902, p. 1677.
■* See synopsis of cases.

1 193

2 krumbhaar: poliexcephalitis superior

one of his cases was due to sulphuric acid poisoning. He termed it
an " independent, inflammatory, acute, nuclear disease in the region
of the oculomotor nuclei. It is progressive, resulting in almost total
paralysis of the eye muscles, although the sphincter iridis and levator
palpebrffi are uninvolved." (Since then, it has been found that the
former is sometimes and the latter frequently involved.) Added to
this were certain general symptoms : a striking disturbance of con-
sciousness, showing a state of marked somnolence either from the
beginning or as a final symptom after a long stage of excitation. The
gait was reeling, a combination of stiffness and ataxia. There was
usuallv photophobia, vertigo, headache, and vomiting, and in all three
an optic neuritis. The autopsies showed capillary hemorrhages in
the floor of the third and fourth ventricles, with infiltration of round
cells about the hemorrhage, and dilated bloodvessels and capillaries.
Since then, as other cases were reported, somewhat varying condi-
tions have naturally been found. The rarity of the condition is shown
by the fact that only 27 reports of cases with autopsy can be found in
the literature on the subject. The following abstract from a case
which was in the service of Dr. Lewis and Dr. Longcope at the
Pennsylvania Hospital is. therefore, of interest, and is also of interest
because there has only been one other case reported due to tuberculosis.

Case. — Wm. INIcA., aged forty years, white, clerk, resident of Phila-
delphia; admitted July 18, 1906, complaining of drowsiness and
langour. Temperature, 98.3° ; pulse, 80; respiration, 20.

Family History. Father, mother, three brothers, two sisters all well.

Previous Medical History. Had whooping-cough and scarlatina as
a child. Then well until two years ago, when he had a fever (kind
unknown) that kept him in bed for three weeks.

Social History. Takes on an average one drink of whiskey in the
morning, and about five of beer during the day. Has been intoxicated
a number of times. Gonorrhea at nineteen. Xo other venereal trouble.

Present Illness. Has been languid for a year, but is now so dull
and drowsy that he does not know anything further about his con-
dition. His sister says that she does not know how long he has been
sick, but that when he visited her two weeks ago he was languid and
dull.

Physical Examination, July 19. Patient will answer when aroused,
l)ut his mind does not seem clear. Skin is dry, but no rash. Pupils
are contracted, and react but slightly to light. The right eye con-

194

krumbiiaar: poliencepiiamtis superior 3

verges, but the left turns out. IMarked nystagmus and photophobia
in both.

Breath foul and urinous. Tongue is pale and covered by moist,
yellowish, white fur, and under surface of tip is badly ulcerated.

Eye grounds show indistinct disks and congested veins. Ptosis of
both lids came on by evening.

(The rest of the routine physical examination on admission is
omitted for the sake of brevity.)

At 3 A.M.. the pulse was weaker, and the patient more restless.
Twelve ounces of blood were removed and two pints of normal salt
solution transfused. Patient got out of bed and fell down. Was
cyanotic and pulse very weak. Helped by stimulation.

During the morning he was stronger, but more comatose. Ptosis
was so great that he had Jo separate lids with his fingers, and com-
plained of dim vision and diplopia. Both internal recti seemed para-
lyzed. No headache or rigidity of neck. Considerable vertigo on
sitting up.

On the 22d the following notes were made by Dr. Longcope : There
is a weakness of all the muscles of the face, the eyelids, and the
muscles of the eyes (except the external recti). The tongue protrudes
slightly to the right. The neck is not stiff, although it was so last
night. No definite weakness of the upper extremities, but some of
the right lower limb. Reflexes in the upper limbs are rather exagger-
ated. Knee-jerks are absent. He has no Babinski sign; has faint
Kernig's sign on right. Patient can distinguish the number of fingers
shown, but not definitely.

July 23. Eyes, face, and tongue about the same. Speech very
thick. Apparently hears, but is very dull. Grip of hands weaker,
especially on right. Definite weakness in both upper extremities;
good motion in shoulders. Reflexes in both upper extremities less
than yesterday. Can hardly lift legs, especially the right one. Re-
flexes in lower extremities absent. No ankle clonus.

July 24. Condition about the same. During the evening pulse
became very rapid and weak, and temperature rose. The next morn-
ing patient died; temperature, 103°. The blood on July 20 showed
10,500 leukocytes, 86 per cent, hemoglobin. The temperature varied
between 97° and 102°, always coming down with hot pack amid free
sweating.

Autopsy (by Dr. Longcope) showed chronic diffuse peribronchial

195

4 krumbhaar: poliexcephalitis superior

tuberculosis of lungs. Tuberculosis of cerebral meninges, intestines,
kidneys, right testicle, seminal vesicles, prostate, and aorta. Cloudy
swelling of myocardium and kidneys.

Spinal cord appears normal, gray matter of pons slightly red.

The Brain. Dura delicate, non-adherent to pia. The latter is some-
what congested, with much edema over the posterior part of the
cortex; especially marked on the base, where there are a few small,
white and gray nodules. (X. B. When I received the tissues, I
was not able to find these, or to demonstrate histologically the presence
of tubercles in the meninges.)

From the brain and cord, sections were made from the sacral,
lumbar, dorsal, and cervical regions, the medulla oblongata, the pons
at three levels, crura cerebri, cerebellum, base of brain, third ventricle,
lateral ventricles at the region of the foramen of ]\Ionro. both para-
central lobules, and both optic tracts and optic disks. Of the cranial
nerves, the optic, trifacial, abducens, and facial of both sides were
studied. (X. B. The sections of brain and cord were stained in
hemalum-acid fuchsin, in thionin, and Weigert's hematoxylin. The
nerves were stained in Weigert only.) To avoid repetition, it might
here be stated that in no sections did the Weigert stain show any
degeneration of the myelin sheath. The ]\Iarchi stain was unavailable
as the tissues had been for over a year in formalin.

(N. B. The oculomotor, trochlear, and hypoglossal nerves could
not be studied, as they had already been removed by Dr. Hutchinson
in blocks of tissue that could not be found.)

Sacral Cord. The pia mater is somewhat thickened, but does not
contain many cells indicating the presence of a chronic inflammation.
The bloodvessels of the posterior roots are much engorged with blood ;
those of the posterior horns moderately so. The cells of the anterior
horn (in the thionin stain) are in only a fair state of preservation;
many stain diffiusely, some appear somewhat swollen, and a few show
more or less displacement of the nuclei toward the periphery. The
dendrites appear lessened in number.

Lumbar Cord. The bloodvessels are less engorged than in the
sacral region, but are still more so than normal. The meningeal
thickening is less distinct. The condition of the ganglion cells is
much the same as in the sacral region ; displacement of the nuclei is
more distinct and the cells are much pigmented. In the posterior
half of the sections are many small, round, homogeneous bodies, vary-

19G .

krumriiaar: poliencephalitis superior 5

ing from 20 to 80 microns in diameter. Their nature and staining
reactions will be considered later.

Dorsal Cord. The same conditions are present here, except that
the engorgement of the bloodvessels is much less marked. The
ganglion cells of the column of Clarke are uniformly pale staining,
with displaced nuclei ; the pigmentation, however, is less marked than
in other regions. This is not an uncommon finding in this group of
cells.

Cervical Cord. The same conditions are also present here, but to
a less extent; the vessels are but slightly engorged, the ganglion cells
are much more numerous, and take the stain better. The amount of
pigment, however, is still more than normal.

Medulla Oblongata. The pia mater shows some chronic thickening
throughout, and at the higher level there is also evidence of acute
inflammation. The meningeal vessels are engorged ; many red blood
cells and leukocytes have extravasated into the surrounding edematous
tissue. The medulla itself is apparently normal; there are no areas
of degeneration, no hemorrhage or engorgement of bloodvessels, the
ganglion cells of the posterior nucleus and nucleus ambiguus of the
tenth nerve are in a good state of preservation. Some of the cells
of the twelfth nuclei on both sides show distinct displacement of the
nuclei and vacuolization of the cell body.

Pons. The inflammatory process of the meninges is less marked
at the lowest level, and disappears at the others. The ganglion cells
of the sixth nuclei are in fairly good state of preservation, much better
than those of the cord. The sections are unfortunately too high to
include the facial nucleus.

At a slightly higher level the bloodvessels in the dorsal half, that
is, below the floor of the fourth ventricle, begin to be more and more
engorged with blood. About one or two capillaries in the locus
cceruleus are small recent punctate hemorrhages. The hyperemia con-
tinues, increasing in intensity, well up into the crura. ]\Iany of the
nerve cells of the pontile nuclei, particularly those in the neighborhood
of bloodvessels contain numerous black, opaque pigment bodies. This
condition will be considered more fully later (Fig. 3).

Crura Cerebri. Numerous small capillary hemorrhages (Fig. i)
are present in the gray matter surrounding the aqueduct of Sylvius.
These hemorrhages vary in size from about a millimeter in diameter
to merely containing forty or fifty red blood cells; they are usually

197

6 krumbhaar: poliencephalitis superior

arranged symmetricaly about a vessel, either artery or vein, and are
mostly situated close to the aqueduct. Some are found, however,
directly in the oculomotor nuclei and some even in the fibers ventral
to it. The hemorrhages are evidently recent; the red blood cells
retain their form and staining capacity, and there is no pigmentation
of the surrounding tissues. In the illustration there is a small area of

■f^

<% ■ .

^«i^

Fig. I. Capillary hemorrhage in the oculomotor nucleus, surrounded by
some necrotic tissue.

necrotic tissue immediately surrounding the arteriole from which the
hemorrhage occurred, which suggests a less recent origin of the
hemorrhage.

The bloodvessels in this region are much engorged ; and in a few
places there is a very slight round-cell infiltration.

The ganglion cells of the oculomotor nuclei present a different kind
of degeneration from those in the anterior horns of the spinal cord
(Fig. 2). The large cells of the lateral nuclei are few in number, and
take the thionin stain but faintly. There is a conspicuous absence
of pigmentation, and the Xissl bodies are mostly present, but are not

198

krUxAidiiaar: poliencephalitis superior (

concentrically arranged, are often clumped together and vary con-
siderably in size. In hardly any of the cells are dendritic processes
visible. On close inspection, with dim illumination, many very pale
remains of cells can be found; in most of them the nucleus and
necleolus is preserved, ])ut much of the cytoplasm has disappeared;
the remainder stains homogeneously, and often the periphery of one

Fig. 2. Degeneration of ganglion cells of oculomotor nucleus: a, slightly
degenerated ganglion cell, with some Nissl bodies remaining; h. old sclerotic
cefl whose nucleus and nucleolus have disappeared; c. much shrunken cells, in
advanced state of karyolysis; d e f, homogeneously staining cells, with partial
loss of Nissl bodies; g, very pale cells, beginning karyolysis; h. normal ganglion
cell from anterior horn of cervical cord; i, vacuolated ganglion cell from crus
cerebri.

side is jagged and irregular. The pericellular lymph spaces are en-
larged, another evidence of the loss of cell substance. In indentations
in the cells are often found two or three lymphocytes or epithelioid
cells lying, suggesting the jihenomenon of neuronophagia (or, as
Marinesco has recently termed it, " necrophagia ").

Differing from the lateral nucleus, where a certain proportion of
the cells remain almost normal, in the smaller celled median nucleus

199

8 krumbhaar: poliexcephalitis superior

the cells are more uniformly affected. The details of the process,
however, are practically the same.

The tissues, especially of the oculomotor region appear rarefied, so
that the network of the neuroglia substance is distended.

Cerebellum. Nothing abnormal found.

Wall of Third Ventricle. Small, punctate hemorrhages are present
in the' walls of the third ventricle, to the depth of a few millimeters,
and the bloodvessels of this region are much engorged. Some of the

-> .. . ^ '^ ^

^■■^ft^

■^-k

" '_'>

■■^

■"'■#t

:##

■^^-

^^ _ :: i^-:-'^

Fig. 3. Hematogenous pigmentation of pontile nerve cells, liiack pigment
lies free in the vessel at the top, and in tissue adjoining.

smaller vessels show a slight but distinct perivascular round-cell in-
filtration.

Walls of Lateral Ventricles. The walls of the lateral ventricles are
apparently normal. The bloodvessels contain about a normal amount
of blood.

Paracentral Lobules. The pia mater over the lobules of both sides
is of normal thickness, the vessels of the meninges and brain sub-
stance are not engorged. The cells of Retz are apparently normal,
although the Xissl bodies are not very clearly shown.

200

KRUMI5HAAR: POLIENCEPIIAIJTIS SUPERIOR 9

Optic Tracts. The optic tracts of both sides are apparently normal.

Cranial A'crz'cs. None of the nerves examined, the second, fifth,
sixth, and seventh of both sides, show any evidences of degeneration.

Optic Disks. The optic disks and distal ends of the optic nerves
are apparently normal. The bloodvessels do not appear congested.

Summary. The examination of tissues has therefore revealed a
slight chronic thickening of the pia mater at some levels of the cord;
a degeneration of the ganglion cells of the anterior horns, more marked
at the lower levels. Engorgment of the bloodvessels of the posterior
roots in the lower part of the cord, and below the floor of the fourth
ventricle in the pons, becoming more marked in the crura. In the
crura about the aqueduct of Sylvius, and in the wall of the third
ventricle numerous recent capillary hemorrhages. The ganglion cells
of the twelfth nucleus arg somewhat degenerated, those of the third
nucleus are much so, and few in number. Presence of numerous so-
called " amyloid " or " hyaline '' bodies in the cord. Perivascular
pigmentation of nerve cells in the pons. Slight perivascular round-cell
infiltration in the wall of the third ventricle and in crura cerebri.

The case, then, is evidently one of Wernicke's poliencephalitis, not
involving any cranial nuclei below the sixth. The pigmented nerve
cells in the pons might point to the absorption of former hemorrhages,
but in the absence of further evidence this cannot be accepted as
proved. The lesions in the anterior horn cells of the cord may be
either an expression of the toxemia accompanying the poliencephalitis,
or an independent condition upon which the acute hemorrhagic proc-
esses of the crura and cerebrum were superimposed. In the absence
of data as to the sequence of the clinical symptoms, this question also
cannot be decided.

Several points, however, are worth noting as dififering from the
ordinary type of the disease. As to its etiology, although chronic
alcoholism has been considered ever since Wernicke's original descrip-
tion as the most common cause, various other intoxications and in-
fections have been recognized. In fact, Gayet's, the earliest case on
record, followed acute sulphuric acid poisoning. In Oppenheim's^
exhaustive monograph on the subject, written in 1896, influenza,
chlorosis endocarditis, cerebrospinal meningitis, poisoning by gas, lead,
nicotine, sulphuric acid, and ptomaines, are all given as possible causes,
with several others as rare possibilities. Tuberculosis, however, is not

^ Nothnagel. Specielle Pathologic u. Therapie. Bd. ix, 2.

201

10 krumbhaar: poliencepHalitis superior

mentioned, and in only one case, that Luce/ reported in 1903, is tuber-
culosis assigned as the cause. In the present case, although there is
a history of moderate alcoholism, it is but fair to assume that general-
ized tuberculosis was the active cause.

As regards the morbid anatomy, the capillary hemorrhages occurred
in classical sites. In most cases, however, they have been of greater
extent, often macroscopic in size. Hyperemia has also been constantly
present, but here it is noteworthy that while the hyperemia was
marked from the upper areas of the crura to a level well down in
the pons, the hemorrhages, except in one or two spots in the locus
coeruleus, did not extend below the level of the third nuclei. The
sharp restriction of the hyperemia to the region near the floor of the
aqueduct and the fourth ventricle should also be noted. Perivascular
round-cell infiltration is said by Oppenheim to be always present, and
has been noted in the great majority of subsequent cases; but in the
present one it was only present in one or two small areas in the wall
of the third ventricle. Degeneration of the ganglion cells, as in the
present case, has been found in most cases, although by no means in
all. In many the nerve fibers were diseased or destroyed, but here
the Weigert stain showed no change. Both these degenerations prob-
ably have a direct relation to the duration of the process.

The duration of the present case is hard to determine. The history
was necessarily so incomplete on this point that the illness is only
known to have existed for two weeks, but it may have been present
for a year. Wernicke's original statement, that the condition was
fatal in ten to fourteen days, has since had to be abandoned, and now
it is recognized that cases may be fulminating or fatal in a few days ;
subacute, lasting some weeks ; chronic, lasting for months or years ;
or may recover partially or completely. In this connection Holden
and Collins'^ recent report of six cases in the Journal of the American
Medical Association, is of interest ; one was fatal in a few days, three
recovered completely, one subacute case recovered partially, and one
had continued a chronic course for seventeen years. It is also note-
worthy that excessive somnolence, a marked symptom in former cases,
was not observed in this series. Several cases of evident polien-
cephalitis, followed by recovery, have been reported (among others

' See synopsis of cases.
' See synopsis.

202

krumbiiaar: poliencephalitis superior 11

by Goldflam/ Bruns,- Guthrie,^ Boedeker,* and Wiener^) ; and, as
Holden and Collins point out, it is very probable that milder, non- fatal
cases are much more common than is generally suspected.

As to the symptomatology of the case, the somnolence and stupor
are quite characteristic. They often, however, are preceded by a period
of restlessness, which in this case may have occurred before the patient
came under observation. Headache, nausea, and vomiting, which
are not noted here, are also more properly symptoms of the onset.
Vertigo is nearly always present, and it is noteworthy that stiffness
of the neck (here present only during one night) may occur without
any associated meningitis.

The ocular symptoms correspond fairly well to the pathological
findings ; the oculomotor nucleus was generally involved, ptosis being
especially marked. The sixth nucleus was observed to be in good
condition, and the history states that the external recti were not para-
lyzed. Slight pupillary reaction to light, as in this case, was noted
by a few observers while Boedeker," Church,' and Holden^ report
cases with absolute failure to react to light. (It will be remembered
that Wernicke originally stated that the levator palpebr?e and sphincter
iridis were never involved.) Nystagmus and photophobia occur in the
great majority of cases.

Deviation of the tongue to the right suggests unilateral involvement
of the hypoglossal nucleus, but microscopically both nuclei were found
to be slightly involved.

Acording to Oppenheim, there is always a disturbance of gait, in-
secure, reeling, staggering. He believes this to be chiefly due to cere-
bellar incoordination, although weakness, ataxia, and tremor may also
have some influence. It could not be looked for in the present case,
as the disease was too far advanced to permit the patient to get out
of bed.

The tendon reflexes have been variously reported as normal, exag-
gerated, diminished, or absent, although in most cases they were found
to be exaggerated. Here the biceps reflexes were exaggerated when

"■ Neurolog. Centralbl, 1891, Nr. 6.

- Ibid., 1895, Nr. 22.

'Report of Soc. for Study of Dis. of Children. 1905.

^ Arch f. Psych, u. Nervcnkr., vol. xl, p. 304.

^ Prager med. Woch., 1895, Nr. 40.

" See synopsis of cases.

' Ibid.

203

12 krumbhaar: poliexcephalitis superior

first seen, later diminished. The patellar reflexes were absent in
every examination, ^^'eakness of the extremities, which is exceptional
in Wernicke's disease, is here probably due both to the terminal process
of the disease, and in the case of the lower extremities to the condition
of the anterior horn cells in the lower parts of the cord.

The numerous small, round, homogeneous bodies that appear at
various levels in the cord, especially in the posterior roots and posterior
columns, are probably of the same nature as Hamilton's^ " hyaline "
bodies, and not far separated from the corpora amylacea of the central
nervous system. Unlike the true corpora amylacea, they have no con-
centric striations, do not stain with osmic acid, and do not give blue
on staining with iodine and sulphuric acid. The iodine reaction, how-
ever, is often not demonstrable in corpora amylacea. They do. how-
ever, give a reddish violet color on being stained with methyl violet.
They also give some of the reactions of mucin ; in thionin they stain a
reddish violet, stain dark blue with methylene blue, and an orange-
yellow with safifranin. Thus far, the staining reactions coincide with
those of the bodies described by Hamilton ; unlike them, however, they
do not stain pale blue with Van Gieson's stain, but instead a pale
pinkish yellow. In Lugol's solution. Hamilton's stained the same color
as the surrounding tissue, and there a reddish brown ; in hematoxylin
and eosin Hamilton's stained blue ; in hemalum and acid f uchsin, these
stain a deep purplish violet.

Redlich- considers also as true corpora amylacea, bodies that are not
concentrically striated, and do not give the amyloid reaction. He
thinks that they come from senile retrogressive changes in the nuclei of
the neuroglia cells, and are normally found in spinal cords of indi-
viduals over forty years of age. Hamilton, however, considers the
bodies that she found (which were in a child aged five years) as prob-
ably the result of a coagulation of an exudate, either postmortem
change, or due to the hardening fluids. This was because she found
them in the bloodvessels and perivascular tissues ; and in regions where
there was edema, but no marked inflammatory changes. Where there
was degeneration of the neuroglia or of the white fibers, no hvaline
bodies were found. Virchow^ found them where neuroglia had proli-
ferated and nerve fibers atrophied : Lubarsch^ thought that they came

* See synopsis of cases.
'Jahrb. f. Psychiat., 1896, Bel. x.
'Cellular Path, (trans.), p. 319.
*Ergeb. d. allg. path. u. path, anat., 1894. Bd. i.

204

KRUMUriAAR: POLIENCEPHALITIS SUPERIOR 13

partially at least from mast cells and leukocytes ; Siegert' said that they
were concretions about degenerated nuclei.

In the present case, a man aged forty years, the bodies seem to be
most numerous in areas unaffected either by degeneration or inflam-
mation ; in hyperemic and hemorrhagic regions they are not present.
This detail, therefore, coincides with Redlich's theory that they may
be normally present after middle life, a view which is unquestionably
correct. The bodies he described, however, presented the same differ-
ences in staining reaction as did Hamilton's, so that they cannot be con-
sidered identical with those of the present case. In fact the bodies
here described do not correspond entirely with any classification
hitherto given.

In view, therefore, of the numerous slight differences that have been
found in the appearance and staining reactions of the coropra amy-
lacea of the central nervous system, it seems probable that they not
only may occur pathologically or normally after middle life, but may
have more than one source of origin, and that their appearance and
staining reactions may differ according to the extent that they have
departed from their original condition.

In the description of the sections from the pons, it was noted that
many of the nerve cells contained numerous deeply pigmented granules.
These cells are invariably in such close proximity to bloodvessels
(although not all the bloodvessels have such cells about them) that it
is highly probable that the pigmentation came from the bloodvessels.
Unlike, however, the pigment-bearing nerve cells described by Weber,-
and again by McCarthy,^ the reaction for iron could not be demon-
strated. Potassium ferrocyanide followed by acid alcohol or hydro-
chloric acid, both in various concentrations of solution and for dif-
ferent lengths of time (from a few minutes to three days), were tried
without producing any blue color in the granules, although the blood
cells in the neighboring vessels turned a faint blue. Also unlike the
cells described by Weber, the pigmented nerve cells are here easily
seen in unstained sections. The granules appear as minute, semi-
opaque, brownish-black bodies, crowding the cytoplasm of the cell, but
not obscuring the nucleus. Comparatively few are found free in the
tissues, except in the space between the vessel wall and the nearest
nerve cells, where they are fairly numerous. They are remarkably

^Virch. Arch., 1892, Bd. cxxix.

"Monatschr. f. Ps\ch. u. Neurol., 3, 1898.

'Contributions from the Pepper Laboratory, vol i, p. 107.

205

14 krumbhaar: poliencephalitis superior

resistant to staining- and dissolving reagents, appearing unchanged in
thionin, Weigert, Van Gieson, hemalnm acid fuchsin. They are un-
affected by immersion for five minutes in acetic, sulphuric, hydro-
chloric acids, weak or concentrated, by potassium hydrate or chloro-
form, and only disappear when immersed in acid alcohol for two or
three days. That these granules have their origin in the blood may
be assumed on account of the strict limitation of the process to the
neighborhood of bloodvessels. Whether their presence in the cells is
due to phagocytic action or to a passive infiltration it is impossible to
state definitely, although their comparative absence from the surround-
ing tissues points to a selective action on the part of the nerve cell.
It may be, however, that there was a general infiltration through the
tissues and that the pigment in the intercellular tissue had since been
removed bv fatty granular cells. Their freedom from iron, as shown
by the ferrocyanide test, cannot be considered as absolutely proved, as,
overlooking the possibility of error in technique, some of the processes
of preparation may have brought about changes that prevented the
necessary reaction from taking place.

In conclusion, I want to express my since thanks to Dr. Spiller, under
whose supervision the preparation of this report was undertaken.

The following cases, arranged chronologically, of poliencephalitis
superior hemorrhagica, with autopsy, have thus far been reported :

Gayet. Archiv. de Phys., 1875.

Wernicke. Lehrb. der Gehirn krankh., 1881, vol. ii, p. 223.

Kojewnikoff. Prog. Med., 1887, Nos. 36 and 2>7-

Thomsen. Arch. f. Psych., 1888, vol. xix, p. 185 (3 cases).

Rissler. Nordiskt. Med. Arch., 1888, vol. xx. (Poliencephalomyelitis,
second case of series of poliomyelitis cases.)

Eisenlohr. Deut. med. Woch., 1892, Nr. 47.

Goldscheider. Charite Ann., 1892, vol. xvii.

Reunert. Dent. Arch. f. klin. Med., 1892, vol. i, p. 222.

Jacobaeus. Deut. Zeit. f. Nervenk., 1894, vol. v.

Dinkier. Deut. Zeit. f. Nervenh., vol. vii. (Poliencephalitis inferior, ac-
companied by hemorrhages in the floor of the aqueduct of Sylvius.)

Boedeker. Arch. f. Psych., 1895, vol. xxvii.

Kaiser. Deut. Zeit. f. Nervenh., 1895, vol. vii, p. 359. (Poliencephalo-
myelitis.)

Kalischer. Deut. Zeit. f. Nervenh., 1895, vol. vi, p. 252. (Poliencephalo-
myelitis.)

Schiile. Arch. f. Psych., 1895, vol. xxvii, p. 295.

Hori and Schlesinger. Arb. a. d. Inst. f. Anat. u. Phys. d. Centralnerv.
(Obersteiner), 1896, vol. iv, p. 263.

Patrick. Jour. Nerv. and Ment. Dis., 1897.

206 .

krumbiiaar: poliencephalitis superior 15

Wilbrandt and Sacngcr. Die Neurol, d. Auges., 1899, vol. i, p. 270.
Oppenheim. Deut. Zeit. f. Nervenk., 1899, vol. vi.
Wijinhoff and Scheffer. Jahresb. f. Neurol, u. Psych., 1900, p. 443.
Sherman and Spiller. Phila. Med. Jour., March, 1900, p. 734- (Polienceph-
alomyelitis.)

Church. Jour. Nerv. and Ment. Dis., 1901, p. 303-

Zingerle. IMonatschr. f. Psych, u. Neurol., 1902, vol. xi, p. 177.

Hamilton. Jour. Med. Research, 1902, p. 11.

Luce. Neurol. Centralbl., 1903, p. 380.

Brissaud and Brecy. Revue de Neurol., 1904, vol. xii, p. 899.

Hunt. New York Med. Jour., February, 1906.

Holden and Collins. Jour. Amer. Med. Assoc, February, 1908, vol. 1, No. 7.

Extracted from the American Journal of the Medical Sciences, May, 1908.

(From the Department of Neurology and the Laboratory of Neuropathology of
the University of Pennsylvania.)

THE SYMPTOM COMPLEX OF TRANSVERSE LESION OF
THE SPINAL CORD AND ITS RELATION TO
STRUCTURAL CHANGES THEREIN

By Alfred Reginald Allen, M.D.

INSTRUCTOR IN NEUROLOGY AND IN NEUROPATHOLOGY IN THE UNIVERSITY OF
PENNSYLVANIA, PHILADELPHIA

Elsewhere^ I have discussed the pros and cons concerning lami-
nectomy in cases of fracture-dislocation of the spinal column. In
studying the literature on this subject one is struck with the absolute
impossibility of being able to determine definitely in many cases, within
any reasonable time, whether the spinal cord has been irrevocably
destroyed as a conductor of afferent and efferent impulses. Many in-
vestigators, chiefly surgeons, hold up the fact that a patient with a
fracture-dislocation showing symptoms of complete transverse lesion
occasionally will recover in the course of time, regaining a fair amount
of power and usefulness in the lower limbs. Upon such a history they
base their arguments as to the possibility of regeneration in the spinal
cord when it is badly crushed. The next step in such logic is, that if
such a patient can occasionally get well without laminectomy how many
who die without this surgical procedure would have lived had their
spinal canal been opened, clots and spicules of bone removed, and the
cord given a chance to bridge with functionating nervous tissue the
area of traumatic softening.

Now, let us keep before us this important fact: The neurons of the
central nervous system, when acted upon by toxic agents or physically
injured by factors either from without (concussion, fracture-disloca-
tion, etc.) or from within (pressure of tumors, exudates, etc.), al-
though structurally damaged, yet are able to exhibit, up to a certain
point, an autorestoration, provided the factor or factors causing this
condition have been removed or ameliorated.

Literature is not wanting in cases presenting symptoms of grave
transverse involvement of the spinal cord the histological examination
of which discloses little and insignificant structural changes. Gordon

^Injuries of the Spinal Cord, Jour. Amer. Med. Assoc, March 21, igo8.
1 208.

ALLEN : TRANSVERSE LESION OF SPINAL CORD 2

Holmes- reports 3 such cases which were the result of the collapse
of tuberculous vertebrae with consequent pressure on the spinal cord.
A fourth case in the same contribution was one of glioma of the pons.
The paraplegia and sensory disturbance were so extreme in the tuber-
culous cases that one would have been warranted in expecting to find a
practically complete destruction of the spinal cord at the level of the
bone lesion, with pronounced secondary ascending and descending de-
generation. In 2 of the cases the paraplegia was present for about
one year before death, in i case for five months. The microscopic
examination of the segments involved revealed but a very moderate
change, and above and below the site of the lesion there was no
secondary tract degeneration found by the Weigert or the March i
method. The fourth case presented at first a right hemiplegia, and
with the further growth of the neoplasm a complete paralysis in all
four extremities obtained for one month before death. Here, again,
the histological examination failed to discover any secondary degener-
ation.

Holmes argues that the loss of the medullary sheath at a given point
is liable to render the axis cylinder non-conducting, but does not neces-
sarily entail the secondary degeneration above or below, as the case
may be. Therefore, in such a case one might examine Weigert or
Marchi preparations functionally distal to the lesion, and find a normal
or almost normal condition of afifairs, whereas in reality the tract
at the point of injury would be minus the myelin sheaths and not a
conductor of impulses. He cites the findings in disseminated sclerosis
to support this view. In this disease there is a loss of myelin for a
short distance only, with preservation of the axis cylinders, and vet a
total break in the conducting ability of the fibers. In other words,
he infers that the myelin is necessary to conduction, that its removal
does not afifect the anatomical integrity of the axis cylinders or its
perikaryon. From these premises he draws the conclusion that repara-
tive process would be possible.

This reasoning of Holmes is all that could be desired, until he comes
to drawing the parallel between the non-conductivity of bare axis
cylinders and disseminated sclerosis. In disseminated sclerosis, al-
though a tract of axis cylinders bereft of their medullary sheaths
remains to the end without medullary cov^ering, the symptoms due to

" On the Relation between Loss of Function and Structural Change in Focal
Lesions of the Central Nervous System, with Special Reference to Secondary
Degeneration. Brain, 1906, iv, 514.

209

3 ALLEN : TRANSVERSE LESION OF SPINAL CORD

non-conduction of impulses in this area of the cord frequently pass
away and there is every evidence that the nerve fibers in this area
are functionating normally or almost so.

Schlagenhaufer^ reports a case in which an endothelioma of the dura
mater situated at the edge of the foramen magnum pressed upon and
grossly distorted the upper cervical segments of the spinal cord and
the medulla oblongata. The duration of this growth was one year
and four months, and before death the patient presented a complete
paralysis of all four extremities, as well as of the bladder and rectum.
Complete anesthesia of the four extremities and the trunk was also
present. Although there was pronounced change in the second and
third cervical segments (alle Stadien einer Compressionsmyelitis), yet
secondarv degeneration was conspicuous on account of its absence.

Bielschowsky,* in a painstaking investigation of axis cylinders when
involved in tumor formation or in compression of the spinal cord, also
thinks that they have the power to regenerate. His conclusions are in-
teresting when compared with those of Ballance and Stewart^ who
were the sponsors in large measure for the neurilemma-nuclei theory
of axis-cvlinder regeneration. Bielschowski found in the outer zone
of infiltrating tumors of the central nervous system, as well as in the
compressed level of the spinal cord, naked axis cylinders which had
been interrupted in their course by the pathological process, but were
still in unbroken communication with their perikaryons. At the ends
of these axis cylinders there were fine fibril-like prolongations ending
in a peculiar oval-shaped structure not unlike the terminal organ of
Held. On account of their oval shape and light centres he speaks of
them as Ende Ringe. He considers these long, fibrillar prolongations
the products of regeneration. The absence from the equation of
neurilemma nuclei is noteworthy, and the fine, newly formed blood-
vessels are supposed to exert the chemotactic influence and to prepare
the path for the newly formed nerve fiber which clings to its outer
surface, showing here the expanded end-bulbs.

Ballance and Stewart*' say : " The more the specimens are studied
the more is the conclusion forced upon the mind of the observer that

Ein intradurales Endotheliom im Bereiche der obersten Halssegmente, Ar-
beiten aus dem Neurologischen Institut, a. d. Weiner Universitiit. 1902. viii, <S8.

* Ueber das Verhalten der Achsenzylinder in Geschwiilsten des Nerven-
systems und in Kompressionsgebieten des Riickenmarks, Jour. f. Psychol, und
Neurol., 1906, vii, loi.

° The Healing of Nerves. ^Vlacmillan & Co., Ltd., 1901.

" Loc. cit., p. 89.

21a

ALLEN : TRANSVERSE LESION OF SPINAL CORD 4

for the regeneration of a peripheral nerve fiber (not only the axis
cylinder, but also the medullary and neurilemma sheaths) the activity
of one variety of cell, and one variety only, is responsible. That cell
is the neurilemma cell." These investigators further submit that the
reasons we do not have any regeneration in the axones of the central
nervous system is that in this locality there is an absence of neurilemma
neuclei.

Marinesco^ holds that there is no rcgcncrcsccncc autogcne in the
sense that there occur islets of new-forming axis cylinders and medul-
lary sheaths in the distal part of a divided nerve. He thinks that the
new nerve fibers in the distal part are prolongations from growing
tissue in the proximal end. In speaking of experimental cases in
which nerves were divided and their proximal portions removed, micro-
scopic examination later demonstrating newly formed fibers in the
distal portion, he evidently cToes not consider that he weakens his theory
by naively stating: Dc parcils cas sorit d'aiifant pins difficiles a inter-
preter que Ics cellules d'origine dii bout arrachc sont complcternent
atrophies.

The raison d'etre of this seeming digression from the title of this
paper lies in the presentment of the leading thoughts on the subject
of nerve regeneration. It makes a considerable difference in the way
we view trauma and tumor involvement of the central nervous system
if we accept the opinion of Bielchowski. As I have said, we have to
admit a capability on the part of the central neuron of autorestoration
(not the regenerescence autogene which Marinesesco denies) ; likewise,
it seems to me that it is axiomatic that the nerve cells and their
processes, axis cylinders as well as dendrites, in the central nervous
system must be amenable to the same laws of wear and tear and death
that govern cells elsewhere in the body. The large, pyrimidal cells of
the motor cortex, for example, are limited in their length of life, and
when their usefulness is past, phagocytic action removes them and their
work is assumed by others. Such a theory presupposes a constant ap-
pearance of new neurons. The supply of the perikaryon might be
from the development of the many small and insignificant somato-
chrome cells with which the gray matter throughout the central nervous
system is studded. But the axis cylinder cannot be so easily accounted
for unless we accept the idea of the growth from the cell body. The
evidence seems to point to the fact that a limited amount of regenera-

' Etude sur le mecanisme de la regenerescence des fibres nerveuses des nerfs
periphriques, Jour. f. Psychol, nnd Neurol., 1906, vii, 140.

211

5 ALLEN : TRANSVERSE LESION OF SPINAL CORD

tion in the central nervous system is possible after trauma under favor-
able conditions. But this regeneration is too insignificant to be of
functional importance.

Through Dr. Spiller's kindness I have been enabled to study the
spinal cord of a case which was in his service in the Philadelphia Gen-
eral Hospital, and which bears interestingly on the question of slight
structural changes accompanied by grave symptoms. The case is one
of II embodied in a paper by Spiller and Weisenburg.*

The patient, a woman, aged about forty years, ten months after the
removal of her left breast for carcinoma, began to suffer from radiat-
ing pains across her chest and back, as well as in the lower limbs.
One month after the onset of these symptoms she suddenly became
paralyzed in her lower limbs, and was confined to her bed. She com-
plained of a girdle sensation, but did not suffer extreme pain. The
upper limbs were not involved. There was no motion whatever in
her lower limbs, which were the seat of flexor contractures, the thighs
on the abdomen, the legs on the thighs. The patellar and Achilles
tendon jerks could not be elicted. Babinski reflex was present on
both sides. Sensation for touch and pain was lost in the lower limbs
and trunk up to a line three inches below the umbilicus. Occasionally
pin prick would be recognized on the dorsum of each foot. Incon-
tinuence of urine and feces was present. She died almost two months
after the onset of her paraplegia, and therefore sufficient time had been
given for secondary degeneration. The diagnosis of vertebral car-
cinoma in the thoracic region was ratified at the necropsy.

In studying the spinal cord microscopically, I expected to find very
w^ell-marked degeneration and wholesale destruction of tissue through
pressure. Sections were made at intervals from the third thoracic
segment down to and including the first lumbar segment and stained
by the Weigert hematoxylin as well as the hemalum-acid fuchsin
methods. To the unaided eye there was none of that yellowish color
in the Weigert sections which betokens degeneration. Under the
microscope there were seen comparatively few swollen axis cylinders
and swollen medullary sheaths in the lateral and posterior columns.
There were also found here and there a few spaces from which it
appeared that axis cylinders had dropped. There was a moderate
number of granular cells. The chief point of interest is that the spinal

* Carcinoma of the Nervous System and the Report of Eleven Cases, Joiir.
Nerv. and Ment. I)is., August, 1906.

212

ALLEN : TRANSVERSE LESION OF SPINAL CORD O

cord which appeared so Httle altered even microscopically had in reality
ceased to be a conducting mechanism.

I think that we are justified in assuming that had this condition
been caused by a fracture-dislocation instead of a rapidly increasing
malignant growth, the patient, in course of time, would have shown
some return of function, which would have been ascribed by certain
observers to regeneration after complete transverse lesion.

It is, indeed, remarkable that the spinal cord can so completely
suspend its function, at the same time presenting so slight evidence
of organic change. And it should make us particularly careful in
giving a positively bad prognosis as to return of function in cases
which have presented for some months symptoms of complete trans-
verse lesion.

213

A CASE OF APRAXIA, WITH AUTOPSY

By John H. W. Rhein, M. D.
neurologist to the howard hospital; physician to the philadelphia

HOME FOR INCURABLES, ETC.

(From the Philadelphia Home for Incurables, and the Department of

Neurology and the Laboratory of Neuropathology of the

University of Pennsylvania.)

Until comparatively recently, by apraxia was meant the in-
ability to use an object because its nature, or its use, was not
recognized (KiJssmahl and Starr (48)). Since Liepmann's ex-
haustive studies on the subject, however, apraxia takes on a dif-
ferent signification. It is a condition entirely independent of
agnosia, or the failure to recognize an object.

According to Liepmann's (23, 24) quite recent opinion, the
apraxic recognizes the object and its use, is able to move the
limbs readily, indicating that the innervation of each limb is in-
tact ; but purposeful movements by the affected limbs are impos-
sible. This, at least, is the definition for pure motor apraxia.

The description of apraxia by Pick (40), Van der Vloet (44),
Margulies (28), and von Monakow (34), and the views held by
Marie (29), especially, are not entirely in accord with this defi-
nition. Psychical disturbances in apraxics ; the appearance of
apraxia in certain forms of insanity ; the element of inattention
and lack of concentration so often observed in the apraxic subject,
all suggest the possibility of the presence of other elements in
the phenomena of apraxia, than are included in Liepmann's defi-
nition.

The whole subject is a difficult and complicated one, and the
following case, while exhibiting certain symptoms in common
with those of some of the reported cases, is generally unlike
any case in the literature at my disposal.

The patient, J. C. G., aged 55 years, married ; by occupa-
tion a railroad builder, and one-time member of the Canadian
Parliament, was admitted to the Philadelphia Home for Incur-
ables, July, 1906, complaining of loss of vision.

The family history was entirely negative. There was no pre-
vious history of illness, excepting the ordinary diseases of child-
hood. He had always worked hard ; had been exposed to the

23 JOHN H. W. RHEIN

elements as a young man, frequently sleeping out at night; and
had always been a moderate, and at times excessive, user of alco-
hol. There was no history of specific disease.

His present disease began with failing vision, about three
years before his admission to the Home. At the end of two years
It had progressed so that he was unable to read.

Dr. David Webster and Dr. J. F. Terriberry (52) reported
this case before the Xew York Ophthalmological Society in 1906.

Dr. \\"ebster stated that the patient came under his observa-
tion August 28, 1905, at which time there was little more than per-
ception of light. When the hand was held before the eyes he
knew there was something there, but did not recognize it. He
was unable to count fingers at any distance, or in any position,
but he did not run into objects when walking about the ward.
Dr. Webster believed that he actually saw objects, but could not
distinguish their form ; in other words, that he was suffering from
" visual astereognosis." At that time the ophthalmological ex-
amination revealed no cause for the blindness.

Dr. Terriberry reported upon the neurological conditions at
the same meeting. At this time the patient was unable to locate
touch, which was, however, perfectly perceived. His sense of
motion \\*as also disturbed, and he was unable to execute any
movement correctly. The muscular sense w^as absent in all the
extremities. There was no disturbance of the sense of heat and
cold ; and taste, smell and hearing were normal. There was no
evidence of palsy.

The vision continued to fail until, at the time of his admission
to the Home, he was unable to distinguish objects, and the blind-
ness appeared to be absolute, as he denied seeing the hand, or a
flame, when it was held before his eyes.

He had had no difficulty in using his arms apparently, as he al-
ways dressed and fed himself, until some time in 1904 (over a year
before admission) when his wife noticed that, if she took his left
hand to lead him, he did not recognize that she held it. He gradu-
ally gave up the use of the left hand on the balusters in going
up and down stairs, and in eating and dressing.

Present Condition : Vision. — Dr. William Campbell Posey
examined his eyes shortly after his admission, with the following
results :

" Eyes appear fixed and expressionless, the patient rotating
them but rarely, in marked contrast to the searching, rolling move-
ments so frequently observed in the blind. Winking is performed
rather less frequently than is normal (Stelwag sign). When re-
quested to regard an object, the patient stares straight ahead, even
though the object toward which he is told to direct his gaze is at
the side, and the width' of the palpebral fissures is somewhat in-
creased on both sides (Dalrymple sign).

" There are no palsies of the extraocular muscles, although

A CASE OF APRAXIA, WITH AUTOPSY 24

it is difficult to make the patient turn his eyes in the direction re-
quired. Both corneas are clear, and the eyes are free from any
traces of recent or old inflammation. Both pupils are 5 mm. in
size, and are quite active to direct and consensual light stimuli.
This reaction is obtainable in diffuse sunlight, and to a much less
degree from the light of an ophthalmoscopic mirror. The
Wernicke pupillary inaction sign was searched for, but was not
present, although the pupils reacted more readily to stimulation of
certain parts of the retina than others ; but these reactions were
not from symmetrical portions of the retina, and were probably
dependent upon a varying degree of atrophy of the optic nerves,
and also upon the impossibility of always confining the light
stimuli to definite and circumscribed retinal regions.

" The media are clear, and the optic nerves exhibit the early
changes of a simple atrophy. As yet, however, there is no true
atrophy, the nerves being dull red-gray, and possessing the appear-
ance so frequently seen in men in middle life who have abused
tobacco and alcohol, or who have cerebrospinal sclerosis. The
blood currents are also" well maintained, and the retina seems well
nourished, and there is not sufficient ophthalmoscopic cause for
the total blindness which is apparently present.

" Loss of vision appears absolute, as the patient denies seeing
the hand when it is held close before his eyes, or even a flame,
and that the blindness is not feigned is evidenced by the fact that
it is not possible to obtain compensatory movements of the eye-
balls by the use of prisms. On account of the absence of total
atrophy, the apparent preservation of the functioning power of the
retina, and the undoubted active reaction of the pupils to light
stimulus, it would appear that the cause of the blindness is cen-
tral, and not peripheral, and that the lesion producing it must be
posterior to the center which regulates pupillary movements."

Sensory Changes : Sense of Position. — The patient was unable
to tell whether he was standing or sitting, although occasionally,
when this symptom was tested, he answered correctly. He failed
to recognize the position of any of his limbs, or that they were
being moved.

Location. — He was incapable of locating, anywhere about his
body, the prick of a pinpoint, light or deep pressure, or heat or
cold.

Sensation. — Tactile sensation appeared to be lost in the left
hand, but was present in the right hand, and elsewhere about the
body.

Pain Sense. — While examinations of the pain sense were rather
unsatisfactory, it was probably preserved. Pricks with the sharp
points of the esthesiometer generally were followed by an ex-
pression of irritation. The right hand was withdrawn, but the
left hand made no movement whatever.

Temperature Sense. — He confused heat and cold on the left

25 JOHN H. W. RHEIN

hand, but elsewhere about the body the temperature sense seemed
to be unimpaired.

Pressure Sense. — He was unable to distinguish between Hght
and deep pressure anywhere.

Stereo gnostic Sense. — He was totally unable to recognize ob-
jects by the sense of touch. He recognized no object placed in
either hand.

Motor Symptoms : The general muscular power seemed to be
fairly good. The dynamometer registered 40 on the right, and
30 on the left. (He was right-handed.) There was no palsy of
any of the limbs, and he was able to walk, although his gait was
peculiar and will be described later.

Apraxia : He presented a few motor manifestations which, in
all probability, w^ere the result of apraxia, although this conclusion
must be somewhat guarded, as the complete loss of the sense of
location of the limbs and the body generally, the complete failure
to recognize objects by the sense of touch, and the loss of the sense
of movement, complicated the study of these symptoms. An-
other symptom which still further complicated the study of the
case, was the behavior of the left arm. It was held in a slightly
flexed position, the fingers semi-flexed most of the time, and
rarely or never was it moved voluntarily. This was not due
to paralysis, as the grasp was fairly good, and at other times the
arm was observed to move freely, apparently without any sign
of paralysis (Seelenlahmung).

Left Hand. — When asked to place to his ear a watch held in
the left hand, no movement whatever of the left arm followed,
but instead the right arm was stretched forward, and the right
hand grasped either his knee or his coat, while at the same time
he believed that he was placing his left hand to his ear.

When asked to squeeze the hand of the examiner with his
left hand he was successful at times, but more often distorted,
or irregular movements of the right arm followed, or he grasped
some portion of his body with the right hand, and squeezed
violently.

When a fork was placed in his left hand and he was re-
quested to feed himself, the left arm remained motionless, or
moved slightly only.

When told to touch his right hand with the left hand, only
a slight movement in the left hand resulted, while the right hand
groped aimlessly, as before described. Or, he would rise to his
feet, without moving either hand, and believed he was making
an effort to obey the command.

On the other hand, on several occasions he was able to take
from his trousers pocket a handkerchief and wipe his nose with
it voluntarily or when requested to do so, with either hand (reflex
movement).

Right Hand. — The right hand was also the seat of disturb-

A CASE OF APRAXIA. WITH AUTOPSY 26

ances of volitional acts. When a watch was placed in his right
hand, and he was told to carry it to his ear, while at times this
was successfully accomplished, he more often carried it to his
mouth, and went through the motions of chewing ; and this, in
spite of the fact that he recognized perfectly that it was a watch
that he was to place to his ear.

In feeding himself a banana he invariably put the banana to
his chin instead of his mouth, if unassisted. In the absence of
ataxia this is interpreted as being an apraxic movement.

He was unable to correctly use the hand for the purpose of
dressing or eating.

In masticating his food the bolus was not moved around
the buccal cavity in the usual manner, the jaw being moved rap-
idly up and down, and the food was often retained in the mouth
a long time before he swallowed it.

When asked to grasp the hand of the examiner with his left
hand, he occasionally succeeded, and at such times he would not
relax his hold and apparently involuntarily, in fact, grasping
more and more tightly, as long as the hand of the examiner
remained within his grasp. This was undoubtedly a manifesta-
tion of perseveration, of the tonic variety described by Liepmann.

Gait. — The gait was peculiar. Short steps were taken, usu-
ally to one or the other side, rather than forward. From this
he never varied, even when every precaution was taken to show
him that there was no danger of his bumping into anything ; in
other words, to eliminate the influence of the loss of vision.

Writing. — When a pencil was placed in his right hand, and
he was asked to write his name he moved the pencil up and down,
from right to left, without forming any letters. He was entirely
unable to make any movements when the pencil was placed in his
left hand.

Mental Condition. — His intellect seemed to be fairly good.
His memory was excellent and he discussed general matters
cleverly and intelligently, apparently understanding everything
that was said to him. He was, however, impatient, irritable and
unreasonable, so that, unfortunately, the examination could not
be carried out in the desired detail.

Reflexes. — The tendon reflexes of the arms were present on
both sides. There was no Babinski sign or ankle clonus. The
plantar reflex was exaggerated on both sides. The patellar re-
flexes were present, and probably exaggerated, but the rigidity of
the limbs prevented much movement of the legs on the thighs.

Station. — With the feet together he stood perfectly.

Contractures. — The left hand was held in a slightly contracteu
position, as above indicated ; that is to say, partial flexion of the
forearm on the arm, and semi-flexion of the fingers. This could
be passively overcome almost entirely, but there developed at the
same time a tremor of both arms, especially of the left. There

27 JOHN H. W. RHEIN

was also a slight but distinct rigidity of both legs and the right
arm, but to a less extent than in the left arm.

Examination of the ears, made by the late Dr. W. G. B.
Harland, gave the following results:

" Cerumen in both ears, but causing him no discomfort, and
I doubt that removal of the masses would change the findings
much. A more important source of error is found in the unre-
liability of his statement. We can say positively that he hears
plainly the ordinary voice, but cannot hear higher tones, this
defect being more noticeable on the left side. Deafness for low
tones is also w^orse in the' left ear, as shown by the tuning forks.

'' He does not know that he is deaf, nor does he suffer from
tinnitus. The results of the examination are what we might
expect in an old man with a mild degree of middle ear disease,
with secondary nerve involvement."

The tongue was protruded straight on command, and in the
median line. It was tremulous and tooth-indented.

The left hand was swollen, and the left forearm was gener-
ally w-asted slightly.

A diagnosis of bilateral cysts in the occipito-parietal region
was made, an exploratory operation advised, and on Feb-
ruary I, 1907, Dr. Edward Martin opened the skull in the right
occipito-parietal region. The pia was much thickened, and what
appeared to be an area of softening was found. The patient
made a good recovery from the operation, but the symptoms
remained unchanged.

On April 4, 1907, efforts to overcome the contracture of the
left arm and hand appeared to give pain. The left arm was very
tremulous, and the rigidity generally seemed to have increased.
His mentality appeared to have suffered, and it was impossible to
make any satisfactory tests of his condition on account of his
extreme irritability.

On May 10, 1907, the contractures of the left hand had in-
creased, and the fingers were pressed forcibly into the palm of
the hand. There was a Babinski sign on the left side at this date.
There was no facial asymmetry. He was confused and violent,
and his passages were involuntary.

There was at no time any vomiting, headache, or pain else-
where in his body.

At this examination his sense of taste was markedly altered.
He could not tell salty foods from sweet, and could not tell the
consistency of food when it was placed in his mouth.

He gradually failed mentally, became at times excited, refused
food, and died of exhaustion.

Autopsy. — At the autopsy there was an intense internal pachy-
meningitis on the right side, which was not observed at the time
of the operation. The inner surface of the dura was covered
with a thick plastic exudate, extending over the entire surfare

A CASE OF APRAXIA, WITH AUTOPSY

of the brain on the right side. The dura was everywhere adherent
to the skull, but was readily dissected away, with slightly more
difficulty over the site of the operation. The bony plate which had
been removed at the time of the operation and replaced was de-
pressed about 3 millimeters below the level of the skull. Bony
union seemed to have occurred in places, but there were two areas
at the site of trephining where no closure by bone had taken place.

A necrotic area was observed on the right side of the brain,
involving the cortex in the region of the occipito-parietal fissure,
extending 3 centimeters in front of, and 15 millimeters behind
this fissure, and laterally 45 millimeters from the longitudinal
fissure. A cross-section in this area showed that the cortical layer
was very narrow — about one millimeter in thickness. In the right
occipital lobe the cortex was not half the normal thickness. On
the left side it was macroscopically apparently normal.

The brain was first placed in formalin, and then in MiJller's

Fig. I. Showing degeneration of white matter in occipital lobe and of
the posterior part of the corpus callosum (right side).

fluid. It was divided horizontally into six approximately equal
portions, and these were blocked and cut in serial sections, and
many of them stained by the Weigert hematoxylin method.

Right Side. — The uppermost levels showed degeneration of
the fibers from the cortex anteriorly and posteriorly to the
occipito-parietal fissure. In this region there was some necrosis
of the cortex and subcortical substance, the section showing slight
loss of tissue, particularly just subcortical.

In sections from a slightly lower level the degeneration was
more apparent, and extended further posteriorly, although not
entirely to the extreme posterior pole. There was no degenera-
tion of the white matter on the median aspect. With a two
thirds objective there were seen much perivascular distension, and
some round-cell infiltration, in the degenerated area, and these
changes were less where the degeneration of the fibers was less
intense.

JOHN H. W. RHEIN

At a still lower level the degeneration extended forward into
the ascending- parietal convolution, and also to the posterior limit
of the convexity.

Still lower, the ascending parietal convolution was slightl}^
involved in its posterior aspect. The degeneration began just
posterior to the interparietal fissure, and extended to the occipital
pole. The median portion of the occipital lobe was not involved.

In sections from block 4 the white matter just posterior to
the ventricle was degenerated, as well as the external surface of
the occipital region, extending to the occipital pole, although
here it was less intense. The fibers coming from the calcarine
region at this level stained well, as did also the inferior longitu-
dinal fasciculus, although it was smaller than in the normal
brain. At this level the posterior part of the corpus callosum

Fig. 2. Showing degeneration of the white matter of the occipital
and second temporal convolutions of the optic radiations and the inferior
longitudinal bundle (right side).

stained less distinctly than normally. The posterior horn of the
lateral ventricle at this level was markedly dilated.

In sections still lower from this block the same condition
existed, except that the degeneration extended to the inferior
parietal lobe. The inferior longitudinal fasciculus, which was
much smaller than normal, was degenerated at this level. The
optic radiations and the tapetum were also degenerated. The
median surface stained normally.

In sections from block 5 there was, in addition to the condi-
tion just described, involvement of the second temporal convolu-
tion to a slight extent, in its posterior portion. The splenium
stained poorly, while the tapetum and inferior longitudinal fasci-
culus appeared to be smaller than normal. The forceps was
smaller than normal, and the optic radiations were degenerated.
The median surface was normal.

In sections from block 6 there was a small area of degenera-

■ A CASE OF APRAXIA. WITH AUTOPSY 30

tion in front of the anterior occipital fissure of Wernicke. The
optic radiations of Gratiolet, the tapetuni, and the splenium were
all degenerated.

In the lowest sections that were cut and stained the degenera-
tion of the white matter did not extend in front of the inter-
occipital fissure. The inferior longitudinal fasciculus was nor-
mal. The internal capsule and basal ganglia showed no abnor-
mality, and the pons, at the level of the red nucleus, which was

Fig. 3. Showing degeneration of the white matter in the occipital lobe
posterior to the interoccipital fissure (right side).

included in the lower sections, stained normally. The foot of
the peduncle showed no degeneration. The zone of Wernicke
was not degenerated and the pulvinar and geniculate bodies
appeared to be normal.

Left Side. — Sections from the uppermost levels (blocks i
and 2) showed only a very slight degeneration in the occipital
region. There was also some slight perivascular distension, and
infiltration of round cells.

Fig. 4. Showing degeneration in the posterior ventricular region,
external portion of occipital lobe, inferior longitudinal fasciculus, corpus
callosum, and optic radiations (left side).

31 JOHN H. IV. RHEIN

In sections from block 3 the degeneration was very slight,
and situated in the external portion of the occipital region. De-
generated fibers could be traced from this region towards the
corpus callosum. The inferior longitudinal fasciculus stained
normally.

Sections from block 4 were degenerated in the posterior ven-
tricular region. The degeneration in the occipital lobe at the
conve-xity, while apparent, was slight. The median surface was
normal. The degeneration did not extend into the parietal re-
gion. At this level the optic radiations, and the inferior longi-
tudinal fasciculus, were slightly degenerated, and the posterior
portion of the corpus callosum markedly so.

In sections from block 5 the degeneration was still apparent
in the external portion of the occipital lobe. The tapetum and
inferior longitudinal fasciculus were smaller than normal, but
stained well, and the posterior horn of the ventricle was dilated.

^_;^.-5^^ .-.

''J-

~"^^'ii

■ ' .:

.'""'I

■.'-■'■^^

^. -

Fig. 5. Showing degeneration limited to the external portion of the
occipital lobe (left side).

A little lower the sections showed degeneration limited to the
external occipital region. The external capsule, the inferior
longitudinal fasciculus, and the tapetum were normal at this
level, excepting that the latter two were smaller than in the nor-
mal brain. The lower temporal lobe was slightly implicated in
its posterior portion.

In sections from block 6 the degeneration was still apparent
in the external occipital region, but otherwise the sections ap-
peared to be normal, including the basal ganglia, the foot of the
peduncle, the pons, and the zone of Wernicke.

The optic chiasm stained normally (Weigert method), as did
also the right and left optic nerves.

Microscopically the cells were unchanged in both parietal
regions (thionin stain). There was a moderate degree of peri-
vascular distension. The blood vessels were thickened within
the brain substance, and a few compound granular cells were

A CASE OF APRAXIA, WITH AUTOPSY 32

found around the blood vessels. The pia was much thickened on
both sides, but less so on the left. On the right side hemosiderin
was found in the pia.

In the paracentral regions the pia was thickened slightly, and
a mild degree of perivascular distension was observed. The
cells stained normally.

In the calcarine region there was slight perivascular disten-
sion, but the cells stained well. The pia was slightly thickened,
the more so on the right.

Sections from the necrotic area in the right occipito-parietal
regions showed the presence of a few compound granular cells,
a number of spider cells, marked perivascular distension, thick-
ened blood vessels, and some perivascular infiltration. A few of
the perivascular spaces contained blood pigment, probably hemo-
siderin.

Sections from the pons and medulla oblongata and the spinal
cord, stained by the Weigert method, showed no degeneration.

Cells in the anterior horns of the cervical and lumbar regions
were slightly diseased. The Nissl bodies were atrophied, and

'^^^,

y0p^

Fig. 6. Showing slight degeneration of external part of the occipital lobe

(left side).

the yellow pigment was increased. Some had lost their pro-
longations, but there were many normal cells.

Summary: A man of 55, at the time of his admission to the
home, was blind ; was totally unable to designate the position of
the limbs ; could not locate touch anywhere ; could not recognize
objects by the sense of touch ; and his touch and temperature
senses were imperfect in the left hand. The left hand, although
capable of some reflex acts, could not be moved voluntarily. The
right hand was apraxic, and apraxic phenomena were present in
chewing and walking.

33 JOHN H. IV. RHEIN

The autopsy revealed the presence of degeneration of the
white matter of the right occipital and parietal regions on the
convexity, and the posterior portion of the temporal lobe, the
calcarine region remaining intact. The inferior longitudinal
fasciculus and the optic radiations were degenerated on the right,
and probably though less markedly on the left. On the left
side there was degeneration in the occipital and temporal regions
to a 'much less degree, leaving the median surface intact. The
corpus callosum, in its posterior portion, was degenerated.
Elsewhere the brain was apparently normal.

The process was probably the result of arteriosclerosis which
caused degeneration in the white matter from deficient nutrition.
The necrosis of the right convexity was probably of the same
origin.

The difficulties of explaining the symptoms presented in this
case are very great, on account of the absolute loss of the sense
of position and movement, the loss of the ability to locate tactile
impressions, and the total blindness.

Was his utter lack of knowledge of the position of his body —
for he could not tell whether or not his limbs were moved, or
whether he was standing- or sitting — due to a loss of muscle
sense, or was it related in some way to the blindness?

Pathologically the general loss of muscle sense was not ex-
plained. The lesion in the right parietal region would account for
the sensory change on the left side, but absence of involvement of
the left parietal region leaves this symptom on the right side un-
explained, for, according to von Monakow, the parietal region is
the center for the muscle sense and the sense of location.

The absence of lesions sufficient to explain in the usual way
the general loss of the sense of position of the entire body leads
us to suspect that the destruction of the fibers going to the visual
centers may play some part in causing it.

A possible analogy is seen in those cases of mind or psychic
paralysis (Seelenlahmung) in which, with the eyes open, a limb
is moved freely, but when the eyes are closed the limb acts as
if dead ; that is to say, when the optic element is cut ofif, or poor,
the movement is disturbed, and the location of the limb is not
recognized. An example of this was present in Liepmann's
patient who, with the eyes closed, lost all knowledge of the posi-
tion of the right arm. This is not unlike the facts presented by
my patient, whose entire body, on account of his blindness, was

A CASE OF APRAXIA. WITH AUTOPSY 34

constantly in the same condition as was the right arm of Liep-
mann's patient, when he closed his eyes.

Or, was the loss of the sense of position of the body a form
of disorientation, analogous to that which is observed in mind-
or psychic blindness (Seelenblindheit) ? Against the acceptation
of this latter view, however (in spite of the fact that the post-
mortem lesion was that which is commonly found in mind-
blindness), is the absence of the characteristic symptoms of
mind-blindness. The mind-blind patient sees objects, and has
light impressions, but is unable to recognize the nature of the
objects which he sees.

In my case, as the examination of Dr. Posey and myself
clearly demonstrated, the man was totally blind. The report of
Drs. Webster and Terriberry does not convince me that he was
suffering, at that time, from mind-blindness. All the symptoms
which he is reported to have presented could be readily accounted
for by pure blindness with slight preservation of light perception.

It is possible that there might have been some light perception
preserved after his admission to the home, in view of the fact
that when the hand was suddenly placed before his eyes, while
he was walking, at times he hesitated or stopped, according to
•the observation made by the orderly; but this observation was
never confirmed by myself, although frequently tested for, and
he was often seen to run into things and had injured himself in
that way. In mind-blindness there is a loss of the recognition
of the nature of the objects, with preserved sensation, and this
is not true in my case.

Apraxia. — The movements which I believe to be probably due
to apraxia are as follows.

1. The curious disturbance of the gait. The patient took
short, rhythmical steps, usually to one side. That this distorted
gait was not due to his blindness is probable, as he did not seem
to have any fear of bumping into things, and the peculiarity of
the gait continued in spite of his being assured that every precau-
tion was taken to prevent his bumping into objects.

2. The false movements. In eating, for example, in an
eft'ort to place to his mouth a banana, he would invariably place
it first upon his chin. This was not due to ataxia, as there was
not the slightest appearance of this symptom in any of his
movements.

?,. Or, if a pen were placed in his hand, and paper before

35 JOHN H. W. RHEIN

him, and he was asked to write, only hacking movements from
right to left resulted (apraxic agraphia?).

4. A watch was placed in his right hand, and he was requested
to put it to his ear. He recognized that it was a watch, though
not bv touch, but instead of putting it to his ear (although he
sometimes succeeded) he usually placed it to his mouth, believing
that he was holding it to his ear, at the same time going through
the movements of chewing.

5. When asked to touch his ear with the right hand, he made
groping movements in the air, or grasped his knee, or his coat,
and pressed tightly, believing that he was touching his ear. Or,
he would stand up and make one or two steps, still believing that
he was touching his ear.

6. Apraxic movements of the muscles of mastication.
Some reserve is necessary in deciding whether or not these

movements were apraxic ; as the studies could not be carried
beyond a certain point, or in great detail, on account of the loss
of the sense of position, the blindness, the loss of perception of
the nature of objects by touch, and the perseveration present in
the left hand. These symptoms, therefore, do not entirely ac-
cord, in all respects with the definition of apraxia given by Liep-
mann, for these reasons.

According to Liepmann's definition of apraxia, an object is
recognized, its use known, the limbs themselves are readily
moved, so that the innervation of each limb is intact, but purpose-
ful movements (Zweckbewegungen) of the affected limb are lost.

The amorphous movements of G. in walking and attempt-
ing to touch the nose, however, correspond to similar movements
in Liepmann's case.

The false movements in eating, in the case of G., which were
present in Kleist's (16) case, and possibly the amorphous move-
ments of the hand, in the effort to write (if this was not pure
agraphia) may be classified under the head of " False Move-
ments."

Agnosia. — Can the element of agnosia be eliminated in ex-
plaining the nature of these movements?

When my patient was eating a banana he recognized the
object, although not by touch, but by a psychical process. He
understood that, in order to eat it, he must put it to his mouth ;
but when he made the effort to place the hand to his mouth he
never succeeded, the banana invariably landed on the chin

A CASE OF APRAXIA. WITH AUTOPSY 36

In attempting to place a watch to his ear he recognized that
it was a watch, not from hearing the tick, or from the sense
of touch, but from the fact that it was a watch that the examiner
would naturally place to his ear for him to listen to. In other
words, he recognized the nature of the objects which he at-
tempted to use. Moreover, he could use his right hand for re-
flex acts. He therefore knew the nature of the object, its use, and
was able to move the right arm, but he could not use the object
properly. It seems to me, therefore, that the false movements that
occurred under these circumstances were apraxic.

The explanation of the symptoms in my case is scarcely to be
found on the basis of agnosia ; at least my conception of agnosia.
As I understand it, agnosia is a loss of the understanding or the
perception of the nature of things, while sensation is preserved, an
illustration of which may be seen in the loss of sensory memory
forms (Wernicke), or a loss of the connections between sensa-
tions and memories in mind-blindness, mind-deafness, and mind-
paralysis, which, according to Hartman (12), are expressions of
agnosia.

Nodet (36) defines agnosia as a disturbance of secondary
identification, with persistence of the primary identification, and
Liepmann, Hartman and others also looked upon agnosia as a
failure of recognition (Erkennen) with preserved sensation.
The identification of the fresh impressions with the memory
forms does not occur, either on account of the loss of the latter,
or on account of a hindered connection between both of them
(Liepmann).

In other words, agnosia is a difficulty of recognizing cortical
sensory impressions, and a difficulty of connecting these impres-
sions with a mental picture. For example : One takes an object
in the hand in which all forms of sensation are preserved ; recog-
nizes its shape, physical, and other properties, and can describe
them fully; but is unable to recognize the use of the object, or
its name. This failure of recognition is due to an interruption
between the memories for objects, and the sensory centers in the
cortex.

These are not the conditions present in my case. There were,
on the left side, distinct sensory changes manifested in the dis-
turbance of the temperature sense, the sense of location, the sense
of movement, and the muscular sense; while on the right side

37 JOHN H. W. RHEIN

the sense of location, the sense of movement, and the muscular
sense were altered.

Felix Rose (43), who believed that my case was one of
agnosia, stated that in agnosia the confusion of movements is
based upon the fact that the patient takes one object for another;
for example, a toothbrush is used as a pencil. He admits that in
motor and ideatory apraxia the substituted movements are ob-
served, but the association between the logical act, and the ex-
ecuted act is evident ; for example, the patient brushes the beard
with the toothbrush. Rose believed that agnosia produces
especially the substituted movements, while this is the exception
in motor apraxia.

In the case of G. there is no analogy with these statements
of Rose.

Substituted movements occurred when my patient made an
efi'ort to touch the ear, for example, when the right arm was used
in a groping manner, as illustrated in Liepmann's case. While
he placed the watch to his mouth instead of to his ear, it was not
because he did not recognize what the watch was. Moreover, in
the case of G. the primary identification was at fault, a condi-
tion the opposite to that described by Nodet and others, who
state that in agnosia primary identification is preserved.

Apraxic Movements. — While the apraxic movements could not
be studied in detail, for the reasons above enumerated, those which
I claim for my patient are similar to those described in at least
some of the cases in the literature.

In Liepmann's (21) case the apraxic movements consisted,
among others, of amorphous movements which were represented
as follows : If Liepmann's apraxic was asked to point to his nose,
the request was followed by a strained position of the arm on the
right side, nodding of the head, or repeated bowing, the patient
thinking all the time that he was touching his nose. This was
analogous to the movements observed in my case, in which
the patient, in an efifort to touch his ear, groped in the air aim-
lessly with his right hand, or grasped his knee, or stood up and
made one or two steps.

When Liepmann's patient was asked to point to the left hand
with the right, he nodded " yes," and lifted an ink well in front of
him. He could not make a fist with his right hand, distorted
movements following instead. He could not dress or undress him-

A CASE OF APRAXIA, WITH AUTOPSY 38

self (which was true m my case), and many special acts, as well as
imitation movements, were unsuccessful.

The similarity of the movements in my case to those of Hart-
man's patients is striking-. In his cases the direction of the move-
ments was missed. When the patient attempted to touch the right
extremity with the left he failed, inappropriate, distorted move-
ments occurring as to direction and form. Taking nourishment
by the mouth was performed very slowly, improperly, or done in
stages.

In Pick's (40) cases the disturbances of movement were some-
what different, and may be looked upon as expresions of idio-
motor apraxia. For example, a patient brings a lighted match
near a candle correctly, lights it, and then finally blows it out.

Again, the patient handles a pistol as if it were a musket ; or,
if given a cigar and a^matchbox, the patient evidently recognizes
the cigar, but opens the matchbox, sticks the cigar into the open
end, and presses upon it as if it were a cigar-cutter. He then
rubs the cigar on the side of the matchbox as if it were a match ;
but finally the movement is made correctly.

Or, a watch is shown to the patient, and he is asked what it is.
After many questions, he takes it in his hand and raises it to his
lips. This resembles the movements in my case.

Bonhoffer's (4) patient struck the cigar on the matchbox.
D'Hollander's (14) patient, when requested to strike a match,
grasped the matchbox in his left hand, took out a match with the
right, hesitated, placed the match in his mouth, then finally re-
placed it in the matchbox again. His case was one of paralytic de-
mentia in which the autopsy revealed the characteristic lesion of
this disease without focal lesions.

In Kleist's (16) case of cortical, or innervatory, apraxia, the
patient was requested to sharpen a pencil with a penknife. He
took the pencil in the left hand, grasped the knife with the right,
sometimes with the back of the blade, and sometimes with the
cutting edge uppermost, and supinated the forearm so extremely
that the knife was held with the edge, instead of out and under,
in and down. When he had succeeded in pronating the arm suf-
ficiently in the effort to sharpen the pencil, the necessary exten-
sion and flexion of the arms brought the position back to supina-
tion. He then pressed and scraped the pencil, then shook his head
with an expression of distress. At another time the patient made

39 JOHN H. W. RHEIN

an effort to cut a piece of paper with a pair of scissors. Instead
of placing the thumb and forefinger in the openings of the scissors,
he held the scissors between the thumb and forefinger, and pressed
these against each other. Later the thumb and finger w^ere
placed in the openings, but in the effort to open them they slipped
out, and he held the scissors as before. Instead of opening them
he pressed the handles together. Once or twice he succeeded in
making the cutting movements, but then lapsed again into the
movements just described (innervatory apraxia).

In Kleist's cortical, or innervatory apraxia, the disturbance is
manifested in all forms of movement. There is an incapacity to
perform those movements which arise from special memories ; in
other words, memories acquired by practice. Antagonistic, or
useless movements appear. Partial acts are undisturbed, but the
preparation of the act is implicated in its innervation. The motor
memories are involved (auto-kinetism), not alone the sensomo-
torium — differing from Liepmann's apraxia in which the senso-
motor " Eigenleistungen " is intact — and therefore not due to
any disturbance of the movement formula.

In Strohmayer's (46) case the patient put his hand, instead
of the spoon, into the soup, or cut with his fork or the back of
his knife, while recognizing the objects and their use.

Bonhoffer's patient, in making an effort to shut the door,
moved the hand in an apraxic manner. He held the key in his
hand, making thrusting movements with it instead of the proper
ones. He was unable to write certain letters, although he knew
them, but produced figures without character. The latter Bon-
hoffer believed was an apraxic agraphia.

In Lewandowski's (17) case of progressive paralysis, the pa-
tient was able to make only three movements with the left arm,
i. e., to the back of the ear, to the mouth, and rubbing movements.
These, however, were probably not true apraxic movements.

In a second case reported by Lewandowski (18) there was
apraxia of the eyelids. The patient w^as unable to close the eyes
on command, neither one at a time, nor both together. This was,
according to this observer, independent of a slight left facial
paralysis.

It will be seen that there is a great variety of abnormal manip-
ulations (" Handlungs," Liepmann ; " Agierns," Abraham (2))
of objects, which are described under the head of apraxia. These

A CASE OF APRAXIA. WITH AUTOPSY 40

may be divided into (i) manifestations of ideomotor apraxia
(ideokinetic, of Liepmann, formerly motor apraxia) ; (2) ideatory
apraxia (ideomotor apraxia — Pick) ; (3) innervatory apraxia
(Kleist).

Von Monakow (34) classifies apraxia as follows: (i) Bi-
lateral apraxia associated with right-sided hemiplegia; (2)
agnosia, or sensory apraxia; (3) amnesic apraxia, as in cases of
progressive paralysis ; (4) unilateral apraxia, as in Liepmann's
case; (5) ideatory apraxia (Pick). He states that in apraxia
movements remain imdisturbed, or are only slightly involved,
in (i) breathing, swallowing and eating; (2) in elementary
movements of orientation, such as turning the eyes and head in
the direction of an irritant, when there is not a concomitant central
optic, or other trouble; (3) in gross reflex acts, as in movements
of defense; (4) locomotor movements, as sitting up, or walking;

(5) in simple acts (moderately free from involvement) ; and in

(6) simple automatic movements, such as unbuttoning a button.
According to Liepmann, motor apraxia affects single limbs,

and is rarely bilateral (cases of Hertzog (10), Liepmann, Stroh-
mayer, and possibly my own case), in contrast to ideatory apraxia
in which both sides may be affected. Motor apraxia appears in
simple acts, such as putting out the tongue, or making a fist.
This is not true of ideatory apraxia, which manifests itself in
complicated acts especially. In motor apraxia the limbs do not
obey the psychical wish. In ideatory apraxia there is a failure of
the psychical conditions for the correct completion of the act, but
the limbs respond properly.

In motor apraxia the motor memories for the extremities are
preserved, but they are insufficiently connected with the other
cortical fields. The ideatory process and the motor memories are
separated. In ideatory apraxia the motor memories are intact,
but the ideatory scheme is at fault.

Resorting to explanation by formulas, Liepmann (19) em-
pioys Wernicke's scheme.

According to Wernicke, the sensory centers (S) perceive sen-
sory impressions. SA represents the psychosensorial pathway, so
that at A there develop end memories (Ausgangvorstellung).
which are discharged at Z. AZ is the intrapsychic pathway. Z
represents the end memories (Zielvorstellung). These excite the
motorium through ZM. ZM is the psychomotor pathway.

JOHN H. W. RHEIN

Liepmann modifies this formula so that Z represents the chief
end memories (Hauptzielvorstelkmg-; ErfolgsvorstelUmg). From
Z there go a number of paths to z^, ^^ s^, z", and from each of
these there goes a path to M, the motorium. He then substitutes
for the M, J the innervation. M = the motorium which becomes
excited to new activity through the action of /, the innervation.

The path Z io z must be reckoned as the intrapsychic region,
so that the horizontal line G (see diagram A) would represent a
division of the intrapsychic and psychomotor regions, not the
vertical line G in diagram B.

-z.^

1a.

Diagram A.

Z_^

- z. — z„

J\ J i J ^

Diaeram. B.

Each memory for a partial act (sS Z', z^, z*, z^) is called a
" Zwischenzielvorstellung." From each z there goes out an in-
nervation / which results in a movement or action. Failure of
reaction in inattention, and the early disturbance of purposeful
movements (Handlung) in brain diseases, show that the Z's
give off false z's, but the proper connection between the z's and
the /'s is maintained. The /'s correspond constantly to the .s's,
and this is the condition that Liepmann believes is present in the
Apraxia of Pick.

Summarizing the whole matter, Liepmann states that purpose-
ful movements (Handlung) in a normal individual, are the result

Diagram C. Memory Complex.

A CASE OF APRAXIA. WITH AUTOPSY 42

of a memory complex made up of partial memories (see diagram
C) ; W, the direction memory (Richtungsvorstellung) ; 0, optic
memories ; K, general kinesthetic memories ; k, limb kinesthetic
memories ; /, the innervation ; B, the external movement. Pur-
poseful movements depend upon a number of these complexes
which replace the s's in the formula.

The same disturbance can be referred to the lapse of these
complexes as to the lapse of the memories themselves. The
subjective result would be that instead of a proper W^^ a false
W^^ would appear, or in a false place. The objective results
would be that a false / and B would appear, but always corre-
sponding to the IV^i'^. The innervation fails proportionately to
the ideation, resulting in ideatory apraxia.

But, if there is a separation within each complex, so that
everywhere the J's, or that / with the immediately adjoining k,
do not any more correspond with the rest of the memory com-
plex, then there results a disturbance of movement which is motor
apraxia.^

In other words, Liepman believes that, in motor apraxia, there
is a dissociation of the kinetic memories by reason of an organic
focal lesion. The psychic element and its associations are pre-
served.

Kleist (15) modifies Liepmann's formula so that each direc-
tion memory contains a general kinesthetic element {K), as well
as optic element (O), claiming that in Liepmann's formula the K
is psychologically incorrect, being properly only a component of
each direction memory, and should be written Wk' He believed
that the partial memories are not a part of the chief end-memories,
but a part of the complex of the total movement memories. His
formula is as given on next page :

Lesions of Apraxia. — In Liepmann's (23) case, which is the
foundation for the newer conception of apraxia, numerous con-
nections between the left central convolutions and the cortex of
the frontal region were severed by a subcortical focus in the
frontal brain. A subcortical focus in the left parietal region
implicated the connections from the occipital and temporal re-

* This is an incomplete resume of this phase of the subject, and refer-
ence should be made to Liepmann's " Ueber Storungen des Handelns, etc.,"
by any one wishing to study the subject more fully. Some of the matter
relating to the elucidation of the subject by formula is translated freely
from the original.

JOHN H. W. RHEIN

gions with the central convok:tions, and destruction of the corpus
callosum, as far as the splenium, separated the central convolu-
tions from the entire right hemisphere. In the right hemisphere
there was also a focus where the gyrus supramarginalis joined

IV,

Ideatory aptaxia.
Diagram D. Kleist's modification of Liepmann's formula.

the gyrus supra-angularis in the right hemisphere; and a second,
focus that destroyed most of the fibers from the left face, arm
and leg centers.

Hartman (12) reported three cases of apraxia, with post-
mortem examination. In the first there was a tumor in the left
frontal region, which spared Broca's region and the neighboring
white matter, and extended to the anterior thalamic levels in the
left corpus callosum. In the second case there was a tumor
which destroyed the corpus callosum from the level of the ante-
rior commissure to the posterior end, and which did not extend
to any part of the brain. In the third case there was a hemor-
rhage in the second right frontal convolution the size of a
walnut.

In Van Vleuten's (52) case of left-sided motor apraxia there
was a cylindrical tumor involving the entire corpus callosum, the
left gyrus fornicatus, and part of the frontal brain. The cor-
tical white matter was not involved.

More recently a case was reported by Liepmann {2y) and
Mass, with left-sided agraphia and apraxia, in which there was
a cyst that began in the left upper frontal region and in the gyTus
fornicatus, and extended posteriorly in the latter to the para-
central region. The left half of the corpus callosum was en-

A CASE OF APRAXIA. WITH AUTOPSY 44

tirely implicated in the left hemisphere. There was also a focus
the size of a pea that interrupted the pyramidal tracts. In the
right cerebellar peduncle there was a focus the size of a lentil,
and one in the pons outside of the lemniscus, and one in the right
thalamus.

In Von Bechterew's (5) case there was a circumscribed
lesion in the left hemisphere in the middle part of the posterior
central region, and in the posterior part of the gyrus supra-
marginalis.

Strohmayer (46) found in his case of apraxia a lesion in the
lower parietal region, where there was an extensive loss of sub-
stance which reached forward to the posterior central convolu-
tion. It extended below to the fissure of Sylvius, and above as
high as the anterior parietal fissure. The vertical section at the
posterior end of the splenium showed that the focus spared the
white matter of the optic region, and that parts of the forceps
major and the superior longitudinal fasciculus were involved.
The striking similarity of Liepmann's case is commented upon.

In Pick's (42) case of senile dementia with apraxia there
was atrophy of the brain accentuated in both frontal lobes, and
the left inferior parietal lobe. It was less severe in the right
inferior parietal lobe, and both temporo-occipital lobes. Nothing
abnormal was observed in the central convolutions, the superior
parietal lobe, the cuneus and precuneus. This case is believed to
have been the result of disturbance of the association centers of
Flechsig.

Westphal (54) recently reported a case of motor apraxia in
which the autopsy showed the presence of internal hydrocephalus,
and several foci of softening in the right frontal and occipital
regions, and in the left optic thalamus.

Abraham (2) described a case of total aphasia, mind-deaf-
ness, apraxia, agraphia and alexia, with autopsy. The brain
was atrophic, especially in the post-central regions. The occipi-
tal lobes were relatively preserved. The lateral ventricles were
distended.

The usual lesion of paralytic dementia was present in
D'Hollander's (14) case of aphasic apraxia, but no focal lesion
was found. There was some hydrocephalus.

Von Monakow (34) has recently reported two cases of
apraxia with autopsy. In one apraxia was a partial symptom

45 JOHN H. W. RHEIN

in a case of progressive sensory and motor aphasia. A tumor was
found in the corpus striatum compressing the entire region sup-
plied by art. foss. Sylvii. The cortical speech centers, central con-
volutions, and the corpus callosum were intact. He looked upon
the case as an example of apraxia due to a subcortical lesion in
the central ganglia.

In a second case the apraxia was a transient symptom, occur-
ring in a case of sensory aphasia, and the autopsy revealed the
presence of a lesion of the right central ganglia, and an infarct
of the posterior third of the temporal lobe, implicating the gyrus
supramarginalis.

A study of these facts shows that a variety of lesions have
been described in cases exhibiting apraxic symptoms, and the
lack of uniformity in the lesions described is striking.

What seems fairly well demonstrated in all this data is that
the destruction of the corpus callosum plays a distinct part in
the causation of left-sided apraxia, probably by cutting off
from the right frontal brain the influence of the left frontal
brain. The predominance of the left frontal brain over the right
side has been demonstrated by Liepmann in cases of left-sided
apraxia in right-sided hemiplegias, and dyspraxia of the left
hand in right-sided apraxia and aphasia.

Manfred Fraenkel (8) described a case of mirror-writing
and apraxia of the left hand with right hemiplegia, and came to
the conclusion that the brain centers on the right side were, to a
certain extent, under the control of the left side of the brain ;
that the left cerebral hemisphere directed not only speech, but
" Handeln " ; that the right cerebral hemisphere did not contain
an independent center for movement memories ; and, finally, that
it was therefore closely related to the lower centers.

Rothman (45) has made similar observations in right-sided
hemiplegia. He believed that the precedence of the left side of
the brain for manipulations was demonstrated.

Liepmann and Mass, in analyzing their recent case, agreed
pathologically with Hartman's second case, and Van Vleuten's
case, i. e., that the left hemisphere exercises a directing influence
upon purposeful movements of the left hand ; and that destruc-
tion of the corpus callosum causes a localizing symptom, /. e.,
dyspraxia of the left hand. Liepmann and Mass claim that it
amounts to the same thing whether the interruption of the corpus

A CASE OF APRAXIA, WITH AUTOPSY 46

callosum separates the left hand from the memory centers of the
left hemisphere, or, if the corpus callosum is a direct pathway
for impulses to the sensomotorium of the right brain. Whether
the chief role is the separation of the left hand center (Liep-
mann) or of the left frontal brain (Hartman), Liepmann believes
the differences in opinion disappear if, as is probable, the kinetic
memories of the sensomotorium of the upper extremity are in
the middle frontal convolution.

Van Vleuten's case showed an intense apraxia of the left,
and relative eupraxia of the right, hand. In this case the corpus
callosum was destroyed, excluding part of the splenium.

In Hartman's case there was grave apraxia in the left hand,
and a suggestion of apraxia in the right hand. The corpus cal-
losum in this case was also destroyed, including the splenmm.

In Liepmann and Mass's case the corpus callosum was de-
stroyed, excluding the splenium. These observers believed that
this demonstrated that the preservation of the posterior fourth
and fifth of the corpus callosum does not guarantee eupraxia of
the left hand.

Liepmann and Mass do not doubt that the splenium plays a
role in the conduction of impulses from the left to the right hemi-
sphere. Van Vleuten's case showed this, and Liepmann and
Mass's case also showed that the posterior portion of the corpus
callosum is of less significance for eupraxia in the left hand than
the middle portion.

Hartman believes, with Liepmann, that there is a center in
the frontal lobes for the mechanism of motor cerebral activity,
analogous to Broca's region in aphasic troubles, and that destruc-
tion of the left frontal lobe causes apraxia. The right frontal
lobe needs the cooperation of the left brain for the outflow of
purposeful movements. Defect in the right frontal region (right
mid-frontal) causes partial conduction-apraxia of the left side,
with preserved movement memories (Hartman's third case).

Felix Rose (44) has recently reported a case of transient
bilateral apraxia of the muscles of the inferior distribution of the
facial nerve, of movements of the tongue and the jaw, and of
the left hand, in a patient suffering from Jacksonian crises of
the face and right arm. He believed that there was a diffuse
meningo-encephalitis (no autopsy) which caused these symp-
toms, and concluded that it must be deduced that while the ri^ht

47 JOHN H. W. RHEIN

hemisphere exercises no influence on the Hmbs of the right side for
unilateral movement, and certain bilateral manipulations, for cer-
tain bilateral habitual motor functions, particularly of the muscles
of the jaw, tongue and face, the integrity of the two hemispheres
and their association pathways is necessary.

In discussing the significance of the corpus callosum in rela-
tion to apraxia, von Monakow (34) stated that for the preserva-
tion of praxic movements the integrity of the corpus callosum
was not necessary, and in support of this opinion cited a case of
intense hydrocephalus, in which the corpus callosum was only
rudimentary and the majority of the fibers of which were degen-
erated. The patient could not only use both hands alternatingly
in housework, but could also write. There was no apraxia, in
spite of the fact that the white matter of the left parietal lobe
and of the left temporal lobe, was defective to a great extent.

Van Vleuten claims that his case supported the view held by
Marie, that apraxia is due not only to a destruction of a move-
ment idea, but to a separation of the corpus callosum connections,
between the material basis of the movement idea in the left hemi-
sphere and in the right hemisphere. The special teaching of his
case was that dyspraxia could be caused by a lesion destroying
simply corpus callosal fibers. In van Vleuten's case the right hand
was not apraxic, and he believed, therefore, that the left-sided
apraxic center has not any influence over the sensomotorium of
the right side.

Von Bechterew (5) believed that apraxia was localized in the
parietal lobe, and was due to a loss of movement memories.

Pick's view in his case, with autopsy, was that there was a
disturbance of the association centers of Flechsig.

Liepmann's opinion is that apraxia results from a dissocia-
tion of the motor centers from many regions of the brain. And
further, that the entire memory of complicated acts is not con-
fined to certain places in the cortex, either in the central or
parietal convolutions. Optic, tactile, kinesthetic, as well as
acoustic, elements, he states, figure in the general memory, and
a circumscribed focus could scarcely cause the loss of the entire
movement memory. The movement memories are collected, he
believes, in the left sensomotorium, and there associated with the
other territories of both hemispheres.

Margulies (28) held, on the contrary, that apraxia could be

A CASE OF APRAXIA, WITH AUTOPSY 48

localized, for, he states, if inability to accomplish simple acts is
present, localization at once becomes evident. In disturbance of
complicated acts the physiologic point of view indicates a pri-
mary localization. Bonhoffer believed the apraxia in his case
was an expression of a focal lesion.

Kleist, in his recent case, claimed that the trouble was in the
innervation, and was a fault of the so-called sensomotor " Eigen-
leistungen," thus differing from Liepmann's case in which the
latter was intact.

Von Monakow (33), in an early opinion, did not subscribe
to the view that apraxia could be a permanent symptom of lesion
of the parietal lobe, and he believed that it was related to cortical
ataxia, as motor aphasia is related to disturbance of articulation.
It is due, he thought, to a series of variable and general factors,
such as is seen in aphtisia, and is the result of " diachisis."

In a more recent opinion von Monakow (34) stated that, in
the pathology of apraxia, the region supplied by the left art.
foss. Sylvii is the locality of predilection. He believed that the left
gyrus supramarginalis plays an important part in the genesis of
apraxia, and that in unilateral apraxia of long duration, though
not necessarily persistent, there is local or diffuse involvement
of the Po, T^, and probably also of the corpus callosuni.

Intellectual Element in Apraxia. — Marie (29) believes that
apraxia is due to an intellectual deficit, and that it really is an
aphasia of feeble intensity. He claims that the patient does not
understand the orders given, nor has he the exact notion of the
gesture corresponding to the order given.

While Liepmann (26) acknowledged that focal disease causes
weakening of the intelligence, he looked upon this as a result of
the lesion causing apraxia, and not the cause of the apraxia itself.
Van der Vloet (50) does not subscribe to Marie's opinion,
and, as a result of the study of 14 cases, believes that dementia
and apraxia are independent of each other.

In this connection the recent views held by Abraham (2),
apropos of a case reported by him, are interesting. He believed
that the psychic element plays an important part in apraxic phe-
nomena, and that this component has not received the attention
it deserves in the study of this subject. The symptoms which,
in his case, he believed proved this were the following. His
patient, in putting on his coat, dropped it accidentally. He then

49 JOHN H. IV. RHEIN

attempted to put the right hand into the sleeve, but could not.
Finally, he made a fold in the coat, put his hand through it, and
was satisfied.

In another instance he put his trousers on wrong side about,
and when his attention was called to it he turned around, believ-
ing that this corrected the mistake. The last. act represents a
psychic- deficiency. Abraham questions whether all cases of
motor apraxia have not more or less psychical disturbances.

The significance of the psychical element in apraxia has also
been emphasized by Margulies (28), who believed that psychical
symptoms are manifested in ideatory apraxia, if an incomplete,
partial motor apraxia or agnosia is present, apropos of Pick's
cases, and of cases of amnesic aphasia in localized senile atrophy.

My own view is that there is always present either a dis-
turbance of intelligence, or agnosia ; for example, the apraxic
believes he is performing an act prop^ly, when in reality it
is being performed in an apraxic manner. He does not recog-
nize the incorrect result, even when he sees it. There is
here more than a suggestion of a loss of perception con-
nected with false interpretation of optic and movement impres-
sions. Whether this is psychic, or a manifestation of agnosia,
is a question. It is possibly both, and, as Liepmann claims,
is the result of the lesion causing apraxia, rather than the cause.
The fact that apraxic symptoms have been observed in cases of
senile and paralytic dementia (Pick, d'Hollander (14), Abraham
(3), Lewandowski (17), Soutzo and Marbe (47), and Marcuse
(31)), and in post-epileptic conditions (Pick (40) and Oppen-
heim (38)) is suggestive in this relation.

Psychic, or Mind Paralysis (Scelenldhmung) . — The behavior
of the left hand in my case resembles, in some respects, the " See-
lenlahmung " described by Nothnagel and Bruns. My patient
could not move the left arm in voluntary acts demanded of him,
and it was held in the same position all day long, with the excep-
tion of occasional movements of a voluntary reflex character. For
example, he could perfectly well put his left hand in his hip-
pocket, take out his handkerchief, wipe his nose, and return the
handkerchief to his pocket, and did so on several occasions.

In Bruns's (6) case of sensory aphasia, alexia, agraphia,
slight paraphasia, and right hemianopsia, with lowered touch,
pain, temperature and position senses on the right side, the right

A CASE OF APRAXIA, WITH AUTOPSY 50

arm was incapable of spontaneous involuntary movements, but
could be used in pure reflex or unconscious voluntary move-
ments. Moreover, by practice other movements were possible.
It made no difference if these movements were controlled by the
eye, ear or hand. My case differs from this in that the arm
was not helped by practice, or assistance from the other hand,
or the hand of the examiner. Bruns believed that in his
case the symptoms were due to softening of the left parietal
and temporal regions, as .well as the gyrus angularis, and the
posterior part of the internal capsule. The rest of the hemi-
sphere, especially the central convolutions, was intact. He stated
that disturbance of the sensory centers, and the subcortical fibers
whereby the destruction of the fibers from these to special motor
centers is occasioned causes an incapability to use the limb in
voluntary movements. If, by reason of the breaking of these
fibers, the psychical center has no longer an influence over move-
ments, there exists a mind-paralysis for voluntary movements,
although these may be unhindered reflexly. Therefore, it is the
result of an imilateral destruction of the sensory centers and their
association fibers.

Somewhat similar cases have been observed by Anton (i)
and Bleuler (7).

Bruns concludes that this condition is a disturbance that
arises alone from the falling out of the memories for movement.

Nothnagel, who was the first to use the term psychic paralysis
(Seelenlahmung), believed it was due to a separation of the cor-
tical fields for motor memories in the parietal lobe from the
motor centers.

Liepmann subscribed to the view held by Monk and Noth-
nagel. Monk defined " Seelenlahmung " as the complete loss
of sensory memories of a portion of the body, which, as Liep-
mann states, corresponds to the more recent view of the loss of
kinesthetic memories, and to Meynert's motor asymboly, Noth-
nagel's " Seelenlahmung," Heilbronner's cortical apraxia, and
Liepmann's loss of limb kinetic memories (Gliedkinetischen-
vorstellung) , and therefore not a manifestation of apraxia. In
Liepmann's case of " Seelenlahmung " the limbs could only be
used skillfully in speaking and gestures.

Perseveration. — When, after some effort, my patient suc-
ceeded in grasping the hand of the examiner, he continued to

51 ■ JOHN H. IV. RHEIN

grasp it with increasing pressure, which would persist as long as
the hand of the examiner remained within his grasp. This symp-
tom, without doubt, was an example of tonic perseveration. •

Perseveration has been classified by Liepmann (19) as tonic.
when contractions of the muscles persist; clonic, when there is
an alternation of contraction and relaxation, without apparent
cause ; and intentional, when an act becomes repeated instead of
the intended new one. Cases of this character have been re-
ported by Veschidi (49), Vurpas, Kleist and Liepmann, and have
been met with in verbal deafness, mind-blindness and cases of
agnosia. According to Liepmann the rare cases of tonic perse-
veration are due to disturbance of the motorium itself. This
opinion seems to be somewhat confirmed by the pathological find-
ings in my case, in which the right ascending parietal convolution
was in part degenerated; that is, providing we believe that the
motor centers are not entirely confined to the precentral region.

Tactile Paralysis (Tasfldhmung). — The failure to recognize
the nature of objects by touch is difficult to explain in my case.
The impairment of sensation on the left side, outside of the loss
of the sense of position, and movement sense, sufficiently explains
its presence on this side, but on the right side all forms of sensa-
tion were intact, except the sense of position, movement and
localization.

There was no ataxia, in spite of the presence of sensory symp-
toms which usually cause this phenomenon. In the absence
of lesions causing tactile paralysis, it is difficult to explain its
presence unless we attribute some significance to the optic mem-
ories, in other words, it may be the result of a separation of the
sensomotorium from the optic centers.

In Nicolauer's (35) case of apraxia and tactile paralysis
(Tastlahmung) in the same limb, the question arose whether both
symptoms were not due to a focal trouble in the right hemi-
sphere, which involved the central convolutions, causing disturb-
ance of touch and other sensations on the one hand, and which
interfered with motor acts bv injury to the conduction of the
nerve impulses required for their proper performance, on the
other hand.

The pathologic cause of the apraxia in my case must remain
problematical, unless apraxia may be caused by lesions of the
occipital and temporal lobes in which, perhaps, are lodged, in
part at least, the memories for purposeful movements.

A CASE OF APRAXIA. JVITII AUTOPSY '52

It occurs to me that perhaps, the memories for purposeful
movement are not centered in any one locaHty, though there may
be a region where all the sensory impressions are congregated,
but this is only a meeting-place, so to speak, for these impres-
sions which have their cortical localization in the primary centers.

If I am correct in my opinion that my patient was not
mind-blind, but actually cortically blind, the lesions of the occipi-
tal lobe, confined to the convexity and leaving the medial areas
intact (including the calcarine region), do not coincide with the
present-day view of the central localization of vision.

If it is true that vision is centered in the calcarine region,
which was not degenerated in my case, why did he not see ?

I am not prepared to make the statement that vision depends
not only on the function of the calcarine cortex, but needs also
the cooperation of the cortex of the entire occipital lobe, but I
am willing to admit that this is a possibility which seems to be
suggested by the postmortem findings in my case, for the inferior
longitudinal bundle and the optic radiation were not entirely, or
completely, degenerated.

In conclusion, it is worthy of note that in a number of the
cases of apraxia with autopsy a certain degree of hydrocephalus
has been found. What significance this may have is conjec-
tural, and I shall at present go no further than to call attention
to the possibility that the association of hydrocephalus and
apraxia may be more than coincidental.

It is with much pleasure that I gratefully acknowledge the
kindness of Professors H. Leipmann and A. Pick, and Dr. Felix
Rose for so kindly sending me reprints of their papers on this
subject.

LITERATURE

1. Anton. Zeitsch. f. Med., 1893, No. 14, p. 313.

2. Abraham. Centralbl. f. Nervenheilk. u. Psych., 1907, p. 161.

3. Abraham. Allgem. Zeit. f. Psych., 1904, No. 61, p. 502.

4. Bonhoffer. Arch. f. Psych, u. Nervenk., 1903, No. 37, p. 800.

5. V. Bechterew. Neurologisches Centralbl., 1906, p. 1016.

6. Bruns. Festschrift d. Proviz. Irrenstadt Nietleben, 1897, p. 375.

7. Bleuler. Arch. f. Psvch. u. Nervenheilk., 1893, No. 25, p. 32.

8. M. Fraenkel. Arch. f. Psych., 1907, XLIII, p. 1275.

9. Heilbronner. Miinch. mcd. Woch., 1906, No. 2, p. 1897.

10. Hertzog. Zeit. f. klin.. Med.. 1904, No. 53, p. 52.

11. Hartman. Neurol. Centralbl, 1906, p. 473.

12. Hartman. Monatssch. f. Psych, u. Neurol., 1907, No. 21, p. 97.

13. Heilbronner. Psych. Abhandl, etc., 1891, Heft 3-4.

14. d'Hollander. Bull, de la Soc. de Med. Mentale de Belg., 1906. Vol.
130, p. 295.

53 JOHN H. W. RHEIN

15. Kleist. Monat. f. Psych, u. Neurol, 1906, No. 19, p. 269.

16. Kleist. Jahrb. f. Psych., I907, p. 46.

17. Lewandowski. Centralbl. f. Nervenheilk.. 1905, p. 705.

18. Lewandowski. Berlin klin. Woch., 1907, No. 44, p. 921.

19. Liepmann. " Ueber Storungen des Handelns.," Berlin, 1905.

20. Liepmann. Medicinische Klinik, 1907, pp. 725-765-

21. Liepmann. Monat. f. Psych., No. 8, p. i.

22. Liepmann. Neurologisches Centralbl., 1906, pp. 284 and 710.

23. Liepmann. Monat. f. Psych, u. Neurol., 1906, No. 19, p. 217.

24. Liepmann. Monat. f. Psych, u. Neurol., 1905, No. 17, p. 289.

25. Liepmann. Munch, med. Woch., 1905, No. 52, p. 2377.
2'6. Liepmann. Neurol. Centralbl., 1907, p. 435-

27. Liepmann and Mass. Jour. f. Psychologic u. Neurol., 1907, p. 214.

28. Margulies. Wien. klin. Woch., 1907, No. 20, p. 473.

29. Marie. La Presse Med., 1906, No. 26, p. 242.

30. Meynert. Vorlesungen u. Psych., 1890, p. 270.

31. Marcuse. Centralbl. f. Nervenh., 1904, p. 72,7-

32. von Monakow. Gehirn. Path., 1905, p. 691.
23. von Monakow. Gehirn. Path., 1905, p. 775.

34. von Monakow. Ergebnisse der Physiologic, 1906, No. 6, Ableit.
I-II, p. 370.

35. Nicolauer. Centralbl. f. Nervenh. u. Psych., 1907, p. 609.

36. Nodet. Les Agnoscies, La Cecite psych, en partic, These.

37. O. Mass. Neurol. Centralbl., 1907, p. 789.

38. Oppenheim. Neurol. Centralbl., 1906, p. 626.

39. A. Pick. Arch. f. Psych, u. Nervenk., 1901-2, p. 896.

40. A. Pick. " btudien u. Motor Apraxie," 1905.

41. A. Pick. Revue Neurol., 1906, p. Bog.

42. A. Pick. Monat. f. Psych, u. Neurol., 1906, No. 19, p. 97.

43. Felix Rose. L'Encephale, 1907, p. i.

44. Felix Rose. Semaine Med., 1907, April.

45. Rothman. Neurol. Centralbl., 1907, p. 371.

46. Strohmayer. Deut. Zeit. f. Nerven., 1903, No. 24, p. 372.

47. Soutzo and Marbe. L'Encephale, 1907, No. 2, p. 355.

48. A. Starr. Med. Record, 1888, No. 2, p. 498.

49. Veschide and Vurpas. Rev. de Psych., 1901, p. 165.

50. Van der Vloet. Jour, de Neurol., 1906, p. 589.

51. Van Vleuten. Neurol. Centralbl., 1907, p. 234.

52. Van Vleuten. Allgem. Zeit. f. Psych., 1907, No. 64, p. 203.

53. Webster and Terriberry. Arch. f. Ophthalmol., 1906, p. 477.

54. Westphal. Allgm. Zeit. f. Psych.. 1907, No. 24, p. 452.

DELAYED APOPLEXY (SPAETAPOPLEXIE) WITH
THE REPORT OF A CASE

By Alfred Reginald Allen, M.D.

OF PHILADELPHIA, PA.

LECTURER ON NEUROLOGICAL ELECTRO-THERAPEUTICS ; INSTRUCTOR IN NEUROL-
OGY AND IN NEUROPATHOLOGY IN THE UNIVERSITY OF PENNSYLVANIA;
ASSISTANT NEUROLOGIST TO THE PHILADELPHIA GENERAL HOS-
PITAL; ASSISTANT ALIENIST TO THE ORTHOPEDIC HOSPITAL

(From the Department of Neurology and Laboratory of Neuropathology
of the University of Pennsylvania.)

In 1878 Duret (i) published his great work, " £tudes experi-
mentales et clinique sur les traumatismes cerebraux," founded
upon seventy-one experiments on the lower animals, many of
which are irrelevant to the subject at hand. The first chapter
deals with twenty-five experiments, mostly on dogs, in which the
cranium was subjected to one or more blows, or in which wax
or some other substance was injected into the cranial cavity
through a small trephine opening, in this way producing a cere-
bral compression. The future effect of this work was so far-
reaching that it seems to me advisable to quote a pertinent part
of his conclusions : " Au moment d'une chute sur la tete, ou par
un coup sur le crane, un flot de liquide est forme autour des
hemispheres et dans les ventricules, qui repercute la violence
subie en un point, dans toutes les regions des centres nerveus,
et plus particulierement au niveau du bulbe rachidien.

" L'action vulnerante du flot aqueux exerce ordinairement
ses effets les plus graves et les plus etendus, dans les lacs arach-
noidiens de la base du cerveau, autour du collet du bulbe, et
principalement au niveau du plancher bulbaire, et sur les corps
restiformes." [Translation : " At the moment of a fall on the
head or a blow on the cranium a flow of liquid is formed about
the hemispheres and in the ventricles which reflects the violence
sustained at one point, in all the regions of the nervous centers,
and more particularly at the level of the medulla oblongata.

" The damaging action of the aqueous flow ordinarily exer-
cises its most grave and extended effects in the arachnoidal

265 ALFRED REGIXALD ALLEN

spaces at the base of the brain, about the collet of the medulla
and particularly at the level of the floor of the ventricle and on
the restiform bodies."]

Although many times there was shown at autopsy a damaged
or hemorrhagic condition of the floor of the fourth ventricle,
there were nevertheless other considerable lesions in the brain
well removed from the locality. This work of Buret's was fol-
io wed, in 1880 by an article by Gussenbauer (2) in the main con-
firmatory in tone of Buret's conclusions. He gives a resume of
theories held prior to Buret's experiments which is interesting
historically. For three years he conducted experiments on the
heads of cadavers in order to arrive at some conclusion as to the
mechanics of concussion of the brain. To this end he made a
number of trephine holes in the skull, all in one direction, and
then introduced deeply into the brain substance pieces of straw,
sticks of wood and long Karlsbad insect needles. He found
that when he struck the head in the direction of the trephine
openings there was a movement in the brain substance of the
introduced bodies, but when the direction of force was not in the
long axis or axis of insertion of those bodies, they remained
stationary. Xow experiments of this kind fail to reproduce the
physical hypothesis of the living animal, on account of the
changes in tissue after death. Moreover, these three years were
spent in proving what is so obvious that it is practically an axiom,
i. e., it is easier for a body to move through a medium in the
direction of its long rather than its short axis, or to use a simile,
a boat makes easier headway if it go either forward or backward
than if it move sideways.

In speaking of general convulsions immediately following a
trauma to the head, as in Buret's experiments, he says : " Sie
sind die Folge der mechanischen Reizung, welche der durch die
aussere Massenbewegung hervorgerufene Strom in der cerebro-
spinal Fliissigkeit am Boden des 4 Ventrikels, insbesondere aber
in den sensiblen Corporibus restiformis bewirkt." [Translation:
" They are the result of the mechanical irritation, which the
stream of cerebrospinal fluid, caused by the external massive
impulse, works on the floor of the fourth ventricle, but particu-
larly on the sensory restiform body."] How he reaches this con-
clusion he does not say and I fail to see. Another statement
which is hardlv borne out in cases of lesions of the floor of the

DELAYED APOPLEXY 266

fourth ventricle in man is : " Langere Zeit andauernde Muskel-
contractionen in dieser ersten Periode der Erscheinungen, deuten
nach den Untersuchungen Buret's durchwegs auf erheblichere
Verletzungen am Boden des 4 Ventrikels." [Translation: " Per-
sistent muscular contractures in this first period of the symptoms
point, according to Duret, to important injury to the floor of the
fourth ventricle."]

In discussing unconsciousness in trauma to the head Gussen-
bauer holds that at the time of and immediately foliowmg ine
injury there is a decrease in pressure in the veins and an increase
of pressure in the arteries, possibly due to general arterial con-
traction. This condition produce? a temporary anemia, which
in turn leads to unconsciousnes>. As soon as the circulation is
restored to normal, consciousness returns. In severe cases there
follows a reflex arterial paralysis which leads to venous stasis.
This can lead to a second loss of consciousness. The reason for
dealing with these views of Gussenbauer will be more apparent
later on.

In 1 891 Bollinger (3) advanced his great hypothesis on late
apoplexy. This was received by all practically without dissent,
and so closely is his name identified with this reasoning from
cause to effect that one frequently meets the term Spdtapoplexie
Bollingers. In his article he cites four cases, all the subjects of
injury to the head. After a latent period, during which symp-
toms were either absent or inconspicuous, the late apoplexy came
on. Bollinger's hypothesis is summed up as follows: First, a
trauma to the head ; second, a degeneration, particularly necrotic
softening, in the cerebrum, pons or medulla ; third, an alteration
of blood vessels secondary to the necrotic softening; finally, the
apoplexy due to three factors: (a) alteration in the vessels, (&)
the weakening of the tissue immediately surrounding the vessels,
thereby decreasing their resistance to internal pressure, and (c)
the raising of arterial pressure.

For twelve years Bollinger's views won nothing but accept-
ance and approbation.

In 1903 Langerhans (4) published his monograph. This
revolutionary work, a most brilliant critique, might be said to
have for its text the following, which I quote : " Da Bollino-er
der geistige Vater der traumatischen Spatapoplexie ist, so lege
ich damit gleichsam die Axt an die Wurzel der ganzen Lehre

.^.^

267 ALFRED REGINALD ALLEN

und setze mich in Gegensatz zu der allgemein herrschenden
Anschauung." [Translation: "Because Bollinger is the spirit-
ual father of the Traumatic Spatapoplexie, therefore I lay at
once the axe to the root of the entire doctrine and set myself in
opposition to the generally prevailing opinion."]

He first takes issue with Bollinger on account of the title of
his paper : " Ein Beitrag zur Lehre von der HirnerschiJtterung."
He calls attention to the fact that Bollinger bases his arguments
on Buret's experiments, in which, after blows on the head, there
were found small punctiform hemorrhages in the aqueduct and
immediate vicinity situated just beneath the ependyma, frequently
the ependyma being torn. Bollinger thought that he had bridged
the gap between these experimental findings of Buret's and con-
cussion of the brain. Langerhans says, in the first place, Bol-
linger's cases failed to show the symptoms of concussion. More-
over as concussion does not necessarily entail the changes in the
aqueduct above mentioned, and as these changes are by no means
constant in their connection with the symptom complex, there-
fore one must come to the conclusion that these changes are col-
lateral in nature only, and in no sense form the anatomical basis
of concussion. For these reasons Langerhans very properly con-
cludes that Bollinger's title is a misstatement.

In dealing with Bollinger's cases Langerhans' criticisms are
as follows: In Case I the fact that there was a considerable
fracture of the skull with meningeal hemorrhage makes it ex-
tremely doubtful what part if any the lesions of the aqueduct
played. This is particularly emphasized by the fact that the
microscopical examination of the brain stem was meager in the
extreme. In Case II there was found in the brain no alteration
of the blood vessels, no area of softening and no traumatic
degeneration to account for the hemorrhage. In Case III the
injury must have been one and a half hours before death or else
is merely supposititious ; there was no alteration of blood vessels
and no area of softening. In Case IV there was no Spatapo-
plexie and no microscopical examination of the isthmus. There
was, however, an area of softening in the isthmus.

The utter demolition of Bollinger's views is so complete that
Langerhans' simile, " die Axt an die Wurzel," is well taken.
How it has been possible for the medical fraternity, usually so
exacting in their demands when anything new is advanced, to

DELAYED APOPLEXY 268

accept without question these utterly fallacious lines of reason-
ing, the glaringly inadequate data ; how it has been possible for
twelve years to pass before the man comes forward to fairly
riddle the preposterous structure of pseudo-reason, are things
vvhich must ever remain wonderful and inexplicable in the his-
tory of medicine.

Langerhans' discussion of Seydel's (5) case is masterly. He
comes to the conclusion that the patient did not suffer a traumatic
late apoplexy (Spiitapoplexie Bollingers), but that on account of
the hypertrophied heart and the arterial condition, miliary aneuris-
mata most likely being present, the injury to the head was enough
to cause an increase of blood pressure sufficient in force to burst
one of the lenticulo-striate arteries.

From a study of the case of Maurermeister Lorenz Gerbl (6),
which Bollinger thouglit one of Spatapoplexie, Langerhans draws
the following conclusions: " (i) L. G. an spontaner Hirnblutung
gestorben ist; (2) ein Zusammenhang der Hirnblutung des L. G.
mit dem Hinfallen, dem angeblichen Unfall nicht bewiesen und
auch nicht wahrscheinlich ist." [Translation: " (i) L. G. died
from spontaneous hemorrhage into the brain; (2) a connection
between the hemorrhage into the brain and the fall, the alleged
accident, is not proven and also not likely."] These statements
seem altogether too strong to me. When one suffers from an
apoplexy, the immediate cause of the broken artery being an
increased blood pressure, and the increased blood pressure having
been preceded by such an accident as L. G. suffered, it is ques-
tionable whether the term " spontaner " can be used with accuracy.
Also the microscopical examination of the brain was such that
miliary aneurism cannot be excluded and therefore I think Lang-
erhans' second conclusion too strongly stated.

In 1903 also appeared the work of Stadelmann (7). This
paper is particularly valuable on account of three conditions which
he advances, coincidence with which he holds as necessary to the
correct diagnosis of late apoplexy. They are important enough
to quote verbatim. " (i) Der betreffende Kranke muss nach-
weislich vorher gesund gewesen sein, keine Zeichen von Gefass-
veranderungen dargeboten haben. Lues, Nephritis, Potus, Herzer-
krankungen miissen ausgeschlossen sein, auch darf es sich nicht
un altere Leute handeln, die sowie so schon an arteriosklerose
leiden konnen, resp. bei denen sie sich spontan entwickeln kann.

%i^'

269 ALFRED REGINALD ALLEN "

(2) Das Trauma muss erheblicher gewesen sein, wenn es auch
nicht nothig zu sein scheint, dass es direkt zur Bewusstlosigkeit
gefiihrt hat. (3) Die Erscheinungen der Gefasserkrankungen,
resp. der weiteren Gehirnerkrankung miissen sich in kiirzerem
Zeitraume und unter unseren Augen entwickelt haben. Liegen
erst Tahre dazwischen, in denen die arztliche Beobachtung fehlt,
so wer-de ich mich nie entschliessen konnen. ein irgendwie be-
stimmtes Urtheil liber den Zussammenhang des Trauma mit den
jetzt zu beobachtenden Erscheinungen abzugeben." [Translation:
"(i) The patient concerned must without question have been in
good health, no signs of alteration in blood vessels having existed,
syphilis, nephritis, alcohol and heart disease must have been ex-
cluded; also old people who so frequently suffer from arterio-
sclerosis cannot be considered ; in other words, those who can
develop it (apoplexy) spontaneously.

"(2) The injury must have been considerable, although it does
not seem necessary that it should have caused unconsciousness.

" (3) The symptoms of the vascular or brain lesion must
have developed within a short space of time and under our own
eyes. If years have intervened in which there has been no med-
ical sur\^eillance I cannot determine with any kind of precise
judgment a relationship between the trauma and the symptoms
now appearing."]

According to Stadelmann, the development of a late apoplexy
is as follows: (a) Disturbance of circulation; (b) softening, and
(c) late hemorrhage in the softened area of the brain.

Inferences drawn from cases such as presented by Bohne (8),
Wimmer (9), and Rupp (10), in which the patients did not come
to necropsy, are all of doubtful value.

Kurt Mendel (11) divides cases of traumatic late apoplexy
into two classes : Class A, those cases in which the vessel in ques-
tion is the seat of an arteriosclerotic process at the time of injuf}^;
Class B, those cases in which the trauma causes a disease of the
wall of a blood vessel, previously normal.

(ad A.) The trauma either causes an immediate rise in blood
pressure, in which case we have a traumatic apoplexy, or it causes
an increase in blood pressure which takes place some time after-
ward, this leading to the late apoplexy. He says that any trauma,
even though it does not directly affect the skull, can cause an
hyperemia of the brain. Also an emotional shock, such as sud-

DELAYED APOPLEXY 270

den fear, can cause a vasomotor disturbance with increased blood
pressure.

(ad B.) He advances the following theories:

1. On account of softening in surrounding tissue the vessel
dilates and its walls become thin,

2. The vessel w-all takes part in the concussion and suffers from
fatty degeneration.

3. Miliary aneurisms are formed on account of changes in the
walls of blood vessels.

4. A disturbance in the nutrition of the walls of the blood
vessels secondarv to circulatory disturbance with consequent for-
mation of aneurisms.

5. A certain relation exists between accident (trauma) and
arteriosclerosis, especially of the vessels of the brain and spinal
cord.

Kurt Mendel has watched the development* of arteriosclerosis
after trauma. He has seen patients who have suffered a trauma-
tism of one side in which headache has been confined to the injured
side and in which the temporal artery of that side has become
tortuous and hard. His final conclusions are that whereas the
clinical history shows a clear relation between trauma and late
apoplexy, pathology has, up to the present, failed to do so. He
thinks that the cases in which late apoplexy are caused solely by
trauma without any preexisting arterial degeneration are exceed-
ingly rare.

Late apoplexy is found in literature according to Kurt Mendel
in patients from seven to seventy years of age. The latent period,
the time elapsing between the injury and the apoplectiform seizure,
can vary from four days to nine months. Usually this latent
period is from one to six weeks and can be absolutely free from
symptoms or show mild mental hebetude and some headache.

I am indebted to Dr. William G. Spiller for the history and
pathological material of the following case :

Annie M., white, female, aged 36 years, occupation, cook ; pre-
sents the following history : Father is living and well ; mother is
dead, cause unknown. Two sisters and one brother living and
well. No brothers or sisters dead. No history of tuberculosis or
malignant disease. Her family were of nervous and excitable
temperament. The history of diseases of childhood could not be
elicited. There is a history of influenza sixteen years ago, also
anemia when a child. Fourteen years aero she was operated uoon

271 ALFRED REGINALD ALLEN

for some uterine trouble the exact nature of which is not known.
She has had four children, the youngest of whom is ten months
old. One child died of diphtheria. No miscarriage and labors
normal. Two years ago she became suddenly violently insane
and had to be confined in an institution for three months, at the
expiration of which time she had recovered sufficiently to be dis-
charged. Her husband states that she frequently complained of
nervous headaches, although her general health had been good.
She never used alcohol immoderately. All venereal history was
denied.

On February 27, 1907, the patient had a quarrel with a neigh-
bor and was struck repeatedly, ten or fifteen times, over the head
and face with the fist. From this time she began complaining of
severe headache which was not localized. Ten days after the
quarrel, while she was lying down with her baby, her husband
heard a sound as if something had fallen and found that she had
dropped her baby to the floor and was lying with the right arm
and leg completely paralyzed and was motor aphasic. She was
not unconscious and had lost control of her bladder and rectum.

She was admitted to the hospital March 12, 1907, in a semi-
stuporous condition. The one word she was able to say was
" No." There is a history of cough, expectoration and pain in
her chest between the time of her injury and her apoplectiform
seizure and her family physician said in this time she had pleurisy
and probably pneumonia.

As she was completely motor aphasic except for the single
word " No," and as she used this word in answer to every ques-
tion, it was hard to determine whether she was word deaf, but the
chances are that she was not; if asked whether her name was
Annie she would say " No." On two occasions she gave her left
hand when asked, but it was impossible to get her to do anything
else. She had a right facial paralysis of central type. She
opened and closed both eyes firmly and equally. It was impos-
sible for her to protrude her tongue but it deviated to the left
while in the mouth. The biceps and triceps reflex were not very
distinct on the left side, but were much more distinct on the right.
Pin prick caused pain over the entire right side. Once or twice
the Babinski -reflex seemed to be obtained on the right, but it was
not constant. Patellar tendon and Achilles tendon reflexes were
present and equal on each side.

On March 15 it is recorded that "her lungs were full of fluid
and her pulse extremely rapid."

The patient died on March 18, at 8 A. M.

Sections of the spinal cord at the level of the fourth lumbar
and fourth thoracic segments were stained by the Weigert hema-
toxylin, hemalum-acid fuchsin and von Lenhossek-Nissl meth-
ods, and showed no pathological alteration. The amount of central
glia substance in the fourth lumbar segment was much greater

DELAYED AFOPLEXY 272

than is usually seen. An examination of sections in the mid-
olivary region showed that the ependymal lining of the fourth
ventricle was, at this level, in perfect condition. Many of the
blood vessels in the sections from the mid-olivary level presented
a lumen packed with erythrocytes. The perivascular lymph spaces
were greatly distended and filled with an unstaining detritus which
in several instances could be seen to contain erythrocytes, but by
far the largest part of this detritus was albuminous coagulum.
In several instances the point of escape from the vessel of the
erythrocytes could be detected. This condition of affairs was not
limited to the immediate subependymal part of the floor of the
fourth ventricle, but is also seen far anterior to this, one such
vessel being located in the hilus of one of the inferior olives. In
this latter case blood pigment was found in the detritus filling the
lymph space. A section at the level of the decussation of the
fourth cranial nerves showed a few of these distended vessels in
the neighborhood of the aqueduct. But the chief point of interest
here is an area anterior to the decussation of the superior cere-
bellar peduncles. This area is located in the mid-line and shows
several large blood vessels with the surrounding unstained detritus
and in addition a pronounced hemorrhagic condition. This lesion
is easily seen with the unaided eye and measures approximately
two millimeters transversely by one millimeter antero-posteriorly.
The ependymal lining of the aqueduct at this level is unaltered.
A section through the cerebral peduncles at the level of the mam-
millary bodies and the middle of the red nuclei shows a slight
tearing at one point of the ependymal lining of the aqueduct and
also a microscopical hemorrhage into the ependymal lining at
another point. Here, as in sections lower down, one finds the dis-
tended perivascular lymph spaces but not in so great numbers. A
section through the optic chiasm and anterior part of the optic
tract shows many erythrocytes free in the ependymal lining of
the third ventricle. The chiasm, optic nerve and optic tracts are
normal.

In the left lenticular region sections were made from blocks
of tissue, the highest level of which corresponded to a plane
passing just above the highest part of the lenticular nucleus.
Over four hundred and fifty sections were made, the lowest plane
being reached when the middle cerebral artery was cut longi-
tudinally. Sections were stained at different levels of the series
by means of the Weigert hematoxylin. Van Gieson's, Weigert's
tlastica and hemalum-acid fuchsin methods.

The lowest sections, those having the middle cerebral artery
cut longitudinally, show the small vessels filled with blood. In
some instances there is a tearing of the intima and media with a
passage of erythrocytes into the perivascular lymph spaces. In
some of the small vessels there is thrombus formation and the
presence of fatty granular cells about the vessels. As successive

273 ALFRED REGINALD ALLEN

levels higher up are examined, the occluded vessels are of more
frequent occurrence and are located chiefly in the globus pallidus
and putamen of the lenticular nucleus. There are found also a
few occluded vessels in the optic thalamus. At about the mid-
level of the lenticula there is found in the globus pallidus a vessel,
the muscular portion of whose wall shows calcification. This is
the only vessel which I have examined in this case which showed
this process. Within the lumen there is seen an hyperplasia of
the endothelial lining. At about the mid-level of the lenticula
there" is found a process of softening which is chiefly confined, at
this level, to the globus pallidus and in which are frequently seen
fatty granular cells. At the highest level of the lenticula the area
of softening has greatly increased and extends well through the
white matter external to the caudate nucleus and optic thalamus
for a distance antero-posteriorly of about five centimeters. There
are also small areas of softening in the caudate nucleus.

Sections of the right and left paracentral lobules were stained
by the von Lenhossek-Nissl method. The large motor cells of
the right side were normal but those of the left side showed
marked degenerative reaction.

Among other things the general post-mortem notes state that
the cortical epithelium of the kidney presents a moderate degree
of cloudy swelling. There was some general kidney congestion
and a slight increase of connective tissue between the tubules was
noted. There seems to be no evidence from the histological ex-
amination of undue arterial change.

Cases of this kind are particularly interesting from a medico-
legal standpoint. There are so many instances on record of trauma
to the head followed after a greater or less time by late apoplexy
that we may be morally certain that a definite relation between
trauma to the head and the apoplectiform seizure does exist. But
are we in possession of every link in the chain of pathological cir-
cumstances so that we can swear on the witness stand that a given
case of apoplexy is a late apoplexy due to the trauma? This
question must be answered unqualifiedly in the negative.

My conclusions, drawn from the above case, together with the
many on record, must of necessity be theoretical and are as follows :

(a) Traumatic delayed apoplexy (Spatapoplexie), in the sense
of the original Greek, dTroTrXr/o-o-etv, is in all probability, an entity.

(&) Delayed apoplexy is not of necessity a condition in which
hemorrhage takes place, but the stroke can have as its immediate
etiological factor the occluding or thrombosis of one or more
arteries.

DELAYED APOPLEXY ^4

(c) The cerebro-spinal fluid does not play a necessary part in
the production of delayed apoplexy and injury to the region of
the aqueduct and fourth ventricle is a collateral circumstance of no
etiological moment.

(d) In cases of delayed apoplexy in which hemorrhage takes
place, the hemorrhage is not necessarily preceded by a process of
necrotic softening about the artery in question, this removing the
outside support (Widerstandsfahigkeit) but the artery itself is
injured as Langerhans holds, and the secondary rise in arterial
pressure, or the normal pressure causes the hemorrhage.

(e) The mechanics of many cases of delayed apoplexy is as
follows : The trauma to the head causes a mechanical agitation to
the brain substance, which falls with greatest severity on the
arteries, small and large, they being filled with an incompressible
fluid. The particular location of the chief action on the vessels
cannot be determined by^the external impact of the blow or the
direction of the force and is impossible of determination until
revealed by symptomatology. At first there is in all probability
a general vasomotor constriction of the cerebral arterial system
followed very shortly by a paresis of the vessel walls. The vessels
particularly injured undergo endothelial proliferation, and throm-
botic processes are set up. Then occlusion, if in a functionally
important area of the brain, can cause an apoplectic attack. To
this class belongs my case.

In considering a case of what may be traumatic delayed
apoplexy, a possible incompetence on the part of the kidney must
be borne in mind and the action of a consequent uremia must be
given full weight. The case I report had a slight amount of
chronic interstitial nephritis and had she not come to necropsy
one could not have positively stated whether there was a hemor-
rhagic or thrombotic condition on the one hand, or a uremic attack.

BIBLIOGRAPHY

_ I. Buret. "Etudes experimentales et eliniques sur les traumatismes
cerebraux," p. 153, Paris, 1878.

2. Gussenbauer. " Uber den Mechanismus der Gehirnerschiitterung "
Prager med. Wochenschrift, 1880, No. 1-3. '

3- Bollinger. " Ueber traumatische Spatapoplexie," Internat. Beitr
zur wissenschaftl. Med. Virchow-Festschr., 1891, Bd. II.

4. Langerhans, R. " Die traumatische Spatapoplexie." Verl v A.
Hirschwald. Berlin, 1903.

5. Seydel. " Fall von traumatischer Spatapoplexie," Aerztl. Sachverst
Ztg., 1902, No. 18.

6. Langerhans. Loc. cit., p. 50.

., i-l

275 ALFRED REGINALD ALLEN

7. Stadelmann, E. " Ueber Spaterkrankungen des Gehirns nach
Schadeltraumen." Deutsche med. Wochenschr., 1903, No. 6.

8. Bohne, Julius. " Ueber einen Fall von traumatischer Spatapo-
plexie." Fortschr. d. Med., 1902, No. 36.

9. Wimmer. " Ueber traumatische Spatapoplexie." Med. Klinik, 1907,
No. 8.

10 Rupp. " Zur Kasuistik der traumatischen Spatapoplexie." Zeit.
fur. Heilk., 1905, Bd. XXVI.

II. Mendel, Kurt. "Der Unfall in der Atiologie der Nervenkrank-
heiten." Verl. von S. Karger, 1908, pp. 48 to 61.

Reprinted from Monthly Cyclopedia and ?^Iedical Bulletin, July, 1908.

PSYCHOTHERAPY: ITS SCOPE AND LIMITATIONS^
By Charles K. Mills, M.D.,

PROFESSOR OF NEUROLOGY, UNIVERSITY OF PENNSYLVANIA

Introductory Remarks

A WAVE of increasing interest in psychic medicine appears to be
passing over our country. An old, old story is being repeated by nev^^
raconteurs; an old, old subject is presented in a garb not entirely new,
but with new trimmings and adornments. Religion is again offering
its services not only to the sick but to the doctor, who should show at
least the courtesy of some attention to those who are offering him aid.
A movement which fills churches and excites the interests of a nation is
deserving of discussion.

Psychic medicine and mystic medicine are terms sometimes used as
if they were interchangeable. This is not the exact truth. In a cer-
tain sense mystic medicine is psychic medicine, but the reverse does
not, or at least should not, hold good. In the incantations of the
medicine men, of the savages, in the appeals to omens and to oracles,
in the calling upon the sun and the stars to relieve the sick and the
helpless, in the ministrations of Mrs. Eddy and her apostles, in the
resort to healing shrines of every description, the psychic element is
easily discoverable. These and other forms of mystic healing appeal
to the superstition or the imagination of the individual ; they play both
upon his normal and abnormal suggestibility. They do this, however,
not from the standpoint of the sane and scientific believer in the proper
use of suggestion. They attribute cures to supernatural interferences,
and in this way deceive in the very act of helping. The psychic medi-
cine in which the doctor should be continuously interested is- that in
which the use of mental influence for the relief or cure of disease is
resorted to on the same scientific principles as is the use of water, medi-
cine, electricity, the surgeon's knife, or the forceps of the obstetrician.

In the brief time at my disposal I can do little more than present my
personal views with regard to the methods, scope, and limitations of

' From the Department of Neurology. University of Pennsylvania. Read
at the meeting of the Philadelphia County Medical Society, March 25, 1908.
1 259

2 MILLS : PSYCHOTHERAPY

psychotherapy. The subject is so ancient, and if elaborately presented
so extensive, that I should become wearisome if I should attempt to do
more than this. On the basis of a considerable experience therefore I
shall open this discussion by somewhat dogmatically presenting my
conclusions regarding the utility of the different methods of psycho-
therapy.

What are to-day the accepted psychotherapic methods — those which
have received the support of medical men of position and influence?
I might say in passing that this support has varied considerably as
regards both its extent and its depth. These methods are: (i) The
use of hypnotic procedures ; ( 2 ) the appeal to suggestibility in the
waking state; and (3) the resort to educational or persuasive measures.
Any one of these may be combined with other accepted therapeutic
measures.

Hypnotism

The subject of hypnotism never seems altogether lacking in interest
for the public, both medical and lay. The treatment by suggestion of
patients in whom have been artificially induced conditions of somno-
lence, letharg}- or catalepsy has again and again attracted the atten-
tion of the profession. Nearly thirty years ago under the stimulus of
the publication of the scientific, and at the same time spectacular, in-
vestigations of Charcot and Richer. I became deeply interested in
hypnotism, studying the new literature, re-reading the old. and making
many personal investigations and contributing papers, reviews, and
chapters relating to the subject. One of my early papers illustrated
by the presentation of patients in whom the phenomena of somnolence,
letharg}-, catalepsy, and automatism at command were exhibited, was
read before this society in 1881, twenty-seven years ago. For some
time I made numerous experiments with hypnosis for diagnostic and
therapeutic purposes, but as time progressed my experiments with this
agency grew less and less. While this is true, during the same period
my respect for scientific psychotherapy increased, but this psycho-
therapy has its wider field of usefulness outside of the domain of
hypnotism.

Science and medicine owe a large debt to Braid. Charcot. Liebault.
Bernheim. Janet. Forel. and others for the light which they have
thrown upon abnormal psvchology and nervous phenomena in general
through their studies in hypnosis, but this fact must not blind us to the
possibility that evil as well as good may have come out of their in-

200

mills: psychotherapy 6

vestigations. Because of the brilliant results obtained by these earnest
and enthusiastic scientific workers the profession has been led to expect
too much and to overlook the unwholesomeness of hypnotic procedures
when thoughtlessly or too generally used. Critically regarded, the
net result in the way of addition to our therapeutic resources which
has come through studies in hypnotism is not large.

Should hypnotic procedures of the sort which produce states of
lethargy or catalepsy be resorted to in the cure of disease, and what is
to be hoped for from their use? What harm if any may result from
employing such methods? These are practical queries.

Putting the matter as briefly as possible, my experience and observa-
tion indicate that little of permanent value is to be accomplished by the
production of profound hypnosis, and that it may be the means of
doing no inconsiderable harjn. Hypnotic procedure, however, even
when used to induce profound somnambulic states, has a field of useful-
ness which is very limited, although it cannot be disregarded. It may
be used, for instance, on the principle of two evils choosing the lesser,
for the improvement of the sad condition of those amnesic and de-
pressed hysterics described by Janet and familiar to all neurologists,
who have a double or dissociated personality and live for the most
part in a state of consciousness in which life is truly a burden. It can
be admitted that it may be right not only to change for a time, for
patients of this sort, their state of consciousness or personality, but even
to allow them to remain in the artificially induced state, the second
condition being better than the first or usual one. At the best however
hypnotism is here only a method of making a life altogether intolerable
a little more tolerable. The temporary ailments or conditions are re-
lieved, but the degeneracy and neuropathy remain and will constantly
reassert themselves.

I have seen not a few examples of the evils actually caused by
hypnotic procedures, used either for amusement or for scientific and
therapeutic purposes. Many years ago two of my professional friends
made a series of interesting experiments on the artificial induction of
convulsive seizures. They brought about their results, as I witnessed
on one occasion, by placing themselves under the conditions of a
spiritual seance or table rapping. With their hands placed in slight
contact with the table, and their minds made as vacuous as possible,
they were able to bring on conditions of catalepsy and clonic spasm.
Wisely they both soon determined that they had had enough of these

261

4 mills: psychotherapy

experiments. One of them told me that on more than one occasion,
while on the street or in some public place, he had been compelled to
back himself into the doorway or against some support to prevent a
spasmodic attack.

A voung man, intelligent and educated, of a highly sensitive nervous
system, out of interest and in the spirit of accommodation, allowed
himself to be used again and again by a psychologist for the purpose
of exhibiting and illustrating the phenomena of hypnosis. States of
lethargy, of catalepsy, and examples of automatism at command were
frequently shown with this youth as the subject. Later, after entirely
giving up his part in these exhibitions, this young man became a victim
of hystero-epilepsy from which he had never suffered before. He did
not recover until some years had passed. Hysterical phenomena
brought out by suggestion under hypnosis can be evoked more readily
the oftener the hypnotic procedures are repeated. It is always possible
that the hypnosis artificially produced may initiate hysterical afTections
which may become chronic. I might give a considerable number of
illustrations of this fact were it worth while.

In considering the possible evil effects on the individuals of repeated
profound hypnotizations it is necessary to direct attention to the in-
fluence on the brain and mind of such procedures. I shall not go into
any discussion of the various theories as to the state of the central
nervous system in one who is hypnotized. Those desiring to dip
deeply into this branch of psychological medicine cannot fail to have
their interest excited, even though their intellects may not be greatly
enlightened as to the physiology and pathogenesis of hypnosis, by
consulting the works of Charcot, Heidenhain, Bernheim, Grasset,
Janet, Forel, DuBois. and many others. The literature of hypnosis
has become so voluminous as to cause embarrassment to the student
of the subject. One thing is evident, namely that when hypnosis
results, through either altru-suggestion or auto-suggestion, the cere-
bral, or at least the psychic resistance of the individual is reduced, and
repeated reductions of this sort must in time permanently diminish the
psychic robustness of the individual. The second, third, and sub-
sequent hypnotizations are usually more readily induced than the first,
and a truly pathological state may eventually be established. In the
language of comparatively recent psychiatry, the phenomena of
hypnosis are sometimes quite lucidly expressed by the statement that
the individual becomes, for the time being, the subject either of nega-

262

mills: psychotherapy o

tive or of positive hallucinations, or of both. When, for instance, he
is told that a chair, a table, a flower, or an individual, is no longer in
the room, and believes the same to be true although they are present
and in full view, and likewise when he is assured that his sciatica, his
headache, or one of his limbs has disappeared and believes this, he is
temporarily the victim of negative hallucinations. In like manner,
when a person, an object, a change in sensation, or impairment of
power, is suggested into existence by acting upon the hypnotic subject,
he becomes the victim of a positive hallucination. The state of hallu-
cination is an abnormal one, whether artificially induced or a symptom
of inherited or acquired insanity, and to repeatedly reproduce ab-
normal psychic conditions is not in the very nature of things a pro-
cedure to be approved and encouraged.

What has thus far been said has had reference chiefly to the thera-
peutic value and to the harmfulness of profound hypnosis. What, if
any, is the value in therapeutics of that degree of hypnosis, sometimes
spoken of as somnolence or light sleep; also what harm, if any, may
befall the patient from the induction of this degree of hypnosis?
These questions are not so easy to answer as those which have refer-
ence to the production of real lethargy and of catalepsy. Some good
authorities, Forel, for instance, would have us believe that hypnotic
somnolence does not dift'er in essence from ordinary sleep, and there-
fore that one is no more deleterious than the other. Into this question
I cannot go on the present occasion. Light hypnotic sleep, unless fre-
quently repeated, is probably not productive of harm. Numerous cases
of transient relief of such symptoms as heaclache, neuralgia, insomnia,
hypochondria, tremor, and various hysterical stigmata, sensory, motor,
and visceral, through suggestion made in light hypnosis are sufficient
to give warrants to the opinion that it may sometimes be used with
advantage and practically without detriment to the patient. It seems
also at times to be of service in mitigating the distressing symptoms
of some organic affections as, for instance, the pains of tabes. In
times gone by I have successfully used suggestion given to patients in
light hypnotic sleep for the relief of such symptoms as hysterical
ptosis, hypesthesia. monoparesis, hemiparesis, aphonia, singultus, and
vomiting. The point to be decided in such cases as these and in others
which might be easily enumerated is whether the method of suggestion
through hypnosis is preferable in these cases to waking suggestion, or
to the use of medicinal or other material remedies. My own convic-

263

6 mills: psychotherapy

tion. after considerable experimentation, is that hypnotic procedures
are not to be preferred to the other methods, and that in some instances
at least harm may result. It is much the same with hypnosis as it is
with the use of drugs with which one has to be careful in order that
a serious habit may not be initiated. Whether a drug or a hypnotic
procedure does harm will depend in part upon the inherited constitu-
tion and proclivities df the patient, and in part upon the wisdom of the
phvsician who uses either as a therapeutic implement.

Suggestion Without Hypnosis

Until the appearance of Bernheim and the exposition of his views
regarding the r^al nature of hypnosis, the world interested in hypnotism
remained to a considerable extent under the influence of the views of
those who, like Mesmer, the elder John K. Mitchell, and many others,
regarded hypnotic phenomena as the result of the influence of some
force like magnetism exerted from without upon the subject. After
a long use of hypnotic procedures it dawned upon Bernheim that it was
possible in many subjects to produce the same effects without the
induction of the artificial somnambulism. Suggestion, and this alone,
was necessary. With others who followed him he held what was
already known to the world at large, although not clothed in scientific
terms in this world, that suggestibility was the common property of
the race. While the percentage of those who are not amenable to
suggestion varies, according to writers on the subject, from 3 per cent,
to 30 per cent., it matters little what the exact figures, all or nearly
all, are suggestible. This being admitted, one practical result for the
psychotherapeutist is the recognition of the truth that hypnotic pro-
cedures, known sometimes to be injurious, are in reality seldom neces-
sary. The therapeutist must simply learn how to act upon the sug-
gestibility of the concrete case before him.

While suggestion is potent, it is so within certain limitations. The
suggester, whether priest, charlatan or scientific physician, may, by
appealing to blind faith, lift the cloud or dissipate the poisonous atmos-
phere which surrounds the patient, but it is not always true that in do-
ing this he eflfects a permanent cure. The truth is not as Du Bois ex-
presses it, that one is cured as soon as he believes himself to be cured,
but that he is cured when the conditions which have caused, and which
tend to reproduce his sickness, have been removed. Suggestion there-

264

mills: psychotherapy /

fore while a powerful agent for good in the hands of the wise, needs
in most instances to be reinforced by the use of other measures.

With Du Bois the physician must so educate his patient as to give
him an understanding of the true nature of the disorder from which
he suffers and of the best methods of counteracting it; with Mitchell
he must improve the nutrition by measures tending to the improvement
of blood and structure ; with the surgeon he must remove when pos-
sible the irritating source of direct and reflex disturbances; with the
everyday physician he must skillfully and thoughtfully employ those
medicinal agents which are calculated to assist in restoring and im-
proving function.

This much may be said, when comparing the beneficial effects which
result from suggestion through hypnosis and of suggestion without
hypnosis, that the latter is the surer as well as the safer remedial
measure.

While treating of suggestion let me say a few words which are not
inappropriate to the subject in hand about the discussion which has
been going on for some time, especially in Paris, regarding the patho-
genesis of hysterical phenomena. Led by the ever alert Babinski on
the one hand, and on the other by Raymond, the inheritor both of the
mantle and of the opinions of Charcot, a battle royal progresses re-
garding the concept advanced by the former that all hysteria is due
to suggestion and that most, if not all, hysterical phenomena can be
traced to suggestive influence exerted on the patient by others. What-
ever of truth this idea may contain it is not altogether correct. With
Janet I incline to the view that depression and dissociation of person-
ality play a larger role than direct suggestion in the production of
hysterical symptoms, although it must be recognized that suggestion
may enter into the induction of the hysterical phenomena which
demonstrate the dissociation. Of this I am convinced by experience,
that suggestion by the examining physician is not the only or even the
main cause of the stigmata which he finds in his patient. To take for
illustration but one class of patients, again and again I have seen cases
after injury, and have had the opportunity of examining them for the
first time for nervous symptoms, no similar examination for the same
purpose having been made. These patients have without suggestion
indicated the presence of anesthesia or hypesthesia, of impaired power
or impaired vision, of vomiting, tics, of localized pain or of hyper-
esthesia. It is true that suggestion on the part of the physician or

265

8 MILLS : P5YCH0THER.\PY

Others may increase or diminish or transfer phenomena of sensation
in such patients, but that they are thus produced in the first place is
another matter. By what process can disease be made to take this or
that classical form in one who is utterly ignorant of the symptomatol-
ogy of all disease ?

While it may cause us to diverge a little from the main line of
thought in the present discussion, it will not be altogether out of place
to speak a little more at length on this subject of the traumatic
neuroses or psychoses which have a not unimportant place in neuro-
psychic medicine. The cases of injury or of alleged injury to the
nervous system have become so important in the medical and economic
histor\- of our time as to have secured the close attention not only of
medical men and jurists, but also of financiers and public administra-
tors. I have been told that forty or fifty is not an unusual number
of accidents and alleged accidents occurring daily in connection with
the Rapid Transit System of Philadelphia. It is even asserted that the
dividends of this and other companies are practically annulled by the
amount of money required to pay for the damages resulting from
such accidents. Charges of fraud, collusion, and crime are freely
bandied to and fro by those who, on the one hand, accuse lawyers and
physicians of assisting in procuring such damages from the innocent
and suft'ering corporations, and on the other hand, by those who accuse
these corporations of defrauding by methods of jolly, bluff, and de-
ception, clients and patients of the just compensation for injuries
received because of the negligence or carelessness of their ill-paid
employees. Hysteria, neurasthenia and hystero-neurasthenia. hypes-
thesia, ankle clonus, irritable spine and contraction of the fields of
vision are terms becoming as familiar to the claim agent and the
attorney for the plaintiff and defendant as to the doctor and the stu-
dent of medicine and psycholog}-. Litigation symptoms have come
to be looked upon as morbid phenomena to be reckoned with as cer-
tainly as the effects of inflammation or intoxication.

Doubtless some instances of unmitigated fraud may be found in the
extensive list of traumatic cases in which suits at law are brought.
It is difficult at times to discriminate between real but exaggerated and
consciously or unconsciously simulated symptoms, but the experi-
ence of those with open minds shows that the majority of those who
bring actions have received more or less injury and are deser^-ing of
more or less compensation. These injuries may be psychic or physical,

266

mills: psychotherapy 9

or both ; but most frequently perhaps there is a combination of local
injuries with a larger and more important psychic disorder. The
ankle or back under judicious treatment may soon be repaired; the
dislodged kidney or uterus may be replaced by the skillful surgeon ; the
broken bones and even the bruised nerves may soon be healed ; but
the shock to the brain and nervous system, especially in those of
neuropathic constitution, and these are a multiude in every community,
often continues to harass the victim of injuries through and often far
beyond the period of litigation.

The fact that litigation may cause an increase or a continuance in
the clinical phenomena of the traumatic neuroses or psychoses is an
argument in favor of rather than against their reality and their impor-
tance. The speedy settlement of just claims or the recovery of just
compensation after waiting may and does sometimes act as a powerful
incitement to recovery. This well-known fact teaches us that in such
cases, as in many other traumatic cases, psychotherapeutics may be
made to play a large part. Whenever lawyers and physicians get to-
gether and in reasonable spirit co-operate in the adjustment of claims,
much good is done for all concerned. A just settlement is good
psychotherapy for the attorney, for the client, for the physician, and
for the patient, and above all for the corporation which settles.

Educational or Reasoning Methods
Du Bois comes to us saying that the best psychotherapeutic method
is that of reasoning or persuasion, that of informing the patient as to
the nature of his case and of reasoning him into the belief that it is
curable, and that he (the patient) can help out this cure. This is not
a new plan of curing or attempting to cure the sick. It has existed
wherever good physicians have used their mental powers for their
fellows. The doctor of the metropolis, of the town or of the cross
roads, if one well fitted for his vocation, has successfully exercised
this art of persuasion, as he has also that of appealing to blind faith.
A debt however is due to Du Bois, to Prince, to Putnam, and to others
working in this field for concentrating the attention of the profession
on the value of persuasive or reasoning methods, and on the best way
of using these for the relief of nervous ills. Methods of education
and of persuasion have been illuminated, and in so far as they are
more clearly seen and better understood are likely to be more largely
and more efficiently employed. Suggestion probably enters into all

267

10 mills: psvchotherapv

psychotherapy, but in the educational method is more than mere
suggestion.

" I have shown," says Du Bois, " that in this influence (persuasive
or educational) exercised on the patients afflicted with the various
functional troubles of the digestive apparatus, or the heart, or the
respiratory system, there is always an element of suggestion. To
arouse in the patient the conviction of cure is the fundamental indi-
cation. It is impossible for me always to keep the patient from reach-
ing this conviction by blind faith; but the fault, if favdt there be, must
be imputed to the subject. Personally I take care that my statements
are rationally founded ; I transmit to the patient only such convic-
tions as are based on my psychological or physiological views. I try
to make the patient follow the same paths, to explain and to make him
understand as clearly as possible the influence of mental representa-
tions on organic functions."

The educational method is among the most valuable of all psych.o-
therapeutic measures. This method contemplates teaching the patient
what he has, what he has not. what he seems to have, what he can
do, and what he cannot do, and what he simply believes he cannot do.
This expresses briefly what might be much elaborated.

In the educational method, first separating the false from the true,
the real from the imaginary, the inevitable from the merely habitual,
after the manner which has been advocated by Du Bois, Prince, Put-
nam, Taylor, and others, the patient becomes enlightened as to the real
nature of his own case. He obtains this enlightenment through fre-
quent conferences with the physician who talks to him somewhat as a
physician talks to his colleague in a consultation, the method of the
physician and consultant being modified in accordance with the lack
of knowledge which the patient has of the anatomy and physiology
necessary to a scientific understanding of the subject. The physician,
so to speak, popularizes for his patient the knowledge which he pos-
sesses and which another physician would comprehend without the
necessity of such popularization. Understanding his own symptoms
and being led to a full belief in the possibility of their removal, the
patient advances more easily along the path of recovery.

268

mills: psychotherapy 11

The Light Shed by Psychic Medicine upon the Nature of

Disease

Whatever views may be held as to the vahie of psychotherapy, no
one famiHar with the hterature of psychic medicine can fail to recog-
nize the important additions to our knowledge of the nature of disease
which have come from the latter-day study of the psychological side
of medicine. The enlightenment regarding diagnosis which has thus
been obtained seems to us at times even greater than that with
reference to treatment. We have to-day a much clearer insight than
ever before into the underlying pathology not only of such so-called
functional diseases as neurasthenia, hysteria, and hypochondria, but
also a better appreciation of the cause of the most distressing phe-
nomena of many of the diseases universally accepted as organic. One
of the fruits of modern research into abnormal psychology has been
the addition to our nosology of the disease or afifection known as
psychasthenia. The propagandum in favor of the acceptation of
psychasthenia as a clinical entity, if it has done nothing else, has clari-
fied our views regarding neurasthenia on the one hand, and the so-
celled paranoid states on the other. We have learned better to sepa-
rate the mental from the physical, the incurable from the curable or
relievable, and at the same time have learned to give full credit to
the psychic element in our attempts to treat both neurasthenia and
psychasthenia.

The Limitations of Psychotherapeutics

In eras of excitement and exploitation of special methods of treat-
ment the community at large or even the profession is often carried
beyond the confines of reason. It would be easy to recall many illus-
trations of this fact. The great but unrealized or only partially
realized expectations which were evoked by such discoveries as the
tubercular bacillus, diphtheroid bacillus, and the X-ray may be recalled.
Even the advent of single remedies has sometimes awakened anticipa-
tions so extraordinary as scarcely to be believed in later times after
such remedies have taken their real place, often a most important one.
These remarks apply to such drugs as the bromides and the petroleum
products, or going much farther back, to mercury, the iodides, quinine,
and opium. The thousand and one remedies which have come loudly
heralded and have departed leaving no trace except the memory of a

269

12 MILLS : PSYCHOTHERAPY

dishonored language and of unbenefited patients need no reference
in this connection. I am speaking rather of remedies and measures
of real and permanent value.

Who does not recall the high hopes of the suffering and the un-
thinking which were awakened by the propaganda in favor of the
remedial use of electricity — in the early days by the static machine,
the galvanic pile, and the faradic coil in their then crude and clumsy
forms, and in more recent times by the improved Franklinic machine
with its accessory appliances, by the sinusoidal current or the currents
of high tension or great frequency, by the electric vibrator and all the
rest? Who does not recall the joyful anticipations indulged in by the
very sanguine or the more or less feeble-minded when suspension was
announced as a cure for sclerosis and the correction of eye-strain as
the sovereign remedy for epilepsy and everything else of a neurological
character which could not be reached by any other therapeutic method ?
Indeed some of our ophthalmological enthusiasts have I believe gone
so far as to find in the correction of the errors of refraction the
panacea for the ills of every refractory organ, whether in the brain,
the thorax, the abdomen, or the pelvis. In the medical world remedies
and therapeutic measures become the rage, just as in other worlds
horse shows and bridge, bicycles or automobiles, football or stock
gambling, may become the rage. The rage subsiding, a sane residuum
is left, whether in medicine or in the lay world outside of it.

Coming nearer to our subject, the eras of excitement regarding
mesmerism, animal magnetism or hypnotism, which at intervals would
seem almost to recur like panics, under some occult law in periods of
ten or twenty years, will be suggested. They have come and gone,
doing little good and much harm, and leaving behind them a trail in
which harm and good commingle, the former predominating. When
I speak of harm and good I would limit my meaning to the matter
which concerns us most in this paper, namely, that of the cure or
relief of disease. Scientific results which cannot be measured in the
same balance with therapeutic achievements have come from a study
of hypnotic i)henomena. Some years of experimentation and many
years of observation have led me to the belief, already expressed in
other words in this contribution, that hypnotism as a therapeutic
procedure, while of some value, has its decided hmitations. Psycho-
therapeutics, of which the practice of hy])notism for remedial purposes

270

mills: psvciiotiierapv 13

may be regarded as a phase, has also its decided Hmitations. The
thoughtful physician will recognize and act upon these limitations.

On arising from the reading of some of the chapters in the book of
Du Bois, one cannot help feeling either that this writer is occasionally
over sanguine or mistaken in the permanence of some of his results,
or else that he has powers of healing by reasoning and persuasion far
exceeding those possessed by other mortals. I would not for a
moment question the verity of his reports, but it has seemed to me
with regard to some of them that, like the reports of cures from
operations or from new medicinal remedies, sufficient time has not
been allowed before recording to decide on the real merits of the
treatment. It is true that he tells us of the relapses of his patients
and of his occasional failures, but discounting these, his results still
remain apparently so briUiant that it is hard to reconcile them with our
experience, even making all allowance for the superior powers of the
recorder. Take, for instance, his reports of his successes by his
methods of persuasion in the treatment of insomnia and of some of
the obstinate forms of tic. In these disorders, while such measures
are useful as adjuvants and in rare cases efficient for radical cures,
they will I believe fail even when exercised by those who most fre-
quently from their personality or training are capable of using suc-
cessfully.

Religion and Psychotherapy

It is not at all surprising that religion and the art of healing should
so often be asked to clasp hands. Faith is fundamental to the ex-
istence of sects and creeds. Religious belief is the corner stone on
which religious organizations are built. In medicine faith or belief
in those who dispense the gifts of healing is often as essential as is
faith to the existence of the cliurches. ^Moreover in the manner in
which religion is honored, expressed and enforced suggestion in its
most powerful form is exhibited. The spire, the vaulted ceilings, the
painted window, the mild religious light, the robed priest, the choir,
the genuflections and all the rest which go to make up religious cere-
monies and surroundings are among the most powerful suggesters.
The faith which has made ecstatics and stoics, which has enabled men
to go smiling to the stake, could not be otherwise than a powerful agent
for healing if properly applied or if applied to the proper subject.

It not infrequently happens that the doctor can appeal to the clergy

271

14 mills: psychotherapy

for aid in some special case. Every good physician who is at all in
contact with the priest or the preacher sees at times an opportunity
when the latter can render a real assistance. The clergyman, on the
other hand, at times can turn with advantage to the doctor in behalf
of some mentally or physically disturbed or diseased member of his
flock. This sort of reciprocity exercised in proper manner for indi-
vidual" cases is often to be commended and rarely brings about any-
thing but good results. It is not the same however when, on the
one hand, the role of the physician is played or attempted to be played
in its entirety by the clergyman, or on the other hand — what will be
more readily recognized as true — when the role of the clergyman is
attempted to be played by the physician.

Any man or any organization proposing to deal with the cure of
disease, must be prepared to meet it in its diverse guises. It is true
that we have specialties and specialists in medicine — men who treat the
eye, or the ear, or nose and throat, or the nervous system, but it is
also true that these men must be prepared to deal with diseases of all
sorts as they afifect the tissues and organs of the body which they have
selected for their special line of work. Those suffering from diseases,
functional or organic, acute or chronic, curable or incurable, of certain
organs or parts of the body, have a right to expect enlightened atten-
tion and assistance from the physician who declares himself fitted for
the treatment of these disorders. What would we think of the
ophthalmologist who knew how to treat granular conjunctivitis, but
was entirely at a loss when confronted with glaucoma or cataract ; or
of the neurologist who had a sure remedy for chorea or neuritis, but
was entirely nonplussed by cerebral syphilis or poliomyelitis. Who-
ever announces himself as prepared to treat diseases, to relieve or cure
physical pain and distress, enters into a wide field, in which much is
of right demanded of him. In times gone by the prophets and healers
did not limit themselves to functional disorders of particular type.
Whatever their success, they had the courage of their pronouncements,
and called upon all who were sick to come and be healed. The modern
clerical healer is in this respect, at least, wiser in his day and generation
than his religious forebears. While from the very nature of his
vocation, whether directly or indirectly — not to be irreverent — he may
be said to be handing out doses of Divine Providence, he is careful
to restrict his offering of help to those who are sick only in certain
ways.

272

mills: psychotherapv 15

One of the greatest evils which has resulted in the j^ast and will
always result from the admixture of religion and medicine is that
which arises from the use of suggestion. The man who can suggest
cures — usually temporary — in those who flock to his shrine is likely
also to suggest diseases to be cured by the same methods. As a result
endemics and even epidemics of hysteria follow in the wake of the cure
of sporadic cases of the same disorder when these cures are wrouo-ht
with all the sensational accompaniments of a healing shrine. In the
middle ages and before them, and in well-known historical periods
in more modern times, disease and disorders produced by influence or
suggestion, have multiplied when it has become the fashion to deal
with them outside the medical profession. I repeat therefore that
psychotherapy like medicinal or mechanical or surgical or climatic,
or any other sort of therapy, belongs to the physician and not to the
clergyman, however sincere the latter may be in his idea that it is his
duty -to invade the province of his medical brother.

Harm is done, not alone to the conmumity and to the medical pro-
fession by the psychotherapeutic efforts of enthusiastic but misled
clergymen, but eventually to religion itself. Sooner or later he who is
not cured, whose cries for help are not answered by relief, may come
to doubt the truth of that faith which claims omnipotence for its God.
Every neurologist of any considerable experience has had pass through
his hands many cases of uncured disease in individuals of deep re-
ligious sentiment who have called in their extremity upon Christian
Science or some similar healing cult, and who, failing to receive the
benefit for which they have been led to hope, have lost their faith not
only in religious healing, but also in everything spiritual. The strong-
est opponents of osteopathy, faith-cures and divine healing and all
similar non-medical therapeutic methods or organizations, are those
who, misled by great promises, consciously or unconsciously made,
have come back to be cared for and treated by those who only claim
for themselves the powers which are given to them through scientific
study and experience with disease.

2-3

THE SY^IPTOM-COMPLEX OF A LESION OF THE UPPER-
MOST PORTION OF THE ANTERIOR SPINAL
AND ADJOINING PORTION OF THE
VERTEBRAL ARTERIES ^

By William G. Spiller, M.D.

PROFESSOR OF NEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE
UNIVERSITY OF PENNSYLVANIA

In the June, 1908, number of this journal I described the symptom-
complex of occlusion of the posterior inferior cerebellar artery, as it
appeared to me from a microscopical study of the material from two
cases and the reports of cases in the literature. The symptom-complex
is sufficiently characteristic to make a diagnosis easy. In the paper
referred to the following occurs :

" Wallenburg sums up Duret's investigations on the supply of the
vertebral arteries. Each of these arteries, the left the larger, gives
origin about two cm. below their union to the posterior inferior cere-
bellar artery, and higher to the anterior spinal artery. Branches from
the latter artery enter the raphe and nourish the interolivary bundles,
posterior longitudinal bundles, hypoglossus nuclei and other nuclei
below the floor of the fourth ventricle. The pyramids are nourished
by the anterior spinal arteries and frequently by the vertebral arteries.
[It is understandable therefore that when the anterior spinal arteries
are not occluded the central and anterior parts of the medulla oblon-
gata are not affected.] Branches from the posterior inferior cere-
bellar or vertebral artery supply the lateral portion of the medulla
oblongata."

It has seemed to me desirable to emphasize a clinical picture — the
complement of that caused by occlusion of the posterior inferior cere-
bellar artery — alluded to in my former paper and in my concluding
remarks on the discussion of this paper (see this journal, p. 713) ;
especially as I believe I have observed cases that belong to this group.
I hope that this brief article may arouse sufficient interest to make the
symptom-complex a source of observation and study by others.

' From the Department of Neurology and the Laboratory of Neuropathology
in the University of Pennsylvania.

1 274

spillkr: lesion of vi-:rtei:kal artiiriks 2

The anterior spinal arteries arise from the vertebrals about a centi-
meter before the latter unite to form the basilar. The left anterior
spinal artery is frequently the larger, and according to Buret the right
may be absent. I have found that these arteries sometimes descend
several centimeters before uniting, in other instances they unite shortly
after their origin to form one vessel. Many of the fibers of the
hypoglossus nerves leave the medulla oblongata above the origin of
these arteries. Branches of these arteries supply the median portion
of the medulla oblongata, especially the lemniscus and the anterior
pyramids, although the latter may be nourished also from the vertebral
arteries. Buret believed that branches of the anterior spinal arteries
supply the hypoglossus fibers, but it seems probable to me that this
nerve as well as the vago-accessorius is nourished more from the
vertebral arteries. I have seen complete absence of the right vertebral
and anterior spinal arteries.

The important fact is that the area of supply of the upper part of
the anterior spinal arteries is chiefly tlie anterior and middle portions
of the medulla oblongata. Occlusion of these arteries or hemorrhage
from them, including the adjoining part of the vertebral arteries,
should therefore give a very definite symptom-group. This may be
unilateral or bilateral, depending on the union of the two arteries near
their origin or several centimeters below. As these arteries are side
by side shortly below their origin, even when they do not at once unite,
the symptom-complex is likely to be bilateral ; whereas in occlusion of
the posterior inferior cerebellar artery it is always indicative of an
unilateral lesion.

We should expect to find paralysis of all the limbs, trunk and neck,
whereas the reflexes necessary to life would be preserved. The face
would not be aflfected. The tongue might escape, as the hypoglossus
nerve has at least a portion of its origin above the origin of the anterior
spinal arteries, but it might be involved by implication of the vertebrals.
As the lemniscus probably conveys fibers of deep sensation, this form
of sensation would probably be disturbed. Henschen believes the
lemniscus contains tactile fibers. If this opinion be correct tactile
sensation would be affected. If the symptom-complex were unilateral
the disturbance of sensation referred to and that of motion would
probably be on the same side of the body, as all the fibers concerned
with these functions have decussated at the level in question. If the
lesion extended lower sensation might be affected on the side opposite

275

3 spiller: lesion of vertebral arteries

to that of the disturbance of motion, or even on both sides of the body.
The tendon reflexes are not infrequently lost when the cerebellum or
fibers connecting with it are involved, and as in the lesion under con-
sideration the cerebello-olivary fibers would be implicated, it is possible
that the tendon reflexes would be lost instead of exaggerated, although
this loss might be due to other causes. The vagus and glosso-
pharyngeus should escape, at least in great part. Ataxia probably
would not be observed because of the motor paralysis or the escape
of important cerebellar fibers.

Occlusion of the vessels in the posterior part of the brain is more
likely to occur in syphilitic arterial disease, and transitory quadriplegia
might be caused by temporary interference with this vascular supply.

I am well aware that a certain degree of uncertainty is to be ex-
pected in the description of a symptom-complex such as this without
sufficient clinical and pathological material, and I have delayed the
publication of this article many months. The anatomical foundation
seems to be reliable, and I am not without some clinical observation.
It remains to be seen whether the symptom-complex could be uni-
lateral, as both the motor fibers and the sensory fibers of the lemniscus
decussate at about the level under consideration.

I have been able to study a case in the service of Dr. Mills that
probably belongs to this type : A woman, forty years of age, previously
in good health, attempted to rise from her bed during the night, in
April, 1908. She fell to the floor and had the sensation of having no
legs, as she expressed it, these limbs feeling numb and dead-like. She
was unconscious about two days, and then understood what was said
to her, but could not speak for about three weeks. She had incon-
tinence of urine and feces only during the first few days of the attack.
She has had a dull aching, tired sensation in all the limbs since the
attack. Her speech has been peculiar in that certain words are in-
distinct and she has difficulty in finding the word she wants at times,
and yet she does not appear to be aphasic. She is distinctly weak in
all her limbs and equally so in the limbs of the two sides, although
the upper are weaker than the lower. The grip is much impaired
on both sides, and the fingers are held in partial flexion, but can be
straightened by the patient with difficulty, although the wrists remain
slightly flexed. The toes are scraped a little on the ground in walking,
especially the right, and the lower limbs are slightly spastic ; the upper
are not so. The tendon reflexes of upper and lower limbs are exag-

276

spiller: lesion of vertebral arteries 4

gerated. There are no localized atrophies, and no incoordinate
movements. Sensations of touch, pain and temperature are normal
everywhere, but the sense of position is distinctly impaired in the
toes and fingers, especially on the right side. Babinski's reflex seems
to be present at least on the right side.

The patient is intelligent and is positive that she was in good health
before the attack, which occurred suddenly and affected both sides of
her body simultaneously and ecjually. The escape of the tongue and
face is noteworthy. The important features of the case are spastic
paresis of all the limbs with some disturbance of the sense of position.

1:77

Reprinted from The Journal of the American Medical Association, July i8, 190S,

Vol. LI, pp. 216-219.

American Medical Association, 103 Dearborn Ave., Chicago.

HEMORRHAGE INTO THE VENTRICLES

Its Relation to Convulsions and Rigidity in Apoplectiform

Hemiplegia ^

By Alfred Reginald Allen, M.D.

INSTRUCTOR IN NEUROLOGY AND IN NEUROPATHOLOGY IN THE UNIVERSITY
OF PENNSYLVANIA, PHILADELPHIA

The question to what extent the upper motor neurons are initiative
in their function must of necessity be speculative. But since the ad-
vent of the theory of definite cerebral localization for well-ascertained
function, since the increase of our knowledge of the association tracts
and their importance psychologically, the concept of the upper motor
neuron as a free agent in initiating its own stimulus becomes more and
more untenable. To-day it would seem that the normal motor im-
pulses from the Rolandic region are set in motion from impulses from
other parts of the brain transmitted through association tracts.

Now muscular movement — for example, of the right side of the
body, face, arm, trunk and leg — may be volitional, in which case it
can be considered reflex to psychic stimulation. It may be due to the
local action of some circulating poison, either stimulating the peri-
karyon or its neurons directly, or rendering them so hypersensitive to
afiferent impulses, that, whereas normally there would be no reflex
action, in the hyper-excitable condition there is a discharge of motor
impulse; this is probably the case in uremia. Intracranial pressure,
unless very gradual, is able by direct irritation to cause a motor dis-
charge ; and intracranial pressure as well as irritative lesions acting
on the right cerebellar lobe can cause stimulation of the motor neurons
of the left motor cortex, as in Weber's cases.- Electricity applied to
the motor cortex or the internal capsule is also capable of causing

'Read in the Section on Nervous and Mental Diseases of the American
Medical Association, at the Fifty-ninth Annual Session, held at Chicago, June.
1908.

From the Department of Neurology and the Lahoratory of Neuropathology
of the University of Pennsylvania.

'Weber, L. W. : Gleichseitige Krampfe l)ei Erkrankung eincr Kleinhirn-
hemisphare, Monatschr. f. Psychiat. u. Neurol., 1906, xix, 478.

97S

ALLEN : HEMORRHAGE INTO THE VENTRICLES 2

motor functional activity. Lastly, and from the standpoint of this
work of most importance, stimulation or irritation of the high sensory
system, either of their perikaryons in the optic thalamus or other
basal ganglia, or of their axons in the white matter of the centrum,
is in all probability capable of causing motor impulses — the pure
cerebral reflex.

The subject of convulsions and rigidity in apoplectiform hemiplegia
is mentioned in a casual unsatisfactory way by Dejerine,^ Strumpell,*
Brissaud^ and Leube,*' all of whom state that these symptoms point
to the rupture of the hemorrhage into the ventricles. Nowhere have
I been able to find any attempt at a physiologic explanation on this
hypothesis.

Before considering my cases it is necessary to deal with the question
of the possibility of stimulating the axons of the upper motor neurons
when they are separated from their perikaryons. Weber- denies this
possibility in no uncertain language :'

That a direct stimulation of the pyramidal fiber can cause convulsions or
rigidity is against all experimental and clinical experiences.

Against this ex cathedra dictum of Weber's I would call attention
to the work of Beevor and Horsley,** who succeeded in mapping out
the internal capsule of the Bonnet monkey and the orang-outang by
means of the secondary faradic current.

Hoche, in three articles,^ discusses experimental work that he did
on the cut end of the spinal cord in criminals who had just been be-
headed. He used the faradic current, and although much of his work
was negative and seemingly corroborative of Weber's statement, yet

'Dejerine, J.: Semiologie du systeme nerveux, p. 476.

* Striimpell : Lehrbuch der specielle Pathologic und Therapie, ed. 13, 1900,
iii, 477.

° Brissaud : Traite de medecine, 1894, vi, 53.

'Leube, W. : Specielle Diagnose der inneren Krankhciten. ed. 6, 1901, ii, 22^.

' " Dass eine direkte Reizung der Pyramidenbahn Zuckungen oder Kriinipfe
hervorrufen kann, ist nach alien Experimentellen und klinischen Erfahrnngen
nicht anzunehmen."

^Beevor, Charles E., and Horsley, Victor: An Experimental Investigation
Into the Arrangement of the Excitable Fibers of the Internal Capsule of the
Bonnet Monkey (Macacus Sinicus), Phil. Tr. Roy. Soc. London, clxxxi. B, 49-
88; A Record of the Results Obtained by Electrical Excitation of the So-called
Motor Cortex and Internal Capsule in an Orang-Outang (Siinia satxnts), Phil.
Tr. Roy. Soc. London, 1890, clxxxi, B, 129-158.

" Hoche, A. : Zur Frage der elektrischen Erregbarkeit des menschlichen
Riickenmarkes, Neurol. Centralbl.. 1895, No. 14, p. 754; Neurol. Centralbl., 1900,
p. 994; Ueber Reizungsversuche am Riickenmarke von Enthaupteten, Berl. klin.
Wchnschr., 1900, No. 22, p. 479.

279

3 ALLEN : HEMORRHAGE INTO THE VENTRICLES

in the case where he made his test with least delay after decapitation
his result speaks as positively in the affirmative as Weber's in the
negative. Hoche's first article^*^ says:

According to the previous accounts I had expected little action; therefore,
so much the more surprising was the effect. The corpse, which was lying flat,
raised both its arms, bent at the elbowjoint, and with clenched fists, up in the
air, the thorax raised itself in inspiration (so that with repeated stimulation the
stump 'of the neck began again to bleed, due to the pump-like action of the
thorax) and both legs were in tonic extension.

Hitzig" called attention to the fact that the excitability of the
central nervous system rapidly failed after exsanguination. This
accounts, it seems to me. for many of Hoche's negative results. I
have found the lateral columns of the spinal cord in exsanguinated
dogs incapable of stimulation. Morat^^ says:

Vulpian has devised an experiment on this disputed point which is decisive.
In a rabbit or dog, after it has been put under the influence of ether, he lays
bare the spinal cord for a length of six to ten centimeters above its lumbar
enlargement; he cuts all the roots which correspond to this length (in order
that he may not have to take into account the movements which would result
from this stimulation by the diflFusion of stimulating current). He cuts the cord
in the most anterior portion of the region which has been laid bare and, through-
out the extent of the latter, removes the posterior columns, a portion of the
lateral columns, and as much as possible of the gray matter, in such a way that
the anterior or anterolateral columns (according to circumstances) thus sepa-
rated are only connected with the cord by their posterior extremity. If the
anterior extremity of the columns isolated in this way be pricked or compressed,
somersaults are provoked; that is to say, contractions of the muscles of the
hind quarters of the animal and movements of the tail. If, by following the
anterior fissure, the two anterior columns be separated with exactitude, the one
from the other, by a longitudinal incision, it is observed, by stimulating one
of them, that movements much stronger ensue in the corresponding limb than
those arising in the limb of the opposite side.

Vulpian^^ gives a very similar experiment in his " Lecons sur la
phvsiologie generate et comparee du systeme nerzrux,'' but I have not
been able to find the original of Morat's quotation.

" " Nach den bisher vorliegenden Angaben hatte ich mir wenig Wirkung
versprochen ; urn so uberraschender war der eintretende Effect : der flach liegende
Leichnam hob beide Arme mit gebeugtem Ellenbogengelenk und geballten Faus-
ten in die Hohe, der Brustkorb hob sich inspiratorisch (so dass bei wieder-
holter Reizung, Dank der Pumpwirkung des Thorax, der Halsstummel wieder
anfing. zu bluten) und beide Beine geriethen in Strecktonus."

" Hitzig. Edward : Physiologische und klinische Untersuchungen uber das
Gehirn, Berlin. 1904, part i, p. 23.

'■ Moral. J. P. : Phvsiologv of the Nervous System, 1906. p. 279.

" Vulpian. A. : Lcgons sur la physiologic generale et comparee du systeme
nerveux, Paris, 1866. p. 360.

280

ALLEN : HEMORRHAGE INTO THE VENTRICLES 4

I have been unable to cause convulsive movements by stimulating
electrically the lining of the lateral ventricles of the dog.

The pathologic material of the following cases has been generously
given to me by Dr. Spiller, to whose kindness I am very deeply in debt.

Case i— Patient.— H. ]., female, aged 44, while doing her housework on the
morning of Jan. 4, 1905, became faint and sat down on a chair, shortly after-
ward falling to the floor unconscious.

Examination.— At the Philadelphia Hospital on the same day a right hemi-
plegia was deinonstrated. The right upper limb was flexed at the elbow joint
and efforts to straighten it met with much resistance. Both limbs on the right
side could be moved but much less freely than the limbs of the left side. The
patient's speech was thick and unintelligible and her answers were made in
monosyllables. Her mouth was drawn to the left. In testing the reflexes it
was doubtful whether any patellar tendon jerk was present on either side.
Ankle clonus could not be elicited. The Babinski reflex was present. Although
the patient had a tendency to stupor when brought to the hospital, yet she could
be aroused and answered questions sensibly.

Clinical History.— At 11 a. m., January 15, the patient began to have
convulsive attacks with unconsciousness. The convulsions began in the right
side of the face, then extended to the right arm, and after that to the right
leg, the head being turned to the left and the eyes to the right. These con-
vulsive seizures lasted from three to five minutes. Later the convulsions be-
came alternate, first on one side and then on the other. The side which was
not involved remained absolutely flaccid. Rarely the convulsion involved both
sides at once. The tongue was bitten frequently. The levator palpebrarum
muscles, the occipitofrontalis and all the facial muscles were implicated in these
convulsive seizures. At times the mouth was drawn strongly to the left but
never to the right.

At 5 p. m. of this day a luni1)ar puncture was performed and about 15 c.c.
of a reddish cloudy fluid were withdrawn. This at first dropped quite rapidly
but was not apparently under much pressure. By this time the convulsions had
become milder, but were still alternating on each side. The patient died Janu-
ary 15, the day of the onset of the convulsions.

Autopsy. — On horizontal section a large hemorrhage of recent development
was found in the left lenticular nucleus. It extended into the internal caosule
and had broken into the lateral ventricle at its anterior end. The hemorrhage
was found in both lateral ventricles but chiefly the left. The extent of the
lesion posteriorly was such that it involved the entire posterior limb of the
internal capsule and the posterolateral part of the optic thalamus.

The clinical history of this case would indicate that the hemorrhage was of
very gradual development. The convulsions on the left side were probably
due to irritation of the right motor cortex from increased intracranial pressure.
Case 2.— Patient.— C. S., male, aged 50, laborer, white, was brought into the
Philadelphia Hospital unconscious and without data as to past history. Soon
after admission (Oct. 2, 1906) he began having general convulsions involving all
limbs, but the left side somewhat more than the right.

281

5 ALLEN : HEMORRHAGE INTO THE VENTRICLES

Examination. — ^When tested between convulsive seizures the right arm would
drop flaccidly when raised, but the left arm would be replaced by the patient.
The notes recorded under date of October 4 state that there was present con-
jugate deviation of head and eyes to the right. When the right arm and leg
were pricked with a pin there was no movement on the right or left side. On
pricking either limb on the left side, the limb so irritated was drawn up. The
patellar tendon jerks on the right side were marked + and on the left side
-j- -|-. There was no ankle clonus on the right side and a quickly disappearing
ankle ctonus on the left. The Babinski reflex was present on both sides. The
left leg tended to develop a general clonus when the tendon reflexes were
examined. There were involuntary evacuations of the bladder and rectum.
By testing the patient with his feeding-cup a right lateral homonymous hemia-
nopsia seemed to be present. The left pupil was larger than the right and
both reacted to light.

Autopsy. — A horizontal section made through the left cerebral hemisphere,
at a level just above the tenia semicircularis, showed an extensive hemorrhage
entirely destroying the left lenticular nucleus at this region and breaking into
the left lateral ventricle anteriorly and posteriorly and encroaching mesially on
the optic thalamus. A section one centimeter below this showed the site of
the hemorrhage moved outward, the head of the caudate nucleus at this level
being uninvolved, the lateral part of the putamen being the farthest encroach-
ment medianward on the lenticular nucleus. There was at this level a slight
involvement of the posterior part of the posterior limb of the internal capsule.

Case 3. — History. — J. E., male, aged 28 years, white, five weeks prior to his
admission to the Philadelphia Hospital, Sept. 16, igo6, took a dose of quinin,
he thought about fifteen grains, for " chills and fever " from which he had
been suffering about one' week. A " couple of hours " after this dose he felt
very sleepy, lay down, became unconscious and remained so for three days.
When he regained his consciousness again he was paralyzed on the left side,
his speech was thick and he could not see clearly. He regained a fair amount
of power in this paralyzed side. Although he had complete incontinence of
bladder and rectum for one week after the attack yet it is recorded that his
condition had so far improved in this respect that if he attended to himself at
once when he felt the inclination he could control his sphincters. On Nov. 16,
1906, he began to have repeated convulsions. These followed one another
with irregular intermissions until he died the same day. There is no descrip-
tion of the convulsions on his record.

Autopsy. — The brain bulged considerably in the right frontal and right
frontoparietal regions with flattening of convolutions and obliteration of sulci.
On horizontal section there was found a large hemorrhage which had in all
probability started in the lenticular nucleus on the right and had torn its way
both anteriorly and posteriorly into the lateral ventricle. The entire ventricular
system, right and left, was filled with blood. The aqueduct of Sylvius and the
fourth ventricle were distended and filled with blood. The anterior part of the
callosomarginal region on the right had become greatly distended and had
encroached on the left hemisphere. The destruction of tissue in the right cere-
brum was very great and involved the anterolateral part of the optic thalamus.

282

ALLEN: HEMORRHAGE INTO THE VENTRICLES 6

There was also some destruction of tissue in the left cerebrum, chiefly about
the anterior extremity of the lateral ventricle.

Case 4. — History. — W. L., male, aged 52, had an apoplectic attack in Novem-
ber, 1903, with unconsciousness, after which he was hemiplegic on the left side.
He was taken to the Hospital of the University of Pennsylvania. The uncon-
sciousness lasted twenty-four hours, during which time there was a convulsive
seizure. In June, 1904, he had another attack of unconsciousness with convul-
sions and after this, three similar attacks about six weeks apart. He became
able to walk afterward but had only a little control over his left hand. On
Aug. 8, 1905, he had about fifteen convulsions in which the right arm was in
clonic spasms, preceded by a conjugate deviation of the head and eyes to the
right. The convulsive condition then affected the left arm and the lower limbs.
The whole body was then the subject of clonic spasms followed by a brief period
of tonic contraction. Apparently the facial muscles were equally involved. On
Nov. 20, 190S, the patient slipped and struck the right side of his forehead
against a door. He was put to bed in a perfectly conscious state, but gradually
lost consciousness. In about Jnalf an hour after he was unconscious he began
having convulsions starting in the left arm and extending to the left leg, left
side of the face and finally over the entire body. He had a number of these
convulsions following one another in rapid succession and lasting from one-
half to two minutes. At times there existed almost a tonic condition. These
convulsions commenced about i 130 p. m. and the patient died at 5 p. m. The
convulsions stopped some time before death and Cheyne-Stokcs respiration
set in.

Autopsy- — The hemorrhage probably started in the putamen of the right
lenticular nucleus. Mesially it had encroached on and involved the internal
capsule and optic thalamus. Posteriorly it had torn through the posterior part
of the posterior limb of the internal capsule into the lateral ventricle. Ex-
ternally it had torn through and completely obliterated the external capsule and
was in close juxtaposition to the cortex of the island of Reil. Anteriorly it had
torn through the white matter which forms the outer wall of the extreme end
of the anterior horn of the lateral ventricle and had likewise passed externally,
via the white matter, past the anterior extremity of the insula, to within one
centimeter of the cortex of the foot of the third frontal convolution. All the
ventricles were filled with blood.

Case 5. — Patient. — C. H., male, aged 80, born in Germany, was brought to the
Philadelphia Hospital unconscious on March 6, 1906.

Examination. — The right arm and leg were paralyzed, the face was slightly
drawn to the left and the right nasolabial fold was almost obliterated. The
right eye was not completely closed and offered no resistance when an attempt
was made to open it. There was conjugate deviation of head and eyes to the
left. The tongue in the mouth deviated to the left, protrusion, of course, being
impossible. The right upper limb was flaccid but the right lower limb was
spastic.

Clinical History. — At first there was right hemianesthesia Init this was of only
short duration, as Dr. Spiller made a note on March 7 to the effect that a
pin prick was perceived in the right side of the face and in the right upper and

283

7 ALLEN : HEMORRHAGE INTO THE VENTRICLES

lower limbs. By March 8, the right upper limb had become spastic. Slight in-
vohmtary jerkings in the right lower limb were also noted. The stupor was
too great to make any test for hemianopsia. On March lo the right hemian-
esthesia was again noted. The patient died March ii, 1906.

Autopsy. — The hemorrhage, which was in the left cerebral hemisphere was
studied in three horizontal planes about one centimeter apart. The highest sec-
tion was at the level of the top of the lenticular nucleus, and the hemorrhage
here involved the external and posterior part of the thalamus and the white
matter external to it. There was only a thin layer of white matter between
the hemorrhage and the cortex of the highest part of the insula. The section
one centimeter below this showed that the hemorrhage had involved the pos-
terior half of the lenticular nucleus and had also cut the posterior two-thirds of
the posterior limb of the internal capsule. In the lowest section, one centi-
meter below the preceding, there still remained a small hemorrhagic area involv-
ing the posterior edge of the inner portion of the pvitamen and also the posterior
edge of the globus pallidus and destroying the posterior extremity of the pos-
terior limb of the internal capsule and here breaking into the lateral ventricle.

Case 6. — Patient. — N. B., male, aged 60, white, was admitted to the Phila-
delphia General Hospital, April 4, 1903. The diagnosis of his condition on
admission was pseudobulbar palsy and his previous history and examination are
so foreign to the subject at hand that I shall omit them, passing at once to the
record of relevant facts.

Clinical History. — On Sept. 4, 1904, the patient complained of feeling bad and
was apprehensive that something was going to happen to him. On September
S, the orderly noticed that the patient was trembling much more than usual when
being put to bed. At 6:30 p. m. of that evening the patient had a convulsion
lasting about three minutes. There were several convulsive seizures following
this first and lasting about the same time, but they are not described, so the
question of consciousness, location and character can not be determined. At
7:15 p. m. the patient had an attack lasting about one hour, in which the entire
right side of the body was in a state of clonic convulsion, the left side remain-
ing free from movement. During this convulsion he was unconscious, bit his
tongue and frothed at the mouth.. After this seizure he remained quiescent until
I a. m., Sept. 6, from which time to 7 a. m. of the same day he had twelve
convulsions.

He had, in addition to unconsciousness, conjugate deviation of his head and
eyes to the left. A pin prick on either side of the face caused movement of the
face, but if the limbs were pricked there was no response. Dr. Spiller's notes
record that when the right upper limb was raised and allowed to fall it did so as
though dead. Both upper limbs were spastic, though the right was more so than
the left. The biceps tendon jerk was exaggerated in both sides. When the left
upper limb was stuck with a pin there was voluntary movement, but the right,
under the same conditions, exhibited no motion. Both the lower limbs were
spastic, both had exaggerated patellar tendon jerks and Babinski reflex. There
was persistent ankle clonus on the right but not on the left side.

Autopsy. — Examination of the brain revealed a small cortical and subcortical
hemorrhage just posterior and inferior to the angular gyrus. This lesion passed

284

ALLEN: HEMORRHAGE INTO THE VENTRICLES 8

inward to within a few millimeters of the lateral ventricle. The optic thala-
mus was uninvolved.

Case 7. — Patient. — J. W., male, aged t,/, white, was admitted to the Phila-
delphia Hospital on May 10, 1905, suffering from tabes dorsalis. His previous
history and physical examination as to his tabetic trouble I shall omit.

Clinical History. — He was allowed to go out on July 4, and next day, July 5,
he was brought back by the police. He had been drinking and developed that
evening a right-sided hemiplegia without aphasia or loss of consciousness.
Until December there was nothing of moment to record except that his hemi-
plegic condition was much improved. On the evening of December 12 he fell
unconscious. His head and eyes were rotated toward the right. On December
14, it is recorded that the face was drawn toward the right and that the left
nasolabial fold was obliterated. A weakness in closing the left eye was also
noted. The tongue had a fine tremor and deviated to the right. The power in
the upper extremities was diminished. The grip was much weaker in the left
hand than in the right. The left upper extremity was spastic and there was
complete loss of power in the^left lower limb with flaccidity. He was able to
move the right lower extremity freely. The Babinski reflex was present on the
left side. All the other reflexes of the lower limbs were absent presumably on
account of the tabetic lesion. The patient gradually lost ground and without
exhibiting any additional symptoms worthy of note died on December 26.

Autopsy. — A horizontal section through the cerebral hemispheres just below
the highest part of the fornix showed a hemorrhage involving the fibers which
in a section lower down, if the optic thalamus and lenticular nucleus were cut,
would form the posterior part of the posterior limb of the internal capsule.
This hemorrhage had broken through into the lateral ventricle posteriorly. The
center of this hemorrhage was evidently well above this level, possibly posterior
and mesial to the roof of the body of the lateral ventricle. The posterior part
of the corpus callosum was involved in this hemorrhage.

In a section one centimeter below this, there was found in the posterior
part of the putamen of the left lenticular nucleus an old cyst which evidently
caused the right hemiplegia. On the right side there was found a cyst the size
of a kidney-bean involving the anterior part of the putamen of the right
lenticular nucleus and encroaching on the external capsule.

Case 8. — Patient. — G. R., male, aged 77, white, born in Germany, was ad-
mitted to the Philadelphia Hospital on July 19, 1904, with the history that he had
had the night before a sudden attack of unconsciousness with a left hemiplegia.
There was slight deviation of the eyes to the right, the left side of the face was
smoother than the right and the tongue deviated toward the left. The reflexes
were all lost on the left and the Babinski reflex was present on the left and
absent on the right. He could not perceive when stuck with a pin in his left
upper or lower limb. He died on July 25, 1904.

Autopsy. — A horizontal section through the right cerebral hemisphere just
below the corpus callosum showed a hemorrhage destroying the posterior part
of the lenticular nucleus and most of the posterior limb of the internal capsule,
especially its posterior part. A section one centimeter below this showed the

285

9 ALLEN : HEMORRHAGE INTO THE VENTRICLES

hemorrhage extending for about one centimeter into the temporal operculum.
The optic thalamus was uninvolved.

Case 9. — History. — I quote from two letters written to Dr. Spiller by Dr.
Thomas R. Neilson, in regard to the patient, jNliss H., aged 50: "I was called to
see her between 9 and 10 o'clock on the evening of April ig, 1904, and found
her in bed, completely unconscious. And from her brother and his wife I got
the information that she had fallen down in her room some time earlier that
evening. When I saw her, her condition, as well as I now remember it, was as
follows: Complete unconsciousness, complete paralysis of all the extremities;
no response to pricking fingers or toes with a pin ; pupils did not respond to
light and were moderately contracted, respiration puffing but not accelerated ;
pulse 84 with no marked degree of tension. The mouth was, I think, slightly
drawn to the left, but I will not be positive of this. In a short time, say twenty
to thirty minutes, I recollect that pricking the fingers of the left hand excited
muscular contraction of that arm resulting in flexion of the elbow. I saw Miss
H. a second time on April 21, at about noon; she was then unmistakably dying.
Paralysis was complete, temperature elevated, pulse weak and frequent, respira-
tions labored and stertorous. She did die that day." There was no history of
convulsions in this case.

Autopsy. — I quote from Dr. Spiller's postmortem record: "A large hemor-
rhage was found filling the whole of the left lateral ventricle and not extending
into the right ventricle ; the substance of the brain was implicated in the lesion
only near the anterior part of the left lateral ventricle. The attack had lasted
forty-eight hours, and convulsions are said to have been absent."

Case 10. — Patient. — W. U., male, aged 72, white, born in England, presented
a history which does not bear on the question at hand except that he had
been an excessive user of alcohol and had had sugar in his urine for the past
fifteen years.

Previous Attack. — The patient retired at his usual time on the night of
March 14, 1905, and was found next day at noon lying at the side of his bed
in a semi-conscious condition with a left hemiplegia. In this condition ho was
sent to the Hospital of the University of Pennsylvania.

Examination. — Dr. Spiller's notes record that there was a semi-stupor and
that the left side of the face was flattened, the nasolabial fold on that side
having disappeared. The eyelids on the left could not be closed as firmly as on
the right. The forehead wrinkled better on the right than on the left side.
The tongue deviated to the left when protruded. The masseter contracted
equally well on both sides. The left upper limb fell lifeless. The patient made
no movement with his left lower limb. He seemed to present a left homony-
mous lateral hemianopsia for when his feeding-cup was brought to him from
his left side he made no attempt to take it. Sensation to pin-prick on left side
was preserved. The biceps and triceps tendon jerks were not very prompt on
either side. The patellar and Achilles tendon jerks were lost on both sides.
The Babinskki reflex was present on the left but not on the right.

Without any marked change except deepening stupor and Cheyne-Stokes
breathing the patient died on March 26, twelve days after his attack. There is
no record of convulsion.

286

ALLEN: HEMOkUOAGE INTO THE VENTRICLES 10

Autopsy.— On horizontal section there was found a hemorrhage hounded
anteriorly by a line passing through the genu of the internal capsule. Pos-
teriorly the hemorrhage had broken through the posterior part of the optic
thalamus into the lateral ventricle. The posterior limb of the internal capsule
was destroyed.

Summary

Four patients (Nos. i, 2, 3, 4) had convulsions, rupture of the
heinorrhage into the ventricles and involvement of the optic thalamus.

One patient (No. 5) had rigidity, rupture of the hemorrhage into
the ventricles and involvement of the optic thalamus.

One patient (No. 6) had convulsions, no hemorrhagic rupture into
the ventricles but an involvement of the cortex and subcortical white
matter of a sensory region.

Three patients (Nos. 7, 8, 9) had no convulsions and no optic
thalamus involvement. Two of these (Nos. 7 and 9) had ventricular
inundation ; one of them had not.

One patient (No. 10) had no convulsion and had optic thalamus
involvement. (It is but just to say that this last case was not suffi-
ciently under observation so that it could be said with certainty that
there was no convulsive action.)

My conclusions are :

1. Ventricular inundation in cerebral hemorrhage has no etiologic
bearing on the convulsions of rigidity.

2. Purely tentatively and basing my opinion on the findings in these
ten cases, I concluded that convulsions and rigidity in apoplectiform
hemiplegia are frequently due to an involvement of the optic thalamus,
or the corticothalamic sensory fibers, with the necessary proviso that
enough of the posterior limb of the internal capsule remain uninjured
to convey the motor impulses.

3. Convulsions and rigidity in apoplectiform heiniplegia may be
caused by a sudden or rapid increase in intracranial pressure due to
cerebral hemorrhage, even though the optic thalamus and the cortico-
thalamic sensory fibers are uninvolved. In this case, as above, there
must be a sufficient preservation of the motor part of the internal
capsule for the transmission of impulses to the parts concerned.

4. It is altogether unlikely that pressure or chemical change acting
on the motor axons of the centrum or internal capsule — these axons
having been severed from their perikaryons by the hemorrhagic process
— could exert a stimulating action sufficient to cause convulsions or
rigidity.

Reprinted from the Journal of Xervous and ]\Iental Disease, 1908.

ACQUIRED SPASTICITY AND ATHETOSIS.

By William G. Spiller, M.D.

Haupt' remarks that idiopathic or primary athetosis is relatively rare.
Lewandowsky distinguishes between acquired athetosis and similar forms
developing after hemiplegia. The former is not merely a post-hemiplegic
athetosis affecting both sides, or a result of infantile spastic diplegia, but is an
independent peculiar disease, whose pathologj- is not definitely known, but prob-
ably consists of bilateral cerebral lesions. Oppenheim also makes the distinc-
tion. Previous diplegic disturbances should be excluded if the term primary
double idiopathic athetosis is employed. In a case reported by Haupt small
cortical foci were found in the left parietal lobe, and seemed to indicate a
previous encephalitis. A few cases with necropsy in the literature are referred
to by him.

Dr. Spiller presented a patient, a boy tVv-elve j-ears old, in whom during the
past five years he had observed gradually developing spasticity of all the limbs
with athetosis, reaching finalh" such an intensity that the patient was confined
to his chair. The patient first came under Dr. Spiller's observation December
15, 1902. At that time he was seven years old. He has been under Dr. Spiller's
care at intervals since 1902. The following history was obtained in 1902. He
was the first born child. The birth was easy and normal. He was said to have
had convulsions when four months old. The father stated that the boy walked,
ran and jumped as other children until four months previously, but since that
time had gradually been getting lame in the left lower limb, and had been
obliged to wear a brace during the previous four weeks. He had not had any
pain but occasionally had some tremor of the upper and lower limbs.

An examination showed that the boy was unable to stand without supporting
himself by bending back the knee. When he attempted to walk the feet were
wide apart, the knees were close- together and the lower limbs became spastic.
There was no spasticity of the limbs when the boy was at rest. The lower limbs
were somewhat weak when he was walking, but very little if at all when he was
sitting. The grip was good in each hand, and the voluntary power of both
upper limbs was good. The patellar reflexes were prompt but there was no
clonus. The plantar and Achilles reflexes were normal. \\ hen lying down the
lower limbs showed no spasticity on voluntary movement. Sensations to touch
and pain were normal. Each thigh could be moved passively freely. What
weakness was present seemed to be in the extensor muscles of the back and
hip. Electrical reactions in the lower limbs were normal, but in August, 1903,
a quantitative decrease was observed. Scoliosis was present when the boy was
standing. The calf muscles were not enlarged.

At that time the diagnosis was very difficult : by some the condition was
regarded as muscular dystrophy, a diagnosis which Dr. Spiller never accepted,

' Deutsche Zeitschrift fiir Nervenheilkunde, Vol. 33, Nos. 5 and 6. p. 464.
1 288

spiller: acquired spasticity and athetosis 2

and the variahility in the gait from time to time suggested a hysterical element.

The notes of an examination made by Dr. Spiller January 13, 1908, are as

follows : The lower limbs are very spastic but at times this spasm yields, so that

linn-

^5i

^^^HV/

4k^

»••*

"''■"^- 4

^^^. ^'-

taiT

■

ii.

Photographs made by Dr. A. R. Allen in igoS, with very rapid exposure, showing
the extreme spasticity of the limbs.

the limbs can be moved at most of the joints quite freely, thougii not to the
full extent. The right lower limb is usually kept extended, with the foot in

28:)

3 SPILLER : ACQUIRED SPASTICITY AND ATHETOSIS

equino-varus position. The varus position can be overcome, but the contraction
of the Achilles tendon is so great that the foot cannot be flexed at a right
angle with the leg. The big toe is hyperextended. The left lower limb is
partially contractured in flexion at the knee, and the left foot is extended to
the full degree with slight tendency to varus position. The varus deformity is
not so intense as in the right foot. The contracture of the Achilles tendon here
also is so great that the foot cannot be flexed at a right angle with the leg. The
lower limbs are not distinctly wasted, but are poorly developed on account of
disuse. The boy usually lies with the left leg flexed on the left thigh, lying
upon the leg. When he is entirely at rest voluntary jerkings occur only occa-
sionally. But any passive or voluntary movement causes involuntary jerkings
of portions of the upper and lower limbs resembling athetosis, seen especially in
the lower limbs in the right big toe which is slowly and repeatedly hyper-
extended, very much as in athetosis. The spasms of the lower limbs are in-
creased b}' passive movements. The patellar tendon reflex is exaggerated on
the right side and probably also on the left. The spasm of the muscles prevents
the movements of the legs. The x\chilles tendon reflexes are probably exag-
gerated, though the full degree of the exaggeration cannot be determined. The
Babinski sign is very distinct on each side, more so on the right. Ankle clonus
is impossible because of contracture of the calf muscles. He moves the right
lower limb in toes, knee and hip, but with much diminished power, and he has
no movement at either ankle. The movements of the left lower limb are pre-
served in the toes and at the knee. The movements of the knee consist of
slight flexion and extension. He has no voluntary movement of the left hip.

Touch, pain and temperature (heat and cold) sensations are normal in all
parts of the body. The trunk is greatly deformed. When the shoulders are
placed fairly on the bed the body is so distorted that the boy lies on the right
hip with the left hip elevated. The scoliosis is extreme with the concavity
towards the left in the lumbar region.

The right upper limb is moved at all parts and at all joints, but with much
diminished power. There is spasticity of the upper limbs and all movements are
exceedingly incoordinate with increased spasm on movement, resembling
athetosis. There is no contracture in either limb. The limbs are not muscular
but are not atrophied. The biceps and triceps tendon reflexes cannot be de-
termined on either side because of the spasticity. The left upper limb is moved
at all the joints, but with much diminished power, and is weaker than the right
upper limb. Incoordination on voluntary' movement is extreme, and the athetoid
movement of the left hand is very pronounced on any voluntary movement, and
occasionally when the patient is at rest. The left upper and lower limbs are
more involved than are the right limbs.

The pupils are equal and respond promptly to light and convergence. The
extra ocular muscles are normal. The tongue is normal. Facial nerve supply is
normal on each side. There seems to be no positive involvement of the cranial
nerves.

The boy is very intelligent.

Dr. Spiller expressed the opinion that the condition was probably the result
of progressive involvement of the pyramidal tracts.

290

SOFTENING OF THE DENTATE NUCLEI CAUSING SYMPTOMS
OF CEREBELLAR TUMOR.

By William G. Spiller, M.D.

The patient, a male aged eighteen years, was seen by Dr. Spiller about April
23, 1907, in consultation with Dr. M. H. Bochrock, from whom the following
history was obtained. The boy had been in fairly good health until about one
year previously, at which time severe headache began. The pain was felt in
the entire head and most severely in the occipital region. He had some ataxia
in walking and would fall, especially to the right. He was very deaf, had
vomited during several months, had much vertigo and divergent strabismus.
Venereal disease was denied.

An examination of the eyes by Dr. James A. Kearney, April 4, 1907, gave the
following results : " Media clear, the disc protrudes from the posterior wall of
the eye very similar to the apex of a thimble (ampulliform). The vessels of
the apex of the disc are engorged, especially the veins, and about four milli-
meters of their length is plainly seen. The vessels are then enveloped in the
disc tissue and emerge at the base where they are of normal character. The
difference in refraction between the apex and the base of the disc is two
diopters. The refraction of the fundus is sphere plus five diopters. The char-
acter of the retina is normal l)Ut slightly irritable. The above examination is
of both eyes."

The patient's condition at the examination by Dr. Spiller was as follows :
when sitting in a chair the head was thrown far backwards, the neck muscles
were stiff, the seventh, twelfth and fifth nerves were not implicated, deafness
was intense and bilateral, stupor was pronounced, the iridic reflex to light was
very feeble if present at all in either eye, the eyeballs were moved in all
directions, but it was impossible to get the patient to make extreme move-
ments of the eyeballs in any direction, nystagmus was not observed, ataxia
was present in each upper limb, sensation to pin prick was preserved all over
the body, the limbs were not weak, the patellar reflexes were lost even on rein-
forcement, the Achilles jerk was feeble on each side, the boy was unable to
stand alone and would fall backwards if not supported, hcmiasynergia and adia-
dococinesia could not be tested for because of the stupor, the corneal reflex was
present, the Babinski reflex was very uncertain.

The diagnosis of a lesion of the cerebellum was made and as the symptoms
indicated that the progress was gradual in development, a tumor was supposed
to be present.

Decompression was performed by Dr. Nassau and was followed rapidly by
death on April 25, 1907.

Only the cerebellum and a portion of the pons were obtained for examina-
tion. A cavity was found in the interior of the right dentate nucleus, and the
1 291

2 spiller: softening of the dentate nuclei

left dentate nucleus did not appear to be normal. The small vessels of both
cerebellar lobes near and m the dentate nuclei were much congested, and
numerous small hemorrhages were found about them with some perivascular
round cell infiltration. The vessels of the pons were congested and here also
a few small hemorrhages and slight perivascular round cell infiltration were
found. As the necropsy was necessarily so incomplete it was impossible to say
whether any other intracranial lesion was present or not.

This case in its findings resembles the case reported by E. F. Buzzard in
Brain, Vol. 29, p'. 508, in which thrombosis affecting, and probably destroying the
functions of, the' dentate nuclei was found.

292

A BRAIN TUMOR LOCALIZED AND COMPLETELY

REMOVED, WITH SOME DISCUSSION OF

THE SYMPTOMATOLOGY OF LESIONS

VARIOUSLY DISTRIBUTED IN

THE PARIETAL LOBE.

From the Department of Neurology of the University of Pennsylvania.

By Charles K. Mills, M.D.,

PROFESSOR OF NEUROLOGY IN THE UNIVERSITY OF PENNSYLVANIA; NEUROLOGIST
TO THE PHILADELPHIA GENERAL HOSPITAL, AND

Charles H. Frazier, M.D.,

PROFESSOR OF CLINICAL SURGERY IN THE UNIVERSITY OF PENNSYLVANIA;
SURGEON TO THE UNIVERSITY HOSPITAL.

The rapidity with which the symptoms indicating- serious
cerebral disease developed ; the presence of a well-defined symp-
tom-complex showing a lesion situated at the junction of the
parietal and occipital lobes, and the complete success which re-
sulted from surgical interference, mark the case which is the
basis of this paper as one of the most instructive that has yet
appeared in the literature of cerebral localization and intracranial
surgery. For the opportunity of seeing the case, we are indebted
to Dr. H. a. Spangler, of Carlisle, Pa., who before it was seen in
consultation, had recognized the nature and probable location oi
the disease from which the patient was suffering. We shall first
present the clinical history of the case, including a resume of the
operation with comments, and shall then briefly discuss its bear-
ings upon the subject of the focal diagnosis of lesions, especially
tumors variously distributed in the parietal lobe.

The patient was a married woman, forty-five years old, who
had enjoyed average good health until October, 1907, although
two to three years before she had had several attacks of what she
described as dizziness or lightness in the head. She had not
however suffered with headache, nausea or vomiting. She went
to Philadelphia on October 26, 1907, and while there had an
attack which she regarded as a bilious spell, and in which she
suffered from dizziness, nausea, and vomiting, but no headache.
About a week later she had a severe attack of vertigo or dizziness,
in which she did not fall or lose consciousness. One week after

75 CHARLES K. MILLS AND CHARLES H. FRAZIER

this she had another attack of dizziness, with a little headache,
but without nausea and vomiting. She also at this time showed
a little forgetfulness of some details in packing her trunk.

During the few days following she did not feel very well,
occasionally being a little dizzy and having headache. On Satur-
day, November 30, while in Philadelphia she was in fairly good
health, but on her way home the next day she was very sick at
her stomach and suffered much from headache and dizziness in
the cars. She reached home feeling very badly.

On November 30 she almost ran into a tree, and later into
a post. These objects were on her left. On the same and the
following day she failed to notice persons passing her on her left.

After her return to Carlisle, Dr. Spangler discovered the ex-
istence of something wrong with her sight, and with her nervous
system and at his suggestion she went to Philadelphia to see Dr.
G. E. de Schweinitz, whom she first saw December 19, 1907.
The report of Dr. de Schweinitz, made at about this time, showed
the presence of left lateral homonymous hemianopsia, and begin-
ning optic neuritis. The fields are shown in Fig. i.

She returned to Carlisle, where she continued under the treat-
ment of Dr. Spangler, and where she was seen by Dr. Mills in
consultation December 30. In the meantime, during the month
of December, before she was seen by Dr. Mills, she had headache,
worse at times, and was occasionally dizzy. About this time and
later she had every two or three days attacks of nausea and vomit-
ing, with dizziness and headache. During the night she would
suffer with headache, and in the morning would be sick at her
stomach and vomit, wath some dizziness, the headache continuing
but abating somewhat at noon and returning again in the night.
She began to drag the left leg a little, and did not use the left
hand and arm as skilfully as the right, this impairment and
awkwardness varying considerably at dift"erent times. Ever since
her first symptoms were noticed in October, the patient had had
a peculiar puffing sound in the left ear, and a singing or buzzing
sound in the fight, although her hearing had not changed. Her
memory and powers of attention continued good.

On December 30, when the patient was examined by Dr. Mills
in conference with Dr. Spangler, at Carlisle, the chief cerebral
symptoms presented were left lateral homonymous hemianopsia ;
quite moderate hypesthesia of the left extremities ; hypastereog-
nosis on the same side as tested in her hand, some ataxia with
atactic tremor of the left upper extremity ; impairment of the
ability to use her left leg and arm, this being due to incoordination
rather than to loss of strength. Central vision was little if at
all affected ; she had no word deafness or word blindness ; no
aphasia, sensorv, motor, or mixed. Her reflexes were not ab-
normal, or only slightly so, the Babinski response being absent
and the response to plantar stimulation being slight or not present

A BRAIN TUMOR LOCALIZED AND REMOVED

77 CHARLES K. MILLS AND CHARLES H. FRAZIER

The patient continued at her home under the care of her family
physician from December 30 until February i. From time to
time reports regarding her were made by Dr. Spangler, these
showing a very gradual increase in the symptoms indicating the
cerebral lesion. Examinations made of her urine during this
time showed no albumin and only a few hyaline casts. During
this period Dr. J. Walter Park, of Harrisburg, made three sepa-
rate reports regarding her eyes, each revealing about the same
condition, except that the last one showed some increase in the
optic neuritis. The ophthalmic findings of Dr. Park summarized
were as follows : Vision in the right eye was 20/30 and in the left
20/15 ; she read number 4 minion type with right eye and number
2 pearl with the left eye. Her field of vision of each eye showed
a hemianopsia of the nasal side of the right eye and of the tem-
poral side of the left eye. The ophthalmoscope showed consider-
able optic neuritis ; the veins were tortuous and full, sometimes
disappearing under the edematous retina, and then reappearing.
At Dr. Park's second examination he found two fatty or albumi-
noid spots, three or four mm. in size, showing in the right eye, one
slightly below, and the other above the disc. They had no well-
defined edges, were flame-like in appearance, and fairly whitish.

During January she was under treatment with iodide and
bromide in moderate doses. This treatment had indeed been tried
previously, but her stomach did not stand it well and she made
no improvement under its use.

After a conference by letter it was decided to bring the patient
to Philadelphia, where she came February i, and was at once
admitted to the University Hospital. An examination made soon
after her arrival showed the same conditions as were present
on December 30, with some additions. The dominating symptoms
were still the left hemianopsia and hemiataxia. The results of
the examination in detail were as follows:

The patient's left palpebral fissure was wider than the right.
The left pupil was slightly larger than the right. Both were
round. Associated upward movement seemed to be limited, but
was not lost. There was no paralysis or weakness of the indi-
vidual superior muscles of either eye. Lateral movements seemed
to be well performed. Downward movements, both individual
and associated, were well performed. Convergence movement
was very slight on the first test, but after several attempts the
movement improved. Nystagmus was absent. The pupils re-
sponded to light stimulation and to convergence.

The brow was wrinkled equally well on each side. The right
eye was forcibly closed better than the left. Resistance to open-
ing was greater on the right than on the left. Slight flattening
of the left nasolabial fold was present, with a drooping of the
angle of the mouth on that side. The teeth were displayed a
little better on the right than on the left side, and puckering the

A BRAIN TUMOR LOCALIZED AND REMOVED 78

lips for whistling- was better done on the right than on the
left. Expression of emotion, as in laughing, was equal on the
two sides. Voluntary movement of the right side of the face
was better than that of the left. The mouth opened straight, no
deflection, as in fifth nerve paralysis, occurring. Power was
well retained in the masseters and temporals.

In the left upper extremity resistance to passive movements,
as compared with the right, was possibly somewhat impaired,
and the patient showed a little awkwardness in some of the
finer movements.

Light hypesthesia was uncertainly shown on the left, as com-
pared with the right. As the patient herself expressed it, the
difference was trifling, and she sometimes seemed to be un-
certain about it. She had slight hypastereognosis on the left, at
least this was the conclusion from the tests. She recognized
everything, but with a little less readiness on the left than on
the right. She had some loss of the sense of position and of
movement on the left. If movements were made of parts of the
Imib on the left side, she could not repeat the same on the right
side; but if the movements w^ere made on the right side, she
could repeat them on the left. The sense of location was un-
doubtedly better on the right than on the left. Again and again
she failed to recognize the place where the finger was touched
on the left, whereas when tested on the right she quickly indi-
cated the position. She had very distinct ataxia in the finger
to nose test on the left side, hovering and uncertainty being
easily observed, although not pronounced. No ataxia was shown
when the test was made with the right forefinger. The grip on
each side was good, somewhat better on the right, but it nuist
be remembered that the patient was right-handed.

She had no Romberg symptom. She had some awkwardness
of gait, due to the impairment of muscular sense in the left lower
extremity.

No Babinski was present on either side. Slight plantar flex-
ion was shown on plantar stimulation on the left, and the same on
the right. The knee jerks were prompt on both sides, prob-
ably a little rnore so on the right. Ankle clonus was absent.

Ophthalmic examinations were made on several occasions
by Dr. G. E. de Schweinitz. He reported on February 3, 1908:
The optic neuritis of the right eye is now five diopters in
height, an increase of two and one-half diopters since the nine-
teenth of Januarv. The optic neuritis of the left eye, which was
just beginning, is now three diopters in height, an increase of
fully two diopters. An entirely new process is the development
of numerous hemorrhages, which have appeared thickly on the
swollen discs and in the neighboring- retinal areas, and give the
impression of being the result of thrombi in the retinal veins, or,
in other words, thrombotic hemorrhages, although it is perfecth'

79 CHARLES K. MILLS AND CHARLES H. FRAZIER

possible that some of them, owing to the difficulty of venous
return, are the representatives of a true diapedesis. Both proc-
esses are the common etiological factors in this type of hemor-
rhage, I think. There is absolute left lateral hemianopsia, that is
to say, the blind fields are blind for form-sense, color-sense, and
light-sense (Fig. 2). This may be of some importance in your
localization. Wernicke's symptom is not present ; in point of fact,
if there is any difl:'erence in the reaction of the pupil to the
changes of light and shade, the light reaction is a little more
active when the light falls upon the blind side of the retina, —
certainly there is no difference. There has been marked failure
of direct visual acuity, which has fallen from practically normal
to 6/12, or one-half, on the right side, and to 6/7.5 on the left
side, that is to say, a little better than two-thirds."

The effects of the operation (to be hereafter described) on
the hemianopsia and optic neuritis, as shown by examinations
made by Dr. de Schweinitz at intervals after the operation, were
very interesting.

On February 13 he reported as follows:

" I examined your patient this afternoon, and have to report
to you that while the hemianopsia continues, there is a distinct
gain, and the blind area has lessened in size (Fig. 3). ^Moreover,
the contraction of the preserved fields, which was present when
I took the fields of vision on the nineteenth of December and the
first of February, has disappeared, so that the preserved halves
are now fully normal in extent. This would seem to me a very
favorable sign as indicating that the pressure, on the one hand,
has not been severe enough to entirely destroy the optic radiations,
and that we may have fair hopes that there will be still further
gain, and on the other hand, that the intracranial pressure has
been so materially lessened that the preserved halves have en-
larged. There is no change in the size of the discs, which are
still swollen five diopters. There is one large fresh hemorrhage
in the right retina, in addition to those which I have previously
described. It should be remembered, however, that in many of
these cases, not only as we have seen them but as they have
been reported elsewhere, the real subsidence of the neuritis did
not begin until the fourteenth day, and if I mistake not. in some
of Paton's cases as much as five and six weeks elapsed before
there was marked lessening of the neuritis."

On March 9 the report was as follows :

" I have great pleasure in reporting to you that the letter
vision is normal in each eye, that there is almost complete dis-
appearance of the optic neuritis, each disc being now swollen not
more than one diopter. The margins are beginning to appear
quite clearly on the temporal sides. In the right eye they have
disappeared. While there are still homonymous losses of the
visual fields upon the left sides, there has also been a gain in

A BRAIN TUMOR LOCALIZED AND REMOVED

8i CHARLES K. MILLS AND CHARLES H. FRAZIER

this respect, particularly of the left eye, which now in some
meridians is not far from the normal boundaries."

On May 8 Dr. de Schweinitz further reported as follows:
" I examined your patient yesterday, and found the condi-
tions the same as when I reported to you last — practically full
field on the right side, with partial hemianopsia upon the left,
central vision sharply normal, no hemorrhages of any kind in
the retina, and only a very slight haziness of the nasal margins
of the disc to suggest the former choked disc" (Fig. 4).

The operation was performed by Dr. Charles H. Frazier on
February 5.

Surgical Memorandum.

The technique and method of procedure differs in no respect
from the routine which we have adopted in the clinic of the Uni-
versity Hospital. The following is a brief description of the
operation : Under nitrous oxide ether anesthesia preceded by the
administration of one-sixth of a grain of morphine and one
hundredth of a grain of atropine the patient was placed upon the
operating chair in the erect posture. The incision was made so
as to expose portions of the occipital, parietal and temporal lobes.
The flap was so fashioned that its base was directed towards the
temporal region, the superior margin being one inch from the
median line, the anterior margin one and a half inches anterior
and the posterior margin two inches posterior to the parieto-
occipital fissure. The bone was sectioned with the spiral osteo-
tome and the osteoplastic flap reflected. As soon as the dural
flap was reflected the brain at once bulged considerably through
the opening, thus assuring us that there was a decided increase of
intracranial pressure, the cause for which we at once began to
investigate. Upon palpating the exposed surface of the brain an
area was discovered in the superior posterior angle of the open-
ing, which, compared with the normal brain structure, seemed
much softer if not cystic. It was noted furthermore that in the
same region the surface was of a different color. The region thus
described did not exceed that of a twenty-five-cent piece and its
margins were so sharply defined that it was not difficult to differ-
entiate the normal from the abnormal brain tissue. The line of
demarcation was much more distinct after the pia mater had been
peeled off. With the handle of the scalpel the line of cleavage
between the tumor and surrounding structure was easily found,
and we at once proceeded to enucleate. While thus engaged there
was a sudden spurt of clear straw color fluid and the tumor was
now found to be a cyst collapsed. One finger was inserted into
the cavity of the cyst as a guide and with but little bleeding the
entire cyst wall was removed intact. During this process there
was a progressive fall of blood pressure and the table was
lowered, changing the position of the patient from the vertical

A BRAIN TUMOR LOCALIZED AND REMOVED

**
#

83 CHARLES K. MILLS AND CHARLES H. FRAZIER

to the horizontal posture. A small rubber tube was introduced
into the residual cavity in order to prevent the retention of blood
and a possible cyst formation. The dural wound was closed with
interrupted silk' and the scalp with silk-worm gut sutures. A
small groove was cut in the skull just to the median side of the
superior margin of the flap and an opening in the scalp made to
provide a means of exit for the drainage tube.

From the time the operation began until the bone flap was
reflected six or seven minutes had elapsed ; within ten minutes
the dural flap had been reflected ; five minutes more were occu-
pied in determining the seat and margins of the lesion and fif-
teen minutes for enucleating it. Thus with the exception of
closing the wound in the dura and scalp the operation was com-
pleted in thirty minutes.

The condition of the patient at no time gave us any cause
for apprehension. By the time she had been taken to her room
she had recovered consciousness sufficiently to recognize her
physician and answer questions intelligently. Her excellent
condition at the conclusion of the operation was due in part to
the short duration of the operation, to the avoidance of exposure,
and to the skill and care with which the anesthetic was ad-
ministered. In Fig. 5 is shown a photograph of the patient
taken four days after operation, showing the size and position of
flap. It is needless to call attention to the fact that disregard to
certain precautionary measures will have a deleterious effect
upon the patient and may lead to a fatal issue. The advantage
of conducting the operation expeditiously in a reasonably short
time need not be dwelt upon, nor need we emphasize the im-
portance of conserving body temperature, by having the patient
warmly clad and last but not least of entrusting the ether always
to a trained skilled anesthetist.

The operation was perfomed at a single sitting. We have
never been advocates of the two step operation, so popular with
some surgeons abroad because experience has shown that the
actual removal of a brain tumor has little if any harmful in-
fluence, as indicated by the pulse or blood pressure. With this
case the question of postponing the removal of the cyst to a
second sitting was not considered. The additional risk of a
second anesthetization and of infection must be reckoned with
in the two-step procedure. To be sure there are exceptional
instances, particularly in cerebellar lesions, in which by the time
the lesion has been discovered the condition of the patient may
be such as to make it advisable not to proceed.

The cyst, when filled with fluid, measured eight centimeters
in length and four in diameter (Fig. 6.) Its shape was not
unlike that of an egg and the greater portion of it was sub-
cortical. Fortunately a small portion of it, what might be said
to be one pole, presented on the surface ; otherwise it might

A BRAIN TUMOR LOCALIZED AND REMOVED

•- ^

•C

.jj

bl)

<x:

1-. >

Oh „

c
15

CHARLES K. MILLS AXD CHARLES H. FRAZIER

have escaped detection. \Miile the cyst appeared in the surface
in the superior portion of the occipital lobe the greater portion
of it extended into the parietal lobe, thus accounting not only for
the visual disturbances but for the hemiataxia.

The report from the Laboratory of Surgical Pathology de-
scribes the cyst as follows :

Fig. 5. Photograph of patient taken four days after operation, show-
ing size and position of flap.

The specimen consists of a thin-walled cyst measuring about
8x4 cm. in size. When received in the laboratory it contained
a ragged rent along one of the surfaces. The outer surface was
smooth, even, and of a pinkish-gray color, with a few very
small vessels ramifying over the surface. The wall was gen-
erally about one or two mm. in thickness and very friable; the

A BRAIN TUMOR LOCALIZED AND REMOVED

interior was smooth except at three places where some soft
tissue was adherent to the wall, but could be peeled from the
same with ease. This tissue was of a darker color than the wall.
When received there was no fluid in the sac, and scrapings from
the walls failed to reveal any evidence of echinococcus.

Microscopic examination of portions of the wall and of the
tissue adherent to its inner wall failed to show any trace of
brain tissue, or resemblance to any glioma or other tumor tissue.
The wall was composed of several layers of fibrous tissue fairly
rich in nuclei, and having an abundant blood supply; the inner
surface was rough and ragged, while the outer layers seemed
to have condensed to form the smooth outer surface. The

Fig. 6. Cyst, drawn to scale.

tissue masses represented blood clot with fibrin, red cells, and
pigment, and a few round cells, probably lymphocytes.

As to the pathological diagnosis there seemed to be little
question from the macroscopic and microscopic features that
we were dealing with a simple serous cyst. Cysts resulting
from hemorrhage and softening are much more irregular in
shape and have not a well developed sac. There was not the
least suspicion that we were dealing with a cyst as part of the
development of a morbid growth, such as sarcoma or glioma.
The wall of the cvst throughout was composed of fibrous, not
of tumor, tissue.

A word or two might be said as to the blood pressure (Fig.
7). Before the operation began the blood pressure registered
210 mm. of Hg; as soon as the flap was reflected, within five
minutes, it dropped to 150, while the cyst was being removed

CHARLES K. MILLS AND CHARLES H. FRAZIER

to lOO, and at the conclusion of the operation registered i8o.
Whether the high pressure at the beginning was due to a gen-
eral arteriosclerosis or to increased intracranial tension is a
matter of conjecture. On two occasions during the convales-
cent period the systolic pressure was i8o and 140 respectively.

ANESTHESIA AND BLOOD PRESSURE RECORD.

N4« %4J c^

II^UJ^ Cjt nnna Indei Fme No. J'^0^

ANESTHESIA CHART.

i;>..

IS KVa\K\ :■ :u,UiB

SK a «

« fO^HiW

?..

"...

a «;■

"...

PllIU

... 1 „„

ISO

* i

U- .-,

180

^T-

1 ^

170

: 1

leo

" i i

ISO

~rT~

140

T>-71

! M : ) ! M 1 i

130

itVHj M M : ! M i J>i.|-I i i

120

110

; j i * j ! ■ ^ i i ^

100

-rfiT-rl.. ..;,;...- .,.. fi ' : ^ i

-ITi-n : 1 : .^ M i M. M

iii ; M . i M M M M M

rr i i 1 1 h i I i h ! ! \\

! \ !—-

1 i i i i i M 1 i : , i 1 ! 1 ■ I M

.50

. , ! ; : ■ : . ■ i . : ^ j : i i i ; •

.-..- - . ^ . , , ■■ \ . ^- ■ \

. ■

Km^ .

ioii,ii^l^iJMii'^ii<i''M^ MiiXiM : 1 . i

i— 1

Opemion Suned IJiUOm Optmlion Ended Z -f/Jl?^ ^

Anmhrtic ^Oin'^ ^d&V

Tine to An«h«.ie ^THlHuMi, Amt to Ancslhttue R«s 'Total Ai»t. Used

Exlmination of Chcsl (btlore) 9lt<1ll£n>t,

/ BLOOD PRESSURE CHART

Tm. 1 Hogn 2H

►.'Sits

r„ ,„:«,»_,,!.

220

i • \ .11^ i J M : i ' i ' ■ : " : ■ i ' i !

1 !

1 >

210

'^^'^LtT^Tr'""' - * ^ ■ -^ ' ' ■ ■■

200

too

-' ^\\l'-:\ '^- \ ' \

ISO

170

' ' S^ i ''■ r \^ ^ a •'• M i '

leo

~ i' ' t ' . ■ ■•' ■ i i

ISO

i i ,;^ V i i 1 i ! : ■ : ^

140

-- M i \i . ill 1 .■■]■■■"

ISO

_!_[■ 1 iW / U.1 -J.. !-..-

120

' i ' i\ J. .- .-..i--^ , - :. J

110

■"■•1- TT \T t Ml

100

i M V ~ .

• 0

j 1 i ! ! M ! - M M • i M !

««

i '■ 1 ! 1 i i • i ■ ; • i ! i i i M '

i i M j i i • i i M i i t M ! M

. ' : ' ' ! ■ " : : ; : ! i : i : ;

mu.

M2l>iH^ii22Mi1^"i'l'i''iV HtJiilU ; 1 • 1 ,

'-I i i : i 1

Fig. 7. Blood pressure chart, showing fall in blood pressure after
removal of cyst.

We are rather disposed to believe that in this case the intracranial
tension was an influential factor, although in numerous and re-
peated observations in other cases of brain tumor or internal
hydrocephalus the blood pressure has not been appreciably dis-

A BRAIN TUMOR LOCALIZED AND REMOVED 88

turbed. When the pressure had fallen from 210 to 100 mm.
of Hg durinj? the course of the operation the position of the
patient was changed from the vertical to the horizontal posture.
This we have found almost invariably has a favorable mfluence
upon the circulation and in this case the pressure at once began
to rise until at the conclusion of the operation it registered 180.
With the opportunity to observe the pressure throughout the
operation we have no hesitation in placing the patient in the
vertical posture. Apart from the fact that this position is more
convenient for the operator it is very efficient as a means of
controlling hemorrhage.

The recovery of the patient from the effects of the operation
was prompt; during the convalescence the temperature did not
rise above 99.4° F., nor the pulse above 96 (Fig. 8) . The patient
was sitting up in bed on the third and walking about her room
on the fourth dav, and with the removal of the stitches on the
fifth day was 'concluded a brief but interesting surgical
experience.

While the rapidity with which the signs of disturbed function
disappeared constitutes one of the most striking features of the
case, the absence of any immediate aggravation of symptoms was
equally noteworthy. In many instances the trauma to the brain
tissue, incidental to the removal of a growth, causes a transitory
disturbance of function in the structure adjacent to the lesion.
This is more particularly the case when the tumor is situated in
the motor cortex. In a case recently operated upon, following
the removal of a tumor the size of a hickory nut from the motor
region, even though the growth was sharply defined and easily
enucleated, the patient had a complete hemiplegia.

From the standpoint of the surgeon this case was of especial
interest because it presented a benign, accessible and operable
lesion. Perhaps the most discouraging feature of the surgery
of brain tumors is the comparative infrequency of operable
tumors. According to Buret's frequently quoted statistics, based
on a series of 344 cases, but 10 per cent, were said to be operable
and of these in two thirds the seat of the tumor was not deter-
mined ; thus reducing the percentage of operable cases to 6.5 per
cent. Many of the statistical tables, however, are based upon
necropsy findings, when the tumor is far enough advanced to take
the patient's life. This " autopsy method " of determining the
operability of tumors, as we have had occasion to say before, is
open to serious objection, and while the percentage of tumors
suitable for radical operation will always be a small fraction of

CHARLES K. MILLS AND CHARLES H. FRAZIER

the whole, the reckoning- should be made from the statistics of
the surgical clinic and not of the pathological laboratory. In
the clinic of the Hospital of the University of Pennsylvania
we have had all told, nine cases of operable tumors ; six of
the cerebrum and three of the cerebellum. These represent
approximately 20 per cent, of our series of tumor cases. While
the decompressive operation is wonderfully effective in palliating

TEMPERATURE RECORD.

M^. <^

Hospital '*W?:tf/(!;S«^«i Index File No.^-?*?-^'©!

1 1

TEMPCRATURC.

1 SI

SS 9e 100 ICrt 102 103 101 105 106 101

J-H.J

fcS /(o

M tP/71

fi . A _ : _ .

. lOn 71*m.»$i«n j i

1-1 ;. c K fMtr.

• 1 f

! !

• '

70 ^0 . M ,iVW -

. • 1 ._

1/t/jie.

^."■^

m±.-J

J.^

~lr, kr. E ■?/? W

7^ ;^>i M iPjn

Uuac

Ai.£l

nv+

yt^ AH T. 'inm

7k :>.k M tPW.

>•

■at A¥ E /JKTT)

"S&^sx

f Of^TttlCTl

jiK.kK M srm

1 "•

JlfitT

o^rat/en.^

11 /.H ji ^pm

•;

9/k Af M ^W .

: ^^. .

%% Af. F. iP.'n

^»

¥-< A'.'.M 9pm

.v.-

sf« x^ E un

. . . .

t¥ ^c,M .^/?m.

1 . .•

9A ;!f E 477771

• ,«"

l/rine

k.«,J

96 j^-i." 9^m_

• -^

sc ;io E /a TO

SJ AA M 3P?n

1 ""•

f? AA E (,FTn

••

f% AA.M qPTTl.

?</ ^0 E /ATI

J? Ac M 3;/i7>\ ,

?? ^0 E 7>7 7n

1 •

l/r^^

Ity/jS

■

5? ^c,M .9ATn.

•

()

J •

•■iH /if E 1A73 .

i.>

f^ ;i* M f/jw)

1 1

i/riue .l^"JL

II 1

9X ;t,* r ivm

' t

i« Xc .M iP7n_

•

It Xt t -ipm

in 7,0 K (,Rm

■-"

- -

Stittii

VrjK

Awu

?s f.u E if>m

( 1

^l,M.Jt .(orm.

•

?y 7,H T qnm

l'ri« ^Jriv

?c.;^o M iTTn

i-A. ..

9,0 AO E -^PTD

«c.;iA.M mm

1 1

?o /<o E yPTxi

SC ac.M ,tp»i,_

fC-AO E !$H»7

. . .

Vri.Y

M"^

;,,,

. . . .

'" 1

Fig. 8. Temperature chart, showing how slight the effects of the
operation were.

symptoms and prolonging life, and some of the results of the
radical procedure appear discouraging, no case should be branded
inoperable until so proved by a thorough exploration through a
liberal opening. Only by early diagnoses and by a resort without
procrastination to well-executed craniotomies, even though there
may be some doubt as to the precise location of the tumor, can

A BRAIN TUMOR LOCALIZED AND REMOVED 90

we determine whether or not we are justified in relapsing into
that state of pessimism which characterized the attitude of the
profession not many years ago.

Examinations of the patient were made with the view of deter-
mining the effects of the operation and removal of the cyst upon
her nervous system from time to time during her stay at the
hospital. On February 8, three days after the operation, a note
was made that the ataxia was fast disappearing and that the
patient's mental condition, although never dull to the extent of
being seriously affected, was becoming much brighter. On
February 12 the record was made that all functions of the arm
and leg were restored. The ataxia had disappeared. Testing for
astereognosis, the sense of location and position, and cutaneous
sensibility the results were negative. Motor functions were nor-
mal in both arms. The patient seemed to have a little less volun-
tary control of the left side of the face, but she stated that this
had always been the case. Hemianopsia was much less marked
as was shown by various tests. On the fifteenth the affected
limbs appeared to be entirely normal, and the patient evidently
saw much better. She was able at this time to detect much smaller
objects about her room.

The examinations of Dr. de Schweinitz, already detailed, give
more explicitly the facts as to her eyes and vision.

The completeness of the removal in this case of a large parieto-
occipital cyst, and the entire disappearance of the severe and
threatening symptoms produced by it, entitle it to be regarded as
one not merely of palliation but of cure. Apparently the cyst was
benignant in character. We have seen the patient within two
weeks of the presentation of this report. In every particular she
was in excellent health, being with the exception of the very
partial hemianopsia, free from signs of cerebral disease.

From the standpoint of physiology, especially as regards the
functions of the parietal lobe and the possibility, in considering
lesions of this lobe, of subdividing it functionally into several
parts, the case also is of much interest.

We have had the opportunity of studying at least four classes
of cases, giving dififerent symptom complexes, according to the
degree of implication of the various portions of the parietal lobe.
Possibly in time more than this number of syndromes can be
separated for diagnostic purposes. These symptom complexes

91 CHARLES K. MILLS AXD CHARLES H. FRAZIER

are: (i) Pronounced hemianopsia and ataxia, combined with
pressure symptoms varying in intensity, such as hypesthesia,
hypastereognosis, and sHght paresis of the face and Hmbs ; (2)
astereognosis and ataxia, combined with symptoms showing vari-
ous degrees of involvement of cutaneous, muscular, and arthroidal
sensibility, but without hemianopsia and with no or only slight
paresis; (3) hemianopsia and hemiataxia, with hypesthesia and
hvpastereognosis and pronounced paresis, especially of the face
and upper extremity ; and (4) astereognosis and ataxia, with
hvpesthesia and pronounced paralysis, especially in the lower
and upper extremities. These symptom complexes, more or less
pure at first, become complicated as the tumor or cyst increases,
and with it the variety and extent of the symptomatology. From
cases already recorded, illustration of each of these symptom
groups might be given.

In considering the question of the position and extent of an
opening for the removal of a tumor or cyst, the osteoplastic flap
should be planned somewhat differently in accordance with these
svmptom complexes. For the first, in which pronounced
hemianopsia and hemiataxia are the dominating symptoms, the
incision for an opening three and a half or four inches in length
for its superior boundary should be made, about one inch from
the mesal edge of the hemisphere and in such manner that about
one half of it should be cephalad and the other caudad, of the
line of the parieto-occipital fissure. The base line connected by
the somewhat converging sides of the opening should be over the
upper part of the temporal lobe. \\'ith the second symptom com-
plex in view — that in which astereognosis and ataxia, without
hemianopsia, are the chief guiding symptoms — the opening should
be carried as close as possible to the middle line of the skull, and
should be an inch more anterior. The incision for the upper line
of the third opening, for a case giving the syndrome in which
hemiataxia, sometimes with hemianopsia and also with hypesthesia,
impairment of the muscular sense and hemiparesis or hemi-
paralysis, especially in the face, are present, should extend about
one third in front and two thirds behind the central fissure, and
about one inch or one and one half inches from the median line,
the base, as in the first opening, being over the temporal lobe.
The incision for the upper limit of the fourth opening should be
as near as possible to the mid-line of the skull, while its sides

A BRAIN TUMOR LOCALIZED AND REMOVED 92

should be in about the same relative positions to the central fissure,
that is so as to uncover the cerebral surface about one third in
front and two thirds behind this fissure. By having the lines
of the opening in the manner here indicated, the surgeon will
probabl}- find the main portion of the growth near the center of
the field of operation.

Reprinted from the Journal of Nervous and Mental Disease, 1908.
(From the Department of Neurology and the Laboratory of Neuropathology
of the University of Pennsylvania.)

OSSEOUS PLAQUES OF THE PIA-ARACHNOID AND

THEIR RELATION TO PAIN IN

AKROMEGALY

By S. Leopold, M.D.

ASSISTANT IN NEUROPATHOLOGY IN THE UNIVERSITY OF PENNSYLV'ANIA

Since Marie in 1886 first named and described the symptom-com-
plex known as akromegaly, many and numerous have been the obser-
vations reported. The pathological findings as well as the symptoms
have varied. The major clinical features, such as increase in size of
the extremities, nose, lips and lower jaw, however, we now consider
as distinctive.

Among the lesser symptoms, pain seems to play an important role.
It was Sainton and State (i) who first called attention to its promi-
nence. In analyzing the cases in literature, they found it present in
50 per cent, of the cases. Sousa Leita (2), Sternberg (3) and others
considered it for the most part as a transient symptom. Osborne (4)
believes that almost every case of akromegaly has pain more or less
constant in some portion of the body, but he included head pains.

As to the pathology of these pains, Sainton and State believe them
to be due to the presence of osseous plaques in the spinal pia and to
the changes, mechanical and irritative, caused by them upon the nerve
roots and substance of the cord. This I do not believe to be correct,
and it is the object of this article to show that there is no definite
pathology of the spinal cord in akromegaly, and also to show that
these plaques play little or no part in the production of the pain of this
disease.

The pain referred to by Sainton and State were distributed some-
times in the extremities, sometimes in the spinal column, and some-
times in the joints. They divided them into five classes — osteo-
arthritic, neuralgic, muscular tabetic and akro-paresthetic. The find-
ings in the case they report include the osseous plaques in the spinal
cord together with degenerations of Goll's tract and Cowers' tract.
The role of these plaques they suggest, is probably mechanical, and
they cite the similar findings in cases reported by Duscheneau, Henriot,
Finzi and others.

1 313

2 LEOPOLD: OSSEOUS PLAQUES OF PL\-ARACHXOID

To observe whether mechanical pressure exerted any influence I
examined several nerves lying under these plaques and found no evi-
dence of thickening or any degenerative change. The spinal cord in
two cases, examined in the lumbar and cervical regions, showed only
a slight increase of the neuroglia, and the presence of arteriosclerosis;
this latter accounting for the neuroglia increase. Other cases in litera-
ture showed a similar picture. A review of the pathological findings,
as_collected by Sternberg (5), Hinsdale (6), Brooks (7), Arnold (8)
and others shows that changes in the spinal cord are inconstant, the
cord being normal in many cases — Packard (9), Cagnetto (10),
Striimpell (11), etc.; in other instances showing varying degenera-
tions of the tracts — Sainton and State (12), Adler (13), Cagnetto,
Arnold; in others syringomyelia, and in Barrett's (14) case the picture
of a severe anemia. In the case I report only arteriosclerosis was to
be noted.

Furthermore, plaques are very frequently present in many diseased
states and in conditions in which no pain is present. Virchow (15)
long ago emphasized this. " For a long time," he says, " they were
thought to be the cause of tetanus, chorea and all other forms in which
pain was a prominent symptom." They reasoned that the presence of
the bone and its processes caused a strong irritation of the nervous
system. " This is an error because we frequently find them present
in individuals who have not the slightest symptoms."

Zanda (16) was able to collect sixty-three cases showing osseous
plaques and found that half occurred in mental disease, that they were
frequent in retrogressive changes, as old age, chronic insanities, dis-
eases of the spinal cord, tuberculosis, marasmus and chronic suppura-
tion.

The giant, Wilkins, reported by Bassoe (17), gave no history of
pain throughout his life and yet many thickened, partly calcified
plaques were found, one even adherent to a nerve root. Micro-
scopically, there was a moderate amount of diffuse degeneration of
the spinal cord. Linsmayer's (18) case of akromegaly showed osseous
plaques with no definite microscopical changes in the spinal cord or
nerve roots save arteriosclerosis and clinically there were no symptoms
of pain. Striimpeirs (19) and Cagnetto's (20) cases showed a similar
picture. In the case reported by Pearce Bailey (21) pain was a promi-
nent symptom in the hands and feet, radiating to the shoulder and to
the hips ; yet the spinal cord, grossly, showed no changes, and micro-

314

LEOPOLD: OSSEOUS PLAQUES OF PL\-ARACHNOID 3

scopically showed only thickening of the arteries. In Packard's (22)
case, sections of which I have studied, the pains were only present
during- the period of onset, a duration of three to five years. Since that
period the patient had been free from pain. The examination of the
spinal cord by Dr. Spiller (23) showed grossly numerous plaques, and
microscopically no degenerations.

It seems to me that we must look elsewhere for the explanation of
these pains. In reviewing the pathological findings of these cases of
akromegaly, the prominent feature of arteriosclerosis should be noted,
and the trophic changes incident thereto, in the joints, muscles, viscera,
skin and spinal cord co«ld easily explain this symptom. In the arthritic
pains the excessive production of lime salts, dependent upon arterio-
sclerosis and also upon the retention of lime salts and phosphorus in
akromegaly, as shown by Moraczewski (24), explains the pathology of
pain in the joints.

Arnold (25) and Cagnetto (26) and others have examined the
muscle and have shown the presence of connective tissue overgrowth
and degeneration of the muscle substance itself. The presence of a
chronic myositis here might easily explain the incident of muscle pains
in any period of the disease.

The question as to the origin of these plaques is of some interest.
Virchow (27) long ago thought them direct transformations of con-
nective tissue into bone. Rokitansky (28), Ziegler (29), Schmaus
(30) simply mention their frequency in the pia-arachnoid and describe
their gross appearance. Zanda (31) has found that the bone forma-
tion takes place from the dura after adhesion with the arachnoid is
formed. In the cartilaginous period they have no connection with the
dura. In the stage of ossification, blood vessels from the dura grow
into them. Their frequent presence in retrogressive conditions, such
as senility, uremia, mental disturbances, in fact, in all those conditions
in which arteriosclerosis is associated or plays a leading part, indicates
clearly to me the natural overgrowth of connective tissue from passive
congestion and chronic irritation, and in such areas of connective
tissue overgrowth lime salts are frequently deposited. Among the
favorable situations for this process are the blood vessels, lymph nodes,
lungs and spinal cord. The associated finding of arteriosclerosis in
many cases of akromegaly indicates clearly one of the predisposing
factors for the production of osseous plaques.

The first case I wish to report shows the presence of these osseous

315

4 .LEOPOLD: OSSEOUS PLAQUES OF PL\-ARACHNOID

plaques in uremia and arterio-capillary fibrosis. The clinical history
of this case unfortunately is very meagre. The patient, D. B., an old
man, was brought to the Philadelphia General Hospital in an uncon-
scious condition. He lived three days. The physical examination by
Dr. Potts showed no paralysis of face or extremities. The diagnosis
of uremia and chronic interstitial nephritis was made and was cor-
roborated at the necropsy. General atheroma of the cardio-vascular
system was also noted, together with chronic endocarditis. Examina-
tion of the spinal cord showed numerous plaques covering the posterior
surface from the cervical region to the lumbar. These measured from
I to 3 cm. and were cartilaginous in appearance ; a few had the firm-
ness of bone.

Microscopical Examination. — Transverse and longitudinal sections
were made of these plaques, and stained with eosin-hematoxylin.
Those cut transversely show a central zone of osteoid material ; sur-
rounding this are layers of fibrous tissue, hyaline in character. In this
are to be noted a few cells, resembling osteoblasts. The upper surface
of the section shows only a few connective tissue cells ; the lower shows
a more active proliferation of connective tissue. In this region the
plaque was in contact with the substance of the cord. This prolifera-
tion occurs in the pia. No evidence of true bone is to be seen.

The spinal cord was studied with Weigert, hemalum and Nissl stains.
A moderate increase of neuroglia in certain parts of the column of
Goll of the cervical section and arteriosclerosis are the chief features
noted. In the lumbar region the arteriosclerosis is not so marked.
There is marked thickening of the spinal pia throughout.

A study of the spinal nerve roots under these plaques showed no
evidence of mechanical pressure. No changes were to be noted by
the Weigert or hemalum stains.

In the second case, one of akromegaly, the clinical features reported
by Dr. Packard in the American Journal of Medical Sciences, 1892,
and by Dr. Spiller pathologically before the Philadelphia Neurological
Society, showed these plagues grossly. Microscopically, by the Wei-
gert, hemalum and Marchi's method no evidences of degeneration were
to be noted.

The clinical notes by Dr. Packard, 1892, may be briefly summarized.
Patient was first seen in 1885. In 1877 had pains all through his body,
which were supposed to be rheumatic. Since that time until 1885 had
vague pains through his body with gradually increasing weakness of

310

LEOPOLD: OSSEOUS PLAQUES OF PL\-ARACHNOID 5

the legs. In 1892 he was again examined. Aside from headache no
other symptoms of pain were noted. Temperature, pain and tactile
sensations in the extremities were good. Knee jerks were absent.
His general health was fairly good. Beside the distinctive features
of akromegaly no other symptoms relating to the subject are to be
noted.

Examinatoin of the cord in the lumbar and sacral regions failed to
show any degeneration.

To summarize briefly :

1. Osseous plaques are frequently present in the pia-arachnoid.

2. They are found in many diseases, such as uremia, tuberculosis,
retrogressive conditions, etc.

3. Arteriosclerosis seems to be the underlying factor in their
causation.

4. The presence of these plaques upon the spinal pia in akromegaly
does not explain the production of pain in that disease.

5. There is no definite pathology of the spinal cord in akromegaly.

I wish to thank Dr. Spiller, under whose direction this work was
undertaken, for the privilege of reporting these cases, and for his
assistance in the examination of the material.

REFERENCES

1. P. Sainton and J. State. Revue Neurologiquc, 1900, p. 302.

2. Sousa Leita. These de Paris, 1890.

3. Sternberg. " Nothnagel's System," Bd. VII, 1901.

4. Osborne. " Handbook of the Med. Sciences," Vol. J, p. 86.

5. Sternberg. Cited above.

6. G. Hinsdale. Medicine, 1898.

7. H. Brooks. Archives of Neurology and Psycho- Pathology, Vol. I, 1898.

8. Arnold. Virchow's Archiv, No. 135, p. i.

9. Packard. Am. Jour. Med. Sciences, 1892.

10. Cagnetto. Virchow's Archiv, 1907.

11. Strinnpell. Deutsche Zeitschrift fiir Nervcnheilkundc, 1897.

12. Sainton and State. Cited above.

13. Adler. Med. News, 1888, Vol. LVIII.

14. Barrett. Am. Jour. Med. Sciences, Vol. 131, 1906.

15. Virchow. " Die Geschwulste," Vol. V, p. 93-

16. Zanda. " Ziegler's Beitrage," 5, 1890.

17. Bassoe. Journal Nervous and Mental Disease, 1903.

18. Linsmayer. Wiener klinischer Wochenschrift, VII, 1894.

19. Striimpell. Cited above.

20. Cagnetto. Cited above.

317

6 LEOPOLD: OSSEOUS PLAQUES OF PIA-ARACHXOID

21. P. Bailey. Phila. :\Ied. Jour., 1898, April 30.

22. Packard. Cited above.

23. Spiller. Journal Nervous and Mental Disease, Vol. 25, p. 42.

24. Moraczewski. Zeitschrift f. klin. Med., 1901, No. 4 (quoted by Bassoe).

25. Arnold. Cited above.

26. Cagnetto. Cited above.

27. Virchovv. Cited above.

28. Rokitansky. " Handbook of Patholog}\"
29'. Ziegler. " Special Pathology."

30. Schmaus. " Vorlesungen iiber die Path. Anatomie des Ruckenmark,''
1901, p. 314.

31. Zanda. Cited above.

318

From the Howard Hospital, and the Department of Neurology and Labora-
tory of Neuropathology of the University of Pennsylvania.

PATHOLOGIC REPORT OF THE NERVOUS SYSTEM IN A
CASE OF SPONDYLOSE RHIZOMELIQUE ^

By John H. W. Rhein, M.D.

NEUROLOGIST TO THE HOWARD HOSPITAL, PHYSICIAN TO THE PHILADELPHIA HOME
FOR INCURABLES, ETC., PHILADELPHIA

A sttidy of the nervous system in cases of spondylose rhizomelique
and allied conditions has been made only in a few cases, those of von
Bechterew,- Leri.^'' Rettter,'' Fraenkel,-'' and ^McCarthy,'' to which refer-
ence will be made later.

In the case which forms the basis for this report the brain, spinal
cord, peripheral nerves and muscles were examined, and pathologic
changes, though slight, were distinctly observed. The addition of
this case, therefore, to the meager literature of the subject, at least
so far as a study of the nervous system is concerned, throws some
light on a subject about which there exists much diversity of opinion.

History. — The patient, Z., was a man. aged 64, whose history, unfortunately,
can not be given, except that he was admitted to the Philadelphia Home for
Incurables, Sept. 29, 1903, and remained there, practically in the same condition
as to the symptoms under discussion, until his death on June 18, 1907. I
examined him a week before his death, although I had previously frequently
observed him without careful examination.

Examination. — The entire spine, including the articulations with the cranium
and pelvis, was ankylosed, presenting the characteristic appearance of so-called
" poker-back." There was ankylosis in both hips, knees and shoulders, although
this was not complete. The arms could be moved about 30 or 40 degrees at the
shoulders. The elbows, hands, ankles and toes were not involved. There was
general emaciation at the time of the examination, so that the presence of local
atrophies could not be clearly determined. There was pain in both sciatic
regions, extending from the knees to the hips, but no pain was complained of
elsewhere about the body. The spinal column stood out prominently, and the

* Read in the Section on Nervous and Mental Diseases of the American
Medical Association, at the Fifty-ninth Annual Session, held at Chicago, June,
igOcS.

" Dcutsch, Ztschr. f. Nervenh., xv, 45: Neurol. Centralbl., 1892, p. 426;
Deutsch. Ztschr. f. Nervenh., xv, 37: Ibid., 1897, No. Ii, p. 327.

^ Rev. neurol. 1905, p. 1085, and Revue de med., 1899, No. 19, p. 597.

* Ztschr. f. Heilk., 1902, No. 23, p. 83.

' Fortschr. a. d. Geb. d. Rontgenstrahlen, 1903-4, P- 62.
" New York Med. Jour., 1905, June.
1 319

2 rhein: nervous system in spondylose rhizomelique

muscles of the back were probably wasted. Tests for pain, touch and localiza-
tion gave negative results. Two months before death he developed tuber-
culosis, which became general, and from which he finally died.

Autopsy. — At the autopsy, when an effort was made to remove the cord,
it was discovered that the entire spinal column presented the appearance of one
bony mass. It was so hard that it was difficult to saw through in the spinal
canal. On removing the spines and arches of the vertebrae a considerable
amount of caseous material was found on the outer surface of the dura, extend-
ing from the lower thoracic to the lower cervical region. There was no evi-
dence of tuberculous bone disease, or tuberculous abscess, and macroscopically
there were no tubercles.

The dura was adherent to the skull, and a few tubercles were found over
the convexity of the brain. Both lungs were infiltrated by tubercles and were
the seat of many cavities. The right kidney was also implicated.

The brain and spinal cord, the plantar nerves, sections of the sciatic and
smaller sciatic nerves, and some of the muscles from the plantar surface of the
foot were preserved for examination. The spinal cord was hardened in Miiller's
fluid, and stained by the Weigert method, and in hemalum, acid fuchsin, and
thionin.

The white matter of the cord itself showed no abnormality. There was,
however, a slight degeneration present in several of the roots. In the lumbar
region the right anterior and left posterior roots were slightly degenerated. In
the lower thoracic region there was slight degeneration of the anterior roots.
Elsewhere the roots stained normally.

The cells of the spinal cord in the spinal and cervical regions showed the
following changes : Some were rounded, had lost their prolongations, and
contained an excessive amount of yellow pigment. Clumping of the chromatic
substance was present in some, and in a number of them the nucleus was dis-
located, and the yellow pigment excessive.

The small sciatic nerve was slightly degenerated in cross sections. An abun-
dance of fatty tissue was seen, and there was increase in the connective tissue.
The plantar nerves were also degenerated. The large sciatic nerve was normal.

In the left paracentral region, there was marked perivascular distention, and
slight infiltration of the pia. The same condition was found on the right side,
where there was also some change in the pyramidal cells. Some of the cells
were swollen, and the nuclei eccentrically placed.

A study of the muscle tissue from the plantar surface showed an increase
in the number of the intramuscular connective tissue cells ; irregularity in the
size of the muscle fibers ; loss of the transverse striations in some of the fibers
and lumpiness of the muscle substance of individual fibers. There was no
vacuolization. The blood vessels showed no endarterial thickening, or sclerosis.

A portion of the spinous process of one of the vertebrae was studied micro-
scopically. The bone trabecula was compact but not unduly so. There was
no evidence of any abnormal condition whatever. In fact the specimen pre-
sented the normal appearance of bone.

Summary. — A man of 64 presented the characteristic clinical manifestations
of spondylose rhizomelique, i. e., rigidity of the spine, and partial ankylosis of

320

RHEIN : NERVOUS SYSTEM IN SPONDYLOSE RHIZOMELIQUE 3

the shoulder, hip, and knee joints. Pathologically there was slight degeneration
of some of the anterior and posterior spinal roots, slight peripheral neuritis, and
muscle taken from the plantar portion of tlie foot was degenerated.

While Marie and Leri' still assume that spondylose rhizomelique
is a disease entity differing from the other forms of ankylosing dis-

Microscopic tindings in a case of spondylose rhizomelique, showing degen-
erated spinal root at A (Weigert method).

ease of the spinal column — and especially separated from von Bech-
terew's type — there are many who maintain that these two varieties
are but manifestations of one and the same disease.

' Nouv. iconog. de la Salpetriere, IQ06, ig, p. 32.

:}2l

4 RHEIX: XERVOUS SYSTEM IX SPOXDYLOSE RHIZOMELIOUE

The differences between the van Bechterew and the Strumpell-]\Iarie
types may be stated, according to Zesas,* as follows :

In von Bechterew's type (i) the spinal column is rigid; (2) kyphosis
is always observed; (3) irritation symptoms are always present; (4)
the joints of the extremities are intact; (5) etiologically there is a
history of inheritance, trauma and lues.

In 'the Striimpell-Marie type (i) the entire spinal column is rigid;
(2) kyphosis is not always present; (3) symptoms of irritation of the
roots are usually absent; (4) there is an ossifying process in the joints
of the extremities; (5) etiologically a history of rheumatism and in-
fectious diseases is obtained.

In von Bechterew's- original case, with autopsy, there was no
primary lesion of the vertebrae, and the rigidity of the spinal column
was attributed to a secondary paretic condition of the muscles, the
result of a compression of the nerve roots from a pachymeningitis,
von Bechterew also believed that his type of spinal rigidity differed
from that described by Striimpell.^ under the name of chronic anky-
losing spondylitis, in whose cases the head, spinal column and hips were
firmly united and completely stiffened. Cases similar to those de-
scribed by Striimpell have been reported by Marie and Anstie.^*'
While in Striimpell's cases there were no root symptoms, these were
pronounced in von Bechterew's cases, and in the former there was in-
volvement of the great joints, which were intact in von Bechterew's
cases.

Renter,* while admitting that, clinically, there was a difference
between these two types, claimed that this difference was not proved
pathologically. He believes that such cases are due to a bony process
to which he applies the name " ascending ankylosis of the spinal
column, with bow-shaped kyphosis."

Siben,^^ Fraenkel,^ Elliott,^- and RumpeP' did not believe that any
difference could be made between these two types, while Schlesinger"
not only did not recognize any difference between these two types, but
did not differentiate between arthritis deformans of the spinal column
and other cases of rigidity of the spinal vertebrae. Nor. in Oppen-

' Deutsch. Ztschr. f. Chin. 1904. No. 74, p. 467.

"Lehrbuch der Specielle Pathologic, ii. 534; and Ztschr. f. Nervenh.. xi. 338.

'" Presse med., 1898; and Marie: Revue de med.. 1898. p. 285.

"Ztschr. f. klin. ^led.. 1903. No. 49, p. 241.

" Am. Jour. Orthop. Surg., 1905-6, No. 3, p. 303.

"Deutsch. med Wchnschr.. 1905. p. 1130.

" Mitt. a. d. Grenzgeb. d. med. u. Chin. 1900, vi, 247.

322

RHEIN: NERVOUS SYSTEM IN SPONDYLOSE RHIZOMELIQUE O

heim's^^ opinion, was it decided that a difference existed between the
various types of spinal ankylosis, either clinically or anatomically.

Zesas,^ after reviewing the subject very carefully, concluded that
the observations, so far, did not permit the view that, etiologically or
symptomatically, and much less pathologically, these two forms were
independent affections, and, according to Niedner,^*"' the pathologico-
anatomic substratum of the von Bechterew type was similar to that
of the Marie-Striimpell type, and consisted of an ossification process
of the cartilages and ligaments.

On the other hand, Glaser^' reported a case in which the findings
coincided with those of Marie's case, and more recently Marie and
Leri^^ have maintained tliat there is a distinct type which they call
" spondylose rhizomelique."

The autopsies of ankylos-ing disease of the spinal column which have
been reported can be more or less divided into ( i ) those in which the
small joints of the vertebrae are mainly affected, leaving the cartilages
intact, or only slightly involved; (2) those in which the cartilages are
especially altered; (3) those in which the process involves equally the
small joints and the cartilages.

In the first category belong the cases of Marsh, ^^ Glaser,^' Milian,-**
Fagge,-^ Simmonds,^- and Fraenkel.^ In Marsh's case there was
ossification of the ligaments and complete ankylosis of the several
joints. There was no atrophy of the body of the vertebrae, nor any
change in the intervertebral discs.

Glaser cited a case of chronic ankylosing inflammation of the spinal
column, in which there was ankylosis of the articulating process, and
between the ribs and the vertebrae. There was no involvement of
the intervertebral discs, or the body of the vertebrae. He cites the
case of Howard Marsh, in which the findings were similar.

In Hilton Fagge's autopsy (cited by Leri) the intervertebral cartil-
ages were of normal appearance ; the arches of the spinal apophyses
were united by bone, and there was an osseous process, with complete
destruction of the articulations. In Milian's case the vertebral column

'° Lehrbuch der Nervenkranklicitcn, 1905, i, 309.

'" Charite-Ann., 1903, No. 28, p. 45.

" ]Mitt. a. d. Grenzgeb. d. Med. u. Chin, 1901, No. 8, p. 282.

'* Gaz. hebdom. de med., 1899, p. 209.

"Brit. Med. Jour., 1895, ii.

^ Gaz. hebdom. de med., 1899, p. 137.

^In Ehrhardt.

" Fortschr. a. d. Geb. d. Rontgenstrahlen, 1905, v, 51.

6 RHEIN : NERVOUS SYSTEM IN SPONDYLOSE RHIZOMELIQUE

was immovable and bow-shaped, and the hips and shoulders were in-
volved. There was an osseous disease of the articulations of the ribs
and the bodies of the vertebrae. Intervertebral cartilages were normal.

Simmonds reported two cases in which there was intense ossification
of the ligaments and an ankylosis of the joints of the vertebrae.
Fraenkel reported four autopsies of chronic ankylosing rigidity of the
vertebrae, in which the intervertebral cartilages were only slightly in-
volved. The vertebrae were connected by broad bony bands, and the
costo-vertebral articulations were implicated. The articulating proc-
esses were fused together. The process seems to have involved prin-
cipally the articulating processes, including the spinous process.
Fraenkel looked on the process as being a primary afifection of the
joints, that is, the articulating processes, the remaining vertebrae being
intact. In the later stages of the disease there is a bony formation,
which in some cases is confined to the sides of the vertebra or it may
extend around the whole body of the vertebra. Sometimes the arches
of a single vertebra or many vertebrae, and also the spinous processes,
are involved. He stated that the process is an ossification of the
articulations of the joints, and the bony formations are probably
secondary, the vertebra becoming involved by mechanical action.

The cases in the second category are those of von Bechterew,^
Ascoli,^^ Renter,* Schlesinger," Ehrhardt-* and Elliott.^^ In von
Bechterew's case, in which there was a study of the nervous system
as well as of the bones, he believed that the disappearance of the cartil-
ages was due secondarily to the fact that there was a compression of
the cartilages, between the neighboring vertebrae, which was caused
by the kyphosis. This kyphosis originally depended, according to von
Bechterew on the paralysis of the musculature of the thoracic spine.
The compression of the cartilages, he states, naturally causes their
gradual disappearance, and finally the surfaces of the vertebrae resting
on each other fuse together.

The case of Ascoli-^ was cited by Leri. The articular cartilages
were destroyed, and the articular head was the seat of multiple erosions
and atrophy. The lesion was the same in the coxo-femoral, and in
the knee, and vertebral articulations. There was no ankylosis.

Reuter reported a case from the pathologic museum, in which
the articulating processes were united by bony substances, the

-^ In Leri.

^ Mitt. a. d. Grenzgeb. d. Med. u. Chir., 1904-5, P- 226.

324

RHEIN: nervous system in SPONDYLOSE RIIIZOMEr.IOUE 7

heads of the ribs with the bodies of the vertebras, and in which
the intervertebral cartilages were for the most part destroyed, being-
replaced by a spongy, bony substance. The bodies of the vertebrae
were markedly rarefied. The ligaments were also ossified. In a
second case the entire vertebral column was fused together by means
of bony masses, so that the borders of the intervertebral discs could
not be seen. A further study revealed the fact that the intervertebral
cartilages appeared to be ossified in part, and the new bone formation
resembled the substance of the neighboring vertebral body.

In both of these cases Renter believed that there was a chronic
process causing a kyphosis of the vertebral column, bony ankylosis
of the joints of the vertebrae and ribs, and the anterior part of the
intervertebral cartilages. He assumed that it was a chronic inflam-
matory process of the joints of the vertebrze similar to that described
by Ziegler as arthritis ankylo-poetica, a view held also by Elliott and
Siben. In Renter's cases there was a process in the cartilages anal-
ogous to normal bone formation. He acknowledges that this theory
has been advanced by Leri, who found ossification of the ligaments,
hypertrophy and destruction of the joints, and looked on the process
as a healing one. Renter's preparations did not support such a view,
however.

In the eight preparations of spondylitis deformans from the Vienna
Museum, Schlesinger distinguished two forms. In one the process
was confined to the intervertebral cartilages, which were destroyed,
the edges of the neighboring vertebrae being united by supra-cartil-
aginous exostoses. The bodies of the vertebrae were atrophied, and
the small joints and ligaments were involved or remained relatively
free.

In the second group, the more frequent form, the transverse process
and ligamentous apparatus were chiefly afifected. The ligaments were
calcified, and there was proliferation of the synovial membrane leadino-
to an ankylosis of the small points. Siben thinks it doubtful that
both of these forms could be considered as types of the same disease.

Ehrhardt examined the skeleton of a case of chronic ankylosing in-
flammation of the vertebras in which not only the vertebras were anky-
losed, but the sacroiliac and hip joints also. The intervertebral cartil-
ages had disappeared, a spongy, bony substance having replaced them,
and this continued into the substance of the neighboring vertebrae.
There were some osteophites present in the lower thoracic region,

325

8 RHEIX : XERVOUS SYSTEM IX SPOXDYLOSE RHIZOMELIOUE

although nowhere united, as is seen in progressive cases of spondyUtis
deformans. Neither did these exostoses project into the vertebral
canal. The vertebral joints were ankylosed throughout the entire
spine, while the spinous processes in the cervical region were exempt.
The intervertebral foramina were not narrowed, except at the tenth
thoracic vertebra on the left side, and this by an irregular exostosis.
This condition, he claims, explains the nervous symptoms better than
the old conception of von Bechterew, that they were due to thickening
of the meninges.

The ligaments between the ribs and the sternum were unchanged.
The process was, according to Ehrhardt, a syndesmoginous synostosis
of the entire vertebral joints, with ossification of the long ligaments,
the ligamentum flava. and partial ossification of the intervertebral discs.

In ElHott's case the bodies of the vertebrae were normal, while the
discs were atrophied and absent anteriorly. There were no bony
bridges or exostoses, but there was ossification of the superior spinous
ligaments, especially in the lumbar region. The spinous processes
were, in places, ankylosed at their distal ends, and there was an ossifi-
cation of the ligamentum flava. There was some evidence of exostosis
in the lumbar region, where the articulating processes were completely
ankylosed.

In the third category belong the cases of Forestier,-^ Leri,^ Siben,^^
Marie and Leri.^^ Forestier examined 66 skeletons in which 17
presented the symptoms of arthritis deformans of the spine. Osteo-
phites were seen on the anterior and lateral surfaces of the bodies of
the vertebrae. The vertebrae were involved, and the articulating proc-
esses were deformed. The intervertebral foramina were diminished
in caliber. The exostoses of the bodies of the vertebrae were very
large and increased in size downward.

Siben cited a case with autopsy, in which the shoulder and hip joints,
as well as the vertebrae, were involved. The small joints of the
vertebrae were ankylosed, and the intervertebral cartilages were also the
seat of a secondary change. There was evidence of an inflammatory
process, and Siben proposed the name "' chronic ankylosing inflamma-
tion of the vertebrae " after Striimpell.

In Leri's case the principal lesion consisted of an ossification of the
ligaments. There was also a hypertrophy of the articulating processes,
which fused together after all traces of the cartilages had disappeared.

'^ Arch. gen. de med., 1901, Xo. 2, p. 158.

326

RHEIN : NERVOUS SYSTEM IN SPONDYLOSE RHIZOMELIOUE 9

The lesion was a double one, consisting first of an ossification of the
ligaments, and second, hypertrophy and fusion of the articulating
extremities.

In a more recent case, reported by Marie and Leri. the findings con-
firmed the original theories advanced by Leri, /. c, that spondylose
rhizomelicjue consists chiefly of an ossification of the ligaments and
intervertebral cartilages, which is accompanied or preceded by an
osseous rarification.

In the cases of Chiari, cited by Renter, the disease appeared to be
principally confined to the ligamentous apparatus in the cervical region
and was supposed to be due to polyarthritis rheumatica.

The case of Piogey-*' was evidently one of arthritis deformans, in-
volving the spinal cord, to which may be added the case of McCarthy.

This classification, though more or less artificial, shows that there
are some cases in which the* process attacks by preference certain parts
of the vertebrae. For example, in some the small joints are chiefly,
if not exclusively, involved ; in others the intervertebral discs suffer
the greatest change, while in still others there is involvement of all
parts of the vertebras.

The impression which I have about these cases is that they are all
more or less allied so far as their pathologic basis is concerned, though
differing perhaps etiologically ; and I feel inclined to follow the teach-
ing of those who look on the Marie and von Bechterew types as ex-
pressions of one and the same process.

Examination of the nervous system in these cases is quite rare.
\'on Bechterew claimed a nervous origin for his case. He described
a chronic leptomeningitis of the cervical region which compressed the
spinal roots, and as a result of which the motor roots were less in-
tensely degenerated than the sensory roots. There was marked de-
generation of the white matter in the cervical and upper dorsal regions,
especially in the columns of Goll and Burdach. The gray matter
was not involved. The cliangc in the roots was found especially in
the upper thoracic and lower cervical regions. It was not present in
the upper cervical, and was less in the lower thoracic and lumbar
regions. There was diffuse degeneration of the anterior pyramidal
tracts in the upper thoracic and lower cervical cord. The cells of
the spinal ganglion showed degeneration and simple atrophy. The
peripheral nerves were slightly degenerated, which von Bechterew

"' Bull. Soc. anat. de Paris, 1898, p. 296.

327

10 RHEIN: nervous system in SPONDVLOSE RHIZOMELigUE

believed was due to the degenerated roots. There was some fatty
change of the atrophied muscles and disappearance of the striations.

In Leri's case the spinal cord was absolutely normal, except for a
slight diminution in the number of the cells of the anterior horns.
Leri did not believe that in all cases there is an organic lesion of the
nervous system. In one of' Renter's cases the spinal cord was ex-
amined, but showed no pathologic change.

In Chiari's case (quoted by Renter) there was a pachymeningitis in
the cervical region. The medulla oblongata showed a certain amount
of ascending degeneration. In one of Fraenkel's cases the spinal
cord and spinal ganglia were examined, but they appeared to be nor-
mal. The muscular bundles were atrophied, and the muscle paren-
chyma was replaced by fatty tissue, and there was hypertrophy of the
perimysium.

The cases of von Bechterew. Chiari and my own were the only in-
stances showing changes in the nervous system, though in Leri's case
the cells of the anterior horns were diminished in number. There
is, therefore, very little evidence pointing to the nervous origin of
these cases. The lesion in my case was slight and could, I believe, be
due to pressure from the rigid bony canal, possibly by reason of the
narrowed foramen, while the peripheral changes could result from the
degeneration of the roots thus occasioned.

The lesions of the nervous system in the cases of Chiari and my own
were so slight as hardly to suggest that the bone symptoms were an
expression of this lesion. On the contrary, it would appear that the
lesions of the nervous system were caused by the bone disease. The
nervous symptoms and lesions are not prominent in these cases, and
more likely of a secondary nature. A nervous disturbance which
would call forth such marked and localized bone changes would be
expressed in other marked nervous symptoms. It is improbable that
the trophic bony changes would stand out so prominently to the exclu-
sion of other symptoms. I believe, with Leri, that certainly not all
these cases have a nervous origin.

The relationship of arthritis deformans to these cases is, I believe,
remote. The difference is striking, if the results of the autopsy of
the case reported by Piogey-*' are contrasted with the pathologic
changes which have been described above. In Piogey's case of
multiple articular deformity and general ankylosis the characteristic

328

RHETX: XRRVOrS SYSTEM IN SPONDYLOSE RHIZOMELIQUE 11

lesions of chronic arthritis deformans were described. The articulating
processes had completely disappeared in places and the surfaces were
connected by osteophites and lamell?e. A similar lesion was found in
the articulations of the vertebrae. While the metacarpal and scapulo-
humeral articulations were not ankylosed, there were changes in the
cartilages, which were eroded. The recent conclusions of Rimann-''
are interesting in this connection. As a result of a j^athologic study
of arthritis deformans he claimed that:

1. Macroscopically there was hypertrophy and atro])hic change, the
former being characterized by proliferative changes in the joints and
synovia, and the latter by complete failure of these. Regressive
changes in the bones and cartilages were common to both.

2. This change is frequently associated with fibrous metaplasia of
the medullary tissues of the bones and cartilages.

3. Metaplastic changes in the atrophic form are more frequent and
more extensive and result from a local reaction of the pathologic
irritant : the expressions of a general disease of the organism.

4. There are transitional changes between arthritis deformans hyper-
trophica and atrophica.

The changes in the synovial membranes and the production of
osteophites or exostoses, do not appear in the description of the
anatomic specimens of the cases under discussion. Ehrhardt calls
attention to the fact that the ossification causes changes in the shape
of the joints which are dissimilar to those found in arthritis deformans,
and Simmonds, Elliott and Siben hold that arthritis deformans of the
spine is a different process entirely.

On the other hand, Senator,-^ Saenger,-" and Auerbach^" believed
that the disease described by Striimpell is an expression of arthritis
deformans, and Schlesinger and Oppenheim do not recognize any
difference between arthritis deformans and the other forms of stiflfen-
ing of the spinal column.

R. Llewellyn Jones''^ believed that no distinction can be made be-
tween cases of so-called " poker-back " and the polyarthritic type of
arthritis deformans.

While everyone who has seen many cases of arthritis deformans

"Arb. a. d. path. Inst, zu Berlin, 1906, p. 139.
-*Berl. klin. Wchnschr., 1899, No. 47, p. 1025.
^ Miinchen. med. Wchnschr., .1899, No. 47, p. 1509.
^ Miinchen. med. Wchnschr.. 1900, No. 24, p. 750.
" Edinburgh Aled. Jour., 1906. No. 61, p. 103.

329

12 rhein: nervous system in spondylose rhizomelioue

recognizes that the spinal column may also become involved, the whole
clinical picture, outside of the pathologic appearance, seems to be en-
tirely different from that presented by cases of ankylosing rigidity of
the spinal column.

Reprinted from The Journal of the American Medical Association,

Aug. 8, 1908, Vol. LI, pp. 463-467-

American Medical Association, 103 Dearborn Ave., Chicago.

330

ACUTE ANTERIOR POLIOMYELITIS — A

PATHOLOGICAL STUDY OF

THREE CASES.*

IBy WILLIAMS B. CADWALADER, M.D..

PHILADBLPBIA.

rATHOLOGIST TO THB PHILADELPHIA ORTHOPEDIC HOSPITAL AND IN
FIRMARY FOR NBKVOUS DISBASBS.

Although acute anterior poliomyelitis had been
recognized as early as 1784 by Underwood,^ and by
Badham^ in 1835, it was not until Heine^ in 1840
published his observations that it became established
as a definite clinical entity. For many years the
pathological anatomy remained obscure, but in i860
Heine* and also Duchenne," on purely clinical evi-
dence, strongly urged the spinal origin of this dis-
ease.

Cornil' in 1863 was the first to demonstrate dis-
tinct lesions in the spinal cord. Prevosf in 1865
studied the spinal cord of a long standing case and
found lesions confined to the anterior horns of the
gray matter, with changes in the ganglion cells, and
believed that the disease depended upon an acute pri-
mary change of the ganglion cells. This was con-
firmed three years later by Lockhart Clarke.' In
r868 Meyer® first pointed out that acute poliomye-
litis also occurred in adults. Charcot and Joflfroy^"

♦From the Laboratory of Neuropathology of the Uni-
versity of Pennsylvania.

in 1870 upheld the view of Prevost as to the origin
of the disease, but up to this time most observa-
tions had been made upon cases which died long af-
ter all the acute signs had disappeared.

Roger and Damaschino" in 1871 described the
pathological changes found in the spinal cord of
three children dying at intervals of two months, six
months, and thirteen months, respectively, after the
onset of the paralysis. They found perivascular
round cell infiltration, small areas of necrosis, and
degeneration and destruction of the ganglion cells,
together with slighter changes in the white columns,
and concluded that it was due to an acute inflam-
matory condition, particularly involving the anterior
horns.

Archambault and Damaschino^^ in 1883 published
a report of an acute case which was fatal in twenty-
six days after the onset. This seems to have been
the first acute case with necropsy on record. They
found an acute myelitis of the anterior horns.

The first case which I wish to describe occurred
in a child aged two years and seven months who
was admitted to the Orthopedic Hospital and In-
firmary for Nervous Diseases and died on the sev-
enth day after the onset of the paralysis. The sec-
ond and third cases occurred in adults dying on the
fifth and fourth days, respectively, after the first
signs of motor disturbance.

I wish to express my thanks to Dr. Crispin and
Dr. Donhauser of the Pennsylvania Hospital for
the pathological material of the second and third
cases, and also to Dr. Spiller for many valuable sug-
gestions in carrying out this study.

Case I. — White male, two years seven months.
Family history negative. Was admitted to the Or-
thopedic Hospital and Infirmary for Nervous Dis-

eases in Dr. Mitchell's ward under the care of Dr.
Eshner, complaining of fever, pain in back, rigidity
of the neck, headache, and weakness of both legs
and arrns; this condition had lasted two days.

Physical Examination. — Respirations were shal-
low and rapid; temperature 992-5. On respiration
there was a violent upward movement of the larynx.
Pupils were equal and reacted to light and accom-
modation. The thoracic and abdominal organs were
normal. There was a total flaccid paralysis of both
lower extremities. The tendon reflexes were ab-
sent, and there was a flexor response on irritation
of the soles of the feet. There was no atrophy of
any of the muscles. The upper extremities were
both somewhat weak. Sensation was normal every-
where. There was moderate rigidity of the pos-
terior muscles of the back. Patient was very drowsy
and weak. During the next five days the tempera-
ture varied from 99 to loi and the patient was
gradually growing weaker. Finally the respirations
became shallow and rapid and patient died of res-
piratory failure on the seventh day of the disease.

The autopsy was performed the same day by Dr
Hill, the resident physician. Nothing abnormal was
found in any of the abdominal or thoracic organs
excepting for intense general congestion. No cul-
tures were made.

^ Brain.— The Vessels of the pia were much in-
jected; otherwise no gross change was found.

Spinal Cord.— The dura was somewhat discolored
about the cervical swelling, but there was no defi-
nite change which could be seen with the naked eye
On cross-section before the tissue had been put in
formalin there could be seen numerous minute red
dots and lines in the gray matter; some of these
seemed to extend into the white matter as well

This was most marked in the lumbar swelling.
Sometimes the hemorrhages appeared to be more
marked on the left side in the lumbar and sacral re-
gions, and again higher up more marked on the right
side. This appearance extended from the sacral re-
gion all the way up the cord through the medulla and
pons, but gradually diminished in intensity from
below upward.

Microscopical Examination, — Lumbar enlarge-
ment. There is a cellular infiltration which involves
chiefly the anterior horns of the gray matter, but
the intermediate portions and posterior horns are
also afifected to a less degree. The blood-vessels are
dilated and engorged with blood corpuscles. In a
few sections small areas of necrosis are found.
The infiltrating cells are difitusely scattered, but
here and there show a tendency to accumulate in
the perivascular spaces. Many of the cells are of
the lymphocyte type, having a small, round, deeply
stained nucleus and a small amount of homogeneous,
nongranular protoplasm, while others present a
larger oval, indented and irregular nucleus less well
stained, with a relatively larger amount of pale and
vacuolated nongranular protoplasm. A few red
blood corpuscles and polymorphonuclear leucocytes
are found scattered here and there outside the blood
vessels. The ganglion cells of the anterior horns are
few in number ; most of those remaining present va-
rious degrees of degeneration, but occasionally a
healthy cell can be found in the most intensely af-
fected areas, while on the other hand they are gen-
erally less afiFected in the less diseased parts. All
degrees of changes can be seen from a be-
ginning chromatolysis to total destruction. A
few small round cells are found collected
about the periphery of the ganglion cells,

but it does not appear to be a definite neurono-
phagic process. The cells of Clarke's column are
similarly affected, but much less so than those of the
anterior horns.

With the Wiegert method the nerve fibers are
widely separated and swollen ; many are broken and
present a varicose appearance. The anterior and
posterior roots are pale. There is a perivascular cel-
lular infiltration of the white matter, chiefly at the
terminal portions of the blood-vessels in the antero-
lateral columns near the anterior horns. The axis
cylinders and neuroglia cells are somewhat swollen.
There are no definite foci of the inflammatory cells
apart from those about the vessels. The posterior
and posterolateral columns of the white matter are
little affected.

The pia is infiltrated with small mononuclear cells,
chiefly on the anterior surface, but the infiltration
can be traced to the posterior surface as well. The
blood-vessels of the pia are filled with blood cor-
puscles, and their walls are slightly infiltrated.

Sacral Region. — The appearances are similar in
every respect.

Thoracic Region.— Here the process is the same,
but the pia on the posterior surface has escaped.

Cervical Region. — The pia is slightly affected
about the anterior surface in close relation to the
artery of the anterior fissure. The gray matter of
the anterior horns is much less involved than that
of the lumbar region, and the posterior horns are
little affected. The anterior horn cells are few in
number and are similar in appearance to those of
the lumbar cord There is only very slight perivas-
cular infiltration of the blood-vessels in the antero-
lateral columns of the white matter.

Medulla Oblongata. — In the gray matter a few

■;, t)

distinct, small, and recent hemorrhages can be seen,
and to a less degree in other parts. The walls of the
blood-vessels here and there show slight cellular in-
filtration. The white matter here, as in the spinal
cord, was much less affected than the gray. The
cells of the twelfth nucleus were for the most part
entirely normal, but a few were somewhat misshapen
and stained poorly by the Weigert method. The cells
of the seventh nerve nucleus were little affected.

Pons. — There is a slight diffuse cellular infiltra-
tion with some perivascular infiltration of small
round cells, but much less marked than that seen
in the medulla oblongata and the spinal cord.

Cerebral Peduncles. — A slight perivascular infil-
tration of small round cells could occasionally be
found in the gray matter.

Paracentral Lobule. — Here there was a slight peri-
vascular infiltration and also a slight cellular infil-
tration of the pia.

Cerebellum. — The pia is slightly affected, and
there are a few small hemorrhages in the cortex.

Optic chiasm and cranial nerves showed nothing
abnormal.

Cauda Equina. — The blood-vessels here were
filled with blood corpuscles, and a few small round
cells were occasionally seen infiltrating their walls.

The peripheral nerves and spinal ganglia were not
examined.

Case II. — P. M., adult Italian. The patient was
brought to the Pennsylvania Hospital September 4,
1907, in an ambulance, complaining of pain in his
head, back of neck, both thighs and legs, lasting four
days. He was constipated ; the bowels had not been
moved for three days, and he had not voided urine
for twenty-four hours. His temperature was
103 3-5 ; pulse T04 ; respiration 36. He was unable

to move his lower extremities. His bowels appeared
to have been paralyzed, but there was no loss of
sphincter control.

Physical Examinction. — Patient is a well devel-
oped, fine looking Italian. Face is flushed; pupils
are equal and react to light and in accommodation;
conjunctivae are injected; tongue is protruded in
the mid-line, and there is slight fibrillary tremor;
there is no pulsation in the vessels of the neck. The
chest is well formed, very muscular and with good
expansion. Respiratory movement rather rapid but
not labored. Radial pulses are equal, rapid and of
good volume. The abdomen is soft and tympanitic.
The lower quadrant was greatly distended before
catheterization, when thirty-nine ounces of urine
were removed, after which the abdomen was
everywhere soft He complains of much pain
about the costal margins. There is abso-
lute paralysis of both lower extremities, with
loss of tendon reflexes. No ankle clonus ;
and there is a flexor response on irritation
of soles of feet. Both feet are held in partial ex-
tension. There is a suspicious Kernig's sign of both
legs. The upper extremities are somewhat weaker
than normal and slightly spastic. There is no im-
pairment of sensation and no loss of sense of posi-
tion. At 8:20 p. M. on the same day he requested
that his thighs be flexed on the abdomen and knees
externally rotated until the thighs made an angle
of nearly forty-five degrees with the trunk and the
legs flexed on the thighs, the feet resting on the
bed. He seemed more comfortable in this position.
Later, at 9 p. m., his body was cold and he was per-
spiring profusely, although his temperature was 103.
During the sponging which had been ordered, it was
noted that his finger tips were cyanotic and the pulse

rapid. When the temperature was being taken by
the mouth at 10:05 ^- ^- ^e became very cyanotic
and frothed at the mouth, both arms were moved
toward his head, and there was a distressed expres-
sion about the mouth. The right arm, hand and
fingers were twitching, but there was no movement
of either leg or toes. The pulse was rapid but
strong. Artificial respiration was begun, and it was
found that the arms were very spastic. Cyanosis
developed when artificial respiration stopped. A
mouth gag was inserted and artificial respiration
continued. The pulse was very rapid, weak and
running. He was given 1-150 gr. of atropine injected
into a vein of the arm. The patient died at 10:50
p, M., the fifth day of the disease.

Autopsy loii. — Hospital No. 1635. Adult Ital-
ian, September 4, 1907. Performed by Dr. Crispin.

Anatomical Diagnosis. — Acute endocarditis (mi-
tral vegetations). Congestion of lungs, spleen and
kidneys. Fatty degeneration of liver. Persistent
thymus.

Spinal Cord Microscopical Examination. — Lum-
bar enlargement. There is an intense cellular infiltra-
tion affecting chiefly the gray matter, but the white
matter and pia are also involved. In some sections
the most marked changes are found in the central
and basal portions of the anterior horns, while in
others the process is more diffuse, the anterior horns,
the posterior horns, and intermediate portions all be-
ing affected alike. The blood-vessels are prominent
and filled with blood corpuscles, many of which have
wandered into the surrounding tissue. The peri-
vascular spaces are filled with small round cells.
This is most marked about the anastomotic branches
of the anterior spinal artery, which in some sec-
tions can be seen cut longitudinally in the gray mat-

ter at the base of the anterior horns. The central
canal in sections from the lower part of the lumbar
cord is almost obliterated, but its outline can be dis-
tinguished by the arrangement of the ependyma
cells, which appear normal. At higher levels of this
region the central canal is wide open and some of
the ependyma cells have been torn off. The lumen
of the canal is filled with an indefinite, structureless,
albuminoid material and a number of round cells.
Many of the ganglion cells have entirely disappeared,
and many show various degrees of degeneration.
They are irregular in outline, the nuclei are indis-
tinct and eccentrically placed, and their dendritic
processes are broken. In the anterior and lateral
portions of the anterior horns there are numerous
separate masses of round cells completely surround-
ing ganglion cells," heaped one upon another so that
the nerve cells can be distinguished with difficulty.
The cells of Clarke's column are similarly affected.
The nerve fibers are swollen and appear broken and
widely separated and varicose. The anterior and
posterior roots are paler than normal when stained
by the Weigert method. In the white matter there is
a cellular infiltration chiefly confined to the perivas-
cular spaces, with some proliferation of the neurog-
lia cells and swelling of the axis cylinders. The
anterior, lateral, and posterior columns are all equal-
ly involved, but much less so than the gray matter.
The pia is also infiltrated with round cells through-
out the circumference of the cord( but it is much
more marked about the blood vessels on the anterior
surface and along the artery of the anterior fissure.
The blood-vessels are filled with blood corpuscles,
and their walls are infiltrated with round cells, but
this is chiefly confined to the adventitia.

Sacral Region. — Here the appearance is exactly

the same, except that the gangHon cells are fewer
in number and the grouping of round cells about
those remaining is less striking.

Thoracic Region. — This portion of the cord pre-
sents much the same appearance as the lumbar re-
gion, but the pia is less affected, and the grouping
of round cells about the ganglion cells is also less
marked here.

The cervical swelling is similar to the lumbar
portion, but in addition there are a few small open
spaces in the gray matter where the tissue
has dropped out.

Medulla Oblongata. — Although much less than in
the spinal cord, inflammation can be found through-
out the medulla oblongata, especially in the gray
matter about the fourth ventricle and in close rela-
tion with the blood-vessel, particularly those pass-
ing from the anterior surface through the raphe to-
ward the gray matter. The white matter is slightly
affected. The cells of the nuclei of the cranial
nerves are also slightly affected. The pia is infil-
trated with round cells and the adventitial coat of
the blood-vessels also somewhat infiltrated.

Pons. — There is a considerable infiltration in the
region of the sixth nucleus, and its cells are much
diseased, but in other respects the changes here are
much the same as those of the medulla oblongata.

Cerebellum. — A few small hemorrhages can be
seen in those parts which were in close relation with
the medulla and pons.

Basal Ganglia. — A few scattered perivascular in-
filtrations can be found here.

Postspinal Ganglia. — There are many round cells
scattered between and about the ganglion cells, many
of which show degeneration With the Weigert
method they appear yellowish and some nearly

black; the nuclei of many are indistinct, eccentrical-
ly placed, and some have disappeared. There are
numerous black colored granules to be seen gener-
ally near the periphery of the cells.

The anterior tibial, sciatic, and median nerves and
portions of the brachial plexuses showed nothing
abnormal.

Case III. — J. F., tv^enty-five years old; admitted
to the Pennsylvania Hospital September 25, 1907.
His family history was negative. He had always
been healthy. He had been occupied as a day labor-
er, which he had not been used to, and he attributes
his condition to overwork. He first noticed numb-
ness of his feet September 23, 1907. The next day,
after helping to lift some heavy boxes, his legs
suddenly gave way under him, and he entirely lost
control of them, and there was some disturbance
of the functions of the bladder.

Physical Examination. — On admission: tempera-
ture 102; pulse 102; respirations 48. Anemic look-
ing man with flaccid paralysis of both lower ex-
tremities. Some stiffness and aching of the muscles
of the neck, and he complained of indefinite numb-
ness of the chest and abdomen. Pupils were equal
and reacted sluggishly to light. Eyes, nose, and
throat were normal. Glands were not enlarged.
There was no pulsation of the vessels of the neck.
Chest well formed and expands well. Lungs nor-
mal but respiratory movements were greatly in-
creased. Heart sounds weak. No murmur. Ab-
dominal organs all normal. There was complete
flaccid paralysis of both legs with loss of tendon
reflexes and weakness of both arms. Sensation was
normal. Blood count showed hemoglobin, 80 per
cent.; leucocytes, 28,650. He died September 26,
1907, the fourth day of the disease.

The spinal cord and a portion of the pons and
peripheral nerves were examined.

Autopsy 1018. — Hospital No. 1833. Male, aged
twenty-five years. September 26, 1907. Performed
by Dr. Donhauser one and a half hours after death.
Edema of lungs, congestion of liver, spleen, and kid-
neys.

Spinal Cord Microscopical Examination. — Lumbar
enlargement. There is a marked diffuse cellular infil-
tration throughout the gray matter ; the white matter
and the pia are also affected, but to a much less de-
gree. In many sections the anterior horns, the pos-
terior horns and intermediate portions of the gray
matter are all equally affected, but in others the
posterior horns are less so than the anterior. In-
flammatory cells are seen diffusely scattered in great
numbers and accumulated in the perivascular spaces.
The vessels are only slightly distended and contain
few blood corpuscles. Here and there small open
spaces are seen when tissue has dropped out of sec-
tions. Most of the ganglion cells of the anterior
horns have entirely disappeared, but a few still re-
main which appear much distorted, with an indis-
tinct nucleus frequently eccentrically placed. The
dendritic processes of many are broken. In a few
sections the inflammatory cells are seen densely
packed about the ganglion cells impinging upon their
borders. With the Weigert method the nerve fibers
seem somewhat more widely separated than normal-
ly, but stain well and do not appear swollen except
in a few places when in close relation to a blood-ves-
sel. The anterior and posterior roots just after
they have made their exit from the cord are paler
than normal. The cells of Clarke's column present
the same changes as the motor cells of the anterior
horns. The central canal appears normal. In the

white matter the vessels are distended, and many
round cells are seen in the perivascular spaces.
There is some diffuse infiltration in the white mat-
ter close to the gray matter of the lateral horns,
with some proliferation of the neuroglia cells. The
pia is infiltrated with cells throughout the circum-
ference of the cord, but the greatest change is seen
on the anterior surface. The vessels of the pia are
filled with blood cells, and their walls, particularly
the adventitial coat, are infiltrated with small round
cells.

Sacral Region. — In a few sections the anastomotic
branch of the anterior spinal artery can be seen cut
longitudinally as it enters the base of the anterior
horn of one side, presenting an intense perivascular
cellular infiltration. In other respects the appear-
ance is the same £s that of the lumbar region.

Thoracic Region. — The gray matter and the white
matter are similar in every respect to that of the
lumbar region, but the pia is slightly affected on the
anterior surface only.

Cervical Region. — Here the process is identical
with that seen in the lumbar region, except that the
pia is less affected.

Pons. — Only a very few sections from this part
were studied, and a few scattere(^ perivascular infil-
trations were found, chiefly confined to the gray
matter.

The sciatic and median nerves showed nothing ab-
normal with the Weigert method. The remaining
portions of the nervous system were not examined.

The cells taking part in the infiltrations were the
same in each case and can be conveniently described
at one time.

Under an oil immersion lens when stained with

hemalum and fuchsin there were many round nuclei,
some very large and others smaller, staining a rob-
in's egg blue color, with a few deep staining chro-
matic granules and a thin rim of protoplasm. These
cells were irregularly scattered and widely separated
from one another, never grouped in definite foci.

There were great numbers of cells resembling
lymphocytes identical with those found in the pia,
generally scattered throughout the gray matter and
densely packed in the perivascular spaces, present-
ing a small, dark-staining, round nucleus and a small
amount of nongranular homogeneous protoplasm,
also many larger cells with a large paler staining nu-
cleus of almost any shape but generally oval, curved
or indented, and a larger amount of protoplasm
stained a moderately deep pink color and often vacu-
olated. Very few polymorphonuclear cells were
found, but numerous red blood corpuscles were scat-
tered here and there.

In addition numerous cells were found which did
not resemble any definite type and in all probability
were undergoing degeneration and would eventual-
ly have become typical compound granular cells.

The cases which I have described are typical ex-
amples of acute anterior poliomyelitis considering
both the clinical an^ pathological pictures. One case
of a child and two of adults, beginning rather sud-
denly with fever, pain in the lower extremities and
back, some rigidity of the posterior muscles of the
neck, flaccid paralysis of the lower extremities with
loss of tendon reflexes and no sensory changes, blad-
der and rectal disturbances, the paralysis ascending
rapidly, resulting in death following bulbar involve-
ment. The pathological findings are very similar to
those reported by Dauber. Goldscheider, Siemer-
ling, Lieppman. Redlich. Risseler. Biilow-Hansen

and Harbitz, Sherman and Spiller, Wickman, Har-
bitz and Scheel, Forrssner, and Sjovell.

Dauber^^ had an acute case in a child that died
after five days, presenting infiltration of the pia and
perivascular and diffuse infiltration of small round
cells in the gray matter, also perivascular infiltration
of the ganglion cells and degeneration of the gan-
glion cells.

Goldscheider^* found the most intense changes in
the anterior horns of the lumbar region, slight de-
generation of the anterior and posterior roots by the
Marchi method, and degeneration of ganglion cells,
and considers poliomyelitis to be a localized myelitis
affecting chiefly the anterior horns through the dis-
tribution of the anterior spinal artery.

Siemerling^^ studied two acute cases in children
and found myelitic changes most marked in the lum-
bar and cervical regions, with some degenerative
changes in the anterior and posterior roots by the
Marchi stain, destruction of ganglion cells and slight
perivascular infiltration in the white matter.

Lieppman^'' reported a case which developed dur-
ing a severe attack of furunculosis and died four
months later of diphtheria. He agrees with Gold-
scheider and Siemerling that the process follows the
course of the blood-vessels of the anterior horns.

Risseler, quoted by Kahlden," describes three
acute cases in which there were various forms of
degeneration of ganglion cells with diffuse and peri-
vascular and pericellular infiltration of round cells,
particularly in the lumbar region. He upholds Char-
cot's theory, namely, that acute poliomyelitis is due
to a primary change of the ganglion cells.

Redlich^^ reports one case of a child that died on
the ninth day. The anterior horns were chiefly af-
fected, but the posterior horns and the white mat-

ter and pia also. The infiltrating cells contained
many fat granules, which he thinks are typical of
inflammation in the nervous system. He also found
degenerations of the ganglion cells and of Clarke's
column, which he considers to be of two distinct
varieties of necrobiosis. There were slight peri-
vascular infiltrations in the medulla, cerebral pe-
duncles, particularly in the tegmentum, none of the
cortex itself, but slightly of its pia.

Biilow-Hansen and Harbitz^® had two fatal cases
and one abortive case, brothers and sisters of the
same family, all affected at the same time. Two
died on the fifth and seventh day, respectively. The
usual changes in the gray matter of the cord, pons,
medulla, and slight changes of the cells of the vagus
nuclei were found.

Spiller and Sherman^" reported a case under the
title of "Acute Polioencephalomyelitis," presenting
the clinical picture of Landry's paralysis and
terminating fatally in thirty-eight hours after
the appearance of the first definite symptoms
of motor disturbance. They found intense
cellular infiltration throughout the gray mat-
ter of the anterior horns. The pia was also
affected, and there were a few perivascular foci
within the white columns. The ganglion cells of the
anterior horns were much degenerated. Changes ex-
tended throughout the spinal cord, medulla, pons,
and cerebral peduncles to the basal ganglia, but were
not very definite in the cerebral cortex and sub-
adjacent white matter, although even here the ves-
sels were much dilated. They consider that the
process was essentially a polioencephalomyelitis, al-
though the white matter was not intact, and that
acute anterior poliomyelitis in an adult is similar to
that in a child.

The naked eye appearances of the spinal cord and
brain in my cases when in the fresh state were sim-
ilar to those of most recorded cases, consisting only
in moderate congestion of the cerebral vessels and a
swollen and red appearance of the gray matter of
the cord when seen on cross section. The membranes
of the cord were slightly hyperemic, but no actual
exudation was seen.

Microscopically the pia was infiltrated with mono-
nuclear cells, particularly on the anterior surface,
and always most marked in the lumbar region. This
was found throughout the length of the cord, gradu-
ally diminishing from below upward. In Cases I
and II it was distinct about the medulla oblongata
and pons, and traces could be found over the cerebral
cortex. Rarely a few inflammatory cells were found
on the outer surface of the pia. Buzzard-^ has
called attention to this fact, which is in harmony
with the absence of a true meningeal exudate visible
to the naked eye in poliomyelitis, and in contrast
with what is generally seen in cases of acute septic
meningitis. However the involvement of the pia is
sufficient to explain the rigidity of the muscles of the
neck and pain and soreness of the lower extremities,
which are not uncommon in the early stages of most
severe acute cases of poliomyelitis. There was no
definite relationship between the intensity of the
changes seen in the pia and the changes in the gray
matter. In Case II there was considerably more
perivascular infiltration of the vessels extending
through the posterior white columns of the lumbar
cord than in the other two cases, and here the pia
on the posterior surface also showed greater
changes.

Harbitz and Scheel" found in their cases that foci
in the cord with perivascular infiltration corre-

sponded to the areas of inflammation in the pia at
the same level, while on the other hand inflammation
of the pia could be found without any inflammation
having developed in the cord at the same level.
Therefore, they are convinced that inflammation be-
gins in the pia with a simultaneous infection of the
cerebrospinal fluid and extends into the cord along
the vessels.

The walls of the vessels about the anterior sur-
face of the cord were slightly infiltrated with mono-
nuclear cells, chiefly confined to the adventitia.
Wickman" found that the veins were more affected
than the arteries ; in my cases, however, there was
little if any difference. UnUke Batten's^* cases
there was never any evidence of thrombosis.

A marked perivascular infiltration of the branch
of the anterior spinal artery as it extended into the
anterior median fissure was always found, and fre-
quently this could be traced along its anastomotic
branch as it extended into the base of the anterior
horn of one side.

Throughout the length of the spinal cord the gray
matter was always intensely diseased, the anterior
horns suffering more than the posterior, except in
a few sections of the lumbar region from Case II,
where there was little difference. Degeneration of
the ganglion cells was very marked, extending over
large areas. Generally the degree of degeneration
and destruction corresponded to the intensity of the
disease in the surrounding structures. Occasional-
ly after a careful search a healthy looking ganglion
cell was found in the midst of a severely affected
area.

Interstitial and parenchymatous changes were both
marked and were found side by side. Severe de-
generation of ganglion cells was never found far dis-

tant from interstitial changes; generally where in-
terstitial changes were less marked ganglion cells
were not severely diseased.

In Case II neuronophagia was very striking, al-
ways confined to the spinal cord, particularly in the
cervical and lumbar regions. In Case III it was oc-
casionally found in slight degree. No attempt was
made to trace the various stages of this process in
detail ; the general appearances, however, were very
like those described by Forrssner and Sjovell'"^ in
two acute cases of poliomyelitis.

In the cervical and lumbar enlargements and es-
pecially the sacral region they found many ganglion
cells completely submerged beneath heaps of round
cells ; also round cells were seen after having pene-
trated into the substance of the ganglion cells. As
the process advanced the ganglion cells degenerated
and the round cells had gained in the quantity of
their protoplasm at the expense of the ganglion cells.

They believe that healthy ganglion cells are never
attacked, but that there is some chemical substance
generated during the course of the disease which has
killed or decrea^d their vitality and which has a
positive chemotactic action for the round cells, and
therefore they attack the ganglion cells and complete
their destruction.

Neuronophagia unquestionably plays a very im-
portant part in the destruction of ganglion cells in
certain cases of poliomyelitis. In the most intensely
affected parts of the gray matter it was never found,
therefore one might infer that the process had been
already completed and is one of the earliest changes
to take place. Forrssner and Sjovell suggest that it
may explain the very rapid and sudden onset of
paralysis. On the other hand early acute cases have
been examined with marked degenerative changes of

ganglion cells and no evidence of neuronophagia, so
that we must admit that there may be some other
cause for the rapid destruction of ganglion cells
which as yet we know little about. The posterior
spinal ganglia were only examined in Case II ; mod-
erate degenerative changes were found. After a
careful search of the literature I have been able to
find but one reference to examinations of the spinal
ganglia in poliomyelitis. Forrssner and Sjovell, in
the same article which has already been quoted, de-
scribe finding a few round cells outside but in close
relation with the small blood vessels and no changes
in the nerve cells. On account of so slight a lesion
they assume that this may explain the preservation
of sensation.

We as yet have little definite knowledge as to the
manner in which lesions found in poliomyelitis are
produced. Many observers believe that the infec-
tious agent is carried to the anterior horns of the
gray matter by way of the anterior spinal artery.
In many instances the distribution of the lesions in
the gray matter corresponds very closely to the dis-
tribution of the blood vessels. The white matter, on
the other hand, is but little affected, and here the
blood supply is derived from the smaller vessels
extending inward from the pia. Kadyi-^ has shown
by his experiments that there is no definite anasto-
mosis between the area supplied by the anterior
spinal artery and that supplied by the pial vessels,
and that there is an area along the adjacent borders
of the gray and white matter which is partly supplied
with blood by one and partly by the others. Wick-
man is inclined to favor the lymphogenous route of
infection as most probable, and refers to the simi-
larity of rabies and poliomyelitis.

If we accept the teaching that true perivascular

lymph channels do exist, we can understand how
lymph-borne infection may cause lesions correspond-
ing to the distribution of the blood-vessels of the
nervous system similar to those frequently found in
acute anterior poliomyelitis.

Conclusions. — '( i ) Acute anterior poliomyelitis is
essentially an acute polioencephalomeningomyelitis.

(2) The process is the same during infancy and
adult life.

(3) The process is most marked in the lumbar
and cervical enlargements of the cord and frequently
may extend upward as far as the cerebral cortex.

(4) Interstitial changes predominate and occur
together with parenchymatous changes. Parenchy-
matous changes never occur without interstitial
changes.

(5) The localization and intensity of cellular infil-
tration depend upon the distribution and vascularity
of the area affected.

(6) Neuronophagia is an important factor in the
destruction of ganglion cells.

REFERENCES.

1. Underwood : A Treatise on the Diseases of Children,
1784.

2. Badham : London Med. Gazette, 1836.

3. Heine : Beobacht. iiber Lahmungszustande der unteren
Extremitaten und deren Behandlung, 1840.

4. Heine : Ueber spinale Kinderlahmung. 2 Aufl., i860.

5. Duchenne (de Bologne) : de I'electrisation localisee,
1855. 2 ed., 1861.

6. Cornil : Compt. rend, de la Soc. de Biolog., 1863, p.
187.

7. Prevost: Compt. rend, de la Soc. de Biolog., 1865,
XVn., p. 215.

8. Johnson and Lockhart Clarke: Med. Chir. Trans.,
XLI., p. 249, 1868.

9. Meyer: Die Elektricitat in ihrer Anwendung, u.s.w.,
3 Aufl., 1868, s. 209.

10. Charcot et Joffroy : Archives de Physiol, norm, et
path., 1870, III., p. 134-

11. Roger et Damaschino : Comp. rend, de la soc. de
Biol., 1871, sme serie, t. III., p. 49.

12. Archambault et Damaschino : Revue Mensuelle des
Maladies de I'Enfance, 1883, Vol. I.

13. Dauber: Deutsche Zeit. fiir Nervenheilk., Bd. IV.,
1893, s. 200.

14. Goldscheider : Zeit. fur klin. Med., Bd. XXIII., 1893,
s. 494.

15. Siemerling: Arch. f. Psych., 1894, Bd. XXVI., s. 267.

16. Lieppman : Deutsche med. Wochensch., Bd. XXXIV.,

1893, s. 823.

17. Kahlden: Centralhl. f. allgemeine Path. u. path.
Anatomie, Bd. V., 1894, s. 729.

18. Redlich: Wiener klin. Wochenschrift, Bd. XVI.,,

1894, s. 287.

19. Biilow-Hansen u. Harbitz : Ziegler's Beitrage, 1889,
Bd. XXV., s. 517.

20. Sherman and Spiller: Phila. Med. J cur., March 31,
1900.

21. Buzzard: Brain, 1907.

22. Harbitz and Scheel: Jour. Amer. Med. Asso., No. 17,
Oct. 26, 1907.

23. Wickman : Arbeiten a. d. path. Institut der Univer-
sitat Helsingfors (Finland), 1905, Bd. I., Hft. 1-2, s. 109.

24. Batten: Brain. 1904.

25. Forrssner and Sjovell: Zeit. f. klin. Med.. Bd. LXIIL,
1907. Hft. I., bis. 4.

26. Kadyi : Quoted by Buzzard, Brain, 1907.
X7IO Locust Strbbt.

Reprinted from the University of Pennsylvania Medical Bulletin, October,
1908.

THE POST-GRADUATE STUDENT^
Bv William G. Spiller, M.D.,

PROFESSOR OF NEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE
UNUTRSITY OF PENNSYLVANIA

Mr. Chancellor of Washington University, Gentlemen of the Faculty,
Doctors in Medicine of the Graduating Class, Ladies and Gentlemen:

Never in the history of the world has the young man had greater
opportunity for advancement in medicine than at the present time.
The discoveries to be made are possibly not so striking and far reach-
ing as some of those of the" past. The period of forty years is widely
regarded as marking an important crisis in man's development, and yet
I cannot but feel that in the acceptance of the teaching that the best
work is done in youth an error has crept in. It is doubtless true
that Morgagni, Corrigan, Stokes, Laennec and many others accom-
plished most in early life, that Washington was a leader at nineteen,
Hamilton a statesman at thirty-two, and Rush a graduate as a Bachelor
of Arts from Princeton College in his fifteenth year. Only recently
one of the United States Senators from the South had just passed the
age of thirty years. It does not follow, however, that the early years
of a man alone are productive. Many of those who in early life have
seen what others before them had not seen, or have thought what
others had not" thought, have simply manifested their genius early, and
frequently have carried out the application of their early teaching dur-
ing many years of useful life. They did not cease their epoch-making
work after one striking discovery. They were great in early life,
but who can say that these same active minds under similar conditions
would not have made equally valuable discoveries if they had been
held back in the pursuit of their studies a few years. Some men are
born with powers of observation ; others with equal vision fail to
observe. Can anyone imagine that so striking a disease as acromegaly
came into existence at the bidding of Pierre Marie.

Xo one can dispute that the brain is more receptive when it is

^ An address delivered at the Commencement exercises of the Medical De-
partment of Washington University, St. Louis, May 28, 1908.

1 353

2 spiller: the post-graduate student

developing. The child learns more readily than the adult, and the
knowledge first acquired is often the longest retained. One may have
mastered several languages, and when a cerebral hemorrhage occurs all
but the one he learned when he first began to lisp, the impressions of
which were deeply embedded in his brain, may be forgotten. The
youth is more active, he may be more inventive, and may enter where
older but possibly less wise men fear to tread. We cannot, however,
deprive middle age and more advanced periods of the honor due them.
The great intellect is interesting in early life, but it may be even more
so in the period of maturity. We have known great men who had
passed the crisis of forty years, but we do not cease to enjoy their
mental power, and, indeed, in many instances we find more delight in
such companionship then we could have done had w^e known them
when their minds were forming.

The interesting and reliable study by Borland of four hundred
celebrities has shown that fifty years is the average age for the per-
formance of the masterwork, that for workers the average age is
forty-seven years, and for thinkers fifty-two years, the latter being also
the average age for physicians and surgeons to accomplish something
lasting. Dorland has found that some of the greatest achievements
have been those of old men long past sixty. Truly, no one can set a
period to man's greatness. As Dorland put it, not infrequently those
mentalities that ripen the slowest last the longest, and often the history
of great men shows a neglect by their fellows until forty or more
years had passed.

It has been suggested that possibly in our present-day methods the
student is carried too far, has been taught during his formative years
too much of what is known at the present time, and sometimes even
incorrectly held. This opinion seems to me fallacious. For purposes
of expediency possibly the course of tuition is too long, but for the
mental development of the student it is not long enough. It is hardly
probable that he has wasted his years of invention and discovery in
studying the knowledge and possibly unrecognized ignorance of others,
and, indeed, I question whether much incorrect instruction is given in
our best medical schools. The man who has the power to see and
think will do so all the better for a thorough grounding in medicine.

The investigating spirit is communicable. We need not expect the
pupils of a great master to make as many important discoveries as
their exemplar has done, but each in his own way often adds to the

354

spiller: the post-graduate student S

general store of knowledge. Can anyone doubt that when Charcot
died his mantle fell on his pupils, and that the influence of this great
master of neurology is felt until the present day? The Germans and
French recognize the truth of this more clearly than we do. The
German professor speaks proudly of the man under whom he received
his instruction, and counts as the greatest honor of his life the privilege
of early acquirement of knowledge from a master mind. It will
usually be found that a man great in medicine in the German or French
school has had a great teacher, and though there may have been many
pupils, possibly only one or two have attained prominence. This, how-
ever, is the law^ of all life — the survival of the fittest. The Frenchman
recognizes so fully the truth of the statement just made that he speaks
of the one who guided his youthful steps as his maitre. The investi-
gating spirit is infectious, and a scientific atmosphere is certainly most
desirable, if not absolutely necessary, for the accomplishment of
original work.

Most of you doubtless will go into general practice and will swell
the great body of those whose life's effort is the healing of the sick.
Truly a noble calling. Far be it from me to lessen the importance of
the general practitioner. His life is one of hard labor, with long hours
and many interruptions, and he occupies an important place in the com-
munity. To him confessions often are made that no priest ever hears.
A certain variety of physician, at one time common when the country
was more sparsely settled than at present, is dying out. I refer to the
man whose practice extended over miles of territory where the rail-
road did not penetrate, who at times was called to set a broken bone
or see a case of measles when the journey extended over fifty miles
or more and required three days on horseback or driving, not infre-
quently necessitating repeated fording of streams. Such a practitioner
I have recently had the pleasure of conversing with. He had been
in his profession over fifty years, had lived in one town and one house
most of that period, and enjoyed a unique position in the community.
He was not only physician to three generations of the same families,
but also friend, father confessor, and judge. A life such as this is
full of hardships, but full also of the rewards of duty well done and
softened by the love and respect of one's associates.

The life of the original investigator when fruitful is of great value
to a great number. One cannot read the life of Pasteur, for example,
without realizing the truth of this remark. He taught others to heal,

:J55

4 spiller: the post-graduate student

and his influence has been felt far and wide. The study of the cause
of disease and of the best methods of its prevention and treatment is
certainly praiseworthy. We look with admiration upon the sacrifice
of men who use their own bodies for experiments aiming to relieve
the sufifering of mankind or to add to our store of knowledge. In our
time we have the division of two sensory nerves by Henry Head in
his own arm in order that he might study the changes in sensation ;
but what shall we say of Jenner, who, in his attempt to overcome small-
pox, experimented on his own babes ! That hardness of heart and
want of paternal affection were not the causes of this apparent rash-
ness the noble life of Jenner clearly shows, and doubtless he felt con-
vinced that his methods were reliable.

It has seemed to me that when the biography of Dr. Osier is written,
the author will perforce dwell largely upon the influence exerted by
this man upon others, and chiefly upon young minds, as one of his
greatest characteristics. There are some born to be great by a dis-
covery of vast importance, and there are others great in the stimula-
tion and inspiration they give to those about them, and possibly the
latter are more valuable than the former, though their influence is so
quiet and even momentarily unrecognized that their greatness is not
always appreciated. I think I have never had a conversation with Dr.
S. Weir Mitchell without feeling that I was in the presence of a master
mind, of an original thinker.

We, as a nation, are just developing along the lines of investigation,
and as yet are hardly out of our swaddling clothes, but the infant is
strong and will certainly grow to be a giant among nations. The
future is full of promise. The American people are beginning to
recognize that men are more valuable than bricks and mortar, that a
great mind, with all its originality and power of inspiration for others,
is more than any buildings ; that the strength of a university is its
faculty. True, indeed, is Osier's statement, " The value of a really
great student to the country is equal to half a dozen grain elevators or
a new transcontinental railway," and yet we as a nation have not
accepted this as a truth. How clearly it was demonstrated when
Pasteur saved the silk industry of his country or worked with the
patriotic resolve of making French beer equal to or better than German
beer, and yet in the irony of fate the enemies of his country probably
benefited by his experiments on beer more than did his own country-

356

spiller: the post-graduate student 5

men, a fact which shows that truth cannot be confined to a narrow
body of men.

American investigators have not always had reasons to feel them-
selves unduly complimented. The Scotchman, Sydney Smith, once
said, " In the four quarters of the globe, who reads an American book?
What does the world yet owe to an American physician or surgeon ? "
In 1876 J. S. Billings wrote : " We have had and still have a very few
men who love science for its own sake, whose chief pleasure is in
original investigations, and to whom the practice of their profession is
mainly, or only, of interest as furnishing material for observation and
comparison. ... Of the highest grade of this class we have thus far
produced no specimens; the John Hunter or Virchow of the United
States has not yet given any sign of existence."

Our wealthy men are beginning to endow richly institutions of in-
vestigation, and such as those given by Rockefeller and Carnegie afford
great promise for the future of American science. A French phys-
ician recently, after a visit to the larger of our medical schools, ac-
knowledged in conversation that the opportunities in this country were
great, wished he could send his son here for his medical education, and
added that in fifty years the tide would turn, and students would cease
going to Europe and foreigners would seek America for their instruc-
tion. As one looks forward with prophetic eye he grows doubtful
whether the fruition of this prophecy is so remote as fifty years.
Already the complaint has been made that fewer American students
are attending the great schools of the capitols of Europe, and the
explanation is ofifered that the need of foreign instruction is yearly
growing less.

There are two chief motives that inspire men in the pursuit of
science ; one, the less noble, is the desire of advertisement, the acquire-
ment of wealth and honors ; and all is done with these ends constantly
in view. The other, and by far the nobler, is the desire of penetrating
nature's secrets, of enriching man's stock of knowledge; and to him
who possesses these motives reward of the highest kind comes. Few
men are so constituted that the applause of their fellows for work vvell
done is distasteful to them, and honors that come are usually appre-
ciated, but the spirit of the man makes the work different. Possibly
the greatest boon in medicine was the discovery of surgical anesthesia,
but we cannot read the sad story without feeling that a black cloud

357

6 spiller: the post-graduate student

hangs over this discovery, although possibly in this we may judge too
harshly.

\\'e can hardly picture the horrors of the early days of surgery
before anesthesia came into use. When the floor of the operating
theatre of the old hospital at Canterbury. England, was torn up a
few vears ago, the rings were discovered through which were passed
the cords for tying patients down on the operating table prior to the
discoverv of anesthetics. In the words of a physician who wrote to
Sir James Simpson and described an amputation of a limb he had
pennitted to be perfomied upon himself, we have language that enables
us to understand in part : " The particular pangs are now forgotten ;
but the blank whirlwind of emotion, the horror of great darkness, and
the sense of desertion by God and man, bordering close upon despair,
which swept through my mind and overwhelmed my heart I can never
forget, however gladly I would do so. ... I watched all that the
surgeon did with a fascinated intensity. I still recall with unwelcome
vividness the spreading out of the instruments, the twisting of the
tourniquet, the first incision, the fingering of the sawed bone, the
sponge pressed on the flap, the tying of the bloodvessels, the stitching
of the skin, and the bloody dismembered limb lying on the floor."

Xot only are better facilities afforded us at home, but the class of
students attending our medical schools is changing. It is within our
memory when the medical student was regarded as a hardened sinner,
supposedly capable of any ill deed, and hardly to be described as a
gentlemen ; when terrors of the dissectiong room were held over the
head of the ignorant and crediting negro, and any misdemeanor was
perforce overlooked if committed by a medical student, provided it
did not come too strictly under the law. All this has changed. In
the University of Pennsylvania, of which I am best able to speak, and
I am sure in your own University also, the medical student is as truly
a man of refinement and culture as is the student of any other depart-
ment, and pranks, if committed, are much more common in the more
youthful men of the Department of Arts than in those belonging to
the Department of Medicine.

In the early days of medical history in this country the stories of
the dissecting room were sufficient to arouse much prejudice. Graves
were robbed and bitter feeling was thus created. In 1788 what was
known as the " Doctor's Alob "" occurred. A boy peeping in at a
window of the dissecting room was frightened by what he saw, and

358

spiller: the post-graduate student 7

told his tale to all who would listen. A crowd collected and tried to
take possession of the building, and the students, followed by the mob,
sought refuge in the jail.

In the middle ages, as Roswell Park has stated, the university was
frequented by boys aged thirteen and fifteen years, and it is evident
that the respect due the institution was sometimes lacking, as we read
that punishments were inflicted if an attempt were made to throw a
stone at a master, and that the fine was greater if the attempt were
successful. It became necessary to make regulations against swearing,
playing games of chance, being out after eight or nine o'clock in the
evening, regulations which would not be tolerated by students of the
present day.

The evolution of the surgeon from the barber occurred only about
one hundred years ago, and previous to that time all operations, if
such they might be called, were in the hands of ignorant men.

Students sometimes elected their teachers, and the unsuccessful pro-
fessor occasionally found it desirable to seek another field. Salaries
were not large. Versalius, the great anatomist, received $i,ooo, with
some additional fees. Students often begged or stole to get sufficient
to pay for their tuition and support. Naturally, such practices en-
gendered a great dislike and fear toward medical students which have
not yet entirely disappeared. The English, as you know, still make
distinctions between physicians and surgeons, in that the title of doctor
is given only to the former.

The condition of the student in Russia even at the present day is far
inferior to that of other civilized lands. A recent writer in the St.
Petersbiirger Zeitiing has declared that the universities of St. Peters-
burg and other large Russian towns are nurseries of rebellion, because
of the poverty in which the students live. The Russian student, this
writer asserts, is in most cases a beggar, and people regard such a con-
dition as a matter of course. More than a thousand students are
dropped from the University of St. Petersburg annually because they
are unable to pay their fees. Such notices as the following are not
uncommon : " A starving scholar asks for employment of any sort."
" A student in utter destitution asks for work, even of the most menial
character."

The requirements of the medical course are becoming constantly
more severe, and men of higher ability than formerly are attracted to
this work. Medical knowledge is growing so fast that the question of

359

8 SPILLER : THE POST-GRADUATE STUDENT

a fifth year is being seriously considered, and it will surely come; it
may be optional at first, but it has existed in England many years. In
France the student, to be properly equipped for his work, must devote
at least eight years in medical study.

Possibly some may regret that they have not received a large share
of the world's wealth, and that investigation and observation for them
are impossible, as the pressing needs of existence allow them no time
for aught else than the winning of the daily bread. Great wealth is
often a blight to a brilliant mind, and the history of the world shows
that much of the lasting work has been done under what has seemed un-
propitious conditions. The effort to win one's daily bread is often the
greatest stimulus to work with other aims, and we cannot despise the
blessing of poverty, but it is a blessing of which we sometimes have
too much.

The investigator in these days need not fear the ridicule of his
colleagues. We have grown so accustomed to wonderful discoveries
that our minds are open to almost anything new, unless it is clearly
fallacious. How dififerent it was in the times of Galvani, when the
experiments of this original thinker with electricity earned for him the
title of the " frog dancing master." Sometimes we are too open to
conviction, and accept new ideas without sufficient foundation. There
are fads and fancies in medicine, and many new methods of treatment
have but a fleeting existence. They are accepted for a time with un-
restrained enthusiasm. How well we remember when Koch's tuber-
culin was supposed to be the unfailing remedy for tuberculosis. Xow
we know that this substance has its value, although it is not that
originally claimed for it.

A little hero worship will do us as a nation and as individuals no
harm. I have had opportunity to see the tribute paid by two nations,
the French and the Austrians, at the burial of two men distinguished
in medical work, Pasteur and Billroth. Funerals of scientific men
with such marks of a nation's sorrow could at present not occur in our
land.

This is a day of skepticism in medicine and of reexamination of
long-accepted doctrines. What can be more striking than the unrest
manifested regarding aphasia? For more than forty years no one has
questioned the correctness of the teaching regarding Broca's area, and
yet Marie, Broca's own pupil, has raised a storm of criticism, with
as yet only a moderate support, by his statement that this region has

360

spiller: the post-graduate student 9

nothing to do with speech, and its destruction does not cause motor
aphasia. Or still another example. Hysteria has been the scrap
basket into which has been thrown everything peculiar and not under-
stood, unless positively shown to be organic. The teaching of Charcot,
covering a period of many years, was that hysteria has definite clinical
manifestations. Babinski, one of Charcot's most distinguished pupils,
has recently taken the stand that hysteria is a mental state in which
the individual is more capable of receiving suggestion, and the symp-
toms are produced by suggestion, either from the patient himself or
communicated to him by the examiner, and are removed by persuasion,
a view radically different from that hitherto accepted.

It is well to state that the views of Babinski have gained many
adherents, and that they are likely to alter our conception of one of
the most common, possibly the most common disorder of humanity ;
and, consequently, our method of treatment.

We hear much in these days from certain of the older and, 'ilas !
from some of the younger members of our profession, that medicine
is exhausted, that this or that method has been employed to its fullest
extent, and there is little more to be learned. Do not, I beg of you,
permit yourselves to assume this blase attitude. Such language is a
reproach to us. No method of study is exhausted ; it is true that one
method may be more promising than another, but the man who dis-
credits investigation with the plea that we are at a standstill, and are
in face of an impenetrable wall, is himself, not medical science, dan-
gerously near the limit of his resources.

To some of you the advantage of travel may be given, and if so it
would be folly to neglect it. It is not that one cannot learn in America
what is taught in England, France, Germany, Austria, or other coun-
tries, but the mingling with men of other nationalities, the observation
of how things are done in different lands, the acquiring of a new
language, open a new world. If we learn by travel the ready use of
German and French and nothing else, we accomplish much, but no
one stops with this. Medicine is taught in a very different manner in
different lands, and the student and professor in Germany are not like
those of France. Many of the lectures of Paris are prepared with a
thoroughness that permits publication of them without much alteration.
A German professor once remarked to me in Paris that there were no
lectures in Paris for undergraduate students, only for physicians.
This, however, was merely making the truth somewhat elastic.

3G1

10 spiller: the post-graduate studext

Recenth in New York and Philadelphia the strange sight of a
" prairie schooner " was presented, one of those old-time wagons drawn
bv oxen. A year or more had been consumed in coming from the
Pacific coast. Its owner had gone West in early manhood, and in the
twilight of life had desired to renew his early experience and retravel
the road in his former manner. \M'iat more striking contrast could
be offered than that between a " prairie schooner "' and a Pullman train,
and vet medicine of today has contrasts with medicine of an earlier
period no less startling.

We are so accustomed to the methods of diagnosis in use, and have
so often percussed, palpated, and auscultated the human body, that we
mav not recognize that the discovery of these means of diagnosis is
among the greatest in medicine. Auenbrugger, in the eighteenth cen-
turv. discovered palpation and percussion, and wrote a short paper on
the subject, but it was reserved for Laennec to teach auscultation about
fifty years later. We are likewise so accustomed to applying patho-
logical findings to the symptom complex in seeking for the explana-
tion of symptoms, that we hardly realize that this important method
of study had its real origin in the fertile brain of Alorgagni, whom
Virchow acknowledged as the Father of Pathology.

It seems almost incredible to us in these days that there should have
existed any serious objection to the establishment of a hospital a cen-
tury and a half ago in this country. Such, however, was the case.
When Dr. Thomas Bond returned to his native land enthusiastic from
his studies in Europe, and in 1751 attempted to introduce the hospital
system, he found many obstacles in his way. Some small institutions
existed, but nothing of the style of a large hospital. He was told that
there was little chance of success in his undertaking, and was advised
to see Franklin. Indeed, it was owing to the sagacity and energy
of the latter that the attempt succeeded. Franklin created a demand
on the part of the public, which previously had not existed, and in
this way the Pennsylvania Hospital came into existence in 1752.

It is a gratification to the medical profession to find that its members
are now being honored by the public. There has recently been erected
in the most conspicuous part of Philadelphia, the plaza of the City
Hall, a bronze statute to Joseph Leidy. I look upon this, in connection
with monuments to Gross and Rush, as a striking sign of the times,
and as indicating that we as a people are beginning to honor men
whose lives have been those of students, as worthy of the highest

362

spiller: the post-graduate student 11

recognition. This statue of Leidy is striking in its simplicity. The
noted scholar is represented in the sack coat he usually wore,' and in
the position of lecturing to a class. We who sat under him recognize
the correctness of the pose and dress, and appreciate the wisdon^that
avoided the sweeping folds of the university gown, which lend them-
selves so well to art, in order to produce a characteristic figure of the
man.

In bringing this address to a close I quote from Robert Graves : " It
is no light thing to have life intrusted into your hands; we are all
liable to err, we all commit mistakes; the rules of our art are not
invariably precise and certain ; but they only are guiltv who have not
used every opportunity of acquiring practical knowledge ; he is doubly
guilty who, conscious of Jiis neglect, embarks in practice and com-
mences with the decision and boldness true experience alone can
confer."

It is, indeed, no light thing to have life in our hands. The tendency
IS to grow callous from repeated contact with disease, and to -ive
advice in the enthusiasm for knowledge that may not be for "die
patient's best advantage. Far be it from me to sav that this is a com-
mon fault among surgeons and physicians, but the tendency is some-
times present, and is powerless for evil in conscientious practitioners
Let me warn you, however, to keep your hearts warm and vour heads
cool in the pursuit of scientific medicine.

You will receive unlimited praise and unlimited blame in your pro-
fession. James Jackson, of Boston, in his " Letters to a Young Phys-
ician just entering upon Practice," wrote : " I have often remarked that
though a physician is sometimes blamed very unjustlv, it is quite as
common for him to get more credit than he is justly entitled to; so that
he has not, on the whole, any right to complain." We do not'alwavs
when smarting from a slight, look upon the matter in this philosophical
light. Few of us are geniuses, but all are capable of great accom-
plishment, and the secret of a useful and successful life is well-directed
work. The men who have done most for the world have been those
who in the scriptural injunction of the sweat of their brow have per-
formed unceasingly and with enthusiasm their full measure of labor.
^ It seems almost as though the busiest are those who have the most
time for additional work. The secret of this apparent contradiction
lies in the fact that these men have learned the value of time, the

363

12 spiller: the post-graduate student

value of the odd moments that are wasted by others, what Johann
Muller called the " gold-dust of time."

Let me leave with you a remark made by Pasteur on his seventieth
anniversary : " Whether our efforts in life meet with success or failure,
let us be able to say when we near the great goal, ' I have done what I
could.' " To this I would add one word, which means happiness to all
who accept and adopt its teaching — the word " work." Never forget
that you are members of a great, noble, and unselfish profession, that
while you have a right to expect medicine to serve you, you are in duty
bound to give it your best, to do whatever you can, however insufifi-
cient it may seem to you, to advance its aims.

REFEREN'CES

]\Iakers of ^Modern Medicine, James J. \\'alsh, Fordham University Press,
New York, 1907.

A Narrative of ]\Iedicine in America, James Gregory Mumford, J. B. Lip-
pincott Co., 1903.

Roswell Park, University of Pennsylvania Medical Bulletin, March 1902.

W. A. N. Dorland, Century Magazine, 1908.

^quanimitas with Other Addresses to Medical Students, Nurses, and
Practitioners of Medicine. Wm. Osier. P. Blakiston's Son & Co., 1906.

The History of [Medicine in the United States. F. R. Packard, J. B.
Lippincott Co., 1901.

364

IDIOPATHIC EPILEPSY COMPLICATED BY MOTOR
APHASIA AND DIPLEGIA, WITH NECROPSY ^

By Williams B. Cadwalader, M.D.

pathologist to the philadelphia orthopedic hospital and infirmary for
nervous diseases, philadelphia

Muscular debility following repeated severe epileptic convusions
may be so marked as to give the impression of true paralysis. It is
probably analogous to that described by Todd under the name of " epi-
leptic hemiplegia."

In one of his lectures Todd says :

"A patient has a fit, distinctly of the epileptic kind; he comes out of it
paralyzed in one-half of the body; generally that side which has been more
convulsed than the other or which has alone been convulsed; but the paralysis
may occur when both sides have been convulsed equally. The paralytic stage
remains for a longer or shorter time, varying from a few minutes or a few
hours to three or four days or even much longer. It then goes off, or improves
until the next fit, when a train of phenomena precisely similar recurs with like
result."

He then goes on to describe eleven cases, and suggests that it is
probably caused by exhaustion of the cerebral centers.

As this condition does not see to be very common, the following
case should be of interest. I wish to take this opportunity of thank-
ing Dr. J. K. Mitchell for the privilege of studying and reporting this
case, and also Dr. Spiller for verifying my pathologic examinations.

Patient.— A. R., female, aged 14, was admitted to the Orthopedic Hospital
and Infirmary for Nervous Diseases, Jan. 22, 1908. Her parents and four
brothers were well. The patient was born at full term, after normal labor,
weight 734 pounds. She developed normally and was perfectly healthy ; walked
and talked at i year; was very intelligent and did well at school. She had
measles in May, 1900, and made a good recovery.

Onset of Disease.— Dm'wg July of this year the patient's family noticed
that she was getting nervous, and at times very excitable ; she was then sent to
the seashore, and while there was very much frightened by seeing a friend in
an epileptic convulsion. She returned home in September and seemed well.

' From the Laboratorv of Xeuropatliology of the University of Pennsylvania.
1 ' 365

2 C.\D\VALADER : IDIOPATHIC EPILEPSY

While she was eating, her arm was struck bj- one of her brothers, so that the
spoon was sharply pressed against the muscles of the right side of the mouth ;
then there suddenly began a clonic spasm at the point of injury, which gradually
extended till all the muscles of the right face w^ere involved. This attack was
confined to the right face and lasted only one or two minutes, without loss of
consciousness. Three similar attacks occurred at intervals of a month, always
apparently brought on by contact of a spoon at the angle of the mouth. Dur-
ing the last attack the right arm was violently extended and rotated outward
and consciousness was partially lost. Under treatment the patient was free
from attacks until August, 1902, when there was a series of six typical nocturnal
epileptic convulsions, involving chiefly the right side of the body. For the next
year and a half there were a few right-sided convulsions with loss of con-
sciousness. In 1904 attacks became more frequent and more general, the two
sides of the body being affected equally, followed by difficulty in talking and
with w-eakness and incoordination of the extremities w'hich lasted a few hours
at a time. In Jul}-, 1904, after a severe general convulsion, with loss of con-
sciousness, the patient became totally aphasic. From this time on there were
many major and minor attacks which increasing weakness of the extremities
and incoordination, so that the patient was confined to her bed.

Physical Examination. — The patient lay in bed with a vacant expression, the
mouth half open, saliva dribbling from the right side. The pupils were equal
and reacted to light and accommodation. Ocular movements seemed normal in
all directions. The tongue could be partially protruded, but with difficulty.
There seemed to be difficulty in swallowing. The patient could not speak but
could make sounds, and understood clearly both written and spoken language.
The facial muscles were not paralyzed but contracted sluggishly. Both v.pper
extremities were very weak, but not totally paralyzed. The forearm was
partially flexed on the arm and the hand on the wrist, on account of the spas-
modic contracture of the flexor muscles, but this could be partially overcome by
manipulation. All voluntary movements were restricted, and slowly and
laboriously performed with marked incoordination. The tendon reflexes were
equal and greatly increased. The muscles of the neck and back were weak.
Spasmodic contracture was quite marked in the adductor muscles of the thighs
and extensors of the feet. Movements were restricted, weak and incodrdinate.
Tendon reflexes were equal and much increased. There were distinct Babinski
reflex and ankle clonus on both sides. Sensation for touch and pain was everj^-
where less than normal. Dr. Langdon reported that the pupils were equal in
size and reacted promptly to light and accommodation. The ocular movements
were full and equal in all directions. The fundi were normal. Vision was
normal. Color fields could not be obtained satisfactorily. Dr. Wood reported
that the fauces were congested. Digital examination showed a small adenoid in
the vault, no relaxation of palate, and apparent loss of sensation. Xo evidence
could be found of any mass or growth above the larynx which would obstruct
respiration. Laryngeal examination was impossible on account of the mental
condition of the patient.

Course of Disease. — During the following six weeks, in spite of larger doses

3')6

cadwalader: idiopathic epilepsy 3

of bromid, there were one hundred and tliirty-two severe general convulsions
with loss of consciousness. During the interparoxysmal stage, from time to
time, the contractures were not so marked and there was slight increase of
muscular power, but on the whole there was very little change. Finally the
patient developed a severe follicular tonsillitis and died two weeks later of
what appeared to be clinically a general streptococcic infection.

Autopsy. — This was performed two hours after death. The brain and spinal
cord only were obtained. On removal the brain was moderately soft and the
vessels were intensely congested. There was a small amount of straw-colored
fluid beneath the meninges. The convolutions were well formed. On section
the ventricles were not- distended. There was no localized lesion apparent to
the naked eye. The microscopic appearance of the cord showed notliing
abnormal.

Microscopic Examination. — With the Wcigert method the pyramidal tracts
of the spinal cord were slightly paler than normal, but there was not a distinct
degeneration of the fibers; with the hemalum and fuchsin method there was no
evidence anywhere of inflammation. The blood vessels and pia appeared
normal. With the Nissl rnethod there was a moderate chromatolysis of the
ganglion cells of the anterior horns. The medulla oblongata, pons and basal
ganglia, studied by the Weigert, Nissl, hemalum and fuchsin methods, showed
nothing abnormal except a few scattered minute capillary hemorrhages. Por-
tions of the cortex taken from the motor convolutions of both sides, parietal
lobes, Broca's area, frontal and occipital lobes, studied by the same methods
presented occasionally a few scattered capillary hemorrhages. The pia here
and there showed a slight accumulation of small round cells. No characteristic
pathologic change was found and there was no evidence of hypertropliic
sclerosis.

I consider that the pathologic examination should be looked on as
entirely negative, as the slight changes which were found could easily
be accounted for by an infection which in this case was the immediate
cause of death.

In many respects this case is unique. Paresis of the extremities
and of the muscles of speech was at first slight, but gradually became
more profound as the convulsions increased in frequency, till finally
there was almost total paralysis of all four extremities with flexure
contracture and incoordination and total motor aphasia. When the
patient attempted to stand, her legs gave way and the head hung
forward. The general appearance was not unlike that in infantile
pseudobulbar palsy. All over the body there seemed to be a diminution
of pain sense ; by some authors this has been attributed to the slowing
of associated activities which exists after an attack. Increase of
tendon reflexes, with ankle clonus and Babinski reflex, shortly after an

4 cadwalader: idiopathic epilepsy

attack, has been described by Jackson,- Beevor,^ Yorkastner* and
Gowers.^

During the first few weeks that the patient was under observation
it was frequently noted that when the nurse attempted to administer
food or medicine, as soon as a spoon was brought in contact with the
muscles of the right side of the mouth there immediately began a clonic
spasm of the right side of the face, which spread quickly till the whole
body was equally convulsed, accompanied with loss of consciousness.
Later, after the patient had taken much bromid, the convulsions were
confined to the face and consciousness was partially preserved.
Finally, after some weeks, direct contact at this area was no longer
followed by convulsions. It would seem as if this might fairly be
considered an example of that rare variety of epilepsy associated with
what has been termed the " epileptogenic zone." Motor irritation
over a moderately well-defined area seemed to bring on a convulsion.

An interesting case, presenting somewhat the same phenomena, has
been described by Clark,'' in which motor or sensory irritation over the
biceps muscle was followed by a convulsion. Motor aphasia, partial
or complete, following epileptic seizures, although uncommon, has
frequently been described and is probably due to a condition involving
the cerebral centers concerned in articulation, which is similar in
character to that which causes the paralysis of the extremities. Cases
presenting more or less complete paralysis of one or more extremities
following idiopathic epileptic seizures, have been reported by Fere,'^
Voisin, Dutil,^ Echeverria,'* Hughlings Jackson,- Gowers,^ Pierce
Clark'' and others. ^°

Although a great variety of lesions have been found in the brains
of epileptics, no constant causal pathologic condition has yet been
demonstrated. Todd believed' that paralysis following a fit was due
to exhaustion of the brain from excessive action ; this view has been
accepted by Hughlings Jackson and others. Clark, who has had
abundant opportunities of studying this subject, says: "It is not

■ Med. Times and Gazette, February, 1881.
^ Quoted by Gowers.

*Vorkastner: Diseases of the Nervous System. 1908: Epilepsy.
^Epilepsy and other Chronic Convulsive Diseases, London, 1881.
' Arch. Neurol, and Psycho-pathology, 1899, ii.
' Compt. rend. Soc. de biol., 1896.
'Rev. de med., 1883, P- 161.
® Epilepsy, 1870.

" Todd : Clinical Lectures on the Nervous System, 1856, lecture xiv, Epileptic
Hemiplegia.

868

cadwaladrr: idiopathic epilepsy 5

necessary to invoke any other state than exhaustion to explain the
temporary paralysis in epilepsy."

In the preceding case the persistence of paralysis probably depended
on the rapidity with which the fits followed one on another. Negative
pathologic findings would be additional evidence in favor of exhaustion
as the probable cause of paralysis.

Reprinted from The Journal of the American Medical Association, Nov. 21, 190S,

Vol. LI, pp. 177S-1780.

American Medical Association. 103 Dearborn Ave., Chicago.

369

CEREBELLAR SYMPTOMS IX HYDROCEPHALUS;

With A Pathologic Report of A Case Associated
With Syringomyelia^

By John H. W. Rhein, M.D.

NEUROLOGIST TO THE HOWARD HOSPITAL) PHYSICIAN TO THE PHILADELPHIA HOME
FOR INCURABLES, PHILADELPHIA

Cases of hydrocephalus exhibiting cerebellar symptoms, while not
very rare, have been recorded in the literature with sufficient infre-
quency to warrant a report. The case to be reported is of still further
interest on account of the probable cause of the cerebellar symptoms,
which, if not unusual, has been infrequently described. The presence
of a well-marked syringomyelia, which was not suspected during life
owing to the absence of at least some of the characteristic clinical
manifestations, is of additional interest.

History. — J. G., a boj-, 8 years old, was admitted to the Howard Hospital,
Jan. 8, 1908. The patient first applied to the dispensarj^ of the Howard Hospital
Xov. 2, 1905. The previous history was entirely negative. Ten days before his
first visit he began to have headaches, associated with vomiting, which occurred
before breakfast, although at other times during the day as well. He also
complained of frontal headaches. The physical examination at that time showed
that the head was enlarged. Examination of the eyes by Dr. William Campbell
Posey was negative. Shortly after this there developed a spastic gait, but other-
wise nothing of importance was noted until two j-ears later, Xov. 29, 1907, when
the child returned again to the clinic with a history of vomiting, attacks of
frontal headache and general tremor. Station was poor, and the knee jerks
were increased. There was a history of a general convulsion three years
previously, and of several attacks, in which the patient fell, during the past year.

Examination. — He was admitted to the wards of the Howard Hospital on
Jan. 8, 1908, when his condition was as follows : There was a coarse, irregular
tremor of both hands on voluntary effort. The eyes were prominent, and the
palpebral fissures unusually wide, showing the cornea above the pupil. The
tongue was protruded straight, and there was no facial palsy. There was no
stiffness of the neck, nor apparent tenderness in that region. There was a
coarse, irregular tremor of both legs. The knee jerks were increased, and

' From the Howard Hospital and Department of Neurologv- and Laboratory
of Neuropathology of the University of Pennsylvania.

Read in the Section on Diseases of Children of the American Medical As-
sociation, at the Fifty-ninth Annual Session, held at Chicago, June, 1908.

1 370

RHEIN : CEREBELLAR SYMPTOMS IN HYDROCEPHALUS

equally so. There was no ankle clonus, but the Babinski phenomenon was
present on the left side. Kernig's sign was also present. The pain sense was
generally preserved. Unfortunately a test for the other forms of sensation was
not made, and the need of it was not specially indicated, for the patient did
not give any history of burning himself without being conscious of it until
afterward, a sign so characteristic of the early stages of syringomyelia. xVIore-
over, there were no local atrophies to attract attention to the possibility of the
existence of syringomyelia. The patient staggered when walking, and seemed
to be weaker in the left leg than in the right. The sphincters of the bladder and
rectum were incontinent.

Fig. I. View of the hydrocephalic brain, showing two cysts, A and B.

Jan. 12, 1908, the headache was very severe ; the head was retracted and the
neck stiff. Any movement of the head increased the pain, which appeared to
be general. At this time the knee jerks were slightly diminished, and there was
a tendency to fall to the left, w-ith the feet together and the eyes closed.

The patient died suddenly.

Autopsy. — When the brain was removed, 6 to 8 ounces of cerebrospinal fluid
escaped, and the very much distended brain collapsed to a certain extent. What
appeared to be two cysts were observed at the cerebello-pontile angles (Fig. i, A
and B), tlie one on the left being the larger. These were distended with fluid,
which escaped when the brain was removed.

371

3 RHEIN : CEREBELLAR SYMPTOMS IN HYDROCEPHALUS

The brain was placed in formalin, and the spinal cord in MuUer's fluid. The
entire cord was divided up into 23 approximately equal portions, and sections
from half of these were stained by the Weigert method and by hematoxylin and
acid fuchsin.

Fig.

2. Cross-section of the hydrocephalic brain showing
lateral ventricles.

distension of the

Cord : The spinal cord was the seat of a syringomyelia which extended from
the eighth thoracic to about the second cervical segment.

At the second cervical segment there was a small cavity to the right of the
central fissure. There was slight degeneration of the posterior columns, and the
left crossed pyramidal tract.

In the fourth cervical segment the same condition was observed, and here
also there was a slight dilatation of the central canal. In both posterior horns
an area of loose tissue was observed in which were found several spider cells.

At the sixth cervical segment the central canal was very much distended.
There was slight degeneration of the left crossed pyramidal tract. At this level
there was a cavity in the right posterior horn, and in the wall of this cavity
spider cells were seen.

372

rhein: cerebellar symptoms in hydrocephalus 4

In the eighth cervical segment the central canal was very much dilated and
hour-glass shaped, occupying a large part of the posterior column. There was a
large cavity in the right posterior horn and a small one in the left posterior
horn. On the right side there was hemorrhage into the tissue of the wall of the
cavity. The left crossed pyramidal tract was degenerated. At the second
thoracic segment the central canal was large and round, and there was still a
small cavity in the right posterior horn. Very slight degeneration of the left
crossed pyramidal tract was observed. Both posterior horns were the seat of
loose tissue, presenting the same characteristics as the levels above described.

In the third thoracic segment the central canal was irregular in shape, ex-
tending into the right posterior horn, and occupying a large part of the posterior
columns on both sides of the median line. The degenerative process, present
in the left horn, extended, at this level, into the anterior horn, and was less
marked than in the levels above. In the fifth thoracic segment the right
posterior horn was almost entirely destroyed by the cavity, and also part of the
anterior horn, and the dilated central canal occupied the posterior part of the
left horn.

Fig. 3. Showing, A and B, distended lateral recess of the fonrlli ventricle;
C, distended fourth ventricle.

At the eighth thoracic segment there was a small cavity in the left posterior
horn. At the ninth thoracic segment the cavity disappeared, and the central
canal was not materially distended. There was no trace of degenerated tissue
in the horns. At the segments below this level nothing abnormal was observed.

At the decussation the medulla appeared to be normal, except for some
central loose tissue, probably of gliomatous nature. The ganglion cells of the
anterior horns in the thoracic and lumbar regions, stained by thionin, appeared
to be normal.

Brain: Both lateral ventricles were very much distended (Fig. 2). The third
ventricle was also greatly distended, pushing the tissues of the interpeduncular
space downward. The aqueduct of Sylvius was very much distended, as well
as the fourth ventricle (Fig. 3, C), especially the cerebellar portion.

The lateral processes of the fourth ventricle, and this is to be especially
noted, were very much dilated (Fig. 3, A and B), and were continuous on each
side with the cysts situated at the cerebello-pontile angles. That is to say, the
portions of these recesses which came in contact with the subarachnoid space
were not closed in as usual. The cereI)ro-spinal fluid had apparently forced

373

5 RHEIN : CEREBELLAR SYMPTOMS IN HYDROCEPHALUS

the arachnoid and pia out in such a way as to give the appearance of a cere-
bellar cyst on each side. The foramina of Magendie and Luschka could not
be found.

Microscopically the ependyma of the lateral ventricles was not abnormal.
The wall of the cyst on one side consisted of a pia-like structure, somewhat
thicker, however, than the normal pia.

Summary.

A boy of 8 presented the characteristic symptoms of cerebellar
disease, slightly more marked on the left side. At autopsy a hydro-
cephalus was found, involving all the ventricles of the brain, distend-
ing the lateral recesses of the fourth ventricle and causing cysts at
both cerebello-pontile angles. There was associated with this a syrin-
gomyelia which did not cause the characteristic symptoms during life.

It is not unusual for the symptoms of syringomyelia in children to
be absent, even when well-marked cavity formation is present. This
is illustrated in the case reported by Bullard and Thomas,- as well
as by ni}- own. Bullard and Thomas' patient, a boy 6^ years old, pre-
sented the symptoms of cerebellar lesion, and later there developed
paraplegia and incontinence of urine and feces. The autopsy revealed
the presence of hydrocephalus and syringomyelia, the latter not having
been suspected during life.

In cases of hydrocephalus the possible presence of syringomyelia
should always be recognized, even when the usual symptoms of the
latter are lacking.

The occurrence of cerebellar symptoms in cases of hydrocephalus is
well known. Spiller,^ in 1902, reported a case of hydrocephalus with
symptoms suggesting cerebellar tumor and expressed the view that
the possibility of internal hydrocephalus should always be borne in
mind when tumor of this portion of the brain is suspected. He cited
the cases of Bramwell,* in which the cerebellar symptoms were ap-
parently due to hydrocephalus.

Since then Finkelburg^ reported a case in which cerebellar symptoms
were associated with moderate dilatation of all the ventricles. He
stated that cerebellar gait occurred in chronic hydrocephalus, as well as
with tumors of the central ganglia, as an early symptom. He believed
that normal or lowered tendon reflexes are not against a chronic hydro-

" Am. Jour. 'Sled. Sc, 1899, cxvii, 265.

^ Am. Jour. Med. Sc, 1902, cxxiv, 44.

* Brain, 1899, xxii, 66.

' Deutsche. Ztschr. f. Nervenheilk.. 1905, No. 29, p. 135.

374

RHEIX : CI-:Ki:r.RLLAR SYMPTOMS IN 1 1 VDROCEPHALUS 0

cephalus. Circumscribed tenderness to pressure or blows on the
cranium is found in chronic hydrocephahis, and has, therefore, no
diagnostic vahie.

Schmidt" suggested a symptom which he claimed was of great
diagnostic significance in deciding between hydrocephalus and cere-
bellar tumor. It consists of the appearance of vomiting and vertigo
if the patient lies on the side opposite to a tumor. He reported two
cases of cerebellar tumor in which the patients would constantly lie on
the side of the tumor, and concluded from this that the pressure from
the tumor on the aqueduct of Sylvius, if the patient would lie on
the opposite side, caused closure of this aqueduct, and hence an in-
crease in intracranial pressure. Finkelburg does not subscribe to this
view, as he believed that these symptoms are found in other localized
tumors of the brain. Schmidt believes that the Westphal phenomenon
points to a tumor rather "than to an idiopathic hydrocephalus.

The cause of cerebellar symptoms in hydrocephalus is difficult to
explain in all cases. I believe that the increased intracranial pressure
in my case, which was exerted more particularly on the cerebellum
at the localities at which the cysts were found, explains the presence of
these symptoms in this case and may have had something to do with
the slight predominance of the symptoms on the left side, as the cyst
on this side apparently had compressed the left lobe of the cerebellum.
When the fourth ventricle, however, is not enlarged, and the intra-
cranial pressure is not, therefore, high in the neighborhood of the
cerebellum, this explanation is not sufficient. It must be due to
another cause — probably the weight of the hydrocephalic brain on the
cerebellum.

Descriptions of cysts, such as those in my case, have not appeared
frequently in the literature. Scholz^ divides cysts of the cerebellum
into seven groups, i. e., (i) cysts similar to those in my case, which
will be discussed later; (2) cysts accompanying new growths; (3)
simple, or serous, cysts: (4) blood cysts, that is, apoplexies; (5) cvsts
from pressure or embolism; (6) dermoid cysts; (7) parasitic cysts.
He quotes the case of \^irchow. in which there was a cystic disten-
sion of the diverticulum of the fourth ventricle, and quotes a similar
case by Clarus.

In Virchow's case^ there was a partial dilatation of the fourth

"Schmidt's Jahrb.. 1889. No. 263. p. 137-

' Mittheilung. a. d. Grenzgeb. d. Med. u. Chin. Jena, igo6, xvi, 745.

' Path, des Tumeurs, 1867, p. 180.

375

7 RHEIN : CEREBELLAR SYMPTOMS IN HYDROCEPHALUS

ventricle, which extended laterally, and sent out prolongations on each
side, between the cord and the cerebellum, at which angle a cherry-
sized cyst was found, compressing the facial nerve. He spoke of it
as a hydrocele of the fourth ventricle.

This case undoubtedly is similar to mine, as was also the one
described by v. Recklinghausen,*^ in which a bilateral cyst which com-
municated with the dilated fourth ventricle was found. An opening
three millimeters in length was found behind the lateral angle of
the floor of the fourth ventricle, leading directly into the sac on either
side. The lateral ventricles were moderately distended and the
ependyma was thickened. The wall of this cyst was somewhat thicker
than the pia of the pons, which was continuous with the wall of the
cyst.

J. Bland Sutton^" cited the cases of Virchow and v. Recklinghausen,
and calls attention to the origin of such cysts. He noted particularly
that the cavity of the fourth ventricle is extended laterally by two
tubular prolongations, or lateral recesses, which open into the sub-
arachnoid space on each side. Key and Retzius were the first to call
attention to this, and Sutton refers to the result of obstruction of these
recesses, i. e., dilatations of the obstructed passages and the production
of cysts.

Other than these brief reports, I am unable to find any reference to
this condition in the literature. I believe, however, that these apparent
cysts may readily be overlooked, as they are apt to collapse when the
cerebrospinal fluid escapes at the time of the autopsy.

The cause of the cysts in my case I believe to be, probably, the
closure of the foramina of Majendie and Luschka, which caused an
accumulation of cerebro-spinal fluid in the ventricles of the brain, and,
as a result, the pia arachnoid covering the free borders of the lateral
recesses of the fourth ventricle as well as the recesses themselves was
distended by the excess of cerebrospinal fluid in the form of a cyst on
each side.

The association of syringomyelia and hydrocephalus has been noted
a number of times in the literature. Schlesinger^^ cites a number of
such instances, and Hinsdale, in 150 cases of syringomyelia collected
from the literature, found fifteen cases of hydrocephalus. Schlesinger
looks on the significance of this process in syringomyelia as two-fold.

" Virchow's Arch., 1864, No. 30.
'"Brain, 1886, 1887, No. 9, p. 352.
" Die Syringomyelia, 1902, p. 383.

376

RHEIN : CEREBELLAR SYMPTOMS IN HYDROCEPHALUS 8

He believes that it explains in some cases the general brain symptoms
of paralysis of the cranial nerves, which are frequently attributed to
syring-omyelia ; and that the existing hydrocephalus causes sudden
death in not a few cases of syringomyelia by reason of an exacerba-
tion of the process.

I wish to thank Dr. F. Jacobs for the opportunity of reporting this
case.

1732 Pine Street.

Reprinted from The Journal of the American Medical Association,

Dec. 5, 1908, Vol. LI, pp. 1933-1935-

American Medical Association, 103 Dearborn Ave., Chicago.

Reprinted from The Journal of Nervous and Mental Disease, Vol. 35,
No. 9, September, 190S.

HEMIPLEGIA WITH PARALYSIS OF THE NECK MUSCLES FROM
A SMALL MYELITIC LESION

By William G. Spiller, M.D.

The patient, a woman aged 57 years, was admitted to Dr. Spiller's service at
the Philadelphia General Hospital on February 14, 1907. She gave the history
of having had two attacks of hemiplegia, one about twelve years previously in
which the left side was paralyzed, and one six years previously in which the
right side was affected. She had frequently " rheumatic " pain. She had also
incontinence of urine. The pupils were unequal, the left being the larger.
Iridic reaction was very slow to light and in accommodation, and in the left eye
was probablj- absent. When she came under observation the left upper and
lower limbs were very weak. The tendon reflexes were exaggerated. The
muscles of the neck w-ere rigid but the head was not retracted. Its voluntary
motion was greatly impaired. The woman said she had had pain in the neck
about five weeks, but the face was not expressive of pain w-hen the head was
at rest.

The paralysis and rigidity of the neck muscles in hemiplegia were remark-
able and there was no means of deciding whether they were a part of the
hemiplegic symptom-complex or were a complication.

The woman died March 2, 1907.

The right lenticular nucleus was entirely destroyed by an old cyst which
extended into the inner capsule and destroyed its anterior limb. The anterior
horn of the right lateral ventricle was much enlarged, owing to the destruction
of the head of the caudate nucleus. A small cavity was found in the left
lenticular nucleus.

In examining the cord with the naked eye what seemed to be a small
hemorrhage was found in the left 'anterior horn at the fourth cervical segment.
This area extended through the fourth cervical segment into the third cervical
segment but not into the fifth cervical segment. The first and second cervical
segments were not obtained.

The microscopical examination gave the following results :

Third Cervical Segment. — The alteration was not confined to any one
region, but here and there throughout the section small hemorrhages and
swollen axis cylinders were found. The small vessels w-ithin the cord had
greatly thickened walls. Round cell infiltration was seen within the cord but
not within the pia. The nerve cells of the anterior horn were not numerous
at this level and those that were present were rounded and had imperfect den-
dritic processes.

The cord was less severely affected at the fourth cervical segment and was
about normal at the fifth cervical segment.
1 378

SPILLER : IIKMIPLEGIA WITH PARALYSIS OF NECK MUSCLES 2

Sections from the middle thoracic region appeared normal. Round cell in-
filtration was not distinct.

The anterior pyramids in the medulla oblongata and pons stained well by the
Weigert method and did not appear to be degenerated, neither was there any
round cell infiltration of the pia. The small vessels of the pia had thickened
walls.

It was evident from the microscopic examination that the paralysis of the
neck muscles was caused by this sharply limited myelitis. A myelitis of so
small extent is a very unusual finding.

379

From the Department of Neurology and the Laboratory of Neuropathology it

the University of Pennsylvania and from the Philadelphia

General Hospital

THE ASSOCIATION OF SYRINGOMYELIA WITH TABES

DORSALIS '

By William G. Spiller, M.D.

PROFESSOR OF NEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE
UNIVERSITY OF PENNSYLVANIA

A few cases of tabes with syringomyelia aje reported, and it is
remarkable that wnth one exception (Philippe and Oberthur ) they are
all by German authors. The case that I have studied is therefore the
first to appear in either American or English literature. Several
writers have held that the association of the two processes is not
merely a coincidence, but that one stands to the other in the relation
of cause and effect ; others have expressed themselves guardedly.
It should be remembered that if one of these processes is in causal
relation to the other, the simultaneous occurrence of the two disorders
should be far more frequent than the few reported cases indicate. In
several of these cases the degeneration of the posterior columns has
been of the variety known as syphilitic tabes, with thickening of the
blood vessels of the cord and round cells infiltration of the pia. Much
has been written about this form of tabes^ and it is the form occurring
in my case. Martin Kirschner has written a thesis on the occurrence
of syringomyelia with tabes dorsalis. He divides the published cases
into three classes : ( i ) Those in which the symptoms of tabes pre-
dominate. (2) Those in which the symptoms of each disorder are
pronounced. (3) Those in which neither group of symptoms is so
distinct that a diagnosis of either of the two processes w'ould be
possible.

Under the first heading he puts the cases of Friedreich, Oppenheim
(two cases), Jegerow, Eisenlohr, Nonne, Saxer, and Kirschner. Un-
der the second heading the cases of Fitrstner and Zacher, and Schles-
inger. Under the third heading the cases of Simon, Schiile and
Nebelthau.

Kirschner^ does not venture to form a definite opinion regarding the relation
of the tabes to syringomyelia in his case. Simon's^ case (Case IV.) was one of

' Read at the meeting of the American Association of Pathologists and Bac-
teriologists, May 7, 8, and 9, 1907. Received for publication Jan. 14. igo8.

1 380

SPILLER: SVRINGOiMVELIA WITH TAHES DORSALIS 2

dilatation of the central canal in the lower cervical and upper thoracic regions
with tabes, and without symptoms of these spinal changes. Probably, therefore,
it was not one of true syringomyelia. Moderate dilatation of the central canal
is not a very rare finding. Jegerow's* case is very briefly reported. The
patient was syphilitic and had paraplegia inferior with atrophy, loss of tendon
reflexes, anesthesia, and loss of pupillary reflex to light. lie had tabetic
degeneration of the cord, meningitis of the sacral portion of the cord, and
syphilitic changes of the vessels. The case is so briefly reported that it is
impossible to form a positive opinion regarding it. Redlich,' in his report of a
case of hydromyelia with tabes, without clinical history, expressed the opinion
that the contraction of the posterior columns caused disturbances of circula-
tion and thereby nutritive changes in the peri-ependymal substance of the
central canal. Kisenlohr's" patient had typical tabetic degeneration of the pos-
terior columns, chronic posterior spinal leptomeningitis, and syringomyelia, and
was in the tertiary stage of syphilis. The syringomyelia was in the cervical
and upper thoracic regions. Eisenlohr believed that the gliosis of the left
posterior columns originated^ from the tabetic proliferation of the posterior
columns, and excluded a mere chance occurrence of the two processes, even
though the syringomyelia was in the portion of the cord most commonly
aff'ected by this lesion, viz., cervical and upper thoracic regions. The case
presented only symptoms of tabes, except possibly vasomotor disturbances.
Nonne'^ thought there was some causal relation between the two processes in
his case of tabes with syringomyelia, but expresses himself rather guardedly.
The condition of the ])asilar artery suggested syphilis, although the spinal mem-
branes were intact.

Oppenheim,^ in reporting his second case in 1893, thought it prolialile that the
tabes and syringomyelia had some relation to one another, especially as five or
six well observed cases of the association of the two diseases were on record.
Gliosis has a tendency to implicate the posterior columns and he believed it
probable that in this way the tabes was produced in his two cases, i. e., the tabes
was secondary to the syringomyelia. No important changes were found in the
spinal membranes in Oppenheim's second case, although he speaks of a con-
siderable endarteritis of the anterior spinal artery, and this might suggest
syphilis.

In a case observed by Saxer" (Case V., p. 376) the lesions of typical tabes
were combined with a cavity extending almost the entire length of the cord.
He did not regard the syringomyelia as a chance complication of the tabes. The
destruction of tissue (syringomyelia) he believed was caused by disturbance in
the nutrition, and this disturbance was produced by tabes.

In Case XX., reported by Philippe and Oberthur,'" the lesions of tabes were
associated with a dilated central canal. It was not, therefore, true syringo-
myelia, and no particular attention was called to the association of the two
processes. Nebelthau," in discussing his case of syringomyelia with degenera-
tion of the posterior columns of the cord, expresses the opinion that syphilis was
probably the original or at least predisposing cause of the two processes. His
case was one of syphilitic tabes. In support of this opinion he refers to the
fact that syphilis was the cause of the disease of the posterior columns of

381

3 spiller: syringomyelia with tabes dorsalis

the spinal cord in the cases of Eisenlohr, Jegerow, Nonne, and Westphal.
Ernest Prey's"^ case is very briefly reported. The tabes was associated with
syringomyelia in the cervical region. Syphilitic leptomeningitis was present.
Frey believed an etiological relation existed between the tabes and the syringo-
myelia, but he could not determine whether a syphiloma developed around the
central canal and later disintegrated, or whether the spyhilis only gave an
impulse to the development of the syringomyelia. The meaning of the last
statement is not very clear.

Schlesinger" presents the subject .very clearly. He reports a case in which
the clinical symptoms of tabes preceded those of syringomyelia. The cavity in
the cord in general appeared like a dilated central canal, inasmuch as it had an
epithelial lining. The degeneration of the posterior columns was typically that
of tabes. The central proliferation of neuroglia in places passed diffusely into
the sclerosis of the posterior columns, but in the greater portion of the cord
was sharply defined. He was unable to find any causal relation between the
tabes and syringomyelia, although he believes that each process was modified by
the other. It is possible, he thinks, that a circumscribed, tumor-like gliosis
may arise from the secondary degeneration of tabes ; this, however, seems to
me very doubtful ; but it is more probable, Schlesinger thinks, that such does
not occur, as in no other form of secondary degeneration do we find tumor-
like proliferation of the neuroglia. It is probable that certain unknown differ-
ences exist between the proliferation of neuroglia in secondary degeneration
and that in tumor-like form, greater than a mere quantitative one ; so that one
type does not pass readily into the other. The neurogliar proliferation in tabes
is reparative ; in syringomyelia it is destructive. Schlesinger quotes the opinion
of Philippe and Oberthur, viz. : that the ependyma proliferates very readily in
chronic disease of the spinal cord because of the irritation produced, and believes
that the hydromyelia associated with tabes is secondary. He thinks the prolif-
eration of the ependyma occurs too seldom to be regarded as a secondary
process. He acknowledges, however, that the pulling caused by the contracting
posterior columns, when changes already exist in the gray matter, may exert
such an irritation as mentioned above upon the wall of the central canal, and
may produce circulatory disturbances ; but this does not mean that one process
arises from the other, but that one is modified by the other. It may be ques-
tioned, I think, whether the contraction of the neurogliar tissue in the posterior
columns causes any appreciable pulling upon the central gray matter. It sliould
be remembered also that the vascular supply of the two areas is not the same.

The origin of tabes in syringomyelia is extremely improbable Schlesinger
thinks. Syringomyelia develops mechanically and cannot produce the systemic
degeneration of tabes. The two processes are coordinated, but both may be
secondary to syphilitic disease of the meninges, which causes degeneration of
many posterior roots and also softening and cavity formation in the spinal cord.

He refers also to cases of tabes and syringomyelia observed by Pick, Vucetic
and Roth (these I have not been able to find) and says he has seen the simul-
taneous occurrence of the two processes in two cases and also the preparations
of two other cases, one of which was Redlich's.

The opinions that Schlesinger has expressed correspond very closely

382

spiller: syringomyelia with tabes dorsalis 4

to my own. In the case I have studied the degeneration of the pos-
terior cohunns from the mid-kimbar region downward was typical of
tabes in the preservation of the ventral fields and the small zone
along each side of the posterior septum (septo-marginal tract). At
these levels of the cord no cavity was found. It would be impossible
to attribute this degeneration at these levels to syringomyelia. Where
the cavity first appeared in the upper lumbar region it was evidently
merely a dilatation of the central canal and could hardly have been
caused by the tabes. Still higher where the cavity was extensive and
was more than hydromyelia, the glia in many levels formed a dense
band of tissue about it, much denser than, and at certain levels distinct
from, the neurogliar proliferation of the posterior columns. In some
regions the vessels throughout the greater part of a transverse section
were sclerotic. This may have been caused by the syphilis, but it
seems to me probable that k was in greater part the vascular thickening
not uncommon in syringomyelia, especially when near the thickened
glia about the cavity. I therefore find it impossible to believe that
either process is in causal relation to the other. Were this causal
relation existing we should find the two disorders occurring together
much more frequently, as tabes is a very common disease, and true
tabetic degeneration associated with syringomyelia is certainly a very
rare finding.

The clinical history of my case justified the diagnosis of tabes asso-
ciated with syringomyelia made by me seven or eight years before the
patient's death. The diagnosis of syringomyelia had previously been
made because of the dissociation of sensation, scoliosis and deformity
of one hand. Some seven or eight years ago, after studying the history
and examining the patient carefully, I made the diagnosis of tabes in
addition to syringomyelia, on account of the commencement of the
symptom-complex with ataxia and shooting pains in the lower limbs,
loss of tendon reflexes in these limbs, optic nerve atrophy, myopia and
Argyll-Robertson pupils. Syringomyelia involving the posterior
columns may cause some of the symptoms of tabes and the loss of the
tendon reflexes of the lower limbs might be explained in this way.
The lumbar region of the cord is not likely to be implicated first by
syringomyelia, and even if the process had commenced here the cavity
would probably be in the center of the cord and the reflex arc of the
patellar tendon on each side might be pushed to one side and escape,
as P* have seen occur, in intense syringomyelia of the lumbar region.

383

5 spiller: syringomyelia with tabes dorsalis

The commencement with pain, ataxia, and loss of tendon reflexes in
the lower limbs is very common in tabes, rare in syringomyelia, and
when optic nerve atropy and Argyll-Robertson pupils were found the
diagnosis of true tabes or syphilitic tabes seemed probable. Scoliosis
may occur in tabes, and P^ have reported such a case, but in associa-
tion with the intense sensory disturbances largely of the syringomyelic
character and the atrophy and deformity of the left hand, the diagnosis
of syringomyelia had to be retained. The case therefore seemed to
be one of tabes associated with syringomyelia. The degeneration of
the median fillet of one side resulting from a cavity in the medulla
oblongata and followed as high as the cerebral peduncle is an inter-
esting finding in the case. The history of my case is as follows :

J. S., about sixty years of age, white, male, was admitted to the Philadelphia
General Hospital May 22, 1886, and died there Aug. 9, 1906. He had often
been studied by members of the Neurological staff and was frequently used by
me in my lectures.

The symptoms began in 1875 with staggering while walking in the dark, and
he fell at times. Sharp shooting pains in the lower limbs began in 1889, and
he often complained of these pains to me nearly to the time of his death. In
1891 he had ataxia of his hands and could not hold his pen properly in writing.
Incontinence of urine commenced in 1900. He complained of a sensation as
if he were walking on rubber, and of girdle-like pains across the chest. The
scoliosis was first noticed in 1893, and since that time he had been unable to
walk on account of weakness and ataxia of the lower limbs. He complained of
tingling and numbness in the fingers and forearms, the little finger of the left
hand became contracted, and later all the fingers of the left hand were similarly
affected. Atrophy of the thenar and hypothenar eminences and of the interossei
muscles of the left hand developed. The bowels were usually constipated.
He denied syphilitic infection.

He was intelligent. His speech was not afifected. The cranial nerves, except
the optic, were in good condition. The scapular and deltoid muscles, especially
the muscles of the left shoulder, were much atrophied. The upper limbs were
ataxic and wasted, especially the left, and the alteration was more intense in
the distal part of this limb. The voluntary power of the upper limbs was im-
paired, especially of the left limb, but the upper limbs were not paralyzed except
in the left hand. The tendon reflexes of the upper limbs were lost.

The lower limbs were almost completely paralyzed.

The patellar reflexes were lost and ankle clonus was not obtained. Babinski's
sign was present on each side. The sensation was tested many times, but the
charts show variations in extent and intensity. All forms of sensation were
much affected in the lower and upper limbs and trunk. For a period tactile
sensation was less disturbed. The man complained much of painful contracture
of his lower limbs. These limbs were not much atrophied. Pes valgus was
present on each side.

3S4

spiller: syringomyelia with tabes dorsalis 6

An examination of the eyegrounds made by Dr. de Schweinitz, the date
of which is not given, showed myopic pupils and gray atrophy of the nerves.
A report from Dr. Oliver, dated Jan. 8, 1902, stated that the irides were almost
immobile to light stimulus thrown from all peripheral portions of the visual
fields, and the best response was obtained when the light was thrown from the
nasal side of the left field. The optic nerve heads were quite gray in their
deeper layers, and the retinal arteries were diminished in size. The iridic
response was prompt in accommodation and convergence. The extraocular
muscles were not affected.

Dr. Oliver reported Feb. 23, 1905: Vision of the right eye uncorrected, 5/15;
of the left eye 5/10. Pupil of the right eye two millimeters in diameter ; of the
left eye, 1.5 millimeters. Iris very sluggish to light stimulus, especially that
of the left eye. The extraocular muscles are unaffected.

Death occurred Aug. 9, 1906, while the patient was in the service of the late
Dr. Wm. C. Pickett. Dr. Pickett spoke of giving the material to me as the
diagnosis of tabes with syringomyelia had been made by me, and after the
sad termination of Dr. Pickett's promising career, the brain and cord of the
patient were generously placed iy my hands for examination by Dr. Buckley.

The microscopical examination gave the following results :

Upper and middle sacral and lower lumbar regions. — The posterior
columns are much degenerated and in sections from the higher levels
the crossed pyramidal tracts are also degenerated. The ventral fields
and a zone along the posterior septum are intact. The degeneration
is typical of tabes. Round cell infiltration is present in the pia. The
region of the central canal shows nothing abnorinal.

Mid-lumbar region. — The degeneration of the posterior columns is
characteristic of a marked case of tabes. The cavity in the central
gray matter begins at this level, and seems to be merely a dilatation
of the central canal, as the opening is surrounded at nearly all parts
by a layer of ependymal cells. The cavity formation at this level has
no connection with the posterior columns, and the ventral fields of
these columns still contain many normal nerve fibers. The cavity
becomes extensive at the second lumbar segment, and extends as a
narrow slit through the left posterior horn, and in this portion has
no lining of ependymal cells, but the portion in the central gray matter
is well lined with these cells. The small blood vessels in the central
gray matter have much thickened walls. As the vessels elsewhere in
the section are not much thickened, the sclerosis of those within the
central gray matter is probably caused by the syringomyelia. No
distinct connection can be seen between the central sclerosis and that
of the posterior columns.

At the twelfth thoracic segment the cavity is confined to the central

385

7 spiller: syringomyelia with tabes dorsalis

gray matter and is surrounded by an area of dense neurogliar tissue,
much denser than the sclerosis of the posterior columns and quite
sharply differentiated from it. Much of the cavity is lined by ependy-
mal cells. The small blood vessels in all the white matter show thick-
ened walls.

At the tenth thoracic segment the cavity is a narrow slit extending
laterally through the gray matter. The posterior columns are in-
tensely degenerated ; the crossed pyramidal tracts are only a little less
degenerated as a result of the syringomyelia.

The cavity becomes much smaller at the ninth thoracic segment, and
is surrounded by a denser layer of neurogliar tissue than in the tenth
thoracic segment; and it is much denser than the neurogliar prolifera-
tion of the posterior columns. This dense central area is rarefied at
the center of one of the anterior horns near the central canal.

The sixth thoracic segment shows the cavity again as a narrow slit
extending through the central gray matter and into one posterior horn.

At the fourth thoracic segment the neurogliar proliferation about
the cavity is more pronounced and forms a part of the sclerosis of the
posterior columns.

The cord is very much flattened at the eighth cervical segment and
the cavity extends through the central gray matter and into each an-
terior horn, destroying almost entirely the left anterior horn but
leaving a large portion of the other anterior horn intact. This gives
the explanation for the intense atrophy of the left hand. The pos-
terior columns are intensely degenerated. The round cell infiltration
is pronounced throughout the spinal pia.

A portion of both anterior horns is preserved at the seventh cervical
segment, and the complete destruction of the left anterior horn seen
at the eighth cervical segment is not present here, therefore the left
forearm was not intensely atrophied. The small blood vessels of the
pia are thickened.

The fifth and sixth segments resemble the seventh segment closely.
The cord begins to assume more of its normal shape at the fourth
cervical segment, although the cavity is still extensive.

The cavity disappears at the third cervical segment.

Sections from the medulla oblongata show a narrow slit in the posi-
tion of the intramedullary roots of the ninth and tenth nerves, which
has cut and destroyed the internal arcuate fibers of one side, so that
the fillet of the opposite side is degenerated. This is rather a rare

386

Journal of Medical Research.

Vol. XVIIl. Plate IV.

Spiller.

Syringomyelia with tabes dorsalis.

Journal Or Medical Research.

Vol. XVIIl. Plate V.

ii^sii

^^,

^-i

tag

■

'^^-'"^

^^^■:

Vv...

"Vi. i: .^

*""*'«■•>'"<••"-

^ «

Spiller

Syringomyelia with tabes dorsalis.

spiller: syringomyelia with tabes dorsalis 8

finding in syringomyelia. The degeneration of this median fillet is
followed as high as the cerebral peduncle. The posterior longitudinal
bundle on the side of the degenerated fillet is not so wide as that of
the opposite side, and from this finding one might conclude that this
bundle is formed partly by the internal arcuate fibers.

The optic chiasm is not degenerated. A transverse section of the
left optic nerve shows many atrophied bundles of nerve fibers peri-
pherally situated.

[I am indebted to Dr. Alfred Reginald Allen for the photographs.]

REFERENXES.

1. vide Nonne. Archiv fiir Psychiatric, xxiv, 1892, 526.

2. Martin Kirschner. Syringomyelic und Tabes dorsalis. Inaugural Dis-
sertation, Strassburg, 1904.

3. Simon. Archiv. fiir Psychiatric, v, 1875, 128.

4. Jegcrow. Ncurologisches Centralblatt, 189 1, 406.

5. Rcdiich. Zeitschrift fiir Heilkunde, xii, 1891, 571.

6. Eisenlohr. Society Report, Archiv. fiir Psychiatric, xxiii, 1892, 60.3.

7. Nonne. Archiv. fiir Psychiatric, xxiv, 1892, 526.

8. Oppenhcim. Archiv. fiir Psychiatric, xxv, 1893, 315.

9. Saxer. Ziegler's Bcitragc, xx, 1896, 376.

10. Philippe and Oberthur. Archives de Medccine Experimentale, xii, 1900,

651.

11. Ncbelthau. Deutsche Zeitschrift fiir Ncrvcnhcilkundc, xvi, 1900, 169.

12. Ernest Frcy. Centralblatt fiir Ncrvcnhcilkundc und Psychiatric, 1902, 240.

13. Herman Schlcsinger. Die Syringomyelic. Franz Deuticke, 1902, 2d
Edition, 331.

14. Dercum and Spiller. American Journal of the Medical Sciences.

15. Spiller. Philadelpliia Medical Journal.

DESCRIPTION OF PLATES IV. AND V.

Fig. a. — Syringomyelia and tabes. The intense atrophy of the left hand was
caused by complete destruction of the left anterior horn at the eighth cervical
segment.

Fig. B. — Fourth or fifth lumbar segment. The degeneration of the posterior
columns is tabetic in type.

Fig. C. — Second lumbar segment. The syringomyelia begins to be more
extensive than it was in the segment below.

Fig. D. — Section from the seventh cervical segment. The cavity extends
from one side of the cord to the other.

Fig. E. — Degeneration of the fillet of one side from destruction of the
internal arciform libers of the opposite side, resulting from a slit-like cavity
in the opposite side in the course of the ninth and tenth nerves.

Reprinted from the Journal of Medical Research, Vol. XVIIT., No. i,
March, 1908.

387

Reprinted from the University of Pennsylvania Medical Bulletin. December.
1908.

ADEXOLIPOMATOSIS, WITH THE REPORT OF A CASE ^
Bv Charles K. Mills, M.D.

PROFESSOR OF NEUROLOGY IX THE UNIVERSITY OF PENNS\T-VANIA, AND NEUROLOGIST
TO THE PHILADELPHIA GENERAL HOSPITAL

The following case, for the privilege of seeing which I am indebted
to Dr. W. B. Diefenderfer, of Altoona, and to Dr. E. C. Town, of the
medical service of the Pennsylvania Railroad, it is believed is of
sufficient interest to present to the members of the Philadelphia Neuro-
logical Society for examination and discussion. Although I have
seen several well-marked cases of adiposis dolorosa, including the
original one described by Dr. F. X. Dercum, I have never observed
the exact counterpart of the case here described. It is, however, by
no means unique, although undoubtedly a form of lipomatous disease
far from common. Launois and Bensaude,- to whom we are indebted
for a valuable contribution on the subject, have collected the records
of 26 cases, including 3 which were unpublished before the appearance
of their article. Chantemesse and Podwyssotosky^ in their " Processus
Generaux," fully discuss this and similar affections, especially from
the standpoint of pathology. The reader interested is referred to
these authors for the bibliography.

Our fellow member. Dr. D. J. ^McCarthy,'* has also given detailed
consideration to the subject, including the report of an original case,
in an article prepared but not yet published.

The case was studied by me in the neurological service of the
hospital of the University of Pennsylvania, and I am indebted to Dr.
C. C. Rush, one of the internes at the hospital, for careful notes and
measurements. The photographs were taken by Dr. Joseph ]M.
Spellissy.

^ The report was presented and the patient exhibited at the meeting of the
Philadelphia Neurological Society held October 23, 190S.

" L'Adenolipomatose sj-metrique a predominance cervicale. Xouvelle Icono-
graphie de la Salpetriere. Paris. 1900. xii. Pp. 41-45- '^84-197. 243-249.

^ Pathologic Generale et Experimentale. Les Processus Generaux. Paris.
1900. vol. i. Pp. 274 to 27S.

* On Adiposis dolorosa and allied conditions, in Osier's Modern ^ledicinr.
vol. vii.

1 38S

mills: ADENOLIPO.MATOSIS 2

F. A. p., an American, aged thirty-three years, w^s admitted to the
hospital of the University of Pennsylvania, October 2, 1908, and was
discharged October 20, 1908. The occupation of the patient had been
that of a moulder in a machine shop, where he had been subjected to
intense heat. None of the workmen in the shop had been similarly
affected. There was no family history of any similar disease. He
had seven brothers whose weights averaged between 155 and 160
pounds. The family history was otherwise negative.

Since his boyhood days the patient had drunk beer, never to intoxi-
cation but quite constantly, often five or six drinks a day. Whiskey
he had taken more moderately. He denied syphilis absolutely. Every
winter for the past five years he has had an attack of acute articular
rheumatism. Except for an attack of measles in childhood, the man
has had no other infectious or diathetic diseases.

The patient's present trouble began between three and one-half and
four years ago. He first noticed a little swelling underneath his chin.
This gradually increased in size until at the end of six weeks it was
about half the size of a hen's egg. At about this time a tiny swelling
began to appear at each breast around the nipple. These swellings
very gradually enlarged until five months later he says they were two-
thirds the size of a hen's egg. Other swellings then began to appear
on his upper arms, and about four months later the sides of his chest
and back showed similar enlargements. Since then the swellings
began to develop at lower and lower points. The abdominal masses
did not appear until twelve or fourteen months later than the tumor
on the neck. The last swellings were those upon the thighs, which
were first noticed eight months ago; that is, about three years after
the first swelling in the neck.

From the beginning the tumors, evidently masses of fat, have varied
in size from time to time, but the general tendency has been to increase
in size. At no time have they disappeared entirely. They give the
patient no inconvenience except that of weight.

The man continued to do very hard manual labor until the day of
admission to the hospital. He has no subjective pain in the swellings
which are only moderately painful to pressure at a few points; after
hard labor, however, his arms are painful, but there is no pain in the
other fatty masses. \\'hen at his work the afifected parts were a little
more sensitive to heat than the other portions of his body. The man

389

3 MILLS : ADEXOLIPOMATOSIS

says that he is not quite as strong as when the trouble began. He
has increased in weight from 132 to 146 pounds.

The patient has no dyspnea, headache, nausea, constipation, diarrhea,
or loss of appetite.

The following is a general description of the fatty enlargements
taken at the time of his admission :

Under his chin and extending over the angles of his superior maxillse
as high as the lobes of his ears is a large, soft, pendulous mass. The
swellings in the upper half of the upper arms are large and taper down
and end just below the elbows. The mammae are extremely massive
and pendulous, and the region above them is prominent. The abdomen
protrudes remarkably; the sides of the chest and abdomen and the
back showing great accumlations of fat which cause a number of
transverse folds. The thighs are swollen in front and to the outer
sides, half way to the knees.

The following are the measurements : Circumference. Right arm,
19 inches, left arm. 20 inches. Right elbow, loj inches : left elbow,
II inches. Right forearm, 10 inches; left forearm, 9^ inches. Right
wrist, 6i inches ; left, wrist, 6^ inches. Right thigh, 21 inches ; left
thigh, 20 inches. Right above patella, 13 inches; left above patella,
13^ inches. Right calf, iif inches; left calf 12 inches. Right ankle,
7^ inches; left ankle, 7J inches. Xeck around the mass, 17 inches.
Neck below the mass. 13:^ inches. The body under the arms, 40^
inches. The body over the breasts, 43^ inches. The body under the
breasts, 36^ inches. The body at umbilicus, 36^ inches.

The height of the patient barefooted is 5 feet, 4^ inches.

The veins are prominent on the back of the thighs and legs.

The following is another description of the tumefactions, containing
some facts not included in the one above given :

The swelling in the neck fills the entire space between the jaws and
the clavicle, but does not extend up on the face itself. He has no
enlargement in front of the ears. On the back of the neck he has
a nearly median swelling, it being a little more to the left, about the
size of a lemon, the lower border of which is on a line with the vertebra
prominens. The tumefactions on the shoulders and upper arms are
exceedinging large. The mammary swellings are symmetrical, and
of immense size. The abdominal tumefactions assume the appearance
of an enormous median swelling with the umbilicus deeply sunk in its
center. Posteriorly there is some, but not much, enlargement over

390

mills: aden'olipomatosis 4

and between the shoulder blades ; below the shoulder blades on each
side are symmetrical swellings of moderate size, and below these two
other symmetrical swellings, the pairs being separated by an im-
perfect groove. Symmetrical swellings of considerable size, but not
nearly so large as those on the shoulders and breasts and central
abdomen, are present on the sides of the trunk, on the buttocks, and on
the anterior and posterior aspects of the thighs. The scrotum con-
tains unusual deposits of fat, it being difficult to easily locate and
separate the testicles by manipulation.

Examining carefully for hard masses within the limits of the fatty
tumors, these could not be found. One small nodulated mass is
present below and near the line of the internal border of the left
mammary swelling, of which it does not form a part. It has the
feeling of a collection of small lymphatics.

Pain and touch sensations are not lost over any part of the body.
Possibly they are a little less acute over the swollen parts.

Examination of the eyes shows that the pupils react normally to
light and in accommodation. The ocular movements are normal. No
nystagmus is present. The upper and lower eyelids are thrown into
folds. The eyes are a little ])rominent. and a just perceptible von
Graefe symptom is present. The pulse at times ranges from 90 to 100.

The patient's hearing is defective in each ear.

No paralysis of facial muscles is present. The jaw drops in a
straight line. The tongue is protruded straight and shows a slight
tremor. No paralysis, paresis nor ataxia is present in the face, trunk,
or extremities.

The knee-jerks and the Achilles jerks are prompt with normal ex-
cursion. There is no ankle clonus nor Babinski response. The
biceps and triceps jerks are normal.

The lunsfs and heart are normal. The liver dulness does not extend
below the costal margin. h\irther examination of the abdomen is
negative.

Examination of the urine shows the following: amber colored;
specific gravity, 1020 ; reaction acid ; no albumin, sugar nor casts.

The blood examination gave the following: red blood cells, 2,950,-
000; white blood cells, 5900; hemoglobin, 60 per cent. Dififerential :
polynuclear cells, 63 per cent. ; lymphocytes, 26 per cent. ; mononuclear
cells, 3 per cent. ; transitional form, 5 per cent. ; eosinophils, 3 per cent.

391

5 MILLS : ADEXOLIPOMATOSIS

During his stay in the hospital the patient was placed on a prepara-
tion of thyroid extract, 5 grains, three or four times daily, and a com-
bination of strychnine, arsenic, and iron. Xo noticeable improvement
occurred. The question of operative interference was discussed, but
postponed until later when the patient was expected to return for
further treatment.

This case cannot, I believe, be regarded as belonging to the type of
adiposis dolorosa, although allied to this disease in some of its features.
The four cardinal symptoms of adiposis dolorosa, as pointed out by
Dercum" and Vitaut,'' are the fatty deposit, pain, asthenia, and the
psychic phenomena. The cases of adiposis dolorosa have been divided
by Vitaut into the nodular, the localized diffuse, and the generalized
diffuse. The nodules, often painful to pressure, which have been
observed in adiposis dolorosa, are not present in this case. This case
may be regarded as at first having been of a localized diffuse character,
later becoming more generally diifused, and in this respect it might
be looked upon as coming within the description of some of the cases
of adiposis dolorosa.

Pain is not a marked feature of the case. He has some feelings of
distress or pain in the arms after hard labor, and at some examinations
has complained of very moderate pain on pressure, particularly along
the swellings in the vipper arm. He does not show to a marked extent
the asthenia which has been recorded in cases of adiposis dolorosa,
although he thinks he is is not as strong as he was before the be-
ginning of these swellings. His mental state has not noticeably
changed. He is not unusually depressed nor does he show any of the
slowness or irritability or hallucinations which have been observed in
adiposis dolorosa.

In the main the case here presented corresponds to adenolipomatosis
as described by Launois and Bensaude, and the writers cited by them
in their discussion of symmetrical adenolipomatosis. Probably if seen
earlier the appearance of the patient would have more closely re-
sembled that which has been presented in some of the photographic
illustrations of Launois and Bensaude's cases. It might be well per-
haps, as the disease is not well known in this country, to summarize

° Two cases of adiposis dolorosa, Transactions of the College of Physicians.
Philadelphia, 1902. Several other papers on adiposis dolorosa have been pub-
lished b}' this neurologist.

® Maladie de Dercum, Lyons, 1901.

392

MILLS : ADENOLIPOMATOSIS

393

mills: adenolipomatosis

394

mills: adenolipomatosis

3*15

9 mills: adexolipomatosis

the special features of adenolipomatosis as described by these authors.^

The appearance presented by a case of adenolipomatosis, as given
by Launois and Bensaude, and as illustrated in large part by the patient
here exhibited, is striking and characteristic. In some cases which
have not greatly progressed, the patient merely presents a median
swelling under the chin, having somewhat the form of a crescent with
the horns turned upward. In our patient the swelling has now taken
the form of a large soft mass, filling almost the entire space between
the chin and clavicle. The face sometimes becomes framed in an
adipose pad. The tumefaction may be replaced by two tumors, or
swellings, one on each side. Similar deformations sometimes appear
in the parotid and pre-auricular regions.

The nape of the neck may be the seat of other protuberances. The
lipomatous swellings may extend beyond the supraclavicular region
and fall like large breasts over the chest and upper back. The masses
may be so diffused as to form a large collarette around the neck.

On the arms are sometimes enormous hypertrophies, as in the present
case and in one cited by Launois and Bensaude. They cover the del-
toid and descend to the midarm. The normal forearm appears com-
paratively lean in relation to these fatty casings.

Adipose deposits are frequent and large and in various positions
over the abdomen. The tumefactions may increase in number and in
volume until they cover almost the entire body. The extremities of
the limbs, however, even in these cases, remain immune and afford a
striking contrast to the rest of the body.

Two characteristics are almost always shown: the appearance of the
swellings in symmetrical pairs or in the median line, and their having
all the marks of diffuse lipomata. The skin is usually fine and
normal, retaining its mobility, although some exceptions to this are
noted, such as multiple adhesions to the deep surface of the derma,
lymphatic varices of the skin, and elephantiastic appearance localized
in the armpits. Sometimes the surface is smooth and homogeneous,
but more often it is irregular and lobulated.

Three varieties of tumors may be usually distinguished : the softest
are those of the region below the chin ; the hardest occupy the nape

' The account of adenolipomatosis as here given from Launois and Bensaude,
inchiding the discussion of pathology and diagnosis is largely a translation from
these writers. The quotation marks have been omitted, as, owing to the omis-
sions and condensations, it has been found difficult to use them correcth'. I
wish, however, to give full credit to these authors for the use of their work.

396

mills: adenolipomatosis 10

of the neck, and finally the consistency of the other tumefactions is
midway between these two. Careful palpation some times permits
one to make out, in the middle of a mass as yet not fully developed,
the existence of nuclei poorly isolated and resistant, embedded in adi-
pose tissue. At other times one can feel toward the centre of the
tumor a single firmer nucleus. A comparison of these tumefactions
with the clusters of the lymphatic ganglia is often mentioned by ob-
servers. In the neighborhood of the tumors one sometimes finds small
and hard lymphatic ganglia. One of these was present below the
mammary swelling in the case detailed in this paper.

To sum up, say Launois and Bensaude, the symmetry, the diffuse
form, and the special Iocali::ations, are the three great objective char-
acteristics of adenolipomatosis.

In the immense majority of cases the lipomatous tumefactions con-
stitute little more than a deformity: in addition to the mechanical dis-
comfort which they occasion they do not interfere seriously in the
functions of the organism. When compression symptoms exist they
are generally slightly marked, and are limited to some twitchings and
to some transitory pains.

In numerous observations one finds symptoms due to the com-
pression of the organs of the mediastinum. It seems, indeed, that
besides the subcutaneous masses, others more profound develop around
the numerous lymphatic ganglia clustered in this region. They gen-
erally manifest themselves in slightly marked respiratory disturbances,
hoarseness, cough, slight dyspnea, dilatation of the subcutaneous veins
of the thorax, etc. At other times the symptoms are more threatening.

Adenolipomatosis is not accompanied by any visceral disturbance and
does not react on the general health of the patients.

The lipomatous masses do not coincide with any appreciable modi-
fication of sensibility, or motility, or with any dystrophy. On the other
hand, there exists in man}- patients an abnormal cerebral condition
which manifests itself sometimes by irritability, sometimes by apath\'
or by hypochondria.

Among visceral symptoms are sometimes noticed hypertrophy of the
spleen and acceleration of the beating of the heart.

The analysis of the urine and the blood has been the object of a few
researches. The number of white corpuscles is sometimes normal,
sometimes slightly exaggerated : in their three patients, of whose blood
they were able to make a detailed examination, Launois and Bensaude

397

11 MILLS : ADENOLIPOMATOSIS

were struck with the scarcity of the mononuclear white corpuscles;
in a fourth patient the blood presented no alteration worthy of note.

The beginning of the disease is generally insidious ; since the de-
velopment of the tumors proceeds slowly and unmarked by pain, the
patients may not be able to fix the time of their beginning, nor even
very often the place of their origin.

One of the most interesting peculiarities presented by these tumors
in their development, is that they may at certain times be subject to
alternate increase and diminution in volume. These variations, which
are likewise observed in adenolymphocele, confirm, in the opinion of
Launois and Bensaude, the hypothesis of a vasculolymphatic origin.

No one has ever observed a complete disappearance of the tumors,
although in certain cases the lipomatous masses have been seen to
diminish to the point of becoming almost unrecognizable.

Diffuse symmetrical adenolipomata, like true lipomata, do not par-
ticipate in the general oscillations of the nutrition ; they appear to have
an individuality of their own upon the site where they develop ; the
tumors preserve their volume during loss of flesh, and in the inanition
of wasting diseases, like tuberculosis, cancer, albuminuria, etc.

The differential diagnosis of adenolipomatosis is to be made from
adiposis dolorosa, which has already been considered in stating the
reasons why the case here presented is not to be regarded as an
example of this latter affection. The diagnosis as pointed out by
Launois and Bensuade is also to be made from such affections as
congenital lipomata, which never give rise to the extraordinary de-
formities exhibited in the case under consideration ; from true lipomata,
which are always definitely isolated and encapsulated, and also do
not increase to the enormous masses present in adenolipomatosis;
from lymphadenoma, •" by the consistency of the tumors, by pain, by
general and functional disturbances, and by the progressively invasive
courses of the disease," and also by early fatal termination, and by
relapses always occurring after the removal of the lymphadenomatous
tumors and not after operations on adenolipomata ; from obesity, which
is more diffuse and accompanied by signs of visceral or perivisceral
adiposity.

Some of the hypotheses advanced to explain adenolipomatosis, and
cited by Launois and Bensaude are :

1. That it is a dystrophy connected with disease of the thyroid.

2. That it is due to undue or faulty action of the cutaneous glands.

398

mills: adenolipomatosis 12

3. That the adipose deposits follow the contours of the underlying
muscles.

4. Many authorities have attributed to adenolipomatosis a nervous
origin, citing in confirmation of this view the fact that the disease is
often associated with cerebral or cerebrospinal disorders like general
paresis and tabes, or with nervous affections like sciatica.

5. An hypothesis which seems to have much in its favor is that
which attributes adenolipomatosis to a disorder of the lymphatic vessels
and glands.

An article by McCarthy'^ on the formation of hemolymph glands
from adipose tissue in man is interesting in this connection. This
writer gives some results of his examination, 'gross and microscopic,
of two cases of adiposis dolorosa, in which were found bodies which
microscopically revealed the structure of hemolymph glands, other
interesting changes being, also present. McCarthy briefly discusses the
question of the formation of lymph glands from adipose tissue.

A number of authors hold that alcoholism plays an important part
in the causation of adenolipomatosis. The history of the case here
reported points to this possibility.

'The Formation of Hemolymph Glands from Adipose Tissue in 'Man, Jour-
nal of Medical Research, N. S., 1903, vol. iv, 241-245.

MVM.)

Extracted from the American Journal of the :Medical Sciences, November,
1908.

From the Department of Neurology and the Laboratory of Neuropathology of
the University of Pennsylvania

TUMOR OF THE GASSERIAN GANGLION^

A Repot^t of Two Cases. ^^'ITH Necropsy

F)V ^^'ILLT.\M G. Spiller, M.D.,

PROFESSOR OF XEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE
UNIVERSITY OF PENNSYLVANIA. PHILADELPHIA

Tumor arisino- in the Gasserian cranglion or havino^ its chief cHnical
manifestations in disttirbance of this g-ang-lion is fortunately rather
rare, but doubtless far more common than is o-enerally believed. Brain
tumor is always serious in its symptomatolog'y. but when the Gasserian
ganglion is implicated the pain is usually intense. The first case of
tumor in this reeion recorded in this country, and the second case on
record in which operation was performed, is that reported by Dercum,
Keen, and Spiller (1900). Since then two cases with necropsy, in
one of which operation was done, have come under my care.

Case L — A. K.. aged twenty-five years, was referred to the Univer-
sity Hospital by Dr. H. S. Grouse, of Littlestown, Pa., ^larch 4, 1907.
Dr. Grouse stated that the patient first had paralysis of the right lifth
nerve, with severe pain ; the right optic nerve was then implicated
causing blindness in the right eye. later the left optic nerve became
affected causing complete blindness. Then the left arm became
paretic. At times the mentality was much impaired, but only when the
pain in the head was severe.

The history as obtained after the patient entered the hospital is as
follows :

Chief Complaints. Blindness, intense headache, vomiting.

Social History. Patient is a bricklayer and stonemason. He is un-
married. He uses beer and whiskey, sometimes to excess. He chews
tobacco rather freely. He had gonorrhcEa about a year ago. but has
no history of syphilitic infection.

Family History. Father and mother are living. Two brothers and
three sisters are living and well; one sister died of eclampsia. The

^Read by invitation at a meeting of the St. Louis Surgical Society, :\Iay 29,
1908.

1 400

spiller: tumor of the gasserian ganglion l

patient says the members of his family are all more or less '' nervous."
No family history is obtained of tuberculosis, malignant, cardiac, or

renal disease.

Previous Medical History. Is negative except that he has had a

number of attacks of tonsillitis.

Present Illness. The patient is in an advanced stage of mental
hebetude probablv as a result of bromism, and his statements are
va-ue and unsatisfactory. He says that last winter he began to have
weakness and soreness about the articulation of his jaw (whether the
rio-ht or left side is not stated), the soreness afterward spreading to his
he'kd where it has persisted ever since. About two months ago bhnd-
ness developed in the right eve and this was followed about two weeks
later bv blindness in the left eve. During this same period he has
vomited a number of times. He is not able to walk unless some one
leads him. He believes this inability to walk is caused by his blind-
ness. His general health'has not suffered much. Headaches of late
have been very severe.

Physical Examination. A rather poorly developed and emaciated
male 'subject, looking considerably older than his given age of twenty-
five years. Skin is covered with a profuse, deeply colored, red erup-
tion, probablv the result of bromides. He has no noteworthy glandular
enlargement.' Pulse is regular, volume fair. Chest is poorly de-
veloped; expansion is fair and equal on both sides. Heart and lungs
negative. Abdomen soft and symmetrical. Abdominal organs nega-
tive. .
March 6, 1907. Examination by Dr. Spiller. The left pupil is
much larger than the right. Both irides are immobile to light ; slight
contraction is obtained of each pupil in attempt at convergence. He
says he sees light with each eye separately. He wrinkles the forehead
well, closes the eyelids, shows the teeth, and draws up each corner of
the mouth well, tliereforc he has no involvement of either facial nerve.
Tactile sensation is completely lost in the entire distribution of the
right fifth nerve, provided no pressure is produced. Sensation of pain
is" lost in the same distribution. Sensation is preserved along the
border of the lower jaw in the distribution of the cervical nerves.
The conjunctiva and cornea are anesthetic in the right eye but not
in the left eve. A piece of paper put far up the right nostril is not
felt unless pressure is produced, and causes no lachrymal reflex. The
jaw goes distinctly to the right when the mouth is opened. The right

401

3 SPILLER: tumor of the GASSEKIAX i.iA.\ ..I.IdN

tonsil is swollen and the uvula is absent. Sensation of the left nostril
is normal.

He is distinctly deaf to the voice in both ears. The soft palate
moves very imperfectly on the right side. The sense of smell is
greatly impaired on each side. The tongue is protruded straight.
Taste for salt and sugar probably is lost on the front and anterior part
of the tongue. This may have been only on the right side. The grasp
of each hand is fair. The upper limbs move freely in all parts ; biceps
and triceps tendon reflexes are not distinct on either side. Sensations

Fig. I. — Diagram showing the position of the tumor in Case I.

of touch and pain are normal in each upper limb. Movements in the
lower limbs are free in all parts. Patellar tendon reflex and Achilles
tendon reflex are lost on each side. Babinski's reflex is not obtained ;
the toes are not moved distinctly in either direction. Sensations of
touch and pain are normal in each lower limb. The gait and station of
the patient are very ataxic ; he has marked sway with the feet close
together.

Examination by Dr. de Schweinitz. Palpebral fissures are equal in
width. Left eye is slightly divergent. Movement of the left external

402

spiller: tumor of the gasserian ganglion 4

rectus is preserved. Movements of internal, superior, and inferior
recti are markedly limited. No wheel movement is obtained.

There is loss of movement of the external rectus of the right eye
and marked limitation of movements of the superior and inferior recti:
with almost lost internal rectus movement. Extensive double optic
neuritis is found with large retinal hemorrhages on the right side.

April II. Last night the patient became unconscious; for the last
few days he has not seemed to be quite so well. He was transferred
to Dr. Frazier's service for operation. A lumbar puncture was made
and the cerebrospinal pressure was found to be equal to 27 mm. of
mercury (351 mm. of water), and about 15 c.c. of spinal fluid was
removed until the pressure was down to 5 mm. of mercury (65 mm. of
water).

A decompressive operation was done on each side of the head by
Dr. Frazier just above the eSr, in order to relieve the intracranial pres-
sure. The brain bulged much on the right side. The patient died
April 12, about 2 p.m.

The brain was removed by me. The right temporal lobe was soft
and tightly adherent to the floor of the middle cerebral fossa, and
was loosened with much difficulty. A tumor was seen holding the
base of this temporal lol>e to the dura, and the lobe was removed by
cutting through the tumor. The latter seemed to grow from the dura,
was most developed at the seat of the right Gasserian ganglion, and
nothing was seen of this ganglion, as its position was occupied by the
tumor. The tumor covered the floor of the middle fossa in the region
of the middle lacerated foramen, foramen ovale, foramen rotundum
as far forward at the right sphenoidal fissure, also the inner part
of the petrous portion of the temporal bone in the posterior fossa
internal to the internal auditory meatus, and extended over the basilar
process as a flat growth as far as the left posterior clinoid process.
A round tumor was found invading the base of the right temporal
lobe (as described above), and was detached from the lobe in removing
the brain, as the lobe was soft. The tumor was firm and measured
about 4 cm. in each direction, and extended about 3 cm. into the brain.
The right fifth nerve was enveloped in the tumor at its entrance into
the pons, and the right third, fourth, and sixth nerves also were caught
in the tumor. The tumor did not seem to extend quite far enough to
the left to catch the left third nerve, but came very close to it. It
must, however, from the symptoms, have involved this nerve. The

403

5 spiller: tumor of the gasseriax gaxglion

bone of the skull did not seem to be implicated. A small hard mass
was found at the under part of the right cerebellar hemisphere. The
tumor was flat and thin except in the middle cranial fossa.

Microscopic Examination. The tumor does not extend into the
base of the brain except very slightly at the side of the pons. It covers
the pons exactly at the entrance of the fifth nerve into the pons, so that
the fibers of this nerve become a part of the tumor immediately

Fig. 2. — Photograph of the brain removed in Case I. The right temporal
lobe is invaded by the tumor. A small tumor of nervous tissue containing nerve
cells and medullated nerve fibers is seen on the right side of the cerebellum at
the exit of the seventh nerve.

beneath the pons. The nerve is completely degenerated at its entrance,
as stained by the Weigert hematoxylin method, but the Marchi stain
shows numerous fibers in the process of degeneration of the myelin
sheaths. The pyramidal tracts are not degenerated. The cells of the
motor nucleus of the right fifth nerve are swollen and have peripherally
placed nuclei. The sensory nucleus stands out prominently b>- its
yellow color in the Weigert stain, owing to the paucity of medullated
nerve fibers ; its cells appear soinewhat shrunken and some have peri-
pheral nuclei. A considerable mononuclear cellular infiltration is seen
about some of the vessels of the pons. These may be cells froin the
tumor, but they appear somewhat smaller. The fibers of the motor
root within the pons show partial recent degeneration by the ]\Iarchi

404

spiller: tumor of the oasserfax canglion 6

method. The pia over the pons presents a distinct round-cell infiltra-
tion, suggesting the possibility of syphilis ; the pia over the optic
chiasm also shows slight round-cell infiltration. The spinal root of the
fifth nerve shows degeneration by the Marchi method in the medulla
oblongata, more intensely in its posterior part. A transverse section
was made through the right optic nerve ; it shows much degenera-
tion.

The tumor consists of groups of cells separated by bundles of con-
nective tissue. In the dura from the region of the tumor the cells
form sometimes elongated masses as seen in an endothelioma.

The small tumor found on the cerebellum at the exit of the seventh
nerve, is of very peculiar structure. It appears to have originated in
the choroid plexus, and in one portion shows the convolutions of this
plexus. It is composed chiefly of neurogliar tissue and contains nerve
cells scattered through it irregularly ; some of these resemble the cells
of the spinal ganglia, others are like the cells of the anterior horns,
except that dendritic processes are not so distinct though occasionally
they are seen. The tumor is vascular. The cells contain chromophilic
elements, though these are not so numerous as is usual in nerve cells.
The tumor also contains medullated nerve fibers, as shown by the
Weigert hematoxylin stajn. These are chiefly at the periphery, but here
and there within the substance of the tumor one or two medullated
nerve fibers may be seen.

Case II. — McC. male, aged forty-three years, was referred by Dr.
Biddle to Dr. de Schweinitz's service in the University Hospital, and
examined by Dr. Spiller February 20, 1906.

He had had severe pain variable in intensity in the head about four
months, worse at night, and controlled only by morphine. The pain
was in the distribution of the first and second divisions of the right
fifth nerve and slightly in the third division. No dizziness was felt.
He had been exposed to syphilis about a year previously, and it was
uncertain whether he had contracted the disease or not. He com-
plained of diplopia. The hearing had not been so good in the right
ear as in the left about two years, but he had had repeated colds. The
right side of the head was said to perspire much less freely than the
left. The right pupil was smaller than the left. Reaction to light was
very sluggish, especially in the right eye ; reaction in accommodation
was prompt. He was unable to rotate the right eyeball outward, and
the right eyeball was slightly retracted ; the right palpebral fissure was

405

7 spiller: tumor of the gasseriax ganglion

smaller than the left. The right masseter muscle did not contract so
forcibly as the left, and the jaw deviated a little to the right when the
mouth was opened.

Gait and station were normal. The bladder and rectum were not
afifected. The muscular-strength was not diminished. The patellar
reflex was normal on each side. Xo objective sensory changes were
detected, except that light touch was felt on the right side of the
head -and face as pain, while heavy pressure was less painful.

A hard, swollen gland was found in the left side of the neck at the
anterior border of the sternocleidomastoid muscle.

Dr. Randall found that the reduction in hearing in the right ear
was slight, and was caused in part by middle ear disease. The bone
conduction was not so good as it ought to be, and a nerve lesion could
not be excluded, but the impaired conduction was no more than is
common with such old tympanic deafness.

An examination of the eyes in Dr. de Schweinitz service was made
February 20, 1906. O.D., ^lo. with correction, %2- O.S. %. -f-, with
correction %.5. Hyperemia of tarsal and bulbar conjunctiva. Inter-
nal squint O.D., limitation of movement of O.D. toward right. Pupil
of O.S. larger than that of O.D. Paralysis of external rectus of O.D.
Both irides respond to light. Ophthalmoscopic examination : O.D.
oval disk, temporal half slightly pallid. Low H. O.S. oval disk.
Nerve of good tint.

Dr. Harland. in Dr. Grayson's clinic, reported March 17, 1906: He
has no sinus pain or tenderness. Xasal obstruction has existed three
months. The septum is deviated to the left. Far back on the left side
is a soft, bleeding mass, and a similar mass is found in the vault of
the pharynx on the right side. The small mass in the vault of the
pharynx hanging down on the left side bleeds to the touch. The
lateral folds are thickened. The larynx is congested, but not par-
alyzed. The diagnosis was sarcoma of the nasopharynx.

Dr. Frazier, at my request, removed an enlarged gland of the neck
for diagnostic purposes, and it was found to be the seat of an endothe-
lioma. This was done before the report of the examination of the
throat was obtained.

The pain in the right side of the head and face continued, and relief
by surgical means seemed imperative. Dr. Frazier operated April 5,
1906, and exposed the Gasserian ganglion. Neoplastic tissue was
found about it, a part of which with a portion of the ganglion was

-toe

SPILLER: tumor of the GASSERI.W CAXCLION o

removed. This removal was followed by excessive hemorrhage, pre-
sumably from the internal carotid, and the operation had to be stopped.
A provisional ligature was placed on the common carotid artery. The
patient was not severely shocked at first by the operation, but an hour
later the pulse suddenly became very rapid, reaching i6o, and blood
pressure became 112. By night the pulse fell to 120 and the blood
pressure rose to 120. The man was comfortable the next day.

He complained frequently of pain in the distribution of the right
fifth nerve after the operation, but it was not of the same severity as
before the operation. Touch was not felt in the distribution of the
three branches of the fifth nerve unless it were made with slight pres-
sure, when it was recognized. The right conjunctiva was anesthetic.
Max 24. 1906. the patient tried to get out of bed four times, and
each time fell to the floor and had to be lifted back to bed. He was
out of bed i\Iay 25. but was duller than usual. By mid-day he became
dizzy while walking, and fell to the floor. He became stuporous and
by night was unconscious. Respiration was somewhat labored. He
died at 6:30 a.m.. May 26. There was some suspicion that he might
have obtained an overdose of morphine from some of his visitors.

The brain was very adherent to the dura at the base of the right
temporal lobe, and a tumor mass extended from the base of the skull
into the temporal lobe. The right temporal lobe was oedematous and
very soft, and was torn a little in removing the brain, and still more
so by handling the specimen later. The tumor mass was yellowish in
color and easily separable from the brain. The brain tissue beneath
was very vascular. Recent fluid hemorrhage was in the base of the
skull and apparently did not come from the necropsy. One or two
recent clots were in the tumor. Death possibly was from hemorrhage.
The region of the right Gasserian ganglion was occupied by the tumor,
and no distinct ganglion tissue could be found. The case was one
of tumor of the right Gasserian ganglion, extending into the base
of the right temporal lobe and into the nasopharynx.

My microscopic examination gave the following results :
Teased specimens, stained with osmic acid, of the right twelfth, right
eighth, and right seventh nerves were normal. The right sixth nerve
showed degenerated fibers in teased preparations.

The descending spinal root of the right fifth nerve was partially
degenerated in the medulla oblongata, by the Marchi stain. The right
eighth nerve, by acid fuchsin and Weigert stain, was normal. The

407

spiller: tumor of the gasserian ganglion

right sixth nerve was embedded in the tumor and was partly de-
generated as shown by these stains ; some of the axis cyhnders and
medullary sheaths were much swollen. The sensory portion of the
right fifth nerve was intensely degenerated, as shown by the Weigert
method and also when teased and stained in the fresh state by osmic
acid.

The tumor had the appearance of an endothelioma. The Gasserian
ganglion was embedded in the tumor, and the nerve cells and nerve
fibers of the ganglion were much degenerated. Nerve bundles in
tissue from the region of the right Gasserian ganglion were intensely
degenerated.

The nerve cells of the ganglion were greatly altered ; many were
shriveled masses of granular pigment in which no nucleus could be
detected, or if the nucleus were present it was indistinct.

Fig. 3. — Photograph of the base of the right cerebral hemisphere from Case II,
showing a condition closely resembling that of Case I.

The important features of Case I are : Paralysis of the right fifth
nerve, with severe pain as the first symptom, then blindness of the
right eye, later of the left eye, with optic neuritis, impaired mentality
when the pain was severe, inequality of pupils, the right being the
smaller, loss of reaction to light probably from the optic nerve disease,
bilateral deafness, weakness of the soft palate on the right side, im-
pairment of the sense of small, loss of patellar and Achilles tendon
reflexes, ataxic gait and station, weakness of the left third nerve, still
greater weakness of the right third nerve, and paralysis of the right
sixth nerve. The facial nerve was not affected.

408

spiller: tumor of the gasserian ganglion

The possibility of syphilis was at first considered, but later tumor
of the Gasserian ganglion was the diagnosis.

The important features of Case TI are: The history of a recent
exposure to probable syphilis, the pain and weakness in the distribu-
tion of the right fifth nerve, impaired hearing on the right side, lessened
sweat secretion on the right side of the head, inequality of the pupils,
sluggish iridic reaction to light, with prompt reaction in accommoda-
tion, weakness of the right external rectus muscle, some retraction of
the right eyeball, and narrowing of the right palpebral fissure. Dr.
Randall's examination made the right-sided deafness of doubtful diag-
nostic value, but the discovery of the tumor in the nasopharynx seemed
to point to intracranial tumor as the cause of the symptoms. This
possibility was strengthened by the excision of the enlarged lymph
gland of the neck, and the discovery of the endothelioma within it.
The diagnosis made by me"" was tumor implicating the right Gasserian
ganglion, probably endothelioma. The right sixth nerve evidently
was afifected. The sympathetic paralysis of the right side of the face
might be explained by the lesion of the fifth nerve.

Operation did not promise much, because it was evident that the
tumor was extensive, was both intracranial and extracranial, and its
complete removal was impossible. The pain in the right fifth nerve
distribution was so intense that relief must be obtained. Dr. Frazier
accordingly operated and removed a part of the Gasserian ganglion.

In the case reported by Dercum, Keen, and Spiller,=^ enlargement
of the glands of the left side of the neck, on the side of the trifacial
pain, was an early sign, and some of these glands when examined were
found to be the seat of endothelioma. The patient had shooting pains
in the lower limbs. The patellar reflexes were lost. Pupillary reflexes
were normal, there was no failure in rotation of the eyes, each disk
was somewhat anemic, and there was no congestion, neuritis, or
atrophy. The symptoms of intracranial disease so far as the cranial
nerves were concerned were confined to the fifth nerve. The pain in
the face was still intense immediately after the first operation. The
only change in the objective sensory phenomena was increase in the
hypesthesia, except in the conjunctiva and brow. Some time after the
first operation paralysis of the left external rectus occurred. In the
second operation a large portion of tumor was removed, and still the
pain persisted. The entire trigeminal distribution showed increased

= Jour. Amer. Med. Assoc, April 28, 1900.

401)

11 spiller: tumor of the gasserian ganglion

hypesthesia. The man was still able to appreciate contact, decided
differences in pressure, and differences between a spoon dipped in hot
and one dipped in cold water. The cornea was absolutely anesthetic.
There could be scarcely any doubt that the Gasserian ganglion was
entirely removed.

Hofmeister and Meyer have recently written a paper on tumor of
the Gasserian ganglion. In their case pain in the distribution of the
right fifth nerve was the first symptom, then developed weakness of
the muscles of mastication on the same side, and loss in objective sensa-
tion in this distribution, right-sided choked disk, paralysis of the
right third, probably of the fourth, sixth, and eighth nerves. The
lymphatic glands on the right side of the neck were swollen.

So much of the tumor as could be removed was excised in this case,
and pain ceased entirely, but only for about three months, and then
became severe again. A necropsy was not permitted. They believe
the implication of the carvernous sinus in the tumor caused the motor
disturbance in the right eye and the right choked disk. They think
the tumor may have grown into the orbit. From the necropsies in my
two cases it seems more probable that the paralysis of the ocular
nerves and the choked disks are caused in these cases by direct im-
plication of the nerves in the tumor, and by increase of intracranial
pressure, as well as by disease of the cavernous sinus. After the opera-
tion their patient was almost entirely unable to chew on the right side,
light touch usually was not recognized, pin stick was recognized some-
what better without distinction of head and point.

The diagnosis of a lesion of the Gasserian ganglion is usually easy,
but it may be difficult to determine whether it is syphilitic meningitis
or a tumor. In the case of 'Dercum, Keen, and Spiller, and in my
Case II, the syphilitic infection was probable. One is tempted by his
desires to make the diagnosis of syphilis, knowing that for this
affection relief is often obtained in mercury and iodide ; whereas in
tumor of this region the prognosis is very serious. Tumor of the
Gasserian ganglion seems in most cases at least to be irremovable.
It apparently has its origin in the dura and has the character of an
endothelioma or sarcoma, the distinction between these two forms
depending largely on the interpretation of the growth by each in-
vestigator. It is usually of large size, and while at first it may be
confined to the region of the Gasserian ganglion, it soon extends
chiefly as a flat growth, over the base of the middle fossa, possibly into

410

spiller: tumor of the gasserian ganglion 1'-'

the posterior fossa, and into the orbit. It has a tendency to extend
into the base of the temporal lobe above it, as in my Cases I and II,
without infiltrating- the brain, although firmly adherent to it. This
implication gives no symptoms, unless it be the cause of the loss of
smell which occurred in my Case I. Loss of smell is not uncommon
in brain tumor when intracranial pressure is much increased, but when
the base of the temporal lobe is implicated the lesion is near the sup-
posed centre of smell. Endothelioma arising- in the dura not infre-
quently implicates the adjacent bone.

The diagnosis of syphilis is therefore likely to be made, because
of the history of syphilitic infection in some of the cases, and because
of the knowledge that this disease by the basal meningitis it produces
may readily lead to symptoms of implication of the Gasserian gan-
glion. The mistake in diagnosis is not likely to be so serious as in
tumor of some other location in the brain, as complete removal by
operation seems impracticable in most cases at least. In my Case I
the pain in the distribution of the fifth nerve was not so severe a^^ in
Case II, nor as in the case of Dercum, Keen, and Spiller, and we
believed that possibly a decompression, might be sufficient by relief
of intracranial pressure to lessen the suffering. The man, however,
lived only one day after this operation.

The swelhng of the lymphatic glands of the neck from tumor is a
valuable means of diagnosing intracranial neoplasm, ft is not ob-
served in every case, but was present in the case of Dercum, Keen,
and Spiller, in the case of Hofmeister and Meyer, and in my Case II.
It is not difficult to excise an enlarged gland, and the finding of tumor
within it, endothelioma or sarcoma, is extremely indicative of brain
tumor, when such symptoms as those described above are present.
The glandular tumor may result from a growth in some other part
of the body, but it seems to occur with sufficient frequency in tumor
of the Gasserian ganglion to make its presence of diagnostic value.

In some instances the fifth is the only cranial nerve involved, as
in the case of Dercum, Keen, and Spiller, and then some confusion in
diagnosis with the tic douloureux may occur, aspecially if objective dis-
turbance of sensation in the fifth distribution be slight, as in the case
to which reference has just been made. Some disturbance of ob-
jective sensation is almost always present, the pain is felt in all three
branches of the nerve nearly simultaneously, because the disease is in
the ganglion in which these roots arise, and the motor branch of the

411

13 spiller: tumor of the gasserian ganglion

fifth nerve soon becomes paralyzed. With such a symptom complex
the diagnosis of tic douloureux is impossible. It is in the early stage
of the symptom complex that operation on a tumor of the Gasserian
ganglion is more promising, and it is possible to perform the opera-
tion while the tumor is small. In any case in which pain is felt in all
three branches of the nerve nearly simultaneously and some loss of
sensation is detected in the distribution of the same nerve, it is probable
that the lesion is in the ganglion, and this probability is increased if
paresis of the motor portion of the fifth nerve occurs. It is at this
stage that we might hope to remove the tumor entirely. Pain in the
fifth nerve is almost always an early symptom, and yet it may in rare
instances be slight.

P have studied a case in which each ganglion was embedded in
and infiltrated by a soft ependymoma without distinct clinical signs
of this invasion. The man had had headache only in the left occipital
region extending forward to the left temporal region, and lying on
the left side increased the headache. He had had also soreness on
pressure over the left supra-orbital and left infra-orbital foramina,
but no tenderness over any other exit points of the fifth nerves. There
was never any objective disturbance of either the sensory or motor
portion of the fifth nerves. It is exceedingly questionable whether the
headache could be attributed to implication of the fifth nerve ; it was
not present on the right side of the head, and on the left side it began
in the back of the head and extended to the frontal region.

Recently I had a case with necrops}- in which a glioma of the pons
caused symptoms resembling closely those of tumor of the Gasserian
ganglion. Pain was not present in the face. The absence of pain
in a case such as this should suggest a glioma of the pons, which may
not cause any paralysis of the limbs.

The persistence of pain after removal of the Gasserian ganglion in
some of these cases is puzzling. It was exceedingly striking in the
case of Dercum, Keen, and Spiller, and was observed in my Case II.
Hofmeister and Meyer mention the same phenomenon in their case.
They acknowledged the possibility of attributing the recurrence of this
pain to conduction by the facial nerve, but they think it is more
probably caused by extension of the tumor into the central stump of
the fifth nerve. The anesthesia of the cornea would seem to indicate
that the conduction at least by means of the upper part of the fifth

^ Ainer. Jour. Aled. Sci., July, 1903, and Jour. Nerv. and ]\Ient. Di?., May, 1907.

412

sptllek: tumor of the gasserian ganglion

nerve is destroyed, as the eyeball receives no facial fibers. It is difficult
also to explain the preservation of objective sensation from periph-
eral stimulation. In the case of Dercum, Keen, and Spiller, after
the second operation hypesthesia of the fifth nerve distribution with
anesthesia of the cornea was present. This may indicate that the
seventh nerve may convey sensation. In this case hypesthesia was
present before the first operation, but the patient could distinguish
between the point and head of a pin. and pressure sensation was
everywhere well preserved. All handling of the skin of the face gave
pain and there was marked hyperalgesia.

In some cases of removal of the Gasserian ganglion for tic dou-
loureux all sensation, including that of pressure, is lost, at least for a
period following the operation, but in two cases of removal of the
Gasserian ganglion P have observed, preservation of pressure sensa-
tion persisted with loss of other forms of sensation in the distribution
of the fifth nerve. In some, if not in all, of these tumor cases in
which the ganglion is implicated removal of the ganglion or a large
part of it does not seem to be followed by complete loss of pressure
sensation in the fifth distribution. This preservation of pressure sen-
sation was very remarkable in my two cases, in one of which a large
part of the ganglion had been removed, and was observed also in the
case of Dercum, Keen, and Spiller. In the case of Hofmeister and
Meyer, after the operation light touch usually was not recognized, but
pin prick was felt somewhat better without distinction of head and
point; this may have been caused by preservation of pressure sen-
sation. Microscopic study of the tissue removed at necropsy in my
Cases I and II showed intense degeneration by the Weigert medullary
stain, of the sensory root of the fifth nerve in both cases. It is true
that the Marchi stain showed many fibers in the sensory roots of
both cases in the process of degeneration, but this by no means implies
that these fibers were capable of couduction, as the Marchi reaction is
available for tissue that has been degenerated for months.

I have for a long time believed that the facial nerve may contain
some sensory fibers, and in lecturing on this subject in 1907 to the
students of the University of Pennsylvania I suggested that a mem-
ber of the class might make it a subject of special study. The result
has been an excellent paper by R. H. Ivy and L. W. Johnson,'^ which
contains the evidence pointing to this possibility.

'Jour. Nerv. and Merit. Dis.. 1906. p. 736.
= Univ Penna. Med. Bull., May, 1907, P- 35-

413

15 SPILLER : TUMOR OF THE GASSERIAN GANGLION

It is unquestionably true that in some cases excision of the Gasserian
ganghon causes complete loss of all forms of sensation, but this fact
does not make the transmission of certain forms of sensation through
the facial nerve impossible. It is not improbable that the deep sen-
sation of the facial nerve may be temporarily interfered with by opera-
tion on the trigeminal nerve. Twisting out peripheral branches of the
latter causes much swelling of the face, and probably, thereby, some
impairm'ent of the function of the facial nerve. Excision of the
Gasserian ganglion causes swelling of the axis cylinders and medullary
sheaths of the peripheral branches of the trigeminal nerve, and if
these are intimately associated with branches of the facial nerve, this
swelling may also interfere temporarily with the function of the latter.
Destruction of the sensory root of the trigeminal nerve by a tumor
affords better opportunity for testing the sensation of the face, than
does destruction of some part of this nerve by operation, at least in
the period immediately following operation. There is great need of
further observation regarding the preservation of pressure sensation
in relation to lesions of the trigeminal nerve.

When the pain is intense in these cases, as usually it is, division of
the sensorv root of the fifth nerve or removal of a part or of the
whole of the ganglion when possible is a justifiable operation. It may
lessen the pain and in some cases remove it. but it is not an infallible
means of relieving suft'ering. It is true that as yet no complete
removal of a tumor of the Gasserian ganglion is on record, but com-
plete and early removal might be possible. Operation, however has
been attempted in very few cases.

Very severe hemorrhage followed the removal by Dr. Frazier of
a portion of the ganglion in my Case II, possibly because the tumor
may have weakened the walls of the vessels, and rendered them more
liable to rupture. This fact should be borne in mind in operations
on tumor of the Gasserian ganglion, as after the ganglion is cut from
most of its attachments it is customary to pull it away, and repeatedly
a rupture of the cavernous sinus has occurred. It might be well to
avoid all pulling on intracranial tissue when operation is performed on
tumor of the ganglion, except tearing the sensory root from the pons.
This would be the most desirable procedure when the tumor could
not be removed.

Hofmeister and Meyer" give the literature on tumor of the Gasserian

' Deut. Zeit. f. Nerven., 1906, xxx, 206.

•iU

spiller: tumor of the casserian ganglion 16

gangiion. It is not very extensive. In their case the tumor was a
sarcoma. Marchand" has made the pathology of these growths re-
cently a subject of special study. The cases he collected with necropsy
are those of Giinsburg, Blessig, Petrina and Klebs (two cases), Good-
hart, Hansch and Bezold, Krogius, Hegelstam, Prince, Dercum, Keen,
and Spiller, and Hofmeister and Meyer. To these Marchand adds
another case. It seems remarkable that the cases of tumor of the
Gasserian ganglion are not more numerous. I can only believe that
these tumors are not diagnosticated in many instances when they exist.

The first operation for tumor of the Gasserian ganglion was done
by Krogius.^ An endothelioma the size of a pigeon's egg was re-
moved, but a portion was left behind. In this case the tumor pre-
sented in the nasopharynx. Death occurred from meningitis thirteen
days after the operation.

The tumors in my two^ cases have nuich the appearance of an
endothelioma. As Marchand points out, the ncwgrowths of the Gas-
serian ganglion that have been reported seem to have a very similar
histology, although different names have been given to them. He is
inclined to believe that these tumors arise in the undifferentiated
Aiilage of the ganglion, and that they may be regarded as neurozy-
tomas. In this connection it is interesting to refer to the neuro-
glioma found in my first case at the junction of the pons with the
cerebellum, and distinct from the tumor of the Gasserian ganglion. It
may have been a congenital anomaly, and gave little evidence of
growth, and was very small. It may afford some support for the
opinion that in these cases of tumor of the Gasserian ganglion there
is a portion of the Anlage of the ganglion which fails to become differ-
entiated into ganglion tissue, and later becomes the seat of a neoplasm.
In whatever way these tumors are regarded, the necropsies show that
they are malignant, and that they are likely to invade the bone of
the skull.

' Festschrift f. von Rindfleisch, 1907, p. 265.
* Rev. de chin, 1896, xvi, 434.

415

Reprinted from The jGurnal of the American Medical Association, Dec. 19,

1908. J'ol. LI, pp. 2101, 2102.

American Medical Association, 103 Dearborn Ave., Chicago.

THE DCRATIOX OF LIFE AFTER EXTENSIVE
HEMORRHAGE OF THE BRAIX ^

By William G. Spiller. M.D.

PROFESSOR OF NEUROPATHOLOGY AND ASSOCIATE PROFESSOR OF NEUROLOGY IN THE
UNIVERSITY OF PENNSYLVANIA, PHILADELPHLA

]\Iv attention has been called recently to a diagnosis of apoplexy
in a case in which life terminated in about five or ten minutes after
a severe attack began. The person, a woman, had been in good health
previouslv, was nearly 70 years of age. and after retiring was heard
to groan. She was found within a few minutes with her head hang-
ing over a bath-tub and her face much congested. A physician, called
at once, pronounced her dead and gave apoplexy as the cause of death.

~\l\ experience has not justified a diagnosis of apoplexy in cases in
which life has been terminated so quickly. I have taken thirteen
specimens from my collection of pathologic conditions, in which ex-
tensive hemorrhage of the brain was found at necropsy. ]\Iany of
these were from cases in my service at different hospitals ; others were
from cases in the service of Dr. ^lills. In all instances life had been
prolonged at least hours after the onset of the apoplexy, even when
the hemorrhage was very extensive, had broken into the ventricles.
and filled all the ventricles, even the fourth. In two cases of this
kind (i and 2) hfe was prolonged several hours; in one about five
hours, in the other eighteen to twenty hours.

As a rule extensive rupture into the lateral ventricles is followed
by more rapid death than when the ventricles escape, but a moderate
hemorrhagic exudate into the ventricles is not necessarily rapidly fatal.
In nine cases (3. 4, 5. 6, 7, 8. 9, 10 and iT) the lateral ventricles were
implicated. In Case 3 death occurred after three days ; in Case 4
after eleven days ; in Case 5 after at least five days ; in Case 6 after
at least twenty- four hours; in Case 7 after five or six days; in Case
8 after about five days ; in Case 9 after about a day and a half ; in
Case 10 after sixteen days; in Case 11 after about two days.

* From the Department of Xeurolog>^ and the Laboratory- of Xeuropathology
in the University of Pennsvlvania.

1 ' 416

SPILLKR : DURATION' OF LIl-K AFTKK III-.M ( )RR 1 1 \( ;K OF r.RMX J

Case 10 is remarkable in that the hemorrhage was very extensive in
the left cerebral hemisphere and extended into both lateral ventricles,
and yet the patient lived sixteen days.

In Cases 12 and 13 the ventricles were not implicated; in Case 12
life persisted a little over six days ; in Case 13 almost two months.

It seems to be held by some that life must be terminated within a
few days if the hemorrhage has destroyed a large part of one cerebral
hemisphere, but Case 13 shows this is not necessarily so. In this case,
in which life persisted almost two months, the clot was found partly
encapsulated and measured 7 x 2.5 cm. It destroyed the lenticular
nucleus, a large part of the posterior limb of the internal capsule and
extended to the island of Reil. It is truly remarkable that the brain
may be so severely injured without a speedy termination of life.

The cases, briefly presented, are as follows :

Case i (No. 308). — History. — W. E. L., aged 52, became hemiplegic on the
left side after an apoplectic attack November, 1905, and was unconscious twenty-
four hours. He became able to walk later, and on different dates had a few
convulsions. On Nov. 20, 1905, shortly after noon, he fell to the floor, and was
put to bed perfectly conscious, but very gradually he began to lose consciousness,
and in about an hour after the fall he was entirely unconscious. He then had
a severe convulsion and died about 5 p. m. the same day. He lived about five
hours after the beginning of the apoplectic stroke.

Autopsy. — A large hemorrhage had destroyed the right lenticular and caudate
nuclei, and had filled all the ventricles, even the fourth, and had extended to the
base of the brain. The hemorrhage was chiefly ventral to the lenticular nucleus,
but extended into the front part of this structure, and had broken into the
anterior part of the right lateral ventricle. Much blood was found over the
pons and medulla oblongata.

Case 2 (No. 370). — History. — John E., aged 26, was admitted to the hospital
Sept. 15, 1906, and died Nov. 17, 1906. About five weeks before admission he
took a large does of quinin ; he then became very drowsy and was unconscious
for three days. When he recovered consciousness he was paralyzed on the
left side. He was able to walk about a week later. The notes state that
" yesterday " in the afternoon he had severe repeated convulsions, lasting until
his death. His pulse reached 160; sweating was profuse; he vomited, and died
the following day at 10:30 a. m. He, therefore, lived about eighteen to twenty
hours, or at least from the afternoon of one day to 10:35 o'clock of the next
day.

Autopsy.— The hemorrhage filled all the ventricles completely, destroyed the
greater part of the right cerebral hemisphere, including the region of the lenti-
cular nucleus and the right occipital lobe.

Case 3 (No. SS7)-— History. — Charles S. was brought into the hospital Oct.
2, 1906, unconscious, and did not regain consciousness before his death, on Oct.
5, 1906. He was paralj'zed on the right side. He lived at least three days.

417

3 SPILLER : DURATION OF LIFE AFTER HEMORRHAGE OF BRAIN

Autopsy. — A hemorrhage was found measuring 2X7 cm. It destroyed the
left lenticular nucleus, part of the posterior limb of the internal capsule, and
extended almost from the posterior horn of the lateral ventricle to the anterior
horn, and ruptured into the latter, distending it with a recent clot.

Case 4 (No. 286). — History. — William U., aged 72, had had diabetes many
years. He retired at his usual time the night before admission to the hospital,
and was found at noon, March 15, 1905, lying at the side of the bed. When
seen by his physician at 12 :30 p. m. he was semi-conscious and paralyzed in the
left upper and lower limbs. His speech was mumbling. He was brought to the
hospital Alarch 15 and died March 26. He, therefore, lived eleven days after
the stroke.

Photographs of sections made through the pons in Case 2, in wliich the
greater part of the right cerebral hemisphere was destroyed and all the ven-
tricles were filled with blood. The great distention of the fourth ventricle by
the blood clot is shown. The surrounding tissue was stained from the blood.
The patient lived eighteen to twenty hours after the attack began. (Photo-
graphs by Dr. Alfred Reginald Allen.)

Autopsy. — A large hemorrhage was found in the right internal capsule, optic
thalamus and lenticular nucleus and a clot filled the center and a part of the
anterior horn of the lateral ventricle.

Case 5 (No. 144).— History. — Annie S., aged 35, w-as admitted to the hospital
Nov. 5, 1897, in a semi-conscious condition, with a history of acute alcoholism of
three weeks' duration. Some twitching of the facial muscles was noticed on the

418

spiller: duration of life after hemorrhage of rrain 4

fifth day. The date of death is not given, but the statements show that she
lived at least five days.

Autopsy. — A large hemorrhage was found in the left lateral ventricle, not
extending into the posterior horn. The inner surface of the lenticular nucleus
and the anterior portion of the optic thalamus were disorganized. The hemor-
rhage was evidently recent, the blood being dark colored and soft.

Case 6 (No. 50). — History. — James L., aged 76, was admitted to the hospital
Nov. 5, 1897, paralyzed on the right side and deeply stuporous. In the morning
of the same day he had fallen unconscious while walking. The day following
admission he was more conscious and noticed those about him, but he gradually
became more stuporous until his death. The date of death is not given in the
notes, but the statements show that the patient lived at least until the next day.

Autopsy. — A large amount of clotted blood was found in the left lateral
ventricle. Hemorrhage filled both ventricles, and on the left side had invaded
the thalamus, the posterior portion of the lenticular nucleus, the internal capsule,
and the adjoining part of the occipital lobe.

Case 7 (No. 318). — History^ — James W., aged 37, became paralyzed on tlie
right side during the night of July 4, 1905. He improved greatly. He fell on
the evening of Dec. t2, 1905, and became stuporous, had conjugate deviation of
the head and eyes to the right, and paralysis of the face and limbs on the left
side. He improved considerably. On December 23 it is stated that he had been
more stuporous for a few days and his pulse was weaker. He was still more
stuporous on December 24, did not change his position in bed, and the pulse was
weak and rapid. The pulse was hardly perceptible on December 25, and the man
was very weak. He lived fi've or six days in a very critical condition.

Autopsy. — A hemorrhage, 2.5 X 3 cm., was found in the occipital lobe. It
extended into the lateral ventricle.

Case 8 (No. 336). — ///.j^ory.— Charles H., aged 80, was admitted to the
hospital Oct. 28, 1899, and died March 11, 1906. He was brought from the out
wards March 6, 1906, in an unconscious condition. The breathing was stertor-
ous. The head and eyes were turned to the left. The right upper and lower
limbs and the right side of the face were paralyzed. He died j\Iarch 11, 1906, at
10 140 a. m. He lived about five days.

Autopsy. — The hemorrhage destroyed the posterior part of the left optic
thalamus and of the posterior limb of the internal capsule and lenticular nucleus,
and filled the descending horn of the left lateral ventricle.

Case 9 (No. 273).— History. — Harriet J., aged 44, fell to the floor and
became unconscious on Jan. 14, 1905, at 9 a. m. When she was brought to the
hospital the same day she was stuporous but could be aroused, and was paralyzed
on the right side. Speech was almost unintelligible. From 11 a. m. January 15
she w^as almost continually in convulsions until death. Lumbar puncture was
performed at 5 p. m., and the patient died the same evening. She lived about a
day and a half.

Autopsy.— A large hemorrhage 6.5X4 cm.,«of recent development, was
found in the left lenticular nucleus, extending into the internal capsule, and at
the anterior end of the left ventricle had broken into the ventricle. Hemorrhage
was found in both lateral ventricles, but mostly in the left.

419

5 SPILLER : DURATIOX OF LIFE AFTER HEMORRHAGE OF BRAIX

Case io (Xo. 429). — History. — Ernest W. was admitted to the hospital Jan.
25, 1908, and died Feb. 10, 1908. While working about the hospital on January
25 he suddenly fell and became paralyzed on the right side in face and limbs.
He was stuporous but could be partly aroused. Loud commands were not
heeded. He had difficulty in swallowing, was unable to speak, and wet the
bed. He showed no improvement on February 2. The pulse was 105 and
feeble. He lived sixteen days, a remarkably long time in consideration of the
extensive hemorrhage.

Aiftopsy. — A hemorrhage occupied a large part of the posterior half of the
left cerebral hemisphere, and both lateral ventricles contained blood. The clot
within the hemisphere measured 5 X5-5 cm. and at one level 5.5 X6.5 cm.

Case ii (Xo. 230). — History. — Miss H., aged 50, fell on the evening of April
19, 1904, became completely unconscious, and seemed to be completely parah'zed.
On April 21 the pulse was weak and respiration stertorous. She died in the
afternoon of the same day. Life was prolonged almost two days.

Autopsy. — A large hemorrhage was found filling the w-hole of the left
lateral ventricle but not extending into the right ventricle. The substance of the
brain was implicated in the lesion only near the anterior part of the left lateral
ventricle.

Case 12 (Xo. 241). — History. — Gottlieb R., aged //, w-as admitted to the
hospital July 19, 1904. The attack occurred suddenly during the night before
admission, and the patient was found lying on the floor unconscious. Respira-
tion was of Cheyne-Stokes type. The paralysis was complete on the left side.
The pulse was rapid. The patient was partly conscious on July 20, and im-
proved somewhat later until July 24, but died July 25. He lived a little over
six days.

Autopsy. — A hemorrhage measuring 4X5 cm. had destroj'ed the greater
part of the right lenticular nucleus, the posterior part of the posterior limb of
the right internal capsule, and extended backward beyond the posterior portion
of the optic thalamus.

Case 13 (Xo. 348). — History. — Hesse, aged 40, was admitted into the hospital
in an unconscious condition ^March 20, 1906. Xo history- was obtainable. The
patient had been found in a stable. He had conjugate deviation of the head and
eyes to the left and paralysis of the right upper and lower limbs. He was
stuporous and did not speak at any time. Xo marked improvement occurred
during his long stay in the hospital. An operation was performed and a sub-
dural hemorrhage over the left temporal region of the brain was removed. He
died May 18, 1906, two weeks after the operation. This case was noteworthy
because of the long duration of the hemorrhage.

Aufopsy.^A large blood clot was found in the left cerebral hemisphere,
measuring 7 X 2.5 cm. It had destroj^ed the lenticular nucleus, a large part of
the posterior limb of the internal capsule, and extended to the island of Reil.
The clot had the appearance of long duration and was partly encapsulated.

4409 Pine Street.

420

Published with illustrations in the Review of Xcurology and Psychiatry,
December, 1908.

TUMOUR MALFORMATIONS OF THE CENTRAL
NERVOUS SYSTEM

By William G. Spiller, ALD.
professor of neuropathology and associate professor of neurology in the

UNIVERSITY OF PENNSYLVANIA

Malformations of the central nervous system occurring as tumour-
like structures are recorded in the literature, hut the cases are not very
numerous. Within the past few years two interesting examples of
embryonic structural defect have come under my observation. In one
of these a tumour was found in the cerebello-pontile angle; in the
other, the tumour was at the lower end of the cord.

(a) Malformation of the Cerebello-pontile Angle.— In examining
a brain in which a tumour having the appearance of an endothelioma,
and growing from the region of the Gasserian ganglion, had been
found, I observed a small flat growth about the size of a small bean,
situated on the under surface of the right lateral lobe of the cerebellum,
at tlie angle formed by the cerebellum, medulla oblongata, and pons.
This tumour, when studied microscopically, gave the following
findings : —

Though lying close upon the brain, it did not form an intimate part
of its tissue. It was closely connected with the choroid plexus of the
fourth ventricle, and in a few places was not differentiated from the
pia covering the cerebellum. The choroid plexus was almost every-
where distinct from the tumour, but in a few places it formed intimate
union with it, and the tumour in small areas had a border of cells of
the ependymal type. The groundwork of the tumour was a loose
neuroglia, with irregular, short and rather massive bands of denser
n^euroglia of varying thickness running through it in all directions.
In some areas the grotmd substance was denser than in others. The
tumour contained numerous blood vessels. Scattered all through the
tumour, without any definite arrangement, were nerve cells, round,
elongated, or triangular, resembling in shape and size the cells of Betz
in the paracentral lobule, or the cells of the spinal ganglia. Some of
the cells appeared degenerated, had peripherally-placed nuclei and
1 421

J. spiller: malformatioxs of central nervous system

swollen cell-bodies, and contained few or no chromophilic elements.
Others had chromophilic elements like those of the pyramidal cells
of the motor cortex.

The tumour in places had numerous medullated nerve fibers, as
shown by the Weigert hsemotoxylin stain. These were almost con-
fined to the periphery, and in some portions were parallel with the
border of the tumour, and in others radiated from the periphery a
short .distance toward the center. Most of these fibers had a dis-
tinctly degenerated appearance. In some places they formed a
mesh work.

The interpretation of this tumour was made easy by the excellent
article by Kasimir v. Orzechowski.^ This writer states that his find-
ing seems to be the only one of malformation of the recessus lateralis
reported in the literature. He believes that the so-called acusticus
tumours, and other tumours of the cerebellopontile angle, are probably
remains of the wall of the lateral recess. The tumour that he de-
scribes was covered in places by an endothelial lining, and contained
nerve cells and nerve fibers, and seems to have been similar to the
malformation in my case.

The embryological malformations of this region, as v. Orzechowski
suggests, are probably not so rare as appears at present, and when
attention is directed to the subject, the reports of such conditions will
doubtless become more numerous.

It is difficult to decide whether any relation existed in my case be-
tween the malformation of the cerebello-pontile angle and the tumour
growing from the region of the Gasserian ganglion, but such connec-
tion is possible, inasmuch as Alarchand holds that tumours of the
Gasserian ganglion arise in the undifferentiated Anlage of the ganglion.

I have referred briefly to my findings in this case in a recent paper,
but their importance seems to justify more consideration than was
given to them there. -

{b) Malfonuation on the Sacral Region of the Cord. — The case
was one of extensive carcinoma, and has been reported without refer-
ence to the malformation of the spinal cord.''^

The patient, a man, was admitted to the Philadelphia General Hos-

'v. Orzechowski, Obcrsteincr's Arbciten, vol. xiv., 1908.

• Marchand, "Festschrift fiir Rindfleisch," 1907, p. 265; Spiller, American
Journal of the Medical Sciences. Nov.. 1908.

^Spiller and Weisenhurg. Journal of Nervous and Mental Disease. Aug.. 1906;
and Weiner klinisch-therapeutische Wochenschrift, Nos. 29, 30. and '31, \go6.

422

spiller: malformations of central nervous system 3

pital, December 24. 1904. The face and upper limbs were not affected,
but the lower limbs were almost completely paralysed, although some
movement was possible at each hip and each knee, and the toes were
moved slightly. The patellar reflex was exaggerated on each side,
but ankle clonus was not obtainable. Ilabinski's reflex was distinctly
present on each side. Sensations for touch and pain were preserved
in the lower limbs, but tactile sensation was diminished on the soles
of the feet. Retention of urine was present. There was only one
record of pain, and that was made July 10, 1905.

He came again into my service in January, 1906. At that time his
condition was as follows : He lay in bed with the thighs strongly flexed
on the abdomen and the legs flexed on the thighs. He had slight
voluntary jwwer in flexion of each thigh, but it was very doubtful
whether he had any voluntary movement of his toes. The slight
upward movement of the -toes which sometimes occurred was prob-
ably reflex. The lower limbs were much wasted. The patellar reflex
and Achilles tendon reflex were absent on each side. The Rabinski
reflex was very typical on each side. Tactile and pain sensations were
entirely lost in the lower limbs. He had no control of the urine or
faeces, and the bowels did not move without enema. He moved the
upper limbs freely, but the movements were weak. These limbs were
also wasted. Biceps and triceps tendon reflexes were present on each
side, and about normal, considering the general emaciation. The
pupils were equal. The movements of the eyeballs probably were
good, although it was impossible to get him to respond promptly. No
impairment of cranial nerves was detected. The abdominal muscles
were intensely rigid, and the abdomen was distended. A necropsy was
obtained. Numerovis carcinomata were found in various parts of the
body.

A flat tumour was observed on the anterior part of the cord in the
upper sacral region. It was about 2 cm. long X i-2 cm. broad, and
was covered by the pia. Some of the nerve roots were implicated in
the tumour. It was very friable.

The spinal ganglion of about the ninth thoracic root and this root
also showed a little of the loose tissue seen in the tumour, but here, of
course, it was outside the dura. This tissue contained osseous plates,
here and there a few striated muscle fibers, fatty connective tissue,
numerous vessels filled with red blood cells, and at one part a small
mass of densely-packed round and elongated nuclei between which

423

4 spiller: malformations of central nervous system

were connective tissue fibers. Between these various structures were
loose bands of wavy connective tissue.

The tumour within the pia was of the structure described above.
The bony plates stained purple, especially along the edges, with
hsemalum, and contained cells separated from one another. ]\Iasses
of cartilage-like tissue also were found. These stained very faintly,
had a somewhat hyaloid appearance, and contained numerous cells
with a large amount of protoplasm, much larger and very different
from those within the bony plates. The muscle fibers were striated
transversely and longitudinally, contained many elongated nuclei, and
were like fully-developed muscle fibers. Here also were fat cells and
some connective tissue fibers. Sections were taken from both the
upper and lower ends of the tumour.

A spinal tumour containing striated muscle fibers resembling the
findings in my case is described by J. Graham Forbes.* It was in the
cervical region. The patient, a child aged five years and six months.
had paralysis of both upper and lower limbs, supposed to be caused
by cervical caries. He had always been " tottery " on his legs, had
looseness of the bowels, and frequent and copious micturition. His
head and shoulders drooped when he was three years old. Later he
had pain in the course of the posterior cervical nerves and rigidity
of the neck. An operation was performed, an incision was made
through the dura, and a growth about the size of a haricot bean pro-
truded through the opening, and seemed to grow from the spinal cord.
It was covered by the pia. The bulging portion of the tumour was
removed.

The tumour on its posterior aspect was covered by a layer of dense
fibrous tissue, probably thickened and adherent meninges. On the
reverse side were many small strands of well-defined striated muscle
fibers, portions of which were embryonic, and appeared as long fusi-
form cells with several nuclei arranged in columns toward the tapering
end of the cell. (Such embryonic muscle cells were not found in my
case.) These structures formed the most striking and characteristic
feature of the growth. The strands of muscle fibers and cells were
separated by broad bands of wavy fibrous tissue and small collections
of fat cells. The center of the tumour was occvipied by poorly-staining
connective tissue, interspersed with inflammatory cells and a large
number of oval and round cells with fibrillary network, some of which

* Forbes, St. Bartholomeic's Hospital Reports, vol xli., 1905, p. 221.

424

SPILLER : MALFORMATION'S OF CFXTKAL NERVOUS SYSTEM 5

possibly was neuroglia. In the anterior part of the growth was a
cluster of large multinucleated giant cells resembling the myeloplaxes
or osteoclasts of bone marrow, and apparently indicating the existence
of )'oung osseous tissue. The tumour was richly supplied with vessels.
^lany of the cells were free in the connective tissue ; they stained
poorly and showed an oval nucleus with a hyaline margin of proto-
plasm and shadowy ill-defined processes, and resembled degenerated
nerve cells. The presence of fully-developed muscle fiber, with em-
bryonic muscle cells and osteoclasts, showed that the growth was a
teratoma, and on account of its vascularity it was regarded as a myo-
angioma.

Forbes mentions a case of senile dementia described by Pick, in
which bundles of smooth muscle fibers were found in the thickened
membranes over the posterior surface of the cord. These muscle
fibers were connected by strands with the hypertrophied muscle of the
arterial walls.

Gowers'-"' case of a lipoma, with striated muscle fibers attached to
the conus, is well known.

^ Gowers, Transactions of the Path. Sac. of London, vol. xxvii., 1876.

425

Extracted from the American Journal of the Medical Sciences, December,
1908.

IXFERIOR POLIO-EXCEPHALITIS IX A CHILD OF FOUR
YEARS. WITH RECOVERY

By Charles F. Judsox, }\I.D.,

PHYSICIAN TO ST. CHRISTOPHERS HOSPITAL, PHILADELPHIA, AND

Horace Carxcross, M.D.,

OF PHILADELPHIA

A boy, aged four years, was admitted to Dr. Judson's senace in
St. Christopher's Hospital, October 29, 1907, and presented an unusual
combination of symptoms. His family history was negative. He had
been healthy from birth and had not had any infectious disease.
Three weeks before his admission to the hospital the mother noticed
that he was " stiff " (as she described it) on coming down stairs in
the morning, and thought that he had rheumatism, because he seemed
" stiff and sore." After breakfast, however, the child played about
outside as usual. The mother soon noticed that he staggered and
that his right eye was turned inward. He held his head to the right
side, although he was able to move it in all directions. He continued
to play about, but seemed dull. During this time he had a cold and a
paroxysmal cough. He never had any convulsions, did not complain
of pain or headache, did not vomit, ate well, voided his secretions
normally, and did not appear feverish. It is questionable, however,
whether he did not have a slight rise of temperature. The mother
failed to notice any weakness of his extremities. He was particularly
stupid just before he was brought into the hospital, and staggered a
great deal on the way there.

The examination of the boy showed him to be well developed, and
of good color. There was a slight scalp wound in the left temporal
region, from a fall on the sidewalk five weeks before his admission.
His throat was clear. His lungs, heart, and abdomen were normal.
The spleen was not palpable, the liver barely so. The lymphatic^ and
the epiphyses of the long bones were not enlarged.

The mental condition of the child was very dull, and he had incon-
tinence of urine and feces. There was no loss of power in the
1 426

JUDSON AND CARXCROSS: IiXFERIOR POLIO-ENCEPHALITIS 2

extremities, and the grip was good. There was no wasting. Meas-
urements showed both sides to be equal. The electrical reactions were
normal. There was a marked ataxia of the cerebellar type, and Rom-
berg's sign was present. The knee-jerks and Achilles jerks were
absent. The skin reflexes were normal. There was no Babinski and
the normal plantar reflex was obtained. There were no sensorv or
trophic disturbances and the tactile, pain, and temperature senses, as
well as the sense of position, appeared normal. There was a coarse
tremor of the hands when the patient was disturbed. Coordinate
movements were well performed when the child was in the recumbent
position. In addition to the mental hebetude, incontinence, cerebellar
ataxia, and loss of deep reflexes, there was paresis of both external
recti, more marked on the right side, with deviation of the tongue to
the left and an extremely slight obliteration of the nasolabial fold on
the left side. The other extra-ocular muscles acted normally, and the
pupils were equal ; reacted to light, and accommodated freely. The
eye-grounds were examined by Dr. Kraus, and found normal. Dr.
Stimson found hearing equal and normal on both sides, with a normal
tympanum. Four examinations of the urine gave negative results.
On November 9, 1907, an ounce of clear cerebrospinal fluid was with-
drawn by lumbar puncture, but this failed to present abnormal features.
No cellular elements were found. The temperature showed slight
elevation on admission and this persisted for two weeks. The average
was from 99° to 100°. It then gradually dropped to the normal
line. The pulse ran from 100 to 120, and the respiratory rate from
25 to 30.

A few days after admission the patient became a little brighter, and
a week after admission the paresis of the external recti was less
marked. There was at this time also only an occasional incontinence
of urine and no longer any tremor. The boy continued to grow
brighter, and on November 22 the ocular palsy was much improved.
The tongue at this time was still slightly deviated to the left. The
ataxia persisted and the deep reflexes remained absent. His condi-
tion continued practically unchanged tmtil his temperature again
rose, in the first week in December, when an uneventful attack of
typhoid fever set in, and lasted a little over three weeks.

An analysis of the case quickly shows that we were dealing with
motor symptoms confined to the functions of three different cranial
nerves ; the twelfth on the left side, both sixths, and possibly the left

427

6 JUDSON AND CARNCROSS: INFERIOR POLIO-ENCEPHALITIS

seventh (the latter was extremely slight). The involvement of the
sixth was very much more marked on the right side, while the twelfth
was affected on the left. Other motor involvement there was none,
and the only additional symptoms to help localize were the ataxia and
loss of deep reflexes.

A single circumscribed lesion would not account for the combina-
tion of symptoms ; for the sixth nucleus was much more markedly
involved on the right side, while the involvement of the twelfth nerve
nucleus was on the left. We were, without doubt, dealing with a
pathological condition in the lower part of the pons and the upper
part of the medulla : it would be almost impossible to account for the
symptoms by pressure from elsewhere, such as a tumor of the cerebel-
lum, since that could hardly press upon the hypoglossal and facial with
more marked pressure on the sixth of the opposite side, and this with-
out affecting the pyramidal tracts. As the child failed to present other
usual symptoms of cerebellar tumor, such as optic neuritis and vomit-
ing, that diagnosis was excluded. His subsequent complete recovery
confirmed this opinion. There was no evidence of meningitis at any
time.

We were then obliged, by the combination of the paresis of these
few cranial nerves and the cerebellar ataxia, to locate the disease in
the pons and medulla. There was no history of syphilis or tubercu-
losis, and the boy had been a particularly healthy child. A thrombosis
or embolism could not be accounted for, as there was no cardiac or
arterial disease. The child showed no evidence of syphilis. Further-
more we could not assume that there was a hemorrhage into the pons
caused by the violent straining of a whooping-cough. Also, as the
condition did not come on suddenly, a hemorrhage was extremely
unlikely. Therefore, we were forced to think of polioencephalitis, for
there was irregular involvement of a few motor cranial nerves whose
nuclei are closely related by position in the lower pons and upper
medulla; and the derangement of functions did not extend to the
ventral portion of the pons, but left the pyramidal tracts apparently
uninvolved.

The course of the disease, with mild febrile disturbance and the
marked mental hebetude, which at one time after the boy's admission
to the hospital approached stupor, confirmed the diagnosis of polio-
encephalitis. The disorder also developed at the end of an epidemic

428

JUDSON AND CARNCROSS: INFERIOR POLIO-ENCEPH ALITl^, 4

of poliomyelitis, which occurred in Philadelphia and in other parts
of Pennsylvania in the autumn of 1907.

The modified views of Striimpell^ (whose earlier conception of
encephalitis as a process involving the cortical area alone, was not
concurred in) have been confirmed and it is now an accepted fact
that a certain number of cases of cerebral palsy are due to acute
non-suppurative encephalitis. The presence of this morbid state has
been demonstrated by the pathological findings of Ganghofner, Sachs,
and Fischl at least : and the simultaneous appearance of such foci of
disease in the brain with the lesions of acute anterior poliomyelitis
has been observed by Redlich and others. A number of cases of
acute anterior poliomyelitis in the adult accompanied by inflammation
in the medulla, pons, crura, cerebral ganglia, or cortex have been
studied pathologically. Such was a case in an adult reported by Sher-
man and Spiller- in 1900.^ As a result of his studies Spiller concludes
that " poliomyelitis is closely related pathologically to the non-purulent
form of encephalitis, and to the polio-encephalitis superior of Wer-
nicke," and that " poliomyelitis in the adult is essentially the same
disease as poliomyelitis in the child."

Any part of the brain can be the seat or point or origin of this
trouble. After referring to the frequency with which the central
ganglia alone are affected, Oppenheim^ concludes that the gray matter
in the wall of the third ventricles and the aqueduct of Sylvius is the
seats of predilection, whence the disease may descend to the spinal
cord, that involvement of the cerebellum is less frequent, that the
process may extend to the optic nerves and retina, that as a rule
there are several foci, and, that although the gray matter is principally
involved, the disease may extend in the neighboring white matter.

In this connection it is well to call attention to the fact that the
pathological findings in anterior poliomyelitis have shown that the
inflammatory changes are really a myelitis principally limited to the
gray matter of the cord, particularly the anterior horns, but that the
other adjacent portions of the white matter, or even the membranes,
do not entirely escape. That the cause acts through and upon the
bloodvessels and does not pick out the anterior horn cells and leave the
surrounding tissue unafifected. has been established in spite of the
fact that the clinical manifestations point to the anterior horn cells

^ Practice, thirteenth edition.

" Phila. Med. Jour., November. 19CO.

' Oppenheim in Nothnagel's System.

429

5 JUDSON AXD CARXCROSS: INFERIOR POLIO-ENCEPHALITIS

alone. Oppenheim also says : " It is not infrequent to find a single
circumscribed process within the pons and medulla, but the size and
extent of any focus may vary in wide limits." We think that our case
gave evidence of the presence of inflammatory changes in the lower
part of the pons, creeping slightly into the medulla on the left side
(judging from the clinical signs), and that the lesion was due to an
encephalitis. Polio-encephalitis may run an acute or subacute course,
and, ahhough in severe types it may end in death within two or three
weeks, it is capable of ending in complete or partial recovery and this
in cases of more extensive involvement than ours. Comby"* and ^ledin
report cases which recovered without any remaining paralysis. Abt,^
of Chicago, reports two recoveries with residual paralysis. Frederick
Taylor," of London, records a case of encephalitis which recovered
after a prolonged period of ataxia lasting over three years.

Our case, which was rather mild, ran six weeks (the rise of tem-
perature lasting only two) with decided improvement, when the tem-
perature again rose and a mild attack of typhoid fever set in, during
the course of which there were no new nervous manifestations. Upon
complete recovery from the typhoid the ataxia had entirely disap-
peared, as well as the cranial nerve palsies. The last symptom to dis-
appear was the absence of the tendon reflexes, but by February 1908,
the knee-jerks, although somewhat weak, were unmistakably present.

Pathological study of the lesion shows that there is cellular infiltra-
tion, which may or may not be intense, and is particularly marked
along the vessels and in the perivascular sheaths. The vessels are
engorged and there are frequent hemorrhages. The cellular infiltra-
tion may be marked in the nuclei. The nerve cells and neurogliar
tissue may be. in the beginning, swollen, or the nerve cells may be
shrivelled, with a disappearance of their dendritic processes, and lack
of distinct nuclei. They may ultimately disappear. The essential
pathological process in encephalitis is the breaking down of the
nervous elements, and extensive granular degeneration. From a num-
ber of observations in which the complete function of the nerves was
restored, it seems that the lesions produced by encephalitis may be
completely absorbed with a restoration to the normal condition, or
there may be local necroses or softening, or the focus may be obliter-
ated by connective-tissue formation.

* Archives de medecine des enfants, 1907.
° Archives of Pediatrics, May, 1907.
" Lancet, 1904, ii.

430

JUDSON AND CARNCROSS: INFERIOR POLIO-ENCEPHALITIS 6

When we come to infer the pathology of a case such as ours (of
the inferior type) it is well to remember that the inflammation may
extend farther than the clinical signs indicate.

It is difficult in a short space to give any summary of the symp-
toms of this disease, because they are so varied in their combinations.
The process may begin above, starting, for instance, with the oculo-
motor region, and go down, finally causing atonic paralysis of the
extremities ; or, on the contrary, it may begin below and travel upward,
presenting the course of Landry's paralysis. In typical cases only
motor functions are involved. There may be different combinations,
more or less symmetrical, of paralysis of the cranial and motor spinal
nerves. When the cranial nerves are involved, there may be ophthal-
moplegia and glossopharyngeal-labial paralysis. Thus the signs of
bulbar disease may be difficult articulation, swallowing, or breathing.
A hemiplegia may accompany the other symptoms. The disease in its
acute manifestations may be initiated by headache, vertigo, nausea and
vomiting, fever, general weakness, and more or less stupor. Its onset,
though often very rapid, is, on the other hand, not apt to be as sudden
as in hemorrhage, and the fact that it may develop through days or
weeks is a diagnostic point in favor of encephalitis against hemor-
rhage. Stupor may deepen to loss of consciousness, with restlessness
and delirium, and there may be general convulsions and retraction of
the head. Paralyses occur early, but may not at first be apparent.
Not infrequently the pulse is slow, but the respiration is apt to be
rapid.

The combination of palsies in polio-encephalitis inferior is very
variable, and a search of the literature has revealed a very limited
number of cases resembling the one here reported. In a case of
Leyden's, a boy, aged fifteen years, there was difficulty in swallowing,
rigidity of the neck, ataxia. paralysis of both facials, of the hypoglossals,
and of the soft palate and vocal cords ; later also paralysis of the
sixth nerves. The disease lasted eleven days, and the autopsy showed
an encephalitis in the region of the medulla.

Dinkier" (from Erb's clinic) reports a case of acute inferior hemor-
rhagic polio-encephalitis of wide extent. A healthy child, aged two
and a quarter years, fell down a flight of stone steps, receiving a
slight scalp wound, and was unconscious a short period without vomit-
ing or convulsions. After this he was altered psychically, had head-

' Deut. Ztschr. f. Nervenheilkunde, 1895, vii.

43i

7 JUDSON AND CARXCROSS: INFERIOR POLIO-ENCEPHALITIS

ache, vertigo, enuresis, occasional vomiting, and a very staggering gait.
These symptoms lasted two and one-half years, when, after a vomit-
ing attacic, speech became affected, chewing and swallowing difficult,
and great restlessness set in. The child died, after two feverish days,
in deep coma. The gray substance of the medulla, the posterior horns
of the cervical cord, and the floor of the fourth ventricle showed recent
bloody extravasations with changes and rupture of the bloodvessel
walls. _ Although the last eight pairs of cranial nerves were thus
surrounded the author says they suffered practically no destruction,
because death occurred from intracranial pressure before there was
time for this to take place.

The following is a case of Batten's^ : A child of five years was taken
ill with fever, and two days later suddenly developed a right-sided
facial palsy, with difficulty in swallowing due to weakness of the
right side of the palate. There was no paralysis of limbs or eve
muscles. Vomiting was present, but there was no loss of conscious-
ness. Death occurred three days later from respiratory failure.
There was complete destruction of the right seventh nucleus, in the
region of which there were hemorrhages, thrombosis of smaller
vessels, and round-cell infiltration. The engorgement of the vessels
produced apparently little change in the left seventh or either of the
sixth nuclei. There was also considerable vascular engorgement with
exudation of round cells in the medulla in the region of the ninth,
tenth, eleventh, and twelfth nuclei, without destruction of these.
There was perivascular exudation in the gray matter of the upper cord.
Batten remarks that the lesion was of vascular origin and exactly
corresponded in appearance with that found in an acute anterior
poliomyelitis and in acute polio-encephalitis superior. He alludes to
the fact that the former disease occurred frequently during the pre-
ceding August, and considers this case to be of the same nature.

The cause of polio-encephalitis, excluding alcoholism, which is re-
sponsible so frequently for the Wernicke type of the disease, is mainly
the infections and especially influenza. But such an infection was
absent in our case. We are in the dark, however, as to the specific
cause of anterior poliomyelitis, which so often occurs in epidemics,
and the case herewith reported appeared at the end of a severe epi-
demic of poliomyelitis. It seems hardly justifiable to regard the fall
that the child sustained, five weeks before his entrance to the hospital,

^Lancet, October and December, 1902.

432

JUDSON AND CARNCROSS: INFERIOR POLIO-KNCEPH ALITIS 8

as an etiological factor. It was only on the pavement while he was
playing- in the street, and there were no symptoms of concussion ; he
never became unconscious and was unchanged after this tumble until
the disease began two weeks later. He had been in the hospital four
weeks when his typhoid developed, so that no claim can be made of
any causal relation between the two affections. The total duration of
the disease was about three months, since there was no ataxia nor
any trace of the palsies upon his return, at the end of January, from
the country, where he had been sent for a fortnight's convalescence.

We think this case is of interest because it forges one more link
in the chain that unites poliomyelitis and non-suppurative encephalitis,
because it shows the inferior type of polio-encephalitis, because it
illustrates that this disease may run a comparatively mild course to
complete recovery, and from the fact that it followed an epidemic of
poliomyelitis.

48.".

Reprinted from the New York Medical Journal for December 26, 1908.
Copyright, 1908, by A. R. Elliott Publishing Company.

HYDROCEPHALUS
By S. D. Ludlum, M.D.,

PHILADELPHIA
INSTRUCTOR IX NEUROLOGY AND NEUROPATHOLOGY, UNIVERSITY OF PENNSYLVANIA

From the Aycr Clinical Laboratory, Pennsylvania Hospital, and from the

Department of Neurology and the Laboratory of Neuropathology

of the University of Pennsylvania

The various writers upon hydrocephalus do not agree upon many
points, and therefore the subject is still open for discussion. This
paper records two cases of internal hydrocephalus in which were defi-
nite lesions causing the condition, both in the aqueduct of Sylvius, one
following tuberculous meningitis, and the other the edipemic form of
meningitis. By means of the foramen of Magendie and the two lateral
apertures in the fourth ventricle, the system of ventricular cavities and
the central canal of the spinal cord are brought into communication
with the subarachnoid lymph space. The fourth ventricle drains the
third by the aqueduct of Sylvius, and the third communicates with the
lateral ventricles by the foramina of Monro. A path is thus provided
by which the cerebrospinal fluid secreted within the ventricles by the
various choroid plexuses constantly escapes, and thereby prevents
undue accumulation and distension within the cavities of the brain and
spinal cord. Any one of these passages is open to infective processes.

Bramwell reports the closure of the foramen of Magendie from
meningitis with consequent hydrocephalus. Neurath (Nciirologisches
Centralblatt, 1896, p. 87) also reports a closure of the foramen of
Magendie. There does not seem to be any report of search into the
condition of the lateral apertures flanking the foramen of Magendie.
But there are a few cases reported of involvement of the aqueduct of
Sylvius. Dixly (Nciirologisches Ccntralhlatt, 1899, p. 977) has
studied internal hydrocephalus in the horse, and thinks it is due to
occlusion of that passage. Bourneville and Novy {Le Progrcs med-
ical, 1900, July 14) report chronic hydrocephalus in a child, with the
aqueduct of Sylvius completely obliterated. Jouche (Bulletins et
1 434

LUDLUM : HYDROCEPHALUS 2

mcinoircs de la Socictc mcdicalc dcs hdpitcaii.v dc Paris, 1902, No. 7,
p. 141) has reported a case of hydrocephalus with the aqueduct of
Sylvius obliterated. Spiller (American Journal of the Medical Sci-
ences, July, 1902) has put on record a case of internal hydrocephalus
resulting from closure of the aqueduct of Sylvius by proliferation of
the neuroglia. Spiller and Allen (Journal of the American Medical
Association, April 13, 1907) report a case of partial occlusion of the
aqueduct, probably due to congenital malformation.^ It is not in any
way a new observation, but the cases accurately reported are about as
stated. Hydrocephalus due to occlusion of one or both foramina of
Monro has been reported by W. C. White (Journal of Insanity, Iviii,
No. 3), unilateral; and by Spiller, Unilateral Hydrocephalus Due to
Partial Closure of the Right Foramen of Monro, in The American
Journal of the Medical Sciences, July, 1902. Most of these cases were
examples of chronic hydrocephalus. Both of the cases appended in the
article were acute secondary closures of the aqueduct of Sylvius fol-
lowing meningitis.

Quincke describes an idiopathic internal hydrocephalus as an epen-
dymitis causing a serous effusion and pressure effects, which might be
compared to the serous exudates in the pleura or synovial membranes.
It is hardly an inflammatory process, and Quincke likens it to an
angeioneurotic oedema. This is termed an ependymitis, but in acute
cases the ependyma is smooth and natural looking, and in chronic cases
is thick and sodden. In most of the cases the chorioid plexus is en-
larged and congested.

Among 1,180 insane patients at the Norristown State Institution,
Pennsylvania, there were ^^J cases with dilatation of lateral ventricles ;
the majority of these showed swelling and cystic involvement of the
chorioid plexus, but not nearly such a large proportion have noticeable
involvement of the ependyma. The cases were of the ordinary forms
of insanity with these conditions found at necropsy.

The inference is that the increase of fluid is due to the chorioid
plexus, that it is blocked, and more than a normal quantity of fluid
secreted. Hvaline bodies forming among vessels would do this, and
they are commonly reported. Burr and McCarthy, in a paper in The
Journal of Experimental Medicine, 1899, from the result of experi-

- In Obersteiner's Festschrift Dr. Spiller recorded another case of occlusion
in the aqueduct of Sylvius in a paper on paralysis of associated upward move-
ments, and has mentioned to me the findings in another case in which the occlu-
sion was only partial and did not cause hydrocephalus.

435

O LUDLUM : HYDROCEPHALUS

mental toxic injections, show that the condition of the ependyma in a
case of acute hydrocephalus is similar to a toxic condition of the ven-
tricular fluid. This fact also being in favor of the chorioid plexus
being the important factor of the disorder, and not an ependymitis.
Engorgement of the choroid plexus would shut up the foramina of
Monro and this could cause lateral hydrocephalus. The inflammatory
condition of the plexus with its ensuing exudate could furnish the fluid,
or, as' has been said, a retardation of blood flow by hyaline bodies in
the vessels causing increased transudate ; and the thickened condition
of the ependyma be due to pressure of fluid or the retention of the fluid
becoming more and more toxic.

The characteristic composition of the cerebrospinal fluid — viz., a low
percentage of albumin and a high percentage of potassium salts —
shows that it is not an ordinary transudate, but a secretory product
from certain ceUs, probably those of the chorioid plexuses. (Falken-
heim and Neuryn, Archiv fiir experimentcUe Pathologic, xxii, p. 269.)
The resorption of this fluid takes place mainly in the Pacchionian
bodies and to a lesser extent in the neighboring lymphatics. A men-
ingitis would impede this resorption, production increased and absorp-
tion diminished. So the primary change in Quincke's type may be in
the chorioid plexus, with secondary action upon the ependyma and in
the acute meningitic forms due to closure of one or more foramina.

Alcoholism on the part of parents is supposed to play a part in
hereditary hydrocephalus. Traumatism after birth seems to have pro-
duced certain cases. But all cases would seem to centre upon patency
of openings and amount of secretion from the chorioid. External
hydrocephalus can usually be explained by atrophy of the brain sub-
stance, met with in old age, after haemorrhage, softenings, or sclerosis
in lingering or cachectic diseases. Hydrocephalus due to brain
growths is quite obvious in certain cases and needs no comment.

Case i. — Dr. Henry Hutchinson's Case: The patient was taken to the
Pennsylvania Hospital with fever, there developed stiffness of the neck, Kernig's
sign; with this there were absent knee jerks and abolished Achilles reflexes.
Pupils were equal and reacting sluggishly to light. Lumbar puncture showed
in smears the diplococcus intracellularis of Weichselbaum. Leucocyte count was
19,000. Lumbar puncture at two successive times showed an improvement as the
case progressed. Then the ears began suppurating, the condition of the patient
became worse, and eventually death ensued at the end of seven weeks of
illness.

A. (871), C. R. Autopsy was performed by Dr. Robinson on June nth.
Anatomical diagnosis. Subacute purulent cerebrospinal leptomeningitis ; hydro-

436

LUDLUM : HYDROCEPHALUS 4

cephalus, purulent otitis media; bronchopneumonia, congestion of lungs; cloudy
swelling of liver; swelling of mesenteric lymph nodes.

Body was much emaciated; 169 cm. in length; pupils equal, slightly dilated;
discharge of purulent material from each ear.

Brain: On removing the skull cap, the dura appeared injected and a little
thickened, the convolutions flattened and the vessels of the pia injected. The
tip of the left temporal lobe was swollen and had a cystic feel. All about the
base of the brain was seen yellow pus, especially between the lobes of the
cerebellum. The pia was everywhere thickened. The floor of the third ven-
tricle behind the optic chiasm was purplish blue in color and bulged. On
separating the cerebellum from the cerebral hemispheres a considerable quantity
of clear watery fluid flowed out, in all about 150 c.c. The brain was put into
formalin.

After hardening in formalin, a horizontal cut made through the lateral ven-
tricles showed the latter considerably dilated, especially in the posterior and
lateral horns. The choroid plexuses presented a curious appearance. At the
point where each plexus curved downward into the descending horn of the
ventricle, bands of inflammatory tissue stretched on all sides from the plexus
into the walls of the ventricle. In addition, there was in the left plexus a firm,
tumorlike mass, i cm. in diameter. The temporal lobes were cut into from
below; the wall here was very thin, measuring i to 2 cm. in thickness, and the
descending horn of the ventricle was seen to be much dilated. The chorioid
plexus, seen through the incision, presented the same appearance as that
described from above. The left descending horn was somewhat more dilated
than the right. At , the tip of the left temporal lobe there was a small area of
softening, i cm. in diameter, situated just beneath the gray matter.

Spinal Cord: Dura was somewhat thickened, tough, and opaque. On opening
it there was seen beneath the pia, at various places, small masses of rather dry,
thick, yellow, purulent material almost caseous in consistence. The vessels of
the pia were injected. The spinal cord was well preserved, and was quite firm
in consistence. No enlargemnt of the central canal was seen.

On opening the middle ears, both were found to contain a considerable
quantity of rather thick yellow, purulent material. There was no perforation
of the tegmen tympani.

Grossly, there was found a cyst of the left temporal lobe. Both ventricles
were dilated. There was a tumor in the left chorioid plexus, and attention is
drawn to the fact that on cutting through the peduncles in separating the cerebel-
lum from the cerebral hemispheres there was an escape of 150 c. c. of fluid.
The fact is explained by the infiltration of the Sylvian aqueduct by inflammatory
tissue causing hydrocephalus.

Abscess of the temporal lobe occurs in ninety per cent, of the cases of
chronic otitis media ; it may be encapsulated or surrounded by softened brain
tissue; it may be multiple or single. The abscess in this case came directly from
the meningitis and the ear suppuration following.

Microscopical Examination: Sections through the wall of the lateral ven-
tricles showed marked signs of inflammation ; the subependymal tissue was in-
filtrated with round cells, densely so in the neighborhood of bloodvessels, and

437

O LUDLUM : H\T)ROCEPHALUS

there was very evident proliferation of neuroglia tissue. In a few places the
ependymal epithelium was absent. The choroid plexus was the seat of intense
inflammation. Its vessels were greatly distended with blood, and the whole
tissue was infiltrated with small round cells, chiefly of mononuclear type.

Tumor: The tumor, which was described at autopsy, was put into decalcify-
ing fluid and showed the following : The tissue appeared to be identical with
that of the choroid. Very large numbers of concentrically marked amyloid
bodies were present, especially at the periphery. The centre of the tumor con-
sisted principally of loose, reticulated tissue, not unlike lung tissue in appear-
ance ; the spaces in the reticulum were for the most part empty, though a few
contained a translucent, homogeneous staining substance. A considerable area
of the tumor was occupied by the remains of calcified substance. The blood
vessels were greatly distended with blood, and the whole tumor was surrounded
by densely packed round cells. Sections through the cortex cerebri of the
temporal lobe, in the region of the abscess, showed the pia acutely inflamed,
with its vessels congested, and its substance thickly penetrated with round cells.
The brain substance where it formed the wall of the abscess was also densely
infiltrated ; and between the abscess and the surface of the gray matter, the blood
vessels were everywhere surrounded by masses of round cells. Near the abscess,
haemorrhages into the brain tissue were seen.

The cranial nerves were examined for degenerations by the Marchi and by
the Weigert methods. The optic nerves showed a few degenerated fibres, seen
by the Marchi method. The oculomotor nerves each showed a fair number of
black stained fibres by this method. Degenerations were also present in both
of the facial nerves. Examined by hjematoxylin and cosin, the second, third,
and seventh nerves were seen to be surrounded by inflammatory tissue, the in-
flammation involving the connective tissue trabeculae within the nerves them-
selves. The condition was most marked in the case of the seventh nerve.

It is very probable, judging from the condition of the chorioid plexus and
from the fact that there were such marked bands of adhesions stretched from
these across to the wall of the lateral ventricle at the beginning of the descend-
ing horn, that the flow of cerebrospinal fluid accumulated and caused dilatation
of the descending horns. The aqueduct lesion was in all probability caused by
extension of the infectious process from the abscess in the temporal lobe. On its
extension the process must have first affected the chorioid plexus; and these
structures then underwent changes described, with possible dilatation of the
descending horns of the lateral ventricles, and adhesions. Next, the third
ventricle and the aqueduct of Sylvius were involved, and when the process of
inflammation had succeeded in closing up completely the aqueduct, the hydro-
cephalus came to involve not only the descending horns but also the rest of the
lateral ventricles and the third ventricle. That the process must have been
chronic is shown by the presence in the exudate in the aqueduct, of young con-
nective tissue.

Spinal Cord, lumbar region: The pia is markedly infiltrated with round cells,
and shows distended vessels filled with blood. In the white matter at the
periphery of the cord there was seen some absorption of nerve fibers. Some

438

LUDLUM : HYDROCEPHALUS 6

scattered recent degenerations were present in the posterior columns in Marchi
sections. All the tracts of the white matter stained well by the Weigert method.

Cervical region of cord: The pia showed the same marked infiltration, and
engorgement of its vessels with blood. Marchi sections showed degenerated
fibers in the posterior columns and in the crossed pyramidal tracts, as well as in
the anterior roots. Sections stained normally by the Weigert method.

Pons: Sections through the upper part of the pons showed the lumen of the
fourth ventricle where this passes into the aqueduct of Sylvius, completely closed
up by a mass of round cells. In a few places the normal lining epithelium of
the wall was present, in one or two areas it appeared to be in process of proli-
feration. For the most part, however, the epithelium was absent, the round cells
of the exudate fading into the surrounding tissues. These round cells were for
the most part mononuclear in type ; many had pale, vesicular, large nuclei. The
blood vessels in the surrounding brain tissue were engorged with blood, and
were surrounded by many round cells.

In one of the corpora quadrigemina there was seen a small area of soften-
ing with some absorption of tissue; the walls of the cavity so formed consisted
of necrotic tissue.

Following the case of Dr. Hutchinson I have had a case to examine
from the service of Dr. Lloyd, at the Philadelphia Hospital, which has
been similar in showing a blockage of the aqueduct of Sylvius.

Case II. — A man, colored, aged thirty years, came in a stuporous condition ;
he showed symptoms of meningitis. He died in a few days, and the brain
showed meningitis and some hydrocephalus. This hydrocephalus was caused by
blocking up of the aqueduct of Sylvius with a round celled infiltration extending
in as far as the middle of the superior colliculus.

Sections showed portions distended with round cells and at some points the
canal had broken open and the nervous tissue infiltrated with inflammatory
material. This blockage had caused a moderate distention of the ventricles.
There was some infiltration of the walls of the ventricles, but of a slight grade.
The chief focus in the ventricles seemed to be at the entrance to the aqueduct
and extending into it. There was a marked tuberculous meningitis, especially
at the base of the brain. This process was apparently secondary to tuberculosis
of the other parts of the body, for the autopsy disclosed tuberculosis of the
lungs, liver, spleen, kidneys, and peritonaeum.

There was no change in the ependyma or chorioid, of the third and lateral
ventricles, but there were inflammatory changes in the floor of the fourth
ventricle.

It would seem that the source of infection was via the foramen of Magendie,
and hence into the aqueduct.

439

University of Pennsylvania.
Contributions from the Dept. of
Neurology and the Laboratory of
Neuropathology.

University of Toronto
Library

Acme Library Card Pocket
LOWE-MARTIN CO. limited

'V'^'

Internet Archive Audio

Featured

Top

Images

Featured

Top

Software

Featured

Top

Books

Featured

Top

Video

Featured

Top

Mobile Apps

Browser Extensions

Archive-It Subscription

Save Page Now

Full text of "Contributions from the Department of Neurology and the Laboratory of Neuropathology (reprints)"

See other formats