Lectures on diseases of the spinal cord : Containing two hundred and forty-four woodcuts

Google

This is a digital copy of a book that was preserved for generations on library shelves before it was carefully scanned by Google as part of a project
to make the world’s books discoverable online.

It has survived long enough for the copyright to expire and the book to enter the public domain. A public domain book is one that was never subject
to copyright or whose legal copyright term has expired. Whether a book is in the public domain may vary country to country. Public domain books
are our gateways to the past, representing a wealth of history, culture and knowledge that’s often difficult to discover.

Marks, notations and other marginalia present in the original volume will appear in this file - a reminder of this book’s long journey from the
publisher to a library and finally to you.

Usage guidelines

Google is proud to partner with libraries to digitize public domain materials and make them widely accessible. Public domain books belong to the
public and we are merely their custodians. Nevertheless, this work is expensive, so in order to keep providing this resource, we have taken steps to
prevent abuse by commercial parties, including placing technical restrictions on automated querying.

We also ask that you:

+ Make non-commercial use of the files We designed Google Book Search for use by individuals, and we request that you use these files for
personal, non-commercial purposes.

+ Refrain from automated querying Do not send automated queries of any sort to Google’s system: If you are conducting research on machine
translation, optical character recognition or other areas where access to a large amount of text is helpful, please contact us. We encourage the
use of public domain materials for these purposes and may be able to help.

+ Maintain attribution The Google “watermark” you see on each file is essential for informing people about this project and helping them find
additional materials through Google Book Search. Please do not remove it.

+ Keep it legal Whatever your use, remember that you are responsible for ensuring that what you are doing is legal. Do not assume that just
because we believe a book is in the public domain for users in the United States, that the work is also in the public domain for users in other
countries. Whether a book is still in copyright varies from country to country, and we can’t offer guidance on whether any specific use of
any specific book is allowed. Please do not assume that a book’s appearance in Google Book Search means it can be used in any manner
anywhere in the world, Copyright infringement liability can be quite severe.

About Google Book Search

Google’s mission is to organize the world’s information and to make it universally accessible and useful. Google Book Search helps readers
discover the world’s books while helping authors and publishers reach new audiences. You can search through the full text of this book on the web
ai[http: //books . google. com/|

2 45 O41 4780

THE NEW SYDENHAM SOCIETY.

VOLUME CLII.

Ixstitcten MDCCCLVIII.

vi AUTHOR'S PREFACE.

Pathological anatomy is still a somewhat uncertain guide
as regards numerous points. It should be chiefly based upon a
knowledge of the anatomy of the part in a normal condition,
and on that account I have thought it right to describe in these
lectures the principal facts connected with the anatomy of the
spinal cord. These are discussed at some length, and I trust:
that I have made them more or less clear by the use of numerous
figures, either diagrammatic or from nature. Many of these
were obtained from collections in the Hospital of the Salpétriére.
and used in these lectures with the full permission of my
master, Professor Charcot, to whom I offer once more my
sincere thanks.

Paris, 1892. PIERRE MARIE.

LECTURE V.

AscznvixG Daaeenation Arren TaaxsvyEense Lesions oF THE
Srivan Conn,

A. In the rosrerron conv: « In tho column of Burdach. A, In the column

ascending tract of English anthors), ite sout, form, origin, path, termina-
tion, and degeneration. ©. In the OkEY SUBSTANCE OF THE CORD,

LECTURE VI.

Deoevenation or Tux Neeyxs anp Srixat Corp
APTER AMPUTATION OF A Lime,

Such degeneration seems at first sight opposed bo the doctrines of physiology,

At the sume time changes of this kind are frequent. They have been
shown to oceur by numerons observers, Works of Bérard, Valpian,
Dickinson, Hayem, Dojerine and Mayor, Hayem and Gilbert, Fried~
Inder and Krause, &o. Changos in the central extremity of nerves aftor
amputation of a limb ; frequent increase in size (does not always occur),
increased diamoter of the fasciculi constituting the nerve, Considerable
diminution, in a transverse section, of the fibres containing myelin,
Salets of degeneration not to bo confused with the primary islets, being
im roulity the trnceu of previously existing nerve fibres. ‘Tho islota of
degeneration are formed of a collection of nerve-fibres composed of a
email axis cylinder, and fine primitive sheath, with or without myelin.—
Evolution of those islets, disporsion of tho nervo fibrila, myxoid axpeet of
tho partitions separating the different primary islets from each other,
slight thickening of tho fibrous bundlon placed betwoon tho fasciouli
of the nerve, Uncertainty with regurd to the condition of the sengtia
connected with the nerve roots, and the nerve roots themselves 0h

b

x CONTENTS.
PAGE
LECTURE X,
IxsubAm ScuEnosis.
History: Cruveilhior, , Charcot and Vulpian, Ordenstein,

rapidity of ite rhythm, specially affects the muscles at the root of the
limbs, but often also these of the trunk and meek, and may predominate
tupon one wide of the DOAY ss: csrienrseessresseesnssonsevancsnnver verses versmansnnmennnne 108
LECTURE XI,
Insotam Scienosis: Sravtoms.

B, Seesory symploms.—Disorders of common sensibility (Frosnd) aro rare,
and bnt slightly pronounced ; they vary in form from tingling to hemi-
anwethesia, Disorders of «pecial sensibility; tho hearing, taste and
smell are little and rarcly affected, Disorders of sight of great impor-
tance: works of Gnauck, Parinand and Ubthoff. Nystagmus and nystag-
moid movements; precantions necessary in them. of
‘the muscles of the orbit, assovinted paralyets (Parinacd), Disorders of
the optic disc, myoats associated with excess of iris reflex. Changes in
‘the optio diso, disorders of sight which result from them, changos in the
field of vision. Variable onset of visual disorders, absence of symmetry
in them. C_ Visceral symptoms connected with the stomach, rectum,
bladder, and gomital organ» (Oppenheim) ; these are rare, and but Little
pronounced, D, Trophic disorders, specially amyotrophic, 1L Bunnam

the q

of xpeceh, the articulation is slow, monotonous, scanning, spasinodic.
Vertigo : intellectual disorders, impulsive laughter, apoplectiform,
leptiform attacks ..... es sesasse sassecceneansse DEL

LECTURE XU.

IxsuLaR Scranosts (continued).
Course. Disonosts. AiTIoLGy,

Couns: Onset acute, or gradual and progressive. The course of insular
sclerosis may follow different types : a chronic progressive type, a chronic
type wiih sndden exacerbations, a remittent chronic type, permanent
improvement, and even recovery. Variable duration. Abortive forms,

canse ix infection. Narration of facts, different infections diseases daring
Be ee ee ey Explanations of ca
Faotreneesn erenienroarversanreses

LECTURE XVI.

‘TAnES.—Syaetoms (continued),

C, Disonveks OF kerLEX actION. Patellar tendon reflex, Procwutions to
aril lg rar tba position to be given to the thigh;
percnssion of the ‘the muscalar contraction

‘Tases.—Srmrtoms (continued),

D. Onoans conneotep witu tix sexens.—1, Visual organs. 1. Thove which
‘are externat to the globe of the eye. Paralysia of tho external muscles of the
eyeball, ptosis, ahedding of tears, epiphora, exophthalmos, narrowness of
‘the palpebral opening, diminution of oonlar tone. II, Internal organs of
the eye. Condition of the pupils, inequality, myosis, mydriasia, deformity.
Reflex actions of tho iris; ealeeidaen sight ce nemaicuaGt ae
ok Asal ota eae eRe Optic nerve; optic neuritis; its
onset, symptome,‘and characters. 2. Auditory organs, Diminution in the
power of hearing ; deafness, ite characters ; Méniére’s disease; excessive
ebisetly ft Bhp paithey nerve ly electels emerenta. is Oise eee
Anosmia, 4. Gustatory organs, Loss of taste

LECTURE XVII
‘Tauxs.—Syacrroms (continued).

KE. Trormic prsorpens.—e. Disorders of the general nutrition, 6, Spontaneous
froctures: nature of these fractures; absence of pain, tendency to con-
solidation, tendenoy to forma large quantity of callus: special mtiological
conditions; the slightest injury may produce thom, seat of these fractures,
fmcturen of the apophyser, fractures of the vertebre, their nature.
Lesions of the bones: porosity, thinning of the compact substance,
dilutation of the medullary cavity, dilatation of the Haversian canals,
decaloification of the lamellw of bono, changes in the osteophytes,
embryonic transformation of the marrow in the bones, dimination of the
non-organic, increaso of tho + organia mutorial. Nature of these alter-
ALLO sree.

LECTURE XIX.
‘TAums.—Syarrome (continual).

FB. Arrumorariy 1x TAnK.—ZJistory :_“ Charcot’s joint disease "opposite
opinion of Votkmann. Onset more or lexe sudden: xwolling, doughy
condition, absence of trae adema; cropitation ; absence of pain, Courae
of the affeotion ; mild and severe form, State of the articulation when tho
arthropathy has existed for a cortain time; flattening of the part, abnor-

of
modulla oblongata. TV.—Usmary syerem, A.—Derengements of the
urinary secretion: glycosuria; changes in the amount of urea,

thoaia of the testis, B.—Derangement in the fenale? genital deprossion
genital oxcitement; pain in the genital organs; vulvo-vaginal criso#....- a7

LECTURE XXIV.
Tangs.—Srarroms (continued ),

VI. Cxngnrat srerext,—Hemiplogia. Apoplectiform attacks, Epileptiform
attacks. Acute symptoms connected with the medulla oblongata,
Prychical derangements. Caincidence of general paralysis of the insane.
Course or Tanes.—Prodromal period. Second period, period of inco-
ordination, Third period, confinement to bed. Forms of tabos: bait
cervical, cerebral, mild, and severe forins, .+.ssesesseonesenseneansacsnancenennrse 21

LECTURE XXV.
‘Tanrs.—HT10L0Gy,

Br101007.—Common cavaes: Wet and cold; Diathesea: arthritic, herpetic;
sexual excess; injury. ‘The true wtiological element of tabs is ayphitise
‘The discovery of this fact is due to Fournier (1876). Erb (1879) adopts and
defends this opinion. Statistios published by difforont authors as to the
percentage of tabid patients known to be affected by syphilis. Recent
statistics of Erb including 369 cases giving 89 por cont, of syphili« in the
wtiology of tabes, Nine-tenths at least of tabid patients suffer therefore
from syphilis—Increased number of adversarios to this opinion.
Rofutation of their arguments.—Infnonce of hereditary pre-disporition to
nervous diseases shown by Charcot, Doubtful effect of hereditary syphilis,
Age: greatest. froquency of onact between 90 and 45 yours. Race,
Profession; most often ocours in the liberal professions ; ite Tee Se
in the clerical profession, wens ceeene 298

LECTURE XXV1.

‘Tasus.—D120No8re

“DraoNosrs: Difficulty in making a complete and methodical dingnosis.
Differential character aa rogards: Cerebellar affections, Insular sclerosis,
Asasia-aharia, Syringo-myelia; ax regards Pscudo-tabes; A, Toxic Peoudow

b

LECTURE XXXV_
INFANTILE PaRauyets (continued),
Awwommat yous, Insidious onset; onret during conralesconce after an

occurrence of scoondary progressive muscular atrophy. eee
pyaar Diagwovia: from birth palay : syphilitic prewdo-perralysin: infan-
tile cerebral hemiplegia; myopathio atrophy ; muscular atrophy of the
Charvot-Marie type ; hysterical paralysis with amyotrophy in childhood,
M010: Cold, injury, ordinary causes; dentition probably has some
influence bnt ie not a direct cause. Infantile paralysis really oceurs
from tho influence of a general dineaxe. usnally of infertiow character.
Enumoration of the infections diseasox after which it has been known

INFANTILE PARALYSIS (continued),

PATHOLOGICAL ANATOMY.—History of the discovery of changes in the
anterior horns: Vulpian and Prevost (1865), Lockbart Clarke, Chareet
and Joffroy, Parrot and Joffroy, Roger and Damaschino. (a) Character
of the losions when the affected focusi« of old date, lysions in thoanterior horns,
nataro of these losions many foci usnally exist which are rarely quite

symmetrical ; atrophy of the half of the spinal cord which corresponds to
‘the paralysed side, and in some cases of tho cerebral hemisphere upon the
same side (Rampf, Cololla, Fornario): lesions but slightly pronouncod
in the anterior roots and trunks of the mixed nerves, explanation of Joffroy
und Achard; alterations in tho muscles; alterations in tho bones, diminn-
tion of the diameter of the Haversian ystems; alterations in tho blood
weasels, (18) Character of the lesions when the affected focus is of recent date:
every appearance of a trne inflammatory focus then exists, this foous of
acute myclitie may extend to the adjoining white matter; the reason of
this fact probably connected with tho distribution of biood vessels in that
togion, Norwan Anatomy of the blood vessels supplied to the anterior
horn. ANTERION srixat sTwrkM.—Anterior spinal artery; anterior
median artery; anterior radieular branches: cach of these articles may be
‘the seat of the intramedullary vascular lexion which constitutes infantile
paralysis. Identity of infantile spinal paralysis with infantile cerebral
homiplogia: my t with Vizioli and Stritmpoll, Observation

Mobis, This explanation of what occurs enables the Inte

"of the clinical course of infantile spinal paralysis, whieh
heen considered to be understood, Twxearkuttcs.—Electricity,
hydrotherapy, orthopedic apparatuses. 433.

ANATOMY OF TRE PYRAMIDAL TRACT. - 3

through the centrum ovale subjacent to the motor convo-
Intions.
The fibres follow this convergent direction, and before going

Fig. 1.—Outer surface of tho cerebral hemisphere showing the motor convolu-
tlons (leg. arm, face). (This figure was extracted from the Atlas of Brieaud *
upon the Anatomy of the Frain.)

Fig. 2.—Modian aspect of the cerebral hemixphere showing the motor convolutions.
(This figure was extracted from the Atlas of Brissaud upon the Anatomy of
‘the Brain.)

far form a compact mass, the pyramidal tract, the fibres of which

ean be localized in a more exact manner. his localization is
* have to thank my friend and colleaguo, Brissand, for permission to use these

Agures before the poblication of his Atlas upon the Anatomy of the Brain, from
which they are taken.

ds

Rl

+ LECTURES ON DISEASES OF THE SPINAL CORD.

specially interesting in the infernal capsule on account of the
differentiation which can be made of the numevous tracts which
constitute this cross-road (carrefour, Charcot).

Fig. 3.8, corpus striatam; NC, nuclous caudatus; CO, thalamus options; Flan-
torior limb of the internal capzule; G, knoo of the internal capaul , fibres of
the internal capsule connected with the movements of the upper extromity.
J, fibres of the internal capsule connected with tho movements of the lower
extromity ; C3, sensory cross-road, (This figure was extracted from the Atlas
of Brissaud upon the Anatomy of the Brain.)

The position of the different tracts is most easily studied, as
you know, by the horizontal division of the brain termed the

6 aes:

lobes, and which arc, at times, though very

found to degenerate (Bechterew), the

the pons is reached. Te ca aealt eyo

into secondary tracts, which from within outwards.
respectively for the face, tongue, and limbs. i

Tn the pona eavolii the fibres of the tract (EY) Sea
the crastal portion, being divided into flattened i
are separated from cack other by soie of the iraatrereg SUragem
the pons. ‘The size of the tract (Py) is here much than
in the upper part of the pyramids in the medulla | a
and it seems probable that, as some authors blown: manera
its fibres terminate in the pons,

In the medulla ohfongata, on the other hand, the tracts
as already stated, are completely isolated from the other fi
in that part, and form the prominent bundles known as pyran

Yon will observe, gentlemen, that the summit of these “
mids” is directed downwards, and that at the lower part
medulla oblongata the tract (FPy) disappears from its
remaining no longer at the surface, but passing into the int
of the bulbo-medullary axis, and, for the most part, crossing to
the lateral column of the opposite side. Such is the well-known
decussation of the pyramids, of which it is unnecessary to say
more,

The path of the pyramidal tract in the cord, which it has now
reached, must be studied most carefully, since it is in connection —
with diseases of the spinal cord that I have rapidly summarized
the path which it pursues.

In the lower part of the medulla oblongata, as you know, the
tract (FPy) divides into two bands, one of which does not. change
its direction, being called on that account the direct pyramidal
tract (FPyD), while the other passes into the lateral column
upon the opposite side of the cord, being therefore called the
crossed pyramidal tract (FPyC). Each of these tracts will now
be separately studied.

Tue crossep pyrasipaL tract (FPyC), by far the most
important as regards its anatomy and pathology, extends almost
to the termination of the spinal cord. Some authors (Vulpian,
Léwenthal) believe it to end at the level of the second lumbar
pair of nerves, while others have traced it farther, notably Tooth,
who found it below the fourth lumbar pair, Yery possibly it

: al

ANATOMY OF THE PYRAMIDAL TRACT. ai

varies in Jength, individual differences existing, which are
analogous to those of which I shall have to speak as regards its
variations in breadth.

‘The seat of the tract is as follows: throughont almost its
whole course it is in relation internally and posteriorly with the
posterior horn until almost the extremity of ite path, The
direct cerebellar tract separates it from the surface of the cord
(except, according to Gowers, for a short distance at the level
of the third cervical pair of nerves); the consequence is that in
those parts where the direct cerebellar tract does not yet exist,

Pig. 8. Fig. 8. Fig, 10.

Sections of the spinal cord in which the two direct pyramidal tracts A, and
the crossed pyramidal tracts B, preeent respectively the samme symmetrical
relations (after M. Flechsig}. Fig. 5 at the lovel of the 2nd cervical pair,
Fig. 6 at that of the 7th cervicel. Fig. 7 at that of the 3rddormal. Fig. Sat
that of the 6th dorsal. Fig. 9 at that of tho Ist lumbar, Fig. 10 at that of the
‘Sth bembar pair of nerves,

that is to say, at some point below one of the Inst pairs of dorsal
nerves, according to the subject, the crossed pyramidal tract
(FPyC) extends outwards as far as the surface of the cord.
Anteriorly the tract, even where most developed, does not
exténd beyond an imaginary line, possing transversely through

ll

a

8 LECTURES ON DISEASES OF THE SPINAL CORD.

the posterior commissure, At the same time it will be seen,
when the secondary degeneration consequent upon transverse
lesions of the spinal cord, and the pathological anatomy of
amyotrophic lateral sclerosis are considered, that the limits of
this tract are scarcely as well defined, as has been stated,
anteriorly, and the presence of stray fibres belonging to this
tract in other parts of the antero-lateral column, though not
certain, is ab any rate quite possible, the fact that individual
differences may exist being always remembered.

The form of the crossed pyramidal tract (PPyC) varies at
different heights. It is more or less oval in the cervical
region, being somewhat triangular, with the apex seated antero-
internally in the dorsal, and antero-externally in the lumbar
region.

Its size decreases regularly from above downwards, on account
of the way in which fibres continually leave the tract in order to
pass into the grey matter.

THE DIRECT FYRAMIDAL TRACT (FPyD) differs from the crossed
pyramidal tract both by its different seat in the spinal cord, and

Fig. 11.—Section of the *pinal cord Fig, 12.—Anterior horn and direet
(upper part of cervical region) in a pyramidal tract of the same seetion
caso of homiplogia dae toa cerebral as in the preceding figure more
lesion, A, direct pyramidal tract, highly magnified. (By am on

which hos here « flattened form, and
extends in an unosual way to the
outor sido of the anterior hora. B,
crossed pyramidal tract. ‘The white
portion is affected by sclerosis (Da-
maschino collection).

its shorter course.

error
the part of the designer the patch of
sclerosis A is upon the tight instead
of the left side, aa in fig. 1.) A, the
diroct pyramidal tract with its sim~
gular flattened form (Damaschino

collection).

According to Bouchard, this tract (EPyD)

does not usually extend beyond the mid-dorsal region. Tooth,

:

=

ty LECTURES ON DISEASES OY THE SPINAL CORD.

both pyramids furnishing « direct and crossed pyramidal tract,
which more or lesa correspond with each other. This, according
to Plochsig, is what usually happens (in 75 per cent. of the cases).

At other times the whole pyramid decusentes, and the dinect

me &

Fig. 15.—Seotion of the «piaal cord Fig. 16—Section of the same mond
in which the direct (A) in far larger (upper part of dorwal region) as Sige
than the crossed pyramidal tract 1h and 15, Same comparative sise of
(B), From the samo patient as fig. the direct (A) and crossed pyramidal
M, (After Flechalg.) trots (B), (After Fiechsig.)

Fig, Vi—Soetion of the same cord Fig. 18,—Section of the same cord
(lower part of tho dorsal region) as —(Jeve! of 4th lumbar pair of nerve:
in five. 14, 15, and 16 Samo com as in figs. 14,15, 16, and 17. In this
parative rine of the direct (A) and cord, in which the pyramidal Abres
crossed pyramidal tracts (B). (After are mostly contained in the direct
Flechaig.) pyramidal tract, traces of this tract

(A) are seon in the lowest part of the
lumbar cord, inatoad of its disap.
poaring, as usually happens, in the
lower part of tho dorsal region.
(Aftor Floebaig.)

pyramidal tracts are completely absent. Inversely the direct
pyramidal tracts (FPyD) are in some cases of langer size than
those which cross to the other side (FPyC). (Vide figs. 14, 15,
16, 17, 18.)

ANATOMY OP THE PYRAMIDAL ‘TRACT. i

Ta some cases the right and left pyramidal tracts act differently,
and the decusgation is then “asymmetrical * (Charcot).

‘Thus one of the pyramids, usually the left, may be larger than
the other by as much as about a third of its volume. It may
happen, for example, that the direct tract (FPyD) on the left
is larger than that on the opposite side, In some cases again
the direct pyramidal tract (FPyD) only exists upon one side
(figs. 19 and 20).

HH

Pig. 19.—Seotion of a cord (cervical Fig. 20.—Section of a cord (cervical
"een in whiok the direct and — rejion)in which the direct pyramidal
crossed pyramidal tracts aro sym- tract (A) only exists upon one side,
inetrically unequal in sice. The ‘The crossed pyramidal tract (B’)
crosed pyramidal tract B, which upon the samo sido far exooods that
is «mailer than B', corresponds to af the opposite side in size, an if it
= direct pyramidal tract A, which contained, in addition to its own
in larger than A‘, fibres, those which should have
formed the direct tract which is

absent. (After Flechsig.)

Similar variations in the pyramidal tract occur in animals,
and several observations of Bechterew, who has studied this
subject, and which are contained in his recent work, will be
mentioned.

‘The time of its development differs widely, and while in man
the pyramidal tract is not completely developed until after
birth, in some animals, such as the guinea-pig, the tract is fully
developed when it is born, and the same is the case with other
animals which can ran easily from the first. Thus there is a
eurions correspondence between the state of powerlessness in
which the young of the higher mammalia are placed at birth,
and that which exists in man, in whom the pyramidal tract is

not yet completely developed.

—

‘The size of the pyramidal tract also d
ments which the animal is able to per
_of which the movements are but: sligl
the whale and the elephant, ithe pyeasddia eal a
entirely absent, while in those living beings by which varied
and Velicate movements are performed, oot ee
monkeys, the pyramidal tract attains its full ;
Differences again exist ax regards thé Givi Set amt
and crossed pyramidal tract; some animals, for instanee, such
a the dog and eat, have no direct tract, while in others, euch
se the iin oh he pyramidal a |
segment of the posterior column, ‘These facts are bres
and show that the knowledge obtained f he
ee ee eee

egcad Se towed a wee to it
may now be considered.
Its development is of special interest, since it occurs at quite a

late date. Thus in the human embryo 12 centimetres hae
in length the pyramidal tract is completely

other tracts of the cord can be distinctly perceived,
Those who have investigated this 1 Oe ee
pyramidal tracte are in all probability formed towards ‘the
middle or end of the Sth month of foetal life. At the same time
the sheath of myelin is not found until the end of the 9th month.
This fact is of importance, and enabled Flechsig to study the
precise path of this tract, which was previously unknown. Sines
osmic acid gives a black colour to myelin® the parts which
remain of a grey or white hue when this reagent is applied to
the cord, are those in which no myelin as yet exists, and Flechsig
has thus been able to follow the development of the: different
tracts of the cord in their various stages,

The pyramidal tracts are essentially composed of fibres
parallel to the axis of the cord, which are of considerable length,
some of them extending from the cortex of the brain to the
lower part of the lumbar region of the cord. From these, as
also from the posterior columns, many of the collaterals —
emerge (Golgi, Ramon y Cajal, Kélliker, &e.), ‘These collaterals, —

* Pide Medical Microscopy, by P. J, Wethered, p, 90 (Translator),

al

14 LECTURES ON DISEASES OF THE SPINAL CORD.

tract (FPyD) produces more pronounced paralysis than one in
the crossed tract (FPyC), the paralysis occurring on the side
opposite to that of the lesion. According to Gowers, on the
other hand, the function of the direct tract (FPyD) is specially
connected with the movement of the upper limbs. Thus,
gentlemen, no definite agreement can by any means be said to
exist as yet in connection with the action of the two parts of the
pyramidal tract.

LECTURE IL,
SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT.

Hysror, Cruveilhier, Turck, Charoot, Valpian, Leyden, Cornil, &o.; Memoir
of Bouchard (1868) ; Thesis of Brinaud

Co-existence of secondary degencration of the pyramidal tract with amyotrophy.
‘Statement of such facte by numerous authore whose observations have given
rise to different theories,

Genriemen,—In the preceding lecture the principal facts con-
nected with the anatomy of the pyramidal tract were discussed
in detail. The degenerations of that tract consecutive to cerebral
lesions will now be studied, the portion of the pyramidal tract
in the spinal cord being alone considered.
existence of such degeneration has been known for
many years. Cruveilhier had already observed atrophy of the
pyramid upon one side in some cases of hemiplegia, but had
not perceived that the degeneration extended beyond this
point, The honour of discovering, and in a remarkable way, that
the pyramidal tract degenerates also in the spinal cord must be
ee, deseribed such degeneration in 1851—55.

rages these observations were confirmed and extended

Charcot, Valpian, Leyden, Cornil, &., and in 1866 the

int

observed upon the subject during several years. In 1880 the
Coa ‘thesis of Brissaud was written, this being the most
complete and suggestive account of the subject i in which we are
‘now interested which bas hitherto been given.

Although different varieties of cerebral lesion may produce

secondary degeneration i in the pyramidal tract, the lesion must

SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 17

about the sixth day. Clinical examination enables it to be
traced some time before this. Thus Pitres bas noticed the
existence of the foot phenomenon as soon as 20, or even 10
hours after the occurrence of hemiplegia. At the same time, in
most cases the existence of secondary degeneration cannot be
recognized with certainty until the end of a week after the
occurrence of the cerebral lesion. I shall also have occasion to
mention that the foot phenomenon, or increase of the tendon
reflexes, so soon after the occurrence of hemiplegia is not always
a sign of descending degeneration.

T need not again describe in detail the parts in which
degeneration occnrs. I should merely have to repeat word for
word what I have already said about the anatomy of the
pyramidal tract. It will suffice to place before you a few
different sections of the cord after hemiplegia due to a lesion in
one of the cerebral hemispheres (figs. 21, 22, 23, 24, 25).

We have already seen, gentlemen, in their collective character,
what, from an anatomical point of view, were the characters of
secondary degeneration due to a cerebral lesion. The clinical
features which characterize it deserve equal attention.

Paralysis (monoplegia or hemiplegia) is merely the direct
effect of the cerebral lesion, and in no way indicates secondary
degeneration. The true indication of this, which must not be
forgotten, gentlemen, is solely the spasmodic condition which

occurs,

Fig. 22—Secondary degeneration of
tke direct pyramidal tract A, and
the crossed pyramidal tract B, in a
ease of hemiplegia from a cerebral
lesion {cervical cord),

2

SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 21

bandage being applied to a limb affected by contracture, so a8
to render the muscles and soft parts exsanguine, and on that
account unable to contract, at the end of from five to twenty
minutes the spasm ceased, and any attitude whatever could be
given to the limb. According to Brissaud the spasm was merely
due to permanent muscular action; the spinal cord affected by
ion of the pyramidal tract being, as Charcot

expresses it, “in a condition of spontaneous strychnism,”
According to Vulpian the contracture is due, not only to
increased irritability of the spinal cord on account: of the dis-
of the fibres belonging to the pyramidal tract, but
also and specially to the irritation which the sclerosis consecutive
to degeneration of that tract produces in the centres of the cord

during a number of years.

Tt is certain, gentlemen, that we cannot completely under-
ee ere: OF he Brent tach e
of contracture, until we are better acquainted

ik he roc Hane of tat track
aeons | Soret arginine geet
opinion as to the action of the fibres in the pyramidal tract.

edie Ae idsiots eee, hs irritation of which has for its
¢ the immediate contraction of one or more muscular
fibres? This is but little probable. We know in fact that
these fibres are not directly continuous with the peripheral
nerves, which on the other hand are separated from them by
cella belonging to the grey matter of the cord. It seems there-
fore most probable that the pyramidal tract merely acts upon the
grey matter of the cord.
‘Most authors believe this action to be excito-motor, and such an
opinion will be easily understood if the ingenious comparison
suggyated by Charcot in his lectures be mentioned to you. The
mechanism of musical boxes is so far understood that it is
known that by pressing a-certain button, which corresponds to
‘one or other of the airs named upon the lid, the cylinder is
a rk, and the instrument immediately plays the air
the human organism the spinal cord may be
* 4 musical box auch as the one just mentioned.
of education, on account of the local and special
ch it is endowed, each part of the cord holds
to exewute such or such 4 movement, it might

Might it not be supposed that the fu
tract is one of inhibition, analogous in a p
that of the vagus nerve upon the heart?
sidered to be of this nature the grey sub
still be the true motor power, but a power

hemiplegia there was foot clonus in the leg of the side.
In 1878 Dejerine made the same observation, and in

year Brissaud called especial attention to pets ne)
two lower extremities, which is sometimes found to occur in
hemiplegia, At the same time it is only fair to say that this
subject was specially studied by Pitres, both from an anatomical
point of view, by himself personally (1882—1883), and from a
clinical point of view by the researches which he inspired in his
pupil Dignat (1883—1884).

I shall borrow many statements from these two authors upon
this interesting question. Let us first consider what we lear
from clinical observation, and let us suppose a hemiplegic
patient suffering from contracture, due to secondary’
of the pyramidal tract. The attitude of the limbs on the side
corresponding to that of the degeneration, gentlemen, is known
to you, the upper limb being fixed in a position of flexion and
pronation, the lower limb in one of extension and adduction, whilst
reflex action is very excessive in both limbs. The “sound”
side is by no means unaffected, as one might @ priori suppose.
Dignat, in fact, has on the contrary shown that if the condition
of the limb upon the “sound” side is examined with care the
muscular force in the lower limbs is found to be diminished by
about 50 per cent., and proportionately more as the time of
examination is nearer to that of the occurrence of hemiplegi:
Tn addition to this, the side presents evidence of functional loss
of power, as if the memory necessary for the co-ordination of
movement on the “sound” side was lost. Thus the movements
required in walking are often difficult or impossible upon this
side, although the muscular power needed for their exeention is
amply sufficient. Decided increase of the knee-jerk upon the
‘‘gound” side (already noted by Brissaud), and at times foot
clonns also occur. Lastly, when the tendency to spasm is still
more pronounced, as Brissaud has remarked, hemiplegia may be
accompanied by contracture in the tivo lower extremities.

‘The upper extremity on the * sound ” side is somewhat similarly
affected. Though it is not in a condition of rigidity, and hand
elonus never occurs, the tendon reflexes are more or less
excessive. The muscular force is diminished by about #8 per
cent. (Dignat), this diminution being greater in proportion as
the time of the observation is nearer to the oceurrence of the

b wl

matter. Tile colaaticn os geannlly Suet
atory facts had been published by Carritre, H
Pitres, Brissaud, &e.,* when within the last few ;
in publishing the account of an autopsy in a case of |
stated that no lesion was found, either in the
spinal cord or the peripheral nerves. Shortly
observed a case of the same kind, as did other
Roth, Muratow, Darkschewitech, &c. On ie
Mgjerine found in four cases of atrophy, after h

2) the publication of theo lectures Joffroi asd Achard publi
‘new facts and an interesting theory as regards the ocour
(Arob, de méd. experim., Nov. 1, 1801.)

32 LECTURES ON DISEASES OF THE SPINAL CORD.

, The affected part is of greater size because the pyramidal
fibres being united in a much more confined tract in the
cord than in the brain, in the latter they are much Jess often
injured throughout than in the former. and consequently the
degeneration in the first case is less extensive than in the
second,

Though disinclined, as a rule, to eclectic solutions, I must
acknowledge, gentlemen, that these two opinions seem to me
equally probable; the fibres added to those of the pyramidal
tract will be again discussed at a future time,

Fig. 33.—Section of the cord (lumbar region) from a caso of transverse Tesion
aoated on the doraal region, Degeneration of the two crossed pyramidal tracts
(somi-dingrammatic),

So far, the whole pyramidal tract has been considered ; it need
scarcely be added that degeneration may not only affect the
fibres of the crossed, but also those of the direct pyramidal tract,
the condition being that the transverse lesion is at such a height
in the cord that the fibres of the latter tract have not as yet
ceased to exist.

B. Portion of the antero-lateral column external to the pyramidal
tract.—As already observed, gentlemen, this portion of the cord
contains nerve fibres belonging to other tracts. Most of these
ave affected by ascending, but some by descending degeneration.
‘The latter are, for the most part, anterior to the pyramidal tract
in the middle portion of the antero-lateral column, and Lowenthal
describes descending degeneration of these fibres which he terms
the intermediate tract of the lateral column.

These specially occupy the middle part of the lateral column
on the inner side of the tract of Gowers, and the direct cerebellar

——
DESCENDING DEGENEWATION AFTER TRANSVERSE LESIONS. 33.

tract, a certain number of fibres seeming to join these tracts
internally. It is very probable that some fibres also join

Fig. 34,—Descending degeneration of the pyramidal tract in the cord of m dog
nigsk pst! ats niobig lence rg agente) eae ee ee
ee een eerie ind to the

of the cord.
those of the crossed pyramidal tract. At this point, in fact,
nerve fibres are found which differ much in size,

Fig. 35.—Doecending degeneration in the cord of a dog after division of the
eee reer ese ten ener cervical region.

inal tract,* while the
tract of the lateral columnt The pyramidal Sbres
this degenorated part,

oy. Anterior Peli —In Bids yoolnton also ‘certain fibres are
a after transverse lesions of

# Vide'p. 94 (Pranelator) + Vide p. 32 (Translator).

3

84° LECTURES ON DISEASES

the cord in opposition to the opinion
thought that these were stray fibres
whereas it seems certain (Flechsig and
quite unconnected with this tract, sinee they ¢
after lesions which are confined to the brain. ‘This is the degen-
eration of the marginal tract (Léwenthal). The degenerated
fibres of this marginal tractare found in man and in the monkey
in the part of the anterior column which adjoins the anterior
fissure, which I shall call the suleo-marginal zone. To distin-
guish these fibres from others in the same part, I propose to
name them the descending system of the sulco-marginal sie.

Fig. 36.—Diagram of the principal sents of descending degeneration in casos of
transverse losion of the cord in the dorsal rogion. A, Sulco-marginal
in which two kinds of fibres are found: firstly, those of the direct pyramidal
fract (+ +++); secondly, those of the descending aystem of the sulcomargival
sone(....). B,C, Iatereedist rac of tha lateral colemey ascie Morse Ue ea
are found at the peripheral part of the cord intimately mixod with those of the
tract of Gowers, D, Zone of the eroseed pyramidal tract, ‘This zone contains
two sorts of fibro: firstly, those of the crossed pyramidal tract( + + +); secondly,
those of the intermediate tract of the lateral column (....), which aro intimately
mixed with the precoding fibres but in amaller number.

‘The path and origin of these fibres of the anterior and lateral
columns, which are independent of the pyramidal tract, will now
be considered.

With regard to the former, it may be asserted that they
almost undoubtedly pass into the grey substance of the cord,
and very probably into its anterior and middle part. Some of
these fibres seem to have an exceedingly long conrse, since traces

rah ai

—EEE———

DESCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 35,

of their degeneration are found in the lower part of the cord,
even where the fibres of the direct pyramidal tract have ceased to
exist. It is, on the other hand, very difficult to trace their origin
with precision, It seems very probable that some of these fibres
which are liable to descending degeneration belong to the
system of longitudinal commissural jibres, of which I have already
spoken,* and which, taking origin from a cell in the grey sub-
stance, return to the grey matter seated in a lower part of the
cord, after passing for a longer or shorter distance through the
antero-lateral column, Since the time when the existence of
snch fibres was suggested by Bouchard they have been definitely
proved to exist. Thus, for example, Ramon y Cajal, whose
beautiful works upon the minute anatomy of the central ner-
vous system are of scientific value, describes convmissural cells
ee ee oe in many points of the grey

See Aa tos sate Chie <n. mokoe calle, on axis
cylinder passes through the anterior commissure into the
anterior column of the opposite side. In this it divides into
two branches, one ascending, the other descending, from which
numerous branches proceed in the direction of the grey matter,
dividing there into numerous fibrils, which form an extensive
network round the cells, which are seated therein,

Daring their path through the white substance collateral
branches form. After this description it is useless, I think,
to add that the descending branch is involved in descending

whenever the transverse lesion is seated below the
commissural cell from which it took origin; the ascending
branch, on the other hand, is affected when ascending degen-
eration occurs.

Tt is doubtful, however, whether such a statement can be made

| to all the fibres, and an opinion recently expressed
‘hy Marchit should at any rate be mentioned. This author,
having removed the whole or part of the cerebellum, stadied the
pre degeneration in the cerebellar peduncles. He was
able to trace such Sagar in the inferior cerebellar
Pedunele, and found that at about the height of the olivary
4 ;

4 decors del peduncoli cereSellari (Risista sperimentale di
eg Vol. XVIM., p. 367).

as

38 LECTURES ON DISEASES OF THE SPINAL COKD.

below the point where the compression existed, degeneration
was found to exist in the region of the columns of Burdach,
in the form of a line parallel in direction to the posterior
horns, commencing at a short distance from the posterior com-
missure but not reaching the surface of the cord, from which,
on the contrary, it remained at gome distance. Owing to its
form this degeneration may very justly be termed the eoimma-
shaped degeneration of the posterior columns.

It should be added, gentlemen, that the cases in which this
comma-shaped form of degeneration has been recognized are
by no means numerous, although Schultze states that he has
observed it three or four times. On the other hand, in a memoir
recently published, Barbacci* states that he found degeneration

Fig. 41.—Section of the cord (upper dorsal region) 2 centimetres below i foous of
compression, seated in the middle part of the cervical enlargement. (After
Schultze.) A, Degencrated pyramidal tract; B, Comma-shaped degeneration
in tho posterior columns The original figure indicated a emall islet of degene-
ration in the antero-latoral columns which is omitted here.

in the posterior column after a transverse lesion of the cord, but
that this degeneration was by no means comma shaped. Fibres
were affected throughout the whole breadth of the posterior
columns; lower in the cord these fibres were grouped near the
posterior median fissure, and lastly in the conus medullaris
oceupied the posterior part of the cone. ‘This form of degenera-
tion could therefore be traced to a much lower point than that
indicated by Schultze.

These facts reqnire, not to be confirmed, as there is no doubt
of their existence, but to be explained, and the best explanation
would be that exactly indicating which tract is involved in the
degeneration. his is as yet unknown, so that the uncertainty

* O, Barbaool, Contributo anatomico ¢ aperimentale alls shadio delle Gayenerazions
necnadarie, Ge. (Lo Sperimentale, 1801, parts TIT. and IV., pp. 395 and 408).

| ,

ES =

DESCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 39

still exists. According to Schultze the fibres whose alteration
produces comma-shaped degeneration are the descending branches
of the posterior roots which enter the cord at the level of the
transverse lesion. In opposition to this idea Tooth, not un-
reasonably, suggests that this comma-shaped degeneration is
not observed to occur afler division of the posterior roots, in
addition to which, this degeneration, after a transverse lesion of
the cord, descends lower than the descending fibres of the
Posterior roots are anatomically shown to do. Thus, according
to Tooth, the degeneration would rather be due to destruction

Pig. 45. Fig. 46. Pig. 47.
‘Seetions of the cord from a case of fracture of the tpine with destruction of
the cord betwoen the 8th cervieal and Ist dorsal pair. (After Tooth.)

42.—Transvorse lesion ; the deatraction is almost
; the greator part of the white columns ix affected.
degeneration exists hore also.
in the antero-lateral column the region of the direct
of Gowers is already almost froo from de-
“comma-shaped degeneration" of

f
f

i
H
i

il
H
i

7:

Fe
fH

Hi
i
iitf
Hl
i
:

trnct.
+ the degeneration of the pyramidal tract is still most
the intermediate and even more that of the eulco-marginal

x
j
i

it
i

of the commissural fibres. Possibly, and this is a simple

®uggestion on my part, the condition of the grey matter in the
1 ould be considered, and in future it will be observed

transverse lesion inyolves to a greater or less extent the grey
J 0 -eord. Ifso, this would Le a fresh argument in

40 LECTURES ON DISEASES OF THE SPINAL CORD.

favour of the theory that the commissural fibres are affected in
this form of degeneration.

With regard to the lesions which exist in tabes I shall show
you that in this disease the fibres which correspond to the
“ comma-shaped ” tract remain unaffected for a long time
after the other fibres in the posterior column are more or less
involved,

Ag regards secondary descending degeneration within the GREY
MATTER OF THE CoRD nothing certain is known, although it is
probable that amongst the tracts of fibres which compose it some
ave as liable to degenerate in the descending as in the ascending
direction. Everything, however, points to the fact that such
degeneration extends but a very short distance along the cord.

becomes divided longitudinally into three segu

of which, the median, the central nervous system i |
and notably the molor portions (the motor cell the cord with
their prolongations which subsequently form the anterior roots
and motor nerves).

On each side a lateral segment is found, which separates from
the median segment representing the cord and gives origin to
groups of cella which afterwards constitute the sympathetic system
and ganglia connected with the spinal nerves.

At a later period the groups of cells which form the spinal
ganglia become spindle shaped, and a nerve fibre is connected
with each of their extremities. One of these fibres constitutes a
posterior voot fibre and approaches the cord into which it pene-
trates, and to which it therefore only belongs in a secondary
way; while the other passing outward in an opposite direction
constitutes a peripheral sensory nerve fibre. After this brief
description, gentlemen, one fact will be easily understood,
namely, that the posterior roots in no way belong to the spinal
cord, being merely extensions from the spinal ganglia, while
these ganglia constitute in connection with the system of con-
ducting sensory fibres (peripheral nerves and posterior roots) a
true trophic centre,

‘The development of the cord shows that the posterior roots
enter the cord from without inwards, and the study of their
path in the cord is in reality merely that of the composition of
the posterior columns of the cord.

In each posterior root three varieties of fibres can be distin-
guished, according to the length of their path before
the grey matter, ‘These varieties have been recognized to exist
by different authors, and were very accurately described by
Singer and Miinzer.

The fibres of the first group (short yibres) radiate at once into
the grey substance of the posterior horn, some penetrating
directly into the peripheral extremity of that horn, with which
they appear to join, while the others approach the internal
portion of that horn after having made a slight curve within the
posterior column.

‘The fibres of the second group (filves of medium length) ascend
to a certain height in the posterior column, and as they pass
onwards are seated more and more internally, so that their

ions being:
which produce ramifications around the margins of the ganglia.
On the other hand, these centres of termination become * centres
of origin,” and new fibres issue from them which cross each
other and, passing into the band of Reil, reach the centres of
the brain.

‘The course of these fibres is therefore by no means continusns,
being interrupted in the grey substance of the cord and medulla
oblongata, and in the terminal centres, so that it should be
rather considered as a path divided into stages.

‘The subject which specially interests us at the present time, viz.,
the secondary degeneration of the posterior roots tn the spinal cond,
may now be considered, and it would be unfair to ignore the
service which has been rendered by experiments upon animals.

In reality it is to them that we owe most of our knowledge
about this form of degeneration. Singer, Tooth, and Horsley
have obtained most interesting results which seem of the neces-
sary precision, and their value is greater because they are
entirely in accordance with the observations of human pathology,
specially with those of Kahler, Schultze, &e.

Tf, for instance, as was done by Tooth, one of the posterior roots
is divided in the cord of an animal such as the monkey, and a saf-
ficient length of time (3, 4, or more weeks) is allowed to elapse
before the animal is killed, a small tract of degeneration is found
to exist in the posterior column of the cord above the lesion, the
exact seat of which varies much according to its height in the
cord, Immediately above the lesion this tract is in immediate
contact with the inner margin of the posterior cornu against
which its external border rests. Then when a fresh pair of

* Flochsig and Honel, Die Centrabwindengen, cin Centralorgan der Hinterstraage
(Newrolagie.her ContralBlat, 1890, p. 417).

a ul

————=

ASCENDING DEGENERATION AFTER NERVE-ROOT LESIONS. 47

nerve roots enters the cord, the degenerating tract being pressed
inwards by the fibres of the newly arrived posterior root, becomes
more distant from the cornu, and is entirely contained in the
postero-lateral column or tract of Burdach. As a higher level
is reached the tendency to pass inwards increases, and should
the divided root belong to the lower part of the cord (the lumbar

after being placed round the cord ascond along ite eurfaco until one
after the other penetrates into its substance; B, Degeneration of the posterior
column where it ix in contact with the posterior cornu (entrance of the poa-
‘terior roots into the cord, true external bandlets). Fig, 61; 3rd lumbar; the
posterior column has undergone more degeneration (B) than at the level of
‘Sth lumbar, beeanss at the latter point it had not yet received all the
of the posterior roots which had undergone ascending dogencrution.
‘Whe degenerated zone (B) commences at the level of the 3rd lambar nerve
tals apy ay hegieebae Fig. 52; 1th dorsal; the degeneration (B) has
‘left the posterior cornu in order to approach the posterior median

. ob

or sacral region) the degeneration occupying a more and more
eee finally affects the postero-median column or
| ‘and may then be followed as far as the medulla
| oblongata, where it is seated near the nucleus of the column of

is reached the number of fibres lost in the grey substance is so
great that at last the sclerosis almost entirel

however, many of the posterior roots have been div e
the long fibres which degenerate are so numerous that they can
be followed throughout their entire length, o

It is unnecessary to describe at greater length seat of
secondary degeneration in these cases, the figures certainly
indicating more with regard to this point than a long explana-
tion would do, The one point upon which alone I would dwell
is the following conclusion. When degeneration follows a lesion
in the posterior roots the degeneration of the white substance
is in the posterior columns, in which its seat is nearer to the
posterior median fissure in proportion as the diseased root
occupies a lower position.

The ascending branches of the posterior roots have as yet
been alone considered, but it must be remembered that a5 they
enter the cord a Y-shaped division of the posterior roots oceurs,
an ascending (which has alone been considered) and a descending
branch being thus formed, both of which may undergo secondary
degeneration. In the posterior columns not only ascending, but
a certain degree of descending degeneration may occur after
destruction of the descending branches which result from bifar-
cation of the divided posterior roots. These branches, however,
being shorter, their degeneration i is slighter in degree, Tooth,
as already mentioned, in his observations upon division of the
roots, states that he has never observed such degeneration to
ocour, while Schultze attributes the “‘comma-shaped ” degenera~
tion, described by him as occurring in the posterior columns, to
the change in these fibres having » downward direction,

Tooth also observed a slight degree of degeneration in the
posterior column of the opposite side, which may be attributed
to destruction of those root fibres in which decussation had
occurred, ;

‘The thanges in the grey matter must alao be considered while
the secondary ascending forms of degeneration are being studied.
When sufficient time has passed after division of the roots,
‘the posterior, and even the anterior horn at its base, may be

egeneate are so numerons that they can
be followed throughout their entire length, =
Je i chon

tion would do. ‘The one point upon which alone I would
is the following conclusion. When degeneration follows lesion
in the posterior roots the degeneration of the white substance
is in the posterior columns, in which its seat is nearer to the
posterior median fissure in proportion as the diseased root
oceupies a lower position,

The ascending branches of the posterior roots have as yet
been alone considered, but it must be remembered that as they
enter the cord a Y-zhaped division of the posterior roots occurs,
an aseénding (which has alone been considered) and a descending
branch being thus formed, both of which may undergo secondary
degeneration. In the posterior columns not only ascending, but
a certain degree of descending degeneration muy occur after
destruction of the descending branches which result from bifar-
cation of the divided posterior roots. These branches, ae
heing shorter, their degeneration is slighter in degre. Tooth,
ag already mentioned, in his observations upon division of the
roots, states that he has never observed such degeneration to
occur, while Schultze attributes the “ comma-shaped " degenera-
tion, described by him as occurring in the posterior columns, to
the change in these fibres having a downward direetion.

‘Tooth also observed a slight degree of degeneration in the
posterior column of the opposite side, which may be attributed
to destruction of those root fibres in which decussation had
occurred.

The changes in the grey matter must also be considered while
the secondary ascending forms of ion are being studied,
When sufficient time has passed after division of the roots,
the posterior, and even the anterior horn at its base, may be

52 LECTURES ON DISEASES OF THE SPINAL CORD.

for instance, in the lower part of the cervical region, the
degeneration of the columns of Goll extends over a much
larger area than in the preceding case, which is to be ex-
pected since a much larger number of long fibres are divided
(vide fig. 49).

The difference which exists in the area of degeneration in this
tract according to the height at which the cord is divided clearly
indicates that the column of Goll is not of immutable size, as
the inexperienced are inclined to think, but composed of anited
fibres each of which has its own individuality. Thus the column
of Goll, as Schultze also believes, cannot be absolutely dis-
tinguishe] from that of Burdach, since during part of its path

Fig. 55.—Division of the cord (cervical region) in a case of ascending degeneration
in the columne of Goll (A) due to compression in the dorsal region.

the fibres of the former tract pass within the territory of the
latter.

In this respect the terms employed by some English authors
may represent better what really occurs. They merely divide
the posterior column into a postero-lateral and postero-median
tract, so that the difference between these two columns is less
decided than when they are termed the “column of Goll” and
the “column of Burdach.”

It is a mere question of terms, and if there is general
ment as to the facts of the case, it is unnecessary to dwell upon
this point.

Such, gentlemen, is what T wished to say about ascending
degeneration of the posterior column, but when transverse
lesions of the cord exist, ascending degeneration is by no means

ASCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 53

limited to the posterior columns, but oecupies also, and over a
considerable area, the antero-lateral colomns.

B. In the ANTERO-LATERAL COLUMN two areas of degeneration
may be distinguished, which are confused at certain points, but
clearly separated in other portions of their path. One is that
which corresponds to the direct cerebellar tract (tract of Flechsig),
the other to the antero-lateral ascending tract (tract of Gowers).

a. Direct cerebellar tract—The degeneration of this tract was
observed by Tiirck, who traced it as far as the restiform body,
hut it is to Flechsig that we are really indebted for knowledge
of the origin, path, and termination of the fibres which compose
it. Schultze, Kahler and Pick have also studied the degeneration

Fig. %—Division of tho medulla oblongata at its lower part, in o case of
ascending degeneration of the oolamns of Goll (A) dno to compreesion of the

of this tract, and their works upon this subject are most

interesting.

The direct cerebellar tract occupies the posterior half of the
surface of the lateral column. It has the form of the segment
of a ring representing about the sixth part of the circumference
of the cord. Its posterior extremity (which is in relation with
the posterior horn) is large and, as it were, puffed out, whilst the
anterior extremity is more fine, so that for a certain distance it
has the appearance of being drawn out, The anterior extremity
of this tract is neither very clear nor certain, and it seems to
fuse insensibly with the adjoining tracts.

‘Opinions differ as to the point in the cord at which this tract

Mile

56 LECTURES ON DISEASES OF THE SPINAL CORD.

columns of Clarke are not the only origin of the fibres of the
direct cerebellar tract, some of which (especially in the cervical
region) seem to be directly connected with the posterior roots.
This, however, is quite uncertain.

Whatever the truth may be, let us suppose, if you will,
gentlemen, that the cells of the columns of Clarke constitute the
sole origin of the direct cerebellar tract, and consider what the
consequences will be as regards ascending secondary degeneration.

When the transverse lesion of the cord is seated at 4 position
(dorsal region) in which the columns of Clarke exist, and the
direct cerebellar tract is already formed, the degeneration which
follows division of the fibres in that tract. and that due to

Fig. 58,—Divisionof the cord (cervical region) in a case of transvorse lesion sented
in the dorsal region. Ascending degeneration —A, Columns of Goll; B, Direct
cerebellar tract posteriorly, tract of Gowers anteriorly,

destruction of a more or less considerable portion of the columns
of Clarke, may be confused. If, however, the transverse lesion is
in a portion of the cord (quite the lowest part of the dorsal region)
in which the direct cerebellar tract is not yet formed, although
the column of Clarke is now beginning to appear, a lesion of
the cells in that column is found to produce ascending degene-
ration in the fibres of the direct cerebellar tract, of which these
cells are the trophic centre. This ascending degeneration only
occurs at a certain height above the transverse lesion on account
of the obliquity of these fibres. ‘The facts indicated by Barbucci,*
already mentioned, in which, after a transverse lesion at the
level of the 10th or 12th dorsal nerve, degeneration of the
direct cerebellar tract is only found to occur at some distance
* Vide p. 54 (Translator).

ASCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 57

above the point where the zone of traumatic degeneration ceases
may thus be explained.

Pig 59.—Section of the spinal cord (upper part of the cervical region immodiately:
below the deoussation of the pyramids) in a casc of transverse lesion of the
cord in the dorsal region. A, Columns of Goll; B, Dirvct cerebellar tracts and
tract of Gowers; C, Marginal tract of the anterior column. (Damaschino
collection.)

Pig, 62 Fig. 63.
medalla oblongata from a monkey in which a division had boen
‘of the cord between the 7th and Sth corvical vertebrn (after
lower part of the medulla oblongata (fig. 00) the direct core-
(B) and the tract of Gowers (A) are in contact, and apparently
| fogathor. As n higher and higher point in the modulla oblongata ix
‘the two tracts tend to separate, and in fig. 63 are seen to be quite

nit

of Gowers (antero-lateral ascending tract of English
the altered part which resulted from degene-

fe
if
i

Inhoratory may be, it is an undeniable fact that in most cases
rs tion. of w limb ch hick Taaaeal
found to occur in the central extremity of er ditties
upon the corresponding side ‘ i

are not quite accurate, it is to Vulpian that | ithe honour of first
- describing facts of this kind in

several memoirs written between 1868 and 1872 must be. given.
At a later date this author inspired the interesting researches
of Dejerine and Mayor (1878). In the meantime Hayem (1875)
to similar facts, and as you know: that

oie eee peda again oceupied in a work
written in conjunction with my distinguished colleague and
Bissci Gilet. Tain bok fir 10 nsme Diokinvon, pate the
” a most remarkable description

an as the principal seat of the atrophy, whilst, as
-Vulpian specially localized that atrophy in
th aon an Ido not wish, however, to insist

n given by the authors is new; in fact I must allude
é to this work, though I am far from agreeing
ons which are formed in it.
able to form a clear ides of the changes which
d after amputation, the study of the cord alone
4 extent, abandoned in these lectures, of which
jee is "Diseases of the Spinal Cord,” and the
trunks above the region in which the
red be investigated. We will not, however,
ly the alterations which are shown to exist in

DEGENPRATION OF NERVES AND CORD AFTER AMPUTATION, 65

size, and are most transparent, being in reality those in which
the greatest change has occurred. Upon examining a section
stained by the hematoxylin of Weigert, it is at once recognized
that the lesions are extremely pronounced. Instead of the regular
mosaic pattern which the transverse section of a healthy nerve
presents owing to juxtaposition of the sheaths of myelin, which
are coloured black, 9 very small number of such sheaths is

Hig Gachion 6f ths tol scatia necro n'a cuss of axaprtation of the thigh on
‘the same side 20 yoars previously, A, Primary fasciculus in which degonora-
thon has occurred. B, Primary fasciculus in anormal condition. C, Primary
fasciculus in half of which degeneration has occurred, while the other half isin
*@normal condition, D, Portion of the norve between the fascicule having a
myxold appearance. E_ Blood vessel.

perceived; these sheaths are also scattered throughout every

fisciculus, not being placed in groups, but completely isolated

from each other. In a few fasciculi, however, two or at the
most three, and these the smallest in size, the ordinary mosaic
pattern indicates that their condition is normal; these come
from the nervous branches proceeding to those parts of the

5

Se

SSS

66 LECTURES ON DISEASES OF THE SPINAL CORD,

thigh which are seated above the point of amputation, while
they are completely separate from the affected fasciculi, whose
fibres were, for the most part, destined to parts of the lower
extremity seated below the amputation. In some sections fasci-
culi may be seen divided into two distinct halves separated by a
partition of connective tissue, of which one is degenerated and
the other sound, the former being composed of fibres coming
from the part amputated, while the latter commences above the
point of amputation.

Thus the fibres which contain myelin are very rare in the
most degenerated fasciculi, being replaced by small islets of

Fig 7.—Part of the section of a healthy scintic nerve (stained by the bema-
toxylin of Weigert) (from nature, and semi-diagrammatio), The segments
isolated by the white lines are the primary islets.

yery singular appearance, which have been observed by different
authors, but to which special attention seems only to have been
drawn by Friedliinder and Krause, although, in my opinion, these
authors are far from attributing to them their true signification.

These islets do not become of a black colour when the method
employed, but are distinctly coloured by carmine ;
n size, having usually a mean diameter three or four
times as great as that of a healthy nerve fibre with its sheath of
myelin, ‘They also vary in form. Where sufficiently isolated
from each other, their form is completely round, while, if pressed
against each other, their shape is altered on account of this
reciprocal compression.

DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 67

As to the composition of these islets, I completely disagree,
gentlemen, from Friedlander and Krause, as you will see. If with
a feeble magnifying power (objective 0 or 1 of Vérick) the section
of a healthy nerve is examined, whatever colour is employed,
the nerve fibres are divided by means of connective tissue into
a variable number of groups (primary islels of some authors),
‘These primary islets are much larger in size than the other
“islets” of which I have just spoken, and to which, in order to

Fig. 72.—Seotion of tho left sciatic nerve after amputation of the thigh upon the
same side 20 years previously. AAA, Nerve fibres in a normal condition which
heave retained their axis cylinder and sheath of myolin. BB, Islet of degene-
mation of rounded or polyhodral form containing in their interior numerous
points which are the fine nerve fibres occurring in degeneration. In some of
these islets one or more black points are seen which indicate the presence of
fine fibres containing myclin in the islets. CC, Islets of degeneration in the
feargin of which a ruptare has occurred so that the norve fibres oxcape and
Ieoome separated from each other. D, Blood vessel with thickoned wall
(eich exaggerated in diagram). Tho segmenta soparated by large white tracts
aro tho primary islets, (From noture, semi-diagrammatic, coloured by the
hematoxylin of Weigert.)

distinguish them from those which are primary, I shall give the
name “ islets of degeneration.” According to Friedlinder and
Krause, the islets of degeneration are merely primitive islets,
‘of whieh the fibres have wasted, and with considerable reduction
of their size in consequence. At first sight this explanation

a

68 LECTURES ON DISEASES OF THE SPINAL CORD.

seems extremely probable, but upon examining it more closely
Twas convinced that this is not the case. The primary islets,
far from being diminished in size, are found to be of langer
size in a degenerated than in a sound nerve. As regards the
islets of degeneration, each of them merely representa, in
my opinion, a deyenerated nerve fibre, of which the size has
inereased to three or four times that which it had previously.

If one exposes one of these islets of degeneration to a higher

magnifying power (objective 3 of Vérick) it is found to be com-
posed of a :nass of very narrow fibres with a cylinder axis, whose
diameter is much smaller than would normally be the case, and
a sheath of Schwann similarly reduced in size, and of great
tenuity. As regards the sheath of myelin, it is usually absent;
but islets of degeneration often occur in which the existence of
one, two, or three extremely small black points can be recog-
nized by the method of Weigert, indicating the existence in this
part of a very narrow nerve fibre with a sheath of myelin.”

I am strongly inclined to think that each of these islets of
degeneration is surrounded, at any rate when its evolution is
not too advanced, by the sheath of myelin which previously
surrounded the nerve fibre, of which this islet is the remaining
trace. A large number of nuclei also exist within the islet,
which are very clearly seen in sections made at right angles to
the axis of the nerve, and even more clearly when the sections
are parallel to that axis. They are scattered throughout the
interstices between the nerve fibrils, and their long axis is in
the same direction as that of these fibrils, namely, parallel to the
axis of the nerve.

The pathology of these tracts of degeneration would be shortly
as follows :—Owing to some influence which need not now be
considered, one of the nerve fibres at the central extremity of a
divided nerve degenerates, that is to say, its cylinder axis and
sheath of myelin disappear while the sheath of Schwann remains.
Consecutively within this sheath of Schwann nerve fibrils are
developed having o small axis cylinder, and narrow sheath of
Schwann containing a substance which but rarely gives the
re-action of myelin. The number of these nerve fibrils varies, but

* The existence of there narrow norve fibres kas been mentioned by Hayom,
thoy were very olearly descr; bed in a momoie by Hayem and Gilbert (Arcot,
de Phyriol., 1884, p. 430 et 903.)

— =

DEGENERATION OF NERVES AND CORD AFTER ampUTATION, 69

is sometimes considerable (5, 10, 15, 20, —— Owing to the
of these nerve fibrils, the sheath of Schwann, which

development.
I shall call “ primary,” becomes rapidly distended, and the islets —

g

thus become of much larger size than the sound
nerve fibres by which they were preceded. Owing also to the
of the development of the nerve fibrils and the external

which they exert, the primary sheath of Schwann,
having reached the extreme limit of its extensibility, bursts;
the contained fibrils become free, and being no longer kept
together and united by this sheath, separate from each other.
The islet of degeneration is broken up and cannot be recognized,
completely ceasing to exist when the nerve fibrils which composed
it are so far apart as to have apparently no connection with each

alt

Such, in my opinion, is the way in which evolution of these
islets of degeneration occurs. which will be observed, gentlemen,
to he in direct opposition to that professed by Friedliinder and
Krause; when they believe atrophy of the nerve fibres to occur,
opinion is that these fibres regenerate and multiply, and what
they look upon as the indication of a primary islet, I regard as
the remaining trace of a degenerated nerve fibre,
‘The other parts of the section of the sciatic nerve will now be
examined, and for that object a lower magnifying power will be
. Tn considering the different nerve tracts it is found that
in the degenerated tracts the partitions separating the primary
each other are twice or three times as large as those
ng in healthy tracts; they seem also to be more relaxed,
ped a traly myxoid appearance, An analogous band of
gpl is also fonnd at the surface of the most altered
separating them from the fibrous sheath furnished by the
reciésios, 2 that, when contraction has taken place in the sec-
tion, these tracts have a pronounced tendency to escape from the
7 ormed by the neurilemma. The connective tissue which
parates the different fasciculi from each other is moderately
ickened ‘The spaces surrounded by many degenerated tracts
munch i in size, this seeming expecially due to
‘in the diameter of the surrounding tracts, The blood
| in the degenerated tract are not increased in
| their walls are at most but moderately thickened.
important factor, gentlemen, in increasing the

72 LECTURES ON DISEASES OF THE SPINAL GORD.

amputation, is really congenital alsence af symmetry and totally
unconnected with the amputation.*

Tn opposition to this opinion, which is of a very sweeping
nature, the facts themselves taken collectively need alone be
considered. Absence of symmetry is certainly frequent in the
cord, but is not usually found as accentuated as when observed in
cases of amputation. In every case of amputation Friedlander
and Krause observed such atrophy, and I myself have seen
it three times in three cases. After such correspondence, I
would almost say constancy, in the results, in my opinion it
ix impossible to deny the existence of a direct and not for-
tuitous relation between atrophy of the cord and amputation
of a limb,

‘The figures now before you, borrowed from the memoir of
Friedliinder and Krauge (figs. 73, 74, 75), show the degree which
may be obtained by the atrophy. In amputation of the lower
extremity these authors have observed, in addition to atrophy
of the posterior column, a diminution in the size of the posterior
horn, at least in the lower part of the cord, and the disappearance
of a large number of cells from the postero-lateral group of the
grey matter.t With regard to the anterior corwu it was found
to be perceptibly reduced in size in the lumbar region, and the
number of cells in the ganglia were diminished by a half or
two-thirds.

Lastly, the dimensions of the column of Clarke were smaller
on the side of the amputation, and the cells less numerous by
about a fifth. As regards the anterior horn reference must be
again made to the work of Hayem and Gilbert, from which T
borrow a diagram (fig. 76), which shows most clearly the atrophy

+ Ina recent work (Des affections nereeuses centripites consiewtioes « la vection des
necfe +f aur amputations des membres, Acad, Royale de Med. de Belgique, 189)
Vanlair considers the alteration in the nerve roots and cord inconstant. He thinks
that when existing in man they are duo rathor to tho surgical affection which re-
quires amputation than to removal of the limb. As regards the operations which
hho has performed in animals Vanlair summarisos the results obtained by saying
that “on account of their ago and the absence of any pre-oxisting
lesion, adult animals usually escape, to a great extent, the consequences of
amputation ; in them myolitie due to section of the nerves or even removal of the
limbs is usually absent, It is rarely mot with exoopt in bilateral

+ In the obsorvations published in Arch, de Physiol., inh Eapen adele
stated the fact that when the sciatic nerve is torn away in young mata
atrophy of the nerve cells belonging to the group in the intermedio-lateral
ia fonnd to occur.

—

DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 75

from the posterior roots on the same side as that of the amputa-
tion oceurs. This band, of sclerosis, as it may be named, though
it must be confessed that this is a somewhat exaggerated term,
did not occupy exactly the same position in the cervical region
after amputation of the thigh or arm. Though unable to give a
detailed description of what occurs, I shall place before you
aketches of this band of sclerosis at different heights in the cord.

Pig. 7%—Cord in the dorsal region

Fig. 77.—Cord in the lumbar region

from caso of mmputation of tho
left thigh performed twenty years
previously. In the left posterior
column, viz.,on the side of the ampu-
tation, degeneration has occurred ;

corns, which soems to
jcate that it is due to degeneration
the fibres of the posterior roots
ted below the point of section.
reduction in size of the left balf
ord is very pronounced in

from the same case of amputation of

the thigh as fig, 77. In the loft
posterior column (the same side as
that of the amputation) degeneration
has occurred. In the posterior column
on the opposite side thore was also
an islet of degeneration, which how-
ever was less pronounced in degree
and of smaller sito. It will be
observed that the area of degonera-
tion is mach noaror the posterior
median fiswore than in the lumbar
cord, and that it is clearly seated in
the column of Goll. The diminution
of xize on the left side of the cord
is vory pronounced.

There is one point on which I would specially insist, namely,
upon the slight increase of connective tissue in the posterior
column of the side opposite to that of the amputation, in a part
which is in symmetry with the band of sclerosis upon the side

76 LECTURES ON DISEASES OF THE SPINAL CORD.

of the atrophy. This may possibly indicate that some fibres
of the posterior roots upon the side of the amputation have

Fig. 79.—Spinal cord in tho cervical region from the same case of amputation of
the thigh as figs. 77 and' 78, In the left posterior column degeneration hax
occurred upon the same side as that of the amputation, there is also an islet of
dogeneration, which is very slight in degree, in the posterior column of the
opposite side. It will be remarked that these areas of degeneration are much
lous extonsive and elightor in degree than in the sections of the cord in the
dorval or lumbar region, The diminution in the size of the cord upon the left
ride is lees pronounced than in the preceding figures,

Fig. 80.—Section of the cord (cervical region) after amputation of the right arm.
It will bo remarked that two areas of degeneration (the parts of light colonr)
exist, separated by a band of healthy tissue in the posterior column of each
side.

The dogenoration is again most manifest upon the right side, which corro-
sponds to the seat of the amputation.

crossed each other in the posterior column of the opposite
side. Tooth, Oddi and Rossi having made a section of some of

: : =

DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 77

the posterior roots, have also observed degeneration of the
posterior column on the opposite side, ©. Barbacci, on the
other hand, after division of half the spinal cord in the dog and
cat, also observed ascending degeneration of the posterior column
ou the opposite side. ‘These facts seem to agree fully with that
which I am now stating, and it is for this reason that I thought
it right to mention it.

Such, then, are the lesions which may be observed both in
the nerres and spinal cord after amputation. Atrophy of the
corebral convolutions, which has been mentioned by some authors,
does not come within the scope of these lectures.

How can the occurrence of such degeneration be explained
since it is in opposition to the doctrines of pure or pathological
physiology? The central extremity, for instance, of peripheral
nerves is found to undergo the most extensive degeneration,
when neither the ganglia connected with the nerve roots nor the
anterior horns of the cord are affected by the wound, How does
this accord with the well-known law of Waller that a nerve fibre
a ee degenorates when separated from its trophic centre ?

Friedliinder and Krause have attempted to explain this in a
very manner. According to them the degeneration
which occurs at the central extremity of an amputated nerve
involves the sensory fibres alone and consequently those of which
the function is afferent. All the sensory fibres, however, coming
from parts of the limb seated below the amputation do not
degenerate but merely those which terminate in such special
terminal organs as the tactile-corpuscles, &c., the sensory fibres
which have a free extremity not presenting such a change. In
support of this supposition it is stated that if the sciatic nerve
is examined after amputation of the thigh, or of the leg above
the malleoli, the number of degenerated fibres is found to be
the same in the two cases. This, according to Friedlander and
Krause, is dne to the fact that the tactile-corjnecles being
specially numerous in the akin of the foot, the number of nerve
fibres which terminate in this part and are affected by ascending

is almost the same wherever amputation is per-
formed in the lower extremity, so long as it is above the malleoli.

T have myself had the opportunity of examining sections of
the sciatic nerve, both after an amputation of the thigh and a

‘Supramalleolar amputation, and the result of my examination

Mi

es

‘LECTURES ON DISEASES OF THE SPINAL CORD,

ac

was in direct opposition to the statements made by these two
authors. I found, in fact, that in the cage of ampatation above
seen that after amputation of the thigh a very large number
were involved.

being taken into consideration, it is in my
opinion certain that with a case, such as the one which T have
mentioned, before us it is impossible to maintain that the
sensory fibres are alone involved. This would almost amount to
saying, on account of the small number of fibres which escape,
that the sciatic nerve only contains an infinitesimal number of
motor fibres, whilst as you know, on the ee
A large eupply of motor branches.

iTvodelaiatyediiit’that some Gbres aay undergo Walled
degeneration at ta eee
Krause believe owing to loss of function (their terminal
heing destroyed), but in my opinion because this ‘cabal
terminal organ is for a large number of fibres the muclens of
origin, the true trophic centre,* In any case these fibres are
‘not numerous, and an alteration in tem would not explain such
extensive degeneration as that observed in the trunk of the
sciatic nerve.

How then can this divergence of opinion between different
authors, I would almost say with respect to different cases
observed by the same author, be explained? In my opinion
the knowledge which we possess with regard to Wallerian
degeneration is insufficient to solve this question. There is an
unknown connected with it which the future will make clear,
Pethaps the following fact may be taken as a partial explanation.
All the autopsies upon which the history of these degenerations
is based were connected with patients in whom an ampnta-
tion had been performed a long time previously (10, 20, or
30 years), that is to say, at a time when antiseptic treatment
was almost unknown, The stumps of all, or nearly all, the
patients had only healed after suppuration continuing for a
longer or shorter time; in consequence, the extremities of the
divided nerves were exposed to many and various kinds of
infection. Owing to these facts it seems possible that the

* T shall have occasion to explain this statement more fally in discussing the
pathological anatomy of taber,

DEGENERATION OF NERVES AND CORD AFTER AMPUTATION, 81

Pig. 90 ter —Diagram of the tracts of the cord (at the junction of tio cervical
‘with the doran! region) in connoction with the study of skconpaty DEgENER-
ATION.

A, Pinaies UXARLE To AsceNDrvo DEoRNERAT: 1. Fibres of the ascending
wulco-marginal tract, 5, Fibrox of the tract of Gowers. 7, Fibres of the direst
cerebellar tract. §. Pibres of the corau-commisseral tract. 9, Fibres of the column
of Burdock. 10. Fibres of tho external bandiet properly so called. 11, Fibres
Gf the ome of Limever. 12. Fibres of tho treo! of Goll.

B, Frees Ltapie to DescesDinG DRGENERATION: 2. Fibres of the descending
sulco-marginal trect, 3. Fibres of the direct pyramidal tract. Fibres of the
intermediate tract of the antero-loteral cclumn ; some of these aro stray Hbres, and
scattered through the tract of Gowers, the direct cerobellar tract, and, above
all, the erossed pyramidal tract. 6. Fibros of the crossed pyremisul trot
13. Pibres of the comma shoped tract of Schultze

6

82 LECTURES ON DISEASES OF THE SPINAL CORD.

the nerve fibrils, which are thus dissociated, tends to reconstitute
a complete nerve fibre, and it is this tendency which betrays
itself by the existence of many fibrils in the previously existing
sheath of Schwann.

This lecture, in which so many hypotheses have been men-
tioned, must not be brought to a conclusion, gentlemen, without
my reminding you that these have, in every case, been put before
you as mere suppositions which claim, not blind faith, but a fair
examination. Such is my excuse for mentioning them. When
every current theory fails, it is quite permissible to seek the
truth in some other way.

LECTURE VIII.

SPASTIC PARAPLEGIA.”

History of the term sparmodic tabes dorsalis, Erb: Sparmodic spinal paralysis,
Charcot ; Spasmodio tabes dorsalis. ‘dogeneration of tho lateral columns.

Appearance of the face. Awkwardness of movements. Fibro-muscular re-
tractions. Influence of fatigue, of the emotions.

Gent emEN,—In the course of these lectures, which are
specially devoted to diseases of the spinal cord, I shall be more
than once induced to speak of cerebro-spinal affections, the study
of which is more properly connected with disease of the brain
than with that of the spinal cord, Spastic paraplegia is one of
the number, and when the proper time arrives for making this
statement, when the pathological anatomy of the affection is
being considered, it will be recognized that the lesions of the
cord are of quite a secondary nature. At the same time it is in
my opinion right to speak of this disease at the present moment,
and to associate it with diseases of the spinal cord, because in
the instruction given in these lectures it is necessary, in onder
to ayoid the confasion which must otherwise inevitably occur,
to respect the classifications which have been established by long
usage, specially as regards well-established clinical conditions.
‘These slight concessions may be reasonably made as regards
the form of the lectmres, so long as care ix taken to make no
error and to congent to no sacrifice to current ideas, which are
in any way inexact.

Tt is necessary to review shortly the mstory of this disease in
order that the Sei of spastic paraplegia, from a nosological

& Synonyms: spasmodic tabes dorsalis (Charcot . Spasmodic spinal paralysix
(Erb). Congenital Svinte rigidity of limbs (Little). Primary «pastic paraplegia

(Gowers), Spasmodic spinal paralysis (Eustace Smith), Primary Interal scle-
rosie (Gowers). &0.—(Tranelator.)

~

8h LECTURES ON DISEASES OF THE SPINAL CORD.

point of view, may be understood. In 1875 Erb, and a few
months later Charcot, described an affection of which the special
indication was pronounced spasmodic paresis in the extremities,
without loss of sensation, Erb believed. tha that in all probability
this was due to primary in the lateral colamns of
eee Vere sree ‘same opinion with some modifi-

‘his idea as to the nature of apasmodic spinal paralysis

atholo @ singular |
adult patients in i ebm etagwet of ening:
{aden Solan boon made ere wan fd ctber erste
sclerosis, or transverse, or focal myelitis, or perhaps amyotrophic
lateral sclerosis; not a single case in fact occurred in which
primary ion of the pyramidal tract existed which had
been looked upon as the basis of this new morbid condition.

An inevitable reaction followed, and thus during the last
few years the diagnosis of spasmodic tabes dorsalis has been
searcely ever made.

Should this affection be definitely excluded from a nosological
catalogue? Tf a few cases, said to have occurred in adults
between the ages of 30 and 50 years, had alone to be con-
sidered I should be very inclined to answer this question in the
affirmative, since, except in some cases of general paralysis of
the insane (Westphal), primary and isolated degeneration of
the pyramidal tract occurs but rarely in the adult, As Charcot
very truly observed in his lectures in 1880, the existence of
such primary degeneration in cases of so-called spasmodic tabes
dorsalis had received no direct confirmation, Until the present
time the same desideratam exists.

If, however, instead of looking for this disease in the adult,
one seeks it in childhood it is quite otherwise. You will meet
day with « case of this kind. You will see a child suffering
from general rigidity in the four limbs sufficient to embarrass
the movements and impart to them special characters, but with-
out preventing their occurrence. The tendon-reflexes will be
exaggerated ; at times strabismus will occur, speech will be slow,
and the articulation of a spasmodic nature ; some mental change
or alteration in the character may occur, sensation remaining
absolutely normal, and the special senses quite unaffected.

86 LECTURES ON DISEASES OF THE SPINAL CORD.

are usually in a state of semiflexion and forcibly applied to the
trunk “like the wings of a fowl.” The lower limbs, which are
usually the most affected, present a truly strange appearance ;
strictly speaking this may be perceived in all forms of spas-
modic paraplegia, whatever its cause may be, but is rarely so
characteristic as in this disease. When such a condition occurs
the two lower extremities are rotated inwards, and rigid in a

Fig. $1.—Girl suffering from spastic paraplegia (Damaschino collection). She is
unable to stand without assistance, The forced adduction of the thighs,
the internal rotation of the legs with the position of the foot in talipes
oquino-varns will be observed.

state of slight flexion at the hip and knee joints. The two
thighs are permanently adducted in the most pronounced
manner, being often, as it were, fixed together as far as the knees,
whilst below the knees the legs are separated by a somewhat:
large oval space, owing to their rotation inwards. On account
of the attitude of the hip and thigh the back is usually also
more or less hollowed out, and the muscles in the pelvis and
attached to the t anter, and inner surface of the iliac bones

uewhat rigid. The fect usually present, when the motor

ac!

ll

SPASTIC PARAPLEGIA. 87

| ‘disorders are very accentuated, a manifest tendency to assame
the position of equinus on account of the contraction of the
gastrocnemii muscles.

‘The little girl, upon attempting to walk, presents a spasmodic
form of gait which is absolutely typical. She is seen to draw
the point of each foot firmly and noisily along the ground,
earrying it forward, as it were, by a semicireular movement,

Fig. S2.—Same girl as in fg. 81, soem in profilo, Tho attitude of the lower
extremities and the position of the feet in equino-varas will be remarked

the foot which remains fixed forming, as it were, the centre of
the circle. A strong inclination of the body towards the side
“opposite to that of the foot which is raised will be also observed,
an inclination which is repeated in an opposite direction with
each step, and thus determines a pronounced swaying movement
of the whole trunk, which is specially decided at its upper part.
Another special character in the gait of these patients is the
more or less precipitate manner in which they advance, or rather
are pushed forwards. This is entirely due to the production of
a kind of foot clonus when they walk, in such a way that, owing

88 LECTURES ON DISEASES OF THE SPINAL CORD.

to reflex contraction of the calf muscles, the patient is thrown
forwards at each step at the moment when the point of the foot,
as it touches the ground, produces elongation of the correspond-
ing gastrocnemius, which is immediately followed by reflex con-
traction of that muscle. The knees, and even the thighs, are
also seen to be rubbed against each other on account of the
tendency to adduction to which I have alluded, and owing to
the combination of such adduction with inward rotation, the
feet are often found to cross each other, so that a fall seems
inevitable if the patient is not supported. ‘This is often the
case, but some infants are so secustomed to their infirmity that
they become sufficiently adroit to preserve their equilibrium,
notwithstanding the extremely bad conditions in which they are
placed as far as walking is concerned. It is useless to add that
when the disease is severe, walking and leaping are almost
impossible. 4

There is one other attitude in which I would ask you to
observe this little girl, because the difficulties connected with
the position present singular characters. I mean the seater!
position. It will be observed in what a singular way she main-
tains that position, if she can do so at all, since she may be
regarded as nothing but a solid mass upon the stool where we
have placed her. It is certain that the child merely rests upon
the seat by means of the ischial tuberosities, the thighs and legs
remaining more or less completely extended. ‘This awkward
attitude immediately depends upon the rigidity in a state of
flexion of the hip joint of which I have already spoken, a rigidity
which prevents the patient from giving either to her trunk or
legs the inclination which would enable her to remain firmly
seated. It is for the same reason that these patients have still
more difficulty in sitting upon the ground than upon a chair,
since, finding it impossible to flex the thighs sufficiently upon
the trunk, they necessarily fall in the reverse direction. Ib must
be added that in some patients the spasmodic rigidity of the
muscles of the hip and pelvis is so pronounced that they cannot
remain seated upon a chair, and are on that account confined to
the bed.

Tn the upper limbs, although the spasmodic rigidity is less
pronounced, its existence is obvious. The arms are applied to
the trunk, the forearms flexed, the hands in pronation and

- SVASTIC PARAPLEGIA. 80

Fig, 83.—Attitude of a patient enffering from spastic paraplegia, in the seated
position (collection of Chareot). The lege, which cannot be entirely flexed,
reniain half extondod, and hence equilibrium cannot be obtained,

more or less inclined towards the ulnar border, The hands are

often extended, while hyper-extension not infrequently exists.

As regards the movements of the upper extremities it-is easily

understood that, owing to the spasms which occur in the muscles

producing them. they are diffieult to perform, awkward, slow,
and accompanied by rigidity of a special kind; grasping objects,
supinating the hands, throwing anything to a distance, without
mentioning the more delicate movements (writing, sewing, &.),
aré amongst those movements which the patients tind it the most
difficult to execute. There are fortunately a large number of
eases in which the disease is of slight or moderate severity, when
the upper extremities are quite unaffected or but little involved.

‘Thus, in a somewhat large number of cases spusmodic paraplegia

ean alone be said to exist; this is really the * paraplegia spastica

cerebralis " of Heine.

Tt is not in the muscles of the extremities alone that this
spasmodic rigidity occurs. Almost every muscle of the body,
those of the trunk and abdomen being included, may, according

a

“90 LECTURES ON DISEASES OF THE SPINAL CORD.

to some authors, be equally involved. This statement, taken in
a general way, is trae, but an exception must be mare with
regard to some muscles of the trunk, of which the isolated con-
traction is almost independent of the influence of the will, for
in this disease the rigidity specially involves the muscles which
are moat subject to the action of the will, and, on the contrary,
affects but little those of which the movement is more often due
to reflex action through the cord than to voluntary incitement.
It must. be well understood, however, as the following enumera-
tion will prove, that no absolute rule can be laid down upon this
point,

‘The muscles of the front and hack of the neck ave often involved
and slight extension of the head backwards, or spasmodic lateral
deviation are then observed.

Difficulties in degtutition are mentioned by some authors, which
are due to the existence of a tendency to spasm in the pharyngeal
and qsophageul muscles, and resemble the respiratory and
phonic changes known by the name of laryngismus. and which
may be attributed to spasm of the laryngeal muscles.

‘The disorders of speech are also frequent, and when the affection
is severe the speech is slow. drawling, jerky, and seems “to emerge
with difficulty" from the lips of the patient; it must also be
noted that a large number of these small patients are very back-
ward in learning to speak.

Another group of muscles of which the functions are embar-
rassed ix that of the muscles of the orbit. In more than 30 per
cent. of the cases (Meer) strabismus, usually convergentstrabismus,
occurs, Many explanations of its existence havo been given.
According to some authors it is due to a cerebral lesion. Ziehl
believes it to be caused by a simple error of refraction due to
malformation of the eye, and hypermetropia is in fact known to
be one of the most frequent causes of ordinary strabismus. The
researches of Feer on the other hand led him to believe that the
strabismus was due to the existence of muscular rigidity in the
muscles of the orbit, analogous in character to that which oceurs
in other muscles. I myself am inclined to look upon the two
latter explanations as the most satisfactory. Each of them
possibly contains some truth, and not improbably many cases of

strabismus occurring in this disease depend upon the association
two causes.

‘seen, be dy ;

‘the muscles under the influence of passion. Thus
case of a young boy who was very fond of
2 ns, at this time, or when he became angry,
<- eeihagdaaeelisett lh agli lareseincal
‘this being accompanied by a grin which was
Little on his part observed a case of “ risus

emen, almcst the whole muscular system may be
iniuacles, however, being affected to a different:
e} without becoming notably weaker it is the inability

but at most pseudo-parésis
Days ahis Oech ti te oo rach oer
while at times the more prominent muscles become
enlarged, and a true idea of the illness will be

tion: of w eimilar Kind may be aleo observed in the.
of the orbit when strabismus exists.

the principal disorders connected with motion. It
interesting fact that the tetanic rigidity of the limbs
hes during or even after simple rest; while on the
‘it is increased by fatigue or strong moral emotions

mu |

LECTURE IX,

SPASTIC PARAPLEGIA (continued).

Excesk of tendon-reflex. Foot clonus. Normal cutaneous reflex action.
retained,

Congenital onaot, often unperceived bythe parents, Such children
very buckward in walking. heir intellectual development usually enables
ad ‘to live mubsoquently in the same way as others. Nature of the affection.

‘True spustic paraplogin must be distinguished from the tabid-spaxmodio
conditions ; clinically the first is not accompanied by epileptic attacks, and
‘very rarely by montal chango ; anatomically it ie duo to a failure in the deve-
fia Be of the pyramidal tract, and not to its destruction ; wtiologically it is
specially due to premature birth. Diagnosis from infantile spinal paralysis,
tetanus of the newly born, tetany, Thomsen's disease, the disease of Pott,
transverse myelitis, insular sclerosis, «pastic infantile hemiplegia, Hyeteria.
‘Treatment: methodical education of the limbs, gymnastics, massage, teno-
tomy.

GerNTLEMEN,—As corollary to the study of the muscular
rigidity which has been described in the preceding lecture the
character of the tendon-reflexes must now be considered, since
in the present state of our knowledge it is by them that we are
able to distinguish whether a motor disorder is or is not of a
spasmodic nature, In this little girl all the reflexes are con-
siderably increased, the knee jerk is more pronounced, and
muscular contraction occurs when the tendo Achillis, the tendon
of the biceps or triceps in the arm, or the tendons at the
wrist are strack. ‘The deep reflexes are so much increased in
this patient that the so-called periosteal reflexes occur, pro-
nounced muscular contraction taking place not only when the
tendons, but when the bones, specially those of the forearm, are
tapped. The distinct occurrence of foot clonus should be
noted in connection with these facts, a phenomenon which, as
Pitres and de Fleury have shown, though not in every way
analogous to the tendon reflexes already considered, may in all
eases be looked upon as a distinctly spasmodic manifestation,

4 LECTURES ON DISEASES OF THE SPINAL CORD.

professions. At the same time, the intelligence of some patients
is but moderate in degree, although in every case it is greater
than at first appears. If the intellectual condition, however, is
unaffected, it is by no means the same with the character: this,
in fact in almost every case, becomes most capricious and odd;
the patients are usually irritable, violent, disagreeable, and
impulsive, being also timid and sly; in short, the character
responds to the description which may be given of the mental
condition of those in whom general degeneration occurs, of
which these patients at times present indications. It is uncer-
tain whether this stamp of degeneration belongs to them, owing
to hereditary transmission, or simply depends upon the affection
from which they suffer, which, attacking them in early infancy
at the time of their development, must necessarily produce in it
the most pronounced alterations,

So far, I have carefully placed before you the example of a
typical case of spastic paraplegia showing how, in my opinion,
the affection should be understood. It must now be stated
that the disease does not by any means always present these
characters ; attenuated and even abortive forms are not uncom-
mon, and may be said, in fact, to be even more frequent than
those which are typical. In these cases motion is not so
extremely difficult as was found to be the case in the patients
already considered ; a certain degree of more or leas pronounced
rigidity exists in the limbs, which, however, is insufficient to
cause much inconvenience; at times, indeed, the rigidity is so
slight that it would only be observed by a careful and experi-
enced eye. In almost every case the lower are more affected
than the upper limbs; and often in the mild cases are alone
involved, slight awkwardness of movement being then alone
observed in the upper limbs.

It must be remembered that the tendency of this disease is
to improve during its course; thus when in infanta the four
limbs are involved to a more or less pronounced extent during
the first years of life, the motor disorders progressively diminish
and the upper limbs become almost completely sound.

As regards the onset of the affection, since it dates from the
time of birth, nothing need be said about it from a clinical point
of view, Atthe same time, as you would expect, it is not during
the first days of life that the diagnosis is made; this usually

ll

| SPASTIC PARAPLEGIA. 05

occurs at a later date, as at the end of a few months when the
Le agplp spec npatalpt tate sc!
Thus when he is bathed, instead of kicking his legs in
every direction, as healthy children do, he keeps them tightly
___ pressed one against the other, without ever completely flexing
} cating en, or at a still later date, when he is eighteen
b months or two years old, they are astonished not to see him
walk, It is the rule in fact, gentlemen, in this affection that a
long time elapses before the little patients make the first step,
which they succeed but imperfectly in doing, and another long
period passes before their attempts to walk are crowned with
success. In some cases one of the symptoms which points to
the existence of this affection is the inability of these patients to
remain in a seated position for reasons already mentioned. The
parents alarmed by these signs of evil augury then determine to
, consult the physician. It is not surprising therefore, as has
heen already observed, that these little patients are so often
seen for the first time at a somewhat long period after the time

of their birth.

These observations may be made about the onset of the
disease, while subsequently, as you know, the spasmodic rigidity
May in some cases diminish or even entirely disappear. You
will ask me, however, what becomes of these patients, and what
their subsequent existence will be? Except in the case of those
who are quite infirm, their existence will not be different from
that of others. They may certainly be occasionally in worse
cireumstances at college, in the same way as is the case with
any whose moral and physical development is below the average,
‘and during life their infirmity is often enough the cause of
some disappointment: the eccentricity of their character may
Imcrease. With these exceptions the life of such children is of
the ordinary kind; they join the same classes as other children
of their age, pass through the same examinations, and belong
subsequently to the same professions, those cases alone being
excepted in which some special apparatus is required in order

| that the muscles may be used. They grow in the same way as

other children, the structures and functions of the body develop
dn the same manner, and puberty is reached in the same way as
that of their companions. Subsequently they marry, and their
children are quite free from the disease which affects them,

x

a
96 LECTURES ON DISEASES OF ‘THE SPINAL CORD,

spastic paraplegia as you know not being a hereditary disease.
‘The duration of their life is of good length, and quite inde-
pendent of the illness from which they suffer.

I haye dwelt upon these facts in order that you may not
forget that spastic paraplegia, which so far I have only put
before you as it occurs in children, exists also in adults since the
affected children reach the adult age. For the same reason
it may be observed in old age, though the fact must not be
forgotten that it has existed from the earliest years of life.

‘These being the clinical characters of the affection, what is
its pathology? what position should it occupy in nosology ?
what is its true nature? This is by far the most difficult
question connected with the disease, and as I explained at first
totally different opinions haye been held upon this point, which
is far from being settled,

The cause of this difference is, in my opinion, the attempt
which has been made to unite things which ore quite dis-
similar. If in fact it is true that an affection, sui generis, exists,
which so far we have alone had in view in the description given,
it is equally certain that other very analogous conditions may
be observed of more or less general musenlar rigidity, These
conditions present almost exactly the same characters as those
which have just been mentioned, but are usually associated with
more or less pronounced disorder of the intelligence, and some~
times with attacks of epilepsy. It is also in these cases, for which
I propose the name of tabid-spasmodic cowlitions, that the mal-
formations of the skull are found, which are mentioned by many
authors. We will first: consider the etiology of these different
morbid conditions. It is to Little, who initiated this branch of
clinical study, that we owe the knowledge of two most important
facts connected with the stiology of these spasmodic conditions:
premature birth, or the existence of « severe labour, Such, gentle-
men, are in fact the two great causes of the morbid conditions
*which we are considering, and to these must be added the
tngfluence of certain tnylammatory diseases of the futus or newly-
horn child, which give rise to lesions in the meninges or brain.

The ordinary lesion in every ease is non-development of the
pyramidal tract throughout its whole length ; it should be noted,
gentlemen, that I do not say its degeneration. We have already
mentioned as one of the characters of this affection the fact of

lll

SPASTIC PARAPLEGIA. 07

its being emyenital, Now it is known that the development of
the sheaths of myelin in the pyramidal tract: takes place at a very
late date, viz., at the end of the 9th month of foetal existence,

and that the pyramidal tract is not completely developed until a

still later date; it is not, therefore, surprising that when a child
is born before the proper time, as in the 7th or 8th month for
instance, the development of this tract is more or less completely
arrested. Again, in cases of severe labour with long duration,
when compression of the head occurs during a somewhat con-
siderable length of time, instruments being often used, it is
easy to understand that the brain may be injured. Whether,
as Little supposed, hemorrhage from the capillary vessels
occurs in the brain and spinal cord owing to the manual
operations rendered necessary by the labour, and accompany-
ing asphyxia, or from the occurrence of some other change
is uncertain. We may, however, join Ross and Feer in
remarking that in the brain of newly-born children, which is so
soft as to be almost diffluent, injury or pressure, wherever they
occur, necessarily affect most severely the part which has the
least power of resistance. This is the pyramidal tract, the part
which is the last to develop, and in which the sheath of myelin,
being as yet unformed, cannot protect its fibres in the same
efficient way as those in other parts of the cerebro-spinal
axis.

As regards the third canse mentioned, viz., the oceurrence of
an inflammatory affection in fortal life, or during the firstfew days
after birth, and which gives rise to meningitis or encephalitis,
which may or may not be followed by porencephalia, and in
every case much interfering with the development of the
pyramidal tract, I confess that I much hesitate to give it
a place here. It is certainly true that these cases are often
accompanied by spasmodic rigidity similar to that which has
been described as characteristic of spastic paraplegia, but they
usually present also special symptoms which clearly distinguish
them from the cases which are due to premature labour. These
symptoms are, on the one hand, intellectwal change, which may
reach complete idiocy; on the other hand convulsive attacks,
which have been often noted, and which in my opinion
opposed, however, to that of Feer, are merely due to epilepsy.
Tn cases due to premature birth, which I have exclusively taken

7

98 LECTURES ON DISEASES OF THE SPINAL CORD.

as the type of my clinical description of spastic paraplegia, a
fact which I consider important, no notable intellectual change
or epilepsy exists. This difference in the symptoms presented
justifies, in my opinion, a complete separation between the two
affections,

As regards the cases which are due to severe labour I am not
equally certain, nor can I say whether intellectual change and
epilepsy are of frequent occurrence, or rare, or completely absent
in these patients, and am therefore unable to decide whether,
from a symptomatic point of view, they should be placed with
those due to premature labour or not.

I chould thus be inclined to isolate all the cases in which it
is most improbable that any arrest in the development of the
pyramidal tract occurs, cases which notably differ, as we have
seen, from a clinical point of view, owing to other symptoms of
extreme gravity (intellectual change, epilepsy) being associated
with the disorders of motion. I should give the name of true
spastic paraplegia to the cases which are due to premature
birth, with arrested development of the pyramidal tract, while
the other eases might be termed “ fabid-spasmodic conditions”
whether due to injury at birth, or to destraction of the motor
region of the brain by an inflammatory disease (the most often
infectious).

‘The advantage of making this distinction is that no confusion
then exists between two groups of affections which differ from
each other in many respects. As regards pathological: anatomy,
one of the groups, that of the “ tabid-spasmodie conditions,”
consists of the destruction of a portion of the brain by
encephalitis or meningitis, destruction of which the seat is not
fixed, and which may only occur at the origin of the pyramidal
tract, or involve other portions of the brain as is usually the
case, This explains the frequency with which intellectual
change occurs in this form of disease, being also the cause of the
convulsive attacks, since, owing to its inflammatory nature, the
lesion constitutes, as it were, a thorn within the brain, whose
irritative tendency shows itself at a later date in the attacks of
epilepsy. ‘The other group, represented by one disease alone,
viz., spastic paraplegia, is simply an arrest of development,
exclusively affecting one system of fibres. It is, in fact, essentially
a systemic disease, and it is on this account that the name of

:

diseases are, having in common but one symptom, the congenital
spasmodic rigidity, This should be regarded as a mere coinci-
dence, it being remembered of how many different affections
another symptom, hemiplegia, is the indication, and it must be
admitted that spasmodic rigidity has by no means the same
meaning in all eases, and that these must therefore be necessarily
separated from each other.

‘The varieties of muscular rigidity haying been distinguished,
the praGyosis of the affection must now be made by comparing
with it other affections which resemble either spastic paraplegia
or the tabid-spasmodic conditions.

Infientile paralysis has in reality no characters in common with
this affection since in it the muscles are essentially flaccid,
whereas in this disease the muscles are in a state of spasm.

Tetanus neonatorum need not be discussed at length, bearing
but little resemblance to spastic paraplegia; it will also be
remembered that it begins in the face and is rapidly fatal,

‘Tetany is a contracture with varying manifestations which do
not resemble the spasmodic rigidity which has been so fully
discussed in this lecture.

Thomeen's disease might possibly give rise to a mistake sinee
in this affection also there is muscular rigidity which may incon-
yenience the free use of the limbs. It will be remembered,
however, that in Thomsen’s disease the rigidity only exists at
the commencement of voluntary movements, whereas in spastic

the reverse is usually the case. Again, in Thomsen’s
disease what is so characteristic of the affection we are now
studying, viz., the tendency to permanent spasm, the great
inerease of the tendon-reflexes and foot-clonus are notably
absent.

‘The only affections which in my opinion it may be difficult to

from this disease are those of which the symptoms

include the spasmodic features which are invariably present in

Spastic paraplegia, Amongst these affections the first to be
considered ave :—

Jnagahege the different varieties of compression of the

are distinguished by their course, by not being

SPASTIC PARAPLEGIA. 101

closely resembles that existing in spastic paraplegia, but which
is really due to hysteria alone. These cases, when once known
to occur, may be easily recognised by the facts that the tendon-
reflexes are much less, if at all, increased, that most often the
sensibility is also more or less deranged, and these combined
with the other characteristic symptoms of hysteria enable this
disease to be recognised.

The treatment which should be employed must now be con-
sidered ; that which I would recommend, though apparently not
of much energy, will undoubtedly produce appreciable results,
‘The knowledge which we have of the nature and lesions of spastic
paraplegia enables us to know that we cannot act directly upon
the spinal cord, and that the cautery, blisters, heated points,
and other counter-irritants, are as useless as they are painful.
‘The development of the pyramidal tract cannot be reetified. At
the same time, the fibres of the pyramidal tract whose evolution
is slightly incomplete may be made to act in a proper manner.
With this object methodical education of the limbs, gymnastic
exercives, massage, and pussive movements with or without pre-
ventive tenotomy will be your chief assistants in the treatment.
Should any doubt exist as to the value of such treatment, or the
mode of practising it be unknown, it may be seen in the practice
of Dr. Bourneville at the Bicétre, that, by means of this method
carried on with perseverance and devotion, much improvement
may be obtained in cases of spasmodic cerebral pseudo-paralysis,
even when complicated by more or leas pronounced idiocy.

102

, Ordenstein, Bournerille
Naa acesaseeeeh peers Syaproms: 1. Sivan. A. Motor. Disorders in
the gait, trno xpastic gait, pare cerebellar guit, corobello-spastic gait) their
characters. Spastic paraplegia, A of

root of the limbs, bt often also those of tho trunk and neck, and may pre-

dominate upon ono side of the body,

GENTLEMEN,—It will certainly haye been observed that at the
beginning ofeach lecture devoted to the study of a disease, I am
careful to mention the names of those to whom we should be
grateful for having discovered, or described, or simply studied it
better than their predecessors, for having, in fact, in some way
increased our knowledge. ‘These names are usually few in
number, since by no means all the works written about the
disease are connected with its history, and it is a historical account,
and not a bibliographical index, that I wish to give you at the
commencement of the chapters. Another reason, on account of
which this enumeration may appear small, is that I reserve for
myself the power of indicating, as the lectures continue, the
most recent works and the progress for which we are indebted
to them. It will not, therefore, be surprising if at times the
history of a disease is almost entirely limited to what may be
called the * heroic periods of neuro-pathology.

Such, with your permission, will be the case with regard to
insular selerosis and other affections which will be considered
by us.

‘The lesions of insular sclerosis are undoubtedly seen in
Cruveilhier's admirable atlas of pathological anatomy, in that
of Carswell and in the treatise of Rokitansky. Many obser-
vations upon this affection were published by Tirck, Frerichs,
Rindfleisch, &e., but it was only regarded by them as a patho-
logical curiosity and the account of the autopsies was alone given.

L

INSULAR SCLEROSIS. 103

Tt is Charcot and Vulpian who deserve the honour of having
given to insular sclerosis the diguity of an anatomical and

thesis of Ordenstein, inspired by Charcot, was written in 1867 and
lastly in 1869 the memoir of Bourneville and Guerard appeared,
4 monograph of real value, since which time insular sclerosis
has had a real existence. The works which have been recently
written will be mentioned in the course of these lectures,
What are the Systrroms of the affection? These are usually
divided into spinal, cerebral, and bulbar symptoms. This mode
of arranging them is undoubtedly the most convenient and will
be employed in these lectures, but it should be stated that this
is merely an artificial proceeding, and that with regard to many
symptoms it would be difficult to say in what part of the neryous
ee they originate,

The spinal symptoms will be first considered and studied in the

following order :—
I. SPINAL SYMPTOMS.
A. Motor Syrroms.

‘Amonget the disorders connected with motion the disorder
of the gait must be specially mentioned, This assumes various
forms which are quite distinct from each other, and of which the
principal are the following :—

a, The form which is clearly spustic in character,

B. The form which is purely cerebellar.

vy. The cerebello-spastic form.

a, The first of these forms, which is clearly spastic in character
consists of more or less pronounced paraplegia with tonic con-
traction and strongly marked rigidity, in short, of the same
spastic gait which has been already described as occurring in
spastic paraplegia and which will be also found to exist in other
diseases. It will suffice to recall the principal features of this
condition; the legs are extended and pressed against each
other and the feet seem adherent to the soil so that the patient
finds it extremely difficult to walk or to bring forward the foot
which was previously behind the other. Since the rigidity
of the leg prevents flexion of the knee the progression of

104 LECTURES ON DISEASES OF THE SPINAL CORD.

each foot is accomplished by elevation of the corresponding half
of the trunk and pelyis, and even then the foot is rubbed against
the ground with such force that a noise is produced which alone
enables the character of the gait to be recognized from afar off.
To these phenomena the other so-called “ spastic” symptoms
must be added. hese specially consist, as you know, in
increase of the tendon-reflexes (patellar tendon, Achilles tendon),
and foot-clonus, the latter being often so pronounced as to occur
spontaneously whenever the ball of the foot rests upon the
ground during the patient’s efforts to walk, to which this con-
dition presents an additional obstacle.

In the purely cerebellar form (8), which certainly but seldom
oceurs, the appearance is quite different: what predominates is
the uncertainty of the gait and the loss of equilibrium, while
the movements are all free and the joints can be used without
difficulty ; the direction, however, given to the limbs is doubtful
and uncertain, while the gait is unsteady, resembling that of an
intoxicated person. In this form the tendon-reflexes are not
excessive, being, on the other hand, somewhat diminished.

The cerebello-spastic form (ry) is by far the most frequent: and
characteristic form of insular sclerosis. As its name, proposed
by Charcot, indicates, the characters of the cerebellar and spastic
gait are associated together. The appearance of the patients “is
as follows: the feet are separated in the same way as those of
seamen upon the bridge of a vessel, and the ball of the foot is
not placed alone on the ground as in the purely spastic form,
but the whole sole of the foot, and specially its posterior part.
Hence the heaviness of the gait and the noise produced by the
heels are explained since the patients do not place, but strike
their feet against the ground. Their steps are irregular, the
irregularity showing itself indifferent ways. As regards rhythm,
length, since the steps may be longer or shorter, and direction
which never remains absolutely the same, these patients walk
in such a reeling manner as to present the appearance of an
intoxicated person. These “cerebellar” phenomena are still
more pronounced when the patient attempts to stop suddenly,
or to turn rapidly round. ‘The spastic phenomena are shown by
the fact that the feet seem glued te the ground, on account of
the difficulty with which the legs are moved, while the tendon-
reflexes are more or less in excess, and foot-clonus often exists.

|

105

INSULAR SCLEROSIS,

= = & & Ss

rection of their

"The
at Rope nnd they d
the d

different,

tho foot varies in

it.
Sees

cin

106 LECTURES ON DISEASES OF THE SPINAL CORD.

Such are the different aspects which the gait of patients
suffering from insular sclerosis presents; it is obvious that the
description which 1 have just given will only show in what way
this symptom should be observed, and does not by any means
include all the cases which may be seen; many forms of tran-
sition in fact exist and different associations.

Thas, for instance, walking may be much embarrassed by the
existence of a general tremor which exists throughout the
whole body and renders the maintenance of equilibriam almost
impossible. Oppenheim, in an interesting work upon the dis-
orders of the gait in insular sclerosis, regards these cases as
belonging to a distinct form which he terms ‘‘vacillating”; I
should myself prefer, in order not to multiply indefinitely
the number of forms, to consider this tremor merely ns a
phenomenon added to one of the forms already deseribed;
this, however, is merely a question of detail.

It must not be thonght that disorders in the gait are the
only symptoms observed in the lower limbs. Their movement
may be affected in every way, so that true paraplegia exists,
which obliges the patients to remain in bed. Extensor
spasm of the two lower limbs is usually associated with the
paraplegia, this being in fact but a more pronounced degree
of the disorder which we haye just studied as the “spastic”
gait.

As regards the upper limbs a condition is often observed, not
of paralysis, or spasm, but of spastic paresis, which often pre-
sents itself in the form of extreme awkwardness of movement,
an awkwardness to the existence of which the tremor, which we
shall study presently, largely contributes.

‘The motor disorders of insular sclerosis would again be but
imperfectly understood if the hemiplegia which is often observed,
and has peculiar characters, was not mentioned,

The hemiplegia which occurs in insular sclerosis was mentioned
insome of the earliest observations regarding the disease ; Charcot
nsed to call the attention of his pupils to this symptom, and I
have myself had the opportunity of seeing several examples of
the condition in his service, of which one was extremely
pronounced, and will be found described in the Thesis of
Babinski. The latter author as you know devoted an important
chapter in his Thesis to the description of this symptom, but the

INSULAR SCLEROSIS. 107

fullest description of hemiplegia occurring in insular sclerosis
is undoubtedly found in the Thesis of Miss Blanche Edwards.
‘The conclusions at which onr distinguished colleague arrived
show that this symptom not uncommonly exists in the disease ;
that it usually occurs after an attack of apoplexy; that such
an attack may take place two, three, four, or more times in the

same person, and may or may not be accompanied by aphasia;
Jastly, that the hemiplegia in insular sclerosis is usually a
transient symptom, and may completely disappear.

_ As regards the anatomical changes to which this hemiplegia
‘is due, they are as yet unknown, and the course of the symptom
alone enables us to recognize that the fibres of the pyramidal
tract are not destroyed by the lesion, and that no secondary
degeneration is associated with it.

To give an idea of the frequency of its occurrence, I should
say that I myself found it to exist three times in thirteen cases
of insular sclerosis in which I searched for it. ‘The hemiplegia
tay affect the limbs alone, or the limbs and lower part of
the face simultaneously ; more rarely crossed hemiplegia occurs,
Tastly in some cases the face is alone affected, the muscles
supplied by the lower branches of the portio dura being alone
paralysed. The nature of the hemiplegia will be discusged at
a future time, and you will see, gentlemen, that most often it
is of hysterical origin.

The tremor, which is one of the most frequent and character-
istic symptoms of insular sclerosis, must now be studied, of
which the clinical analysis made by M. Charcot may be
considered a model of its kind.

With your permission this symptom will be considered in
connection with patients suffering from this disease who will be
put before you.

The present attitude of these patients does not show any
tremor to exist; they are seated quietly in their chairs, and no
movement or effort is being made. This in fact, gentlemen, is

‘one Of the characters of the tremor in insular sclerosis, that it
does nwt ocour while the patient is at rest; upon drawing one of
the patients forward so that he does not lean against the back
of his chair, the upper part of the body is at once seen to
‘osillate. It is however specially during voluntary movements
that the tremor occurs, as Charcot has shown, and hence it is

~

108 LECTURES ON DISEASES OF THE SPINAL CORD.

termed “intentional tremor.” The phenomenon will be again
witnessed if one of the patients is asked to take the glass which
ig upon this plate and carry it to his mouth. The hand seizes
it somewhat abruptly so that the liquid contained in the glass
is shaken ; the glass is then raised from the plate, and let us
examine the progress which it makes :—

The oscillations of the hand are at first small and slow, but.
as the glass approaches the mouth increase both in size and to
a smal] extent in rapidity; the water in the glass is agitated
and tossed in every direction, the face and clothes’of the patient
are drenched, and those near the patient must stand aside, or

Fig, 88.—Writing of a patient suffering from insular acleronis (service of M.
Charcot),

will suffer in the same way. It is now when the hand has
nearly reached the mouth that the tremor becomes considerably
increased, and the oscillations of an extraordinary size [20—30
centimetres (5—7 inches), or more] and far more rapid ; it will
also be observed that the upper part of the patient's body which
had inclined forward to meet the glass trembles in a similar
manner to the hand, so that the head is moved by a series of
rhythmic salutations,

An audible sound is produced when the mouth and the glass
meet, and this explains how it is that at times the teeth of the
patient are broken when he attempts to drink. It will be
noticed, however, that in this case the glass, displaced by the
oscillations of the hand, has escaped from the lips, which were

le to seize it, and carried away by its uncontrollable

eV"

INSULAR SCLEROSIS. 109

movements resembling those of a pendulum, strikes at one time
the nose, at another the chin; wearied by his efforts the patient
has seized the right wrist with the left hand, and thus fixes the
right forearm strongly against the chest and prevents it from

whilst at the same time he carefully keeps the glass
in place by means of the lips and teeth, and thus with the head
inclined forward succeeds in drinking a small quantity of the
liquid which remains therein.

‘Now that the patient is at rest again there is no longer any
evidence of tremor, this only occurring, as already explained,
when voluntary movements are made, Some conditions increase
this phenomenon, and these should be known that it may be
understood how to produce tremor in a patient suffering from
this disease. These conditions are: 1. The extent of the move-
ment; thas, when a patient is directed to take hold of an object,
‘as was done in this case, he should be placed at some distance from
it in order that he may be obliged to extend his arm. 2. The
existence of emotion; thus, the idea of being observed, or if the
glass presented to them contains a liquid, the fear of upsetting
it suffices in these patients to considerably increase the tremor.

It is fonnd, when the tremor itself is studied, that from the
nature of its rhythm it belongs to the tremors with moderate
rhythm (those in which from 54 to 7} oscillations occur in
a second). This fact, as I observe, gentlemen, surprises you

the moyements in insular sclerosis seem to be far more rapid
than they really are, the cause of which is that the movements
are of somewhat large size. A simple experiment will prove
this: in the left hand I hold a ruler 30 centimetres (1 foot)
in length, in the right hand a rod 1-5 metres (5 feet) long,
and with both of these I strike the table with equal intervals,
paanown by the sounds produced, the only difference is that the
of the ruler is moved a much shorter distance
than that of the rod. Upon watching the two instruments the
movement of the rod is seen to be very much more rapid than
‘that of the ruler (since its size is greater), although in reality
‘the number of oscillations occurring in the two instruments is
5 the same. This, gentlemen, is what occurs with

ot to the tremor in insular sclerosis, but a moderate number

110 LECTURES ON DISEASES OF THE SPINAL CORD.

of oscillations occar, though they are of such a size as to give at
firet sight the impression that they are very rapid.

To what is the extent of these movements due? To this fact,
that the tremor in insular sclerosis is not limited as in many
cases of tremor, to the extremity of the limb, or to the muscles
of the forearm, the flexors, and extensors of the wrist, but, as
will be seen, affects almost all the muscles of the body ; it may
almost be said that it is specially seated in the muscles of the
trunk and in those of the scapular and iliac girdles. Since, on
account of having this position the tremor principally occurs
near the root of the limb (as in the shoulder and elbow joints),
you will understand, gentlemen, that its extent would be there-
fore multiplied by the whole length of the lever which the arm
aoe constitute for it, and becomes thus of considerable

Such, gentlemen, are the observations which I wished to
make with regard to tremor in the upper limbs. As you know,
the tremor is not confined to these, but occurs also in the lower
limbs, where it is comparatively but little pronounced. The
trunk is also liable to move violently forward or backwards when
the patient places himself in an upright position, or attempts
to walk, or even when he is seated, or in bed if insufficiently
supported. Some patients in whom the movements .are very
pronounced must be “steadied” on every side before they dis-
appear, The neck and head also move forward and backwards
when an effort is made to stand in an upright position, or to
walk, or, as was just observed, when the patient carries some-

to the mouth, In very pronounced cases the tremor in
the head tends not only to occur in an antero-posterior direction
‘but to produce, as it were, a movement of circumduction.

Tn some observations the tremor has been said to be unilateral,
This, however, is rare, although it is often found to predominate
on one side of the body, so that when this symptom is examined
care must invariably be taken to examine the hands upon both

112 LECTURES ON DISEASES OF THE SPINAL CORD.

sense of constriction round the trunk may exist quite analogous
to that which occurs in tabes.

The objective disorders consist, according to Freund, in
abnormal tactile sensibility, a sensation of compression, analgesia,
Aypalgesia or hyperalgesia ; various forms of ancsthesia may also
be observed, disorders of the sensibility to temperature, or muscular
senae, the latter being more rare; in some cases hemi-«nesthesia
has been noted.

‘The sensory disorders are specially seated at the extremities
of the limbs, viz., in the fingers and foes; they are but slightly
eaten: of temporary duration, and liable to change, haying

been permanent in six out of thirty-three patients suffering
Soe tene ceeds (heat,

‘These facts will not be discussed at present, since the relations
between insular sclerosis and hysteria will only be considered
at alater period. At the same time I would repeat. what should
notwithstanding this be kept in mind for daily use, the idea
first mentioned by Charcot, that sensory disorders do not form
port of the clinical features of insular sclerosis, I would not
certainly deny that they may occur, but in my opinion this
happens so rarely that they should merely be regarded as a
clinical curiosity.

B.—SreciaL Senstawuiry.

Different changes have been noted in hearing, notably a
diminution of this function which may increase to complete
deafness. ‘The same may occur as regards faste and smell, but
the troubles of these senses are in reality but rare and seldom
at all pronounced in degree. As regards sight it is quite other-
wise; the disorders are frequent, often much accentuated, and of
great importance as regards the diagnosis of the disease. These
symptoms will therefore be studied in detail. To facilitate this
e ing connected with the organ of vision will be now
considered, its motor functions being included.

The principal symptoms nasociated with the visual finction
have been long known, and are mentioned in the ordinary
manuals connected with this subject. During the Jast few years,
however, attention has been again directed to these symptoms,
and to this we owe the important works of Parinaud, Gnauck

_

INSULAR SCLEROSIS. 113

and Ubthoif, works from which I shall largely quote in the
which Tam about to make.

Almost all the structures of the organ of vision may be
notably affected in insular sclerosis.

Amongst the disorders there is one which is specially impor-
tant a3 regards the diagnosia of the disease, namely Nystagmus,
which consists as you know, gentlemen, of rapid oscillations
of the eyeball produced independently of the will of the patient
and usually without his being ‘aware of its occurrence. The
movement always oceurs in a horizontal direction from left
to right and vice versa; Uhthoff, however, states that he once
saw it occur in a vertical direction, and in two cases seen by
the same author the patients believed that they saw objects
more.

Nystagmus is sometimes so pronounced as to be observed at
ouce when the attention is fixed on the eye, but at other times,
is scarcely perceptible and must be carefully sought.

To do this the eye of the patient should be placed in o
strained position, as for exanyple by his being directed to look at
the finger which is placed at some distance on the outer or
inner side of the eye, without turning the head so that the eye
is as near as possible to the outer or inner angle of the eyelids.
‘The eye being thus placed in a “strained position” the rhyth-
mical movements will be perceptible which cannot be seen in
the ordinary position of the eye. This artifice should be
employed in such cases, upon the utility of which Parinaud
thas justly insisted for many years in his conferences at the
Salpetriére Hospital.

Uhthoff distinguishes two varieties of ocular movement :—
1. those which belong to trve nystagmue, and are directly due
to s lesion of the brain or medulla oblongata; 2. the so-called
uyxtagmoid oscillations, which are not true nystagmus and only
exist in strained positions of the eye, being due to a condition of
slight paresis, involving the nerves of the ocular muscles. In
100 cases of insular sclerosis this author found true nystagmus
in 12, nystagmoid oscillations in 46 cases, that is to say,
thythutical movements were found to occur in 58 per cent. of
the cases. These numbers must be accepted since I know of no
others to compare with them, bub at the same time I must
acknowledge that the number appears to me somewhat too small,

8

114 LECTURES ON DISEASES OF THE SPINAL CORD.

and my own impression is that from 70 to 80 per cent. are thus
affected, since this is one of the symptoms most commonly
observed in insular sclerosis.

Other disorders may occur in the action of the ocular muscles
in this disease, viz., paralysis of the muscles of the orbit. Though
not as frequent as nystagmus this symptom is also of great
importance as regards the diagnosis of the disease (it occurs 17
times in 100 cases—Uhthoff), The condition is far more one of
paresis than of true paralysis, and the paresis is partial, incom-
plete, and transitory, which causes it to have a characteristic
nature. External ophthalmoplegia (paralysis of all the muscles
of the orbit) may also occur, but the 6th nerve is principally
affected in this disease, either on one or both sides, as is also
the 3rd nerve.

According to Parinaud the motor disorders of the ocular
muscles in insular sclerosis almost exclusively consist of par-
alysis or paresis of associated muscles, that is to say, both eyes
are simultaneously affected, and the muscles which act together
in producing a certain movement are involved.

Nystagmus is essentially of this nature. It is a tremor which
indicates paresis of such associated muacles. The extent of the
muscular movements is invariably found to be diminished when
the nystagmus is pronounced. The lateral movements are
usually affected, but all the muscles may be paralysed, and
more or leas pronounced external ophthalmoplegia then exists.
According to the same author paralysis limited to three pairs of
nerves, such as occurs in tabes and syphilis, is on the other hand
very rare.

The natural consequence of such ocular paresis, if sufficiently
pronounced, is double vision, which, however, is most often
transitory, only occurs in certain positions of the eye, and
corresponds, in fact, to the ocular paresia which produced it.
Another consequence of this paresis is that the appearance of
the patient is often characteristic, haying been well described by
Charcot under the name of “ vague expression.”

The action of the iris may be disordered in different ways (11
per cent. of the cases, Uhthoff). Thus myosis, inequality of the
pupils, or a diminution of its action under the influence of con=
vergence or light may occur. Parinaud specially insists upon the
fact that a nervous affection in which myosis with retention,

INSULAR SCLEROSIS. 115

and perhaps excess of the light reflex, as regards the rapidity of
its production, exists, should be suspected to be insular sclerosis
and not tabes.

‘The condition of the fundus oculi and ophthalmoscopic ex-
amination of the optic disc must now be considered. In the
first descriptions made by Charcot the existence of atrophy of
the optic nerve was noted in certain cases. This observer also
noted the fact that such a change did not always occur, notwith-
standing the existence of pronounced amblyopia, and that the
lesions in the dise never corresponded in intensity to the loss
of sight. Since this time Gnauck, Uhthoff, and the principal
ophthalmologists who have considered this question have men-
tioned these facts in detail, and made interesting deductions
therefrom.

The changes in the optic papilla may, according to Uhthoff,
be referred to three different degrees of alteration :—

A. There is atrophy and complete decoloration of the
papilla, the reddish reflection of the normal papilla having
entirely disappeared.

B. There is incomplete decoloration of the whole papilla,
the internal portion of which still preserves to a certain extent
its red reflection.

C. The external, temporal, part of the papilla is alone dis-
coloured, the tint of the internal portion being in every respect
normal.

D, There is the appearance of frue optic neuritis (Parinaud,
Uhthoff, &e.), hypersmia, cloudy appearance of the papilla,
vessels concealed and dilated, papilla prominent, &c. This
condition perhaps is merely one of the first phases of the process
which leads to atrophy and decoloration of the optic papilla.

As you see, gentlemen, the changes in the fundus oculi are
clearly marked in insular sclerosis, they are also frequent,
Uhthoff having found that the fandus oculi was only normal in
48 out of 100 cases, while the sight was diminished in 5 of the
apparently normal cases, Thus this author justly remarks
that “cerebral tamours and tubercular meningitis being ex-
cepted there is no disease of the nervous system (tabes being
included) which is so often accompanied by ophthalmoscopic
changes as insular eclerosis.”

Such, gentlemen, are the lesions of the optic nerve where it

je LETTESS iS JiSEasES GF TRE SPrsal CoRD.

¢xtam3s -: jem =e ~cna ami che sverptoms due to these
sans Wl aw Te urcccmet ks I have already said when

a case of izerlar aernele
Bice, seen estes re freee.
aie
* of visto is free, whit mime. anccema esse. The
acalgoas to those found ix cases of torcactlrspia, Ubthed.
fzore :s borrowed. states that b+ bas cols met with this cane
# visicz had the above form.

ai, ding to the inetraction given by Chareot. the twe will not
te found to entirely correspond in all cases.

INSULAR SCLEROSIS. 7

The disorders of sight vary both in form and intensity.
Parinand, who has specially studied them, describes many
varieties.

A. Slowly progressive diminution of visual power, possibly

\ aa N
; Se

reaching from } to 4; the field of vision is normal but some
dyechromatopesia existe as regards red and green, which can only
be recognised by means of the photometer. This form would

to the commencement of decoloration in the outer

correspond
portion of the papillie.

118 LECTURES ON DISEASES OF THE SPINAL CORD.

_ B. The development of visual disorder is rapid; complete

blindness may occur though this is but transitory; improve-
| ment takes place which is often very great ; dyschromatopsia is

frequent, different alterations may oceur in the field of vision.
The visual disorders are bilateral, ‘These changes correspond
with very pronounced atrophic decoloration of the optic nerve,
which persists notwithstanding the improvement of sight.

©. The impairment of sight is unilateral, more pronounced
and persistent. The field of vision presents an irregular con=
traction ; no dyschromatopsia exists. This form depends upon
white atrophy of the optic nerve which is well marked.

Uhthoff’ has also mentioned the different changes which occur
in the visual field’, and describes the four following varieties :—

A. Central scotoma without change in the periphery of the
visual field.

B. Central scotoma associated with contraction of the peri-
phery of the visual field.

C. Peripheral contraction of the visual field which is irregular,
central vision being comparatively normal.

D. Regular concentric contraction of the visual field analogous
to that occurring in hysteria (the most uncommon variety

‘occurring once in 24 cases).

Charcot again dwelt upon the fact that dyschromatopsia may
be very pronounced in insular sclerosis, that it is analogous to
‘the same condition when it occurs in tabes, blue and yellow
tig ho colours which persist longest, whereas in hysteria it is

different facts, gentlemen, may be looked upon as
‘that changes in the fundus oculi occur frequently in
osis, but are often moderate in degree; the visual
is due to them is usually but slight in degree, being
! e and to pronounced retrogression.
character in the visual disorders is that they
or if bilateral ave asymmetrical.
onset, Uhthoff found that in half the cases
the other half gradual. Sometimes the
cur until a certain time in the evolution
e at other times they constitute the
Lastly, they have at times been
vy parallel with that of the other

INSULAR SCLEROSIS. 119

symptoms of the malady, becoming more pronounced when the
other symptoms increase in severity.

C.—Viscerat Disonpers.

Though less frequent than in tabes, for example, certain
visceral symptoms are quite as liable to occur in insular
sclerosis. In a memoir (presented to the Academy of Medicine
for the Civrienx prize in 1885) I was already able to mention
a certain number of cases in which troubles occurred in the
stomach, rectum, bladder, or genital functions. Cases in fact
were published in which incontinence or retention of the urine or
fivces, or in some patients tmpotence, or more rarely gastric crises
somewhat analogous to those of tabes had ocurred.

Oppenheim, upon making special inquiry as to visceral
symptoms in all the cases of insular sclerosis which came
before him, found them far more freqnent than observations
previously made had led him to expect. According to this
author they occurred in 80 per cent, of the cases. Notwith-
standing the confidence that one should feel in the assertions
of Oppenheim I cannot help fearing, gentlemen, that this
high number was reached by his including cases of visceral
disorder which were but very slightly pronounced. I would
therefore repeat what I have already observed in speaking of the
ehanges which occur in tactile sensibility :—“ As regards the
ordinary clinical features of the disease visceral disorders can
scarcely be included among the symptoms of insular sclerosis,
and even then they rarely become at all severe.”

.—Troraic DisorDERs.

Trophic disorders in the same way cannot be said to
to the ordinary symptoms of insular sclerosis, At the same
time several different kinds may be possibly observed.

Changes in the nails haye been very rarely observed analogous
to those which occur in tabes (?) (Domecq Turon).

Sloughing, which may occur in the gluteal region, or elsewhere
are complications rather than direct consequences of the nervous
affection.

Of ali the trophic digorders the most pronounced is
this may affect the muscles of the hand, and specially the inter

ae

120 LECTURES ON. DISEASES OF THE SPINAL CORD.

ossei; at other times it is more or less general, and to such an
extent that these conditions of muscular atrophy in insular
sclerosis have caused it to be confused with amyotrophic lateral
sclerosis; Pitres drew attention to this fact with respect to a
case Which he observed ; two other cases have been recorded by
Dejerine and Skolosubow, The subject will be again considered
when the diagnosis of the disease is discussed.

T1.—Bvirar Symptoms.

Tt has been already observed that the division of the symptoms
in insular sclerosis into spinal, bulbar, and cerebral is quite
arbitrary, and only of interest from a didactic point of view.
This statement having been made I shall class the following
symptoms amongst those of bulbar origin :—

Difficulties connected (a) with deglutition, and (bh) with masti~
cation, which in some eases, of great rarity, may be so severe as
to resemble those occurring in labio-glosso-pharyngeal paralysis.
Tt will be understood that in such a case, when muscular
atrophy of the limbs coexists, the diagnosis of amyotrophic
lateral sclerosis is probably made, and an error, analogous to
those of which I recently spoke, is difficult to avoid.

c. The tremor in the tongue does not seem altogether analo-
gous to that which exists in the limbs, but rather to consist in a
difficulty in protruding the tongue and in keeping it ont of the
mouth.

d, Glycosuria has been mentioned in a certain number of
observations; their enumeration will be found in two interest-
ing memoirs which appeared almost simultaneously, one by
Richarditre, the other by Miss Blanche Edwards, It is probable
that in these cases the islets of sclerosis were seated in the floor
of the fourth ventricle, at the point where a puncture causes the
presence of sugar in the urine, unless of course true diabetes
happens to exist.

e, Polyuria, which is at times observed, is probably due to a
similar cause,

All the bulbar symptoms, or those which are called by this
name, except perhaps the tremor in the tongue, so rarely occur in
insular sclerosis that in my opinion they need only be mentioned,
their description being quite unnecessary.

INSULAR SCLEROSIS. 121

TU.—Cesesran Symrroms.

Amongst the most important of these symptoms are the dis-
orders of speech. This acconling to clnasical description becomes
slow, monotonous, scanning, and spasmodic,

Tt is very difficult to describe these changes, and the best
method of showing what they are will be°to direct the patients
now present to speak before you. At the same time, an attempt
will be made to analyse the singular characters of the articula-
tion. This patient, whose speech is much altered in the way
already mentioned, will be asked to say a few words—this sen-
tence for example—* toute médaille a un revers.” Observe in
the first place what may be called the “ preparations to speak ";
the lips are thrown forward, the forehead is wrinkled, and a
great effort is made that all may be ready; now remark that
every syllable is spoken in a laboured way, or rather is extracted
from the mouth with difficulty ; the effort first made is continued
throughout the whole sentence, seeming to be always required.
At the same time neither a vowel nor a consonant is omitted,
almost precisely the same stress being laid upon each letter, and
this contributes greatly to the slowness and monotonous character
of the articulation. Observe, again, his great difficulty in pro-
nouncing the syllable “ daille” of the word “ médaille,” it might
be said that a hill must be climbed upon the road which is
already difficult, and that redoubled efforts must be made, This
difficulty in pronouncing the vowels or diphthongs, followed by
two liquid I's, is often very pronounced in insular sclerosis. The
patient is now reaching the end of the sentence, and has arrived
at the last syllable; this is,as it were, abruptly and incontrollably
thrown out in an “explosive” manner. He seems to have badly
calculated his power, and finding that there is now no syllable
to retain him, makes an effort which exceeds those which he has
previously made. It should be observed, gentlemen, that this
desctiption applies to those cases in which the disorders of the
speech are very pronounced; in other patients these characters
are but slightly indicated, and may even be entirely absent in
the caves which are termed abortive.

Another symptom of cerebral origin which often occurs is
vertigo; this symptom may only occur when the patient walks,
and walking may be extremely difficult on this account; it may

122 LECTURES ON OF THE SPINAL CORD.

exist, so to speak, continuously with but slight remissions.
Lastly, in some cases, the vertigo exactly resembles that which
occurs in Méniére’s disease, and it will be seen that the same is
often the case in tabes.

‘The existence of intellectual change has been noted by many
observers. It is undoubtedly true that such change often exists
to a more or less pronounced extent, but this does not show that
all the observations in which it has been recorded are correct.
Far from this I could mention certain cases in which the mental
change was very pronounced, and the lesions were wrongly con-
sidered at the autopsy to be those of insular sclerosis, whereas
in reality they were due to the existence of diffused cerebral
sclerosis. I shall return to this point in a subsequent lecture.
Tt should be remembered that the mental change in insular
sclerosis is but moderate in degree, consisting most frequently
of slight weakness of the intellect, indifference or melancholia,
whereas at times these conditions may be, or appear to be, com-
pletely absent. In some cases only the mental distarbance is
greater, and a kind of delirious idea which the patient has of his
own importance may be observed, or even complete dementia or
deprayation of character if at least true insular sclerosis can be
said to exist in such cases, ‘Io these mental changes the attacks
of spasmodic impulsive laughter may be added which are very
frequent in this disease,

One of the patients presented to you to-day has this symptom
ina pronounced degree. It will be seen that the word ** laughter”
is scarcely spoken by me, than he begins to laugh, one must not
say “ with open mouth,” since on the contrary the same tendency
to spasm exists in this as in many other of the symptoms which
occur in insular sclerosis ; there are small outbursts rather than
peals of laughter, although the tendency to laugh is compulsive
and irresistible, and it is this which causes the peculiar appear-
ance of the patients. In some cases the impulsive, uncontroll-
able nature of this symptom is so marked that the langhter
continues for an unlimited time. Oppenheim, who has specially
studied this symptom, states that he has seen it continue during
several minutes, and be so intense as to produce alarming
cyanosis of the face. This unreasonable laughter does not by
any means indicate that the intellect of the patient is more
seriously affected, and the answers made by this patient will show

INSULAR SCLEROSIS. 123

that his intellectual condition is not so appreciably weakened aa
might be supposed.

Lastly, before ending what should be said with regard to
symptoms of cerebral origin, the apoplectiform or epileptiform
attacks, noted by Charcot, and which may oecur at the onset or
during the course of the disease, must be mentioned; these
attacks do not by any means occur in all cases of insular
sclerosis ; they may almost completely resemble (and for more
information upon this point, reference should be made to the
thesis of Gérandean) an attack of trae epilepsy or apoplexy,
except that in the apoplectiform attack of insular sclerosis the
temperature usually rises to 39° and 40°C, (102° and 104°F,),
whereas the reverse occurs in apoplexy due to cerebral disease.

lll

124

LECTURE XII.
INSULAR SCLEROSIS (continued).
COURSE. DIAGNOSIS, £TIOLOGY.

Counsx : Onset acute, or gradual and progressive. The course of insular aclorosis

progress

can emcees remittent chronic type, permanent improvement, and

even recovery. Varinblo duration. Abortive forms. Diaarosis} A. Of the
abortive forms: with hysteria, paralysis agitans, mercurial tremor, chorea,
general paralysis of the insane, cerebral tumour, transverse myelitis, com-
Pression of the cord, combined lateral and posterior sclerosis, tubes, focal
corebral lesions, amyotrophic lateral sclerosis. B. Of the typical form with
Friedreich's disease, hystoria (coincidence of hysteria aud insular sclerosis).
Br1oL06x : Slightly more frequont in the male sex, most frequent betwoen the
age of 20 and 30 years, docs not occur in old age, very rare, if occurring at all,
in childhood. The special cause is infection, Narration of facte, different
infectious diseases during or after which insular sclerosis has been found to
occur. Explanations of this fact.

GerNTLEMEN,—The symptoms which occur in insular sclerosis
having been described in the last lecture the evolution of the
disease from a clinical point of view has still to be considered.

Firstly the onset occurs in different ways. It is at times
sudden, even then appearing in different forms. At times an
attack of apoplexy, either alone or complicated by hemiplegia,
signalizes the commencement of the disease, or at times hemi-
plegia without apoplexy. At other times vertigo and giddiness
are the first symptoms, while sometimes the disease commences
suddenly and unexpectedly by disorders of the sight.

Sometimes, on the contrary, the onset is slow and progressive,
the symptoms which first attract the attention of the patient or
his friends consisting either of tnereasing difficulty in walking, or
disorders of the speech, or tremor of the upper limbs; while in
other cases, which at the same time are rare, the first symptoms
experienced by the patient are more or less severe, and some-
times lightning pains resembling those which occur at the onset
of tabes.

eS

INSULAR SCLEROSIS. 125

‘The course of the affection also varies very much but may, as
T believe, be included in one of the following types :—

A. A chronic progressive course, that in which the symptoms
are gradually and slowly, though constantly, aggravated. This
form by no means occurs so frequently as some descriptions
imply.

B. A chronic course with sudden exacerbations. Tt is in this
form that some of the symptoms are found to occur which have
been mentioned in connection with a rapid onset, apoplexy,
hemiplegia, sudden troubles of the sight, &e., &e.

©, A remittent chronic course. The symptoms are but slowly
progressive, if they increase at all, and remain stationary during
long periods of time, after which slight exacerbations occur.

D. Permanent improvement, or even recovery. This not un-
commonly occurs, and it is with good reason that Charcot
frequently insisted upon the tendency to improvement exhibited
by insular sclerosis. ‘The disease may in fact be arrested atvany
period of its evolution, and not only cease to increase subsequently
but even diminish to such an extent, according to Charcot, that
actual recovery may occur.

In any case, gentlemen, it should be remembered that the
prognosis of insular sclerosis is not so extremely grave as in
some diseases of the spina! cord which I shall have to describe.

At the same time the disease does not always pursue this
lappy course, and independently of the fact that death may
oceur on account of some intercurrent disease, it may be due to
an apoplectiform attack or some difficulty connected with de-
glutition and bulbar symptoms, or to the patient being completely
wrecked by the disease.

The duration of the affection is essentially variable, and the
patient perhaps dies at the end of one or two years, or may live
for twenty years or more, according as the disease occurs in one
or other of the forms which have been described.

We have just considered cases of insular sclerosis which are
quite clear and more or less complete in character. Other cases,
however, exist in which the symptoms are so little pronounced
or in such an isolated form that the clinical indications of the
affection can scarcely be recognised; it is these cases which
Charcot termed ebortive and in which, interesting as they are,
the diagnosis is at times extremely difficult. It is these cases

Re LECTURES ON DISEASES OF THE SPINAL CORD.

which we should have specially in mind in distinguishing this
Gisease from other affections with similar symptoms, and it is
by considering them that we will begin to study the diagnosis
of insular sclerosis,

A—DIGNosis OF THE ABORTIVE FORM.

‘That is to say the diagnosis of those forms of the disease in
which one symptom exists in an isolated manner or predominates
over the others, to such an extent that it might on that account
receive the name of insular sclerosis, in which but one or few
symploms exist, According as one or other symptom predom-
inates the malady will have to be distinguished from very
different diseases,

a. The predominating symptom is the tremor. The diseases
which must then be distinguished from it are the following :—

(a.) Hysteria, which will be again mentioned when the typical
form of the disease is considered.

(8.) Paralysis ayitans: in this affection the tremor occurs
more slowly than in insular sclerosis (4 or 5 oscillations
occurring in a second instead of 6 or 7); it is also continuous
except during sleep, exists whilst the limbs are at rest, is
diminished rather than increased by voluntary movements, or
whenever it increases during such movements, does so to such
a slight extent as to bear no resemblance to that which occurs
in insular sclerosis. Lastly it is a tremor with but very small
oscillations, only affecting the wrist and fingers, the arms and
forearms remaining pressed against the trunk. The other
symptoms of paralysis agitans, the attitude, face, and gait,
haye not been mentioned, since my endeavour is to enable the
cause of the symptom to be recognised by the characters which
it presents itself.

y- Mercurial tremor. This tremor has been specially studied
by Charcot during the last few years; it is, according to his
description, very analogous to that which occurs in insular
sclerosis. ‘The oscillations, in fact, occur with moderate rapidity
(less than 8 oscillations in a seeond), are often of some size, and
are produced and increased by voluntary movement. That,
however, which enables it to be distinguished is, as Charcot
expresses it, that it only ceases during rest in a vemiffent manner,

|

INSULAR SCLEROSIS. 127

reappearing from time to time without the patient making any
movement, either from the influence of slight emotion or occur-
ring quite spontaneously, whereas in insular sclerosis the tremor
is, as you know, completely absent while the part is at rest,

Fig. 04.—Sketch of the romittent tremor in mercurial poisoning (after Charcot).
‘Whilst the part is at rest (AB) the trembling occurs during short periods of
time (D, E), which soon cease, to occur again, and cease almost immediately.
When a voluntary movement (HC) is made, the tremor is of precisely the mame
character as in insular sclerosis.

8. Chorea.—It seems almost useless to dwell upon the diag-
nosis as regards this affection, in which no real tremor exists,
but a series of irregular and unnecessary movements. There is
no axis of direction around which the oscillations take place,
nor are the movements similar to each other as are those of real
tremor. It is therefore impossible to sketch them graphically
with any exactness. or these reasons, without the other
characters of the disease being considered, it seems to me
impossible to mistake it for insular sclerosis.

128 LECTURES ON DISEASES OF THE SPINAL CORD.

b. The predominating symptom is disorder of speech with or
without mental change,

The disease will then have to be distinguished from general
paralysis of the insane. In these two affections the disorders of
speech are often yery similar, At the same time they may be
distinguished by the fact that in general paralysis the speech is
more tremulous, more hasty and indistinct, that the syllables

ate and that the spasmodic character which exists in
fai sclerosis is absent. In order to perceive these differences
it is usual to direct the patient to pronounce words which are
of some length, and notably the word “artillery.” While this
word will be pronounced “artie-ry” by the patient suffering
from insular sclerosis, the one affected by | general paralysis says,
* aar-tille-e-re-ry.” In the first the speech i is but drawling and
spasmodic, while in the second it is drawling, stuttering, and
weak, a3 if in the first case a hill was being ascended with diffi-
culty but certainty, whereas in the second the gait is interrupted
by false steps which occur unexpectedly, and are tripping,

ce. The predominating symptom is faltering speech with or
without nystagmus and optic neuritis.

These three symptoms, which, as already stated, frequently
exist in insular sclerosis, by their co-existence often indicate
the existence of a cerebral tumour, and it must be confessed that
they are so analogous in these two affections as hardly to be
distinguishable by the characters which they themselves possess.
The existence of other symptoms must therefore be sought,
either thoze of insular. sclerosis or of a cerebral tumour, and
amongst the latter, vomiting, headache, the special characters of
the optic dise, &ke., &e.

d. The predominating symplom is disorder of the gait.

a. The gait is purely spasmodic: the distinction must then
be made from a certain number of lesions in the cord of
which the principal are due, either to transverse myelitis, or
compression of the spinal cord (Potts’ disease, tumour, injury,
&c,). Besides the fact that each of these presents its own
special symptoms, it will be remembered that they are often
accompanied by disorders of the sphincters, which is not the
case in insular sclerosis.

8. The gait is spasmodic and accompanied by disorder in
the ocular muscles (paralysis, myosis, changes in the reflex

-_ &

actions of the iris). In this case the possibility of combined
lateral and posterior sclerosis must be remembered ; and spastic
would then be combined with a certain number of the
symptoms of tabes so that the existence of this affection could

be easily
7. The gait is cerelellar, besides which some disorders exist
connected with the eyo, and there is absence of knee-jerk. In
eases, which however are rare, there may be some

In the latter affection the element “pain” is far more pro-

dinguosis more difficult; an investigation of the special
symptoms which occur in each of these affections should then
be made.

e. The predominating symptom is hemiplegia, Whether hemi-
plegia occurs or not after an attack of apoplexy the diagnosis
between a focal cerebral lesion and insular sclerosis may be,
specially during the first moments after its onset, of great diffi-
enlty. In many casea the subsequent course of the symptoms
can alone settle the question, and with regard to this point
T would remind you that hemiplegia in insular sclerosis is not
followed by contracture which occurs in most cases of focal
cerebral lesion.

Ff The predominating symptom is amyotrophy, which may or
may not he accompanied by bulbar palsy. Tt is these cases which,
as already stated, may resemble amyotrophic lateral sclerosis and
thus give rise to an error in diagnosis. They are, however, so
fare that they may possibly never be seen. Should this, how-
‘ever, occur, it should be remembered that in insular sclerosis
the muscular atrophy never attains a pronounced degree, that it
never produces spasm in the upper limbs, or affects the chin,
lips, or tongue.

—

%

‘130 LECTURES ON DISEASES OF THE SPINAL CORD.

B.—Diacyosis ov THE TyricaL For.

B. Diacyosis or tHe tyricaL Form, When all, or nearly
all the symptoms of insular sclerosis exist in the same patient,
as in the fyjical form, the diagnosis usually presents no difficulty.
Tt may do so, however, in some cases, which will now be slone
considered.

a. Friedreich's disease. Without discussing at this moment
Friedreich's disease, which will be specially considered in a
subsequent lecture, I would remind you that many of its
symptoms exist alsoin insularsclerosis; such are the nystagmus,
the slowness of speech, the diorders of gait, and he tremor
when voluntary movements are made, ke. The possibility of
an error in diagnosis between these two affections is therefore
easily understood owing to the resemblance of the symptoms,
One of the most important differences from an objective point
of view is the spasmodic character of the symptoms in insular
sclerosis, whereas in Friedreich's disease the affected parts are
completely relaxed. This is specially observed in the diminution
or loss of the tendon reflexes (neither the irregular movements
resembling those of chorea, nor the absence of ocular paralysis,
nor the scoliosis will be now mentioned, their description being
reserved for a subsequent lecture).

4, Hysteria, This disease now comes before us for the first
time, a disease to be feared almost as much by the doctor as by
the patient. It will be seen how exactly, 1 might almost say
how artfully, it may imitate most other diseases of the nervous
system, and notably those of the spinal cord, specially insular
sclerosis, The thesis of Souques upon imitutive hysterical
associations, in which numerous illustrative observations will
be found, may be read with advantage by those wishing for
detailed information upon the imitative power of hysteria.
The only fact which I will borrow from them is the follow-
ing: In the six first observations of his work referring to
patients who were known to be suffering from hysteria, the
following symptoms were found to be associated therewith :
vertigo, apoplexy, hemiplegia, intentional tremor (the interesting
thesis of M. Dutil may be consulted on this point), disorder of
the speech, diplopia. It will be recognized that a more perfect

association of the principal symptoms which exist in insular

ih a

INSULAR SCLEROSIS. 131

sclerosis could scarcely be found, and that in this case the name
of imitative hysterical association is amply justified. Thus the
mistakes which haye been made are numerous, and the choice
would be difficult to make even were professed neurologists
alone to be quoted os a proof of this. It is possible that the
occurrence of such a mistake explains the cases published by
Westphal, with regard to which this author states that after
observing most of the symptoms of insular sclerosis in certain
patients he did not find the characteristic lesions of that disease
at the post-mortem examinations, Such a mistake, gentlemen,
may be avoided if it is never forgotten that yest exists,
the fear of which should haunt you whenever a diagnosis is
made, “timor hysterie initium sapientiw.” To know when
to suspect it is the most important point; as regards its re-
cognition this is comparatively easy, owing to the remarkable
works of my master, Professor Charcot, upon the organic

which occur in that neurosis. The *‘ stigmata” of
this disease should therefore be carefully sought: reduction of
the field of vision, achromatopsia, monocular polyopia, areas
of cutaneous anasthesia, hysterogenous zones, &e., &e., and
mistakes of no little importance may then be avoided.

Even, however, when the existence of hysteria is recognized it
‘must not be supposed that all the difficulties are overcome, Cases
occur, and by no means infrequently, in which hysteria and insular
sclerosis are aszociated together, either from insular sclerosis
occurring in a patient suffering from hysteria, or, as is much
more frequent, from insular sclerosis being the cause of hysteria,
an oceurrence which was well discussed hy Georges Guinon in
his thesis for the doctorate. Whatever the chronological relation
between these two affections may be they undoubtedly co-exist.
When this occurs it may be difficult to recognize that such
oceurs, but in my opinion cases should be mistrusted in which,
while symptoms of insular sclerosis exist conjointly with certain
indications of hysteria, the tendon reflexes are truly excessive.

Insular sclerosis having been studied, from a symptomatic
point of view, the conditions of its development will now be
considered.

The 2t1oLoay of this affection presents, as will be seen,
several points which are open to discussion, and therefore of
real interest,

=

INSULAR SCLEROSIS. 135,

the brain or spinal cord. ‘This truly effective cause is the
presence of infection, or rather infections.

With your permission this question will be disonssed in some
detail, since the subject is one which specially interests me.
Even in my first work* upon Insular Sclerosis and the Infections
Diseases (1884), T thought it possible to affirm definitely that
this relation of cause and effect existed between them; at that
time I had collected 25 cases, of which a certain number
were taken from the memoir of Kahler and Pick, in which as
early as in 1879 these authors drew attention to the frequency
with which insular sclerosis occurs after acute disease, It
is true that all the facts stated are not equally convincing,
and I admit that the prudent reserve recommended by our
esteemed master, Professor Jaccond (Clinical Lectures at the
Hospital de la Piti®), was quite legitimate from a purely
scientific point of view. Since that time, however, new facts
have been added to those previously known. Undoubtedly
some of these are more or less incomplete witlr regard to certain
points, but it must be remembered that clinical is not the same
‘a8 experimental investigation, and that being obliged to accept
situations which we have not ourselves created. we must make
use of whatever documents chance puts into our hands, and
‘draw from them the best conclusions we can.

This is what usually occurs: a person of between 20 and 30
years of age contracts an infectious disease (it will presently
be seen in the enumeration which I shall make how different in
nature the infectious diseases may be), and either in the course
of the disease, or during the period of convalescence which follows
it, or perhaps some months later, the first symptoms of the
‘nervous affection occur. In some cases, to which reference will
again be made, when the pathological anatomy is considered,
these symptoms improve, and may completely disappear, the
morbid process not having then produced selerosis, but having
been definitely arrested. In other cases the symptoms which

* The infectious origin of insular aclerosi« is almoat generally admitted, and
Depron fit

origin,
thie opinion ix also acknowledged to be correct, I feel bound to state
conviction

-

Tt is certain that when the nervous symptoms
long as several months after the infectious dise
between the two is far from evident. If such ¢
only been observed three or four times, it might be |
as a mere coincidence, and this would explain to a cet
the frequency of infectious disease and the com
tatat jnlce, y afte thor + oats ed
nature published by different authors is
compares them with those in which re
during the course of an infectious disease, or the c
period of convalescence, it is impossible, in my opinion, to.
that they stand fo ano anciier in tha raat of eet
effect. You will soon perceive that the pathological anatomy of
the disease, far from being in opposition to this “a
an additional reason for believing it to be true,

We will now examine the facts themselves; in the
enumeration the infectious diseases will be named po or
after which, insular sclerosis has been found to occur,

Eateric fever appears to take the lead in a decided manner,
since in 26 observations which I collected for my work on this
subject the disease was met with 11 times.

Pneumonia ia not infrequently connected with the disease,
Tn acase which Richard had obligingly communicated to me,
that of a patient named Rob, who was well known in the
hospitals as a typical example of insular sclerosis, pneumonia
existed on both sides of the chest.

» Malaria is mentioned in several observations, and in the
“ Legons du Mardi” of Charcot you will find that a patient was
interrogated upon this subject, and of his own accord referred
the nervous affection from which he was suffering to an attack of
malaria.

Bruptive fevers must also be mentioned, namely, measles,
scarlatina, and above all, small-~pox. The cases are numerous in
which insular sclerosis has been known to occur during con-
valescence after the latter affection ; tremor in the limbs, with
more or less paresis, disorder of the speech, which becomes slow
and scanning. nystagmus, and in short, all the characteristic

i ,

INSULAR SCLEHOSIS, 185,

symptoms of insular sclerosis may exist. At times these
symptoms cease and entirely disappear, but they may also
continue and confirmed insular sclerosis occur.

Other infectious diseases should alo be named thongh, as
reganis the number of cases connected with them, they are but
of secondary importance. Such are diphtheria, whooping-cough,
erysipelas, dysentery, and even cholera, Lastly, ab quite a recent
date Charcot, in one of bis clinieal lectures, recorded a most
interesting case, in which insular sclerosis seems to have
followed cerebral rhewmatiem,

Before concluding this enumeration, a paragraph must be
devoted to unnamed infections, 80 frequent, so little known, I
might add so much disregarded. There are no special symptoms
at the onset which indicate its existence; fever is known to
have occurred, prolonged discomfort with or withoat gastro-
intestinal symptoms, occasionally jaundice or pulmonary trouble,
nothing else being known about the disease. In such a case,
gentlemen, you must not doubt that this is certainly a case
of infection, but of such a kind that i¢ has not received any
definite clinical name. As regards the patients in whom insular
sclerosis seems to occur from the influence of injury or some
other purely physical cause, my conviction is that these cases
are also due to infection, but that the infection has passed away
completely unperceived, while some less important but more
dramatic incident has alone attracted the attention of the patient
or those who are with him.

Of the manner in which insular sclerosis is produced by
infection it will be better to speak when the pathological anatomy
of the disease is considered.

As to the part which micro-organisms play in this process the
question is an embarrassing one, scarcely any facts existing
which can aid its solution. At the time of my first communi-
eations upon this subject, some of my colleagues, who un-
donbtedly had not done me the honour to read them, pretended
to believe that I had described “the micro-organiem of insular
sclerosis.” On the contrary, I have been careful to avoid
exposing myself to such an accusation, and merely stated the fact
that insular sclerosis occurs in a number of infectious diseases
which are quite distinct from each other, Can it be looked upon
as due to the micro-organism which is epecial to each of these

—

137

LECTURE XI.
INSULAR SCLEROSIS (continuation and endl).
PATHOLOGICAL ANATOMY, NATURE. TREATMENT.

‘The meninges are usnally but slightly, if at all, affected. Insular appearance of
‘the lesions: abundance of the islets, extreme irrogularity of thoir seat, dimon-

ological
nected with this subject. Distinsiten elweea iasnlar wolervela ned eiumed
multilocular sclerosis, Treatment,

GextLemex,—That a clear idea may be formed of insular
sclerosis the study of its lesions is indispensable. The
PATHOLOGICAL ANATOMY of the disease will therefore be now
considered.

A. From a macroscopic point of view the affected parts of the
central nervous system present the following appearance from
without inwards :-—

The meninges are neither thickened nor adherent, the trans-
parency of the pia mater being preserved so that the most
superficial islets may often be seen through the membrane. In
some cases, however, meningeal changes have been said to be
observed. Without being able to affirm that the meninges are
never involved in insular sclerosis I must put you on your guard
with, to these cases, of which the greater number are far
atlides poe examples, if they belong at all to the disease
which is now being considered.

Thé nervous centres themselves, when the meninges have
‘been removed, are found to present different aspects. In some
cases there is nothing abnormal at the surface, while if sections

=—

138 LECTURES ON DISEASES OF THE SPINAL CORD.

are made through the deeper parts islets are found to be con-
tained in them, whereas in other cases no islets are found either
at the surface or more deeply seated, and it is only after

Fig. 95.—Transverse section of the extremity of a cerebral hemisphere in which
‘Sirlots of sclorosia are acon in different parte. One of these (B) is entirely
contained in the white substance. ‘The second (C) is for the most part in the
grey matter. The third (A) in seated partly in the white and partly in the grey
substance, being as it wore astride over one of the flesures of the cortex.

ee.
6.—Section of the crura cereSri (slightly mngnitied). (Damaschino colleo-
tion.) It isin the white «nbatance that the islets of sclerosis have specially
appeared, The islot of sclerosis (A) involves the whole thickness of the part;
another islet is seated near the Srd ventricle,

remaining for a more or less prolonged period in a solution of
dichromate or being immersed for a time in a colouring liquid
that such islets of sclerosis are clearly seen.

INSULAR SCLEROSIS. 139

Usually the natore of what occurs is more simple, and as T
have just said, even before removal of the meninges some of

Fig 97.—Transvorse section of the pons varolii (slightly magnified). (Damaschino
collection.) The white portions of tho figure are those in which the islets of
sclerosis are seated.

Fig. 98—Seetion of the pons varolii in the long axis from a case Of insular
sclerosis (slightly magnified). (Damaschino collection.) The white portions
of the Suro are those in which tha islots of solerosia aro aeatod.

the islets of sclerosis can be very easily distinguished by trans-

‘The islets present some characters which it is important to

140 LECTURES ON DISEASES OF THE SPINAL CORD.

>

Fig. 19.—Section of the lower part of the medulla oblongata (slightly magnified).
‘The groy and whito mattor of tho part aro both oceupied by islota of acleroais,
Tt will be seen that neither the olivary body nor the grey substance in the floor
of the 4th ventricle (D) have boon respected.

Fig. 100.-Section of medulla oblongata parallel to the floor of the 4th
Tentriclo (elightly magnified), (Damaschino collection.) ‘The grey parts are
thone affected by the ialota of sclerosis.

INSULAR SCLEROSIS. ui

know: they are quite irregularly placed, and hence the name
proposed by Charcot, and rightly adopted, of disseminated sclerosis.
‘Thus it is impossible to find two cases which, from an anatomical
point of view, resemble each other. In some cases the seat of
the lesions is unforeseen and odd, being formed according to no
rule, arrested by no obstacle, and confined to no anatomical
system. At one time the islets exist abundantly at the surface
of the spinal cord and convolutions, at another the deep parts
of these organs are specially affected, The islets are un-
doubtedly more numerous in the white than in the grey substance,
although it would be wrong to say, as do some authors, that the
grey substance offers an insuperable barrier to the islets of

Fig, 101,—Section of the spinal cord (cervical region) in # case of invalar sclerosis,
‘Two islets are seen in this section: one (A) of moderate size, the other (BCD)
‘of vory large size is seated on both sides of the cord pasxing beyond tha posterior
fissure, and posterior born on the left aide.

sclerosis which are seated in the adjoining white substance. The
different fissures of the cord again do not prevent its extension,
and in the figures placed before you the lesions are seen to be
at times as it were astride over the fissures. The islets are found
throughout the whole length of the cerebrospinal axis, from the
surface of the convolutions or ventricles, from the crara cerebri,
pons varolii, or cerebellum to the jilwm lerminale, though not
in all parts with the same frequency; thus, for example, they
rarely occupy the large ganglia of the cerebral hemispheres or
olivary bodies.

The word “ disseminated ” indicates that the islets are of small
dimensions (inselfGrmige sklerose in German, insular sclerosis
in English ; polynesic sclerosis of some authors), and they have

142 LECTURES ON DISEASES OF THE SPINAL CORD.

usually the appearance of being flattened. In many cases,
however, the islets of sclerosis have a cuneiform aspect; and in
this case their base is usually turned towards the periphery of
the organ in which they are seated.

‘The number of islets varies considerably, and as many as some
hundreds may exist in the same pati

Their size also differs much, varying from that of a millet
seed to the size of a two-shilling piece.

‘Their appearance is as follows: usually of a slate or greyish
red colour, they become clearly more red after being exposed for
a time to the air; their consistence differs in a similar way,
being mach greater in the large than in the small islets, Tn one
section the islets may appear to be prominent, while in others
they seem depressed. This difference in their appearance pro=
bably depends upon the age of the islets, and: the degrea df
evolution of the sclerosis where they are observed,

So far, gentlemen, the islets seated in the nervous centres
have been alone considered; it must be stated, however, that
some authors have observed them upon the roots of the peri~
pheral nerves which adjoin those centres. They have been also
found upon the nerves connected with the medulla oblongata, not
only upon the optic nerve of which the special structure makes
it to a certain point a diverticulum from the brain, but also
upon the hypoglossal, glossopharyngeal, vagus, &c, The nerve
roots connected with the spinal cord may be also the seat of the
islets.

B. Their microscopical appearance presents certain characters
which should be mentioned.

When examining a specimen with a microscope of slight
magnifying power the clearness with which the islet of sclerosis
separates itself from the adjoining part is at once observed. In
the section now put before you the islet of sclerosis seems almost
to be punched out, and it will be difficult to find an islet of
aclerosia in any organ of which the limits are as little diffused as
thoxe of the islets which we are now studying. What also con-
tributes to give them the appearance of being “ punched ont” is
the decided manner in which the sheaths of myelin disappear
throughout the whole extent of the islet, whereas outside it, and
so to speak without interval, the sheaths are preserved, ‘The fact
must theretore be carefully remembered that if in the disease

nes |

INSULAR SCLEROSIS, 143

which we are studying, sclerosis presents itself in an irregular
form, and essentially disseminated as regards its seat the diffuse

Fig. 102—Seotion of the spinal cord in insular sclerosis (slightly magnified). Tho
ielet of eclerosis (white) ix in this caso singlo, and soated at the surfaco.

Fig. 108.—Section of the spinal cord in a caso of insular sclerosis (magnified to a
moderate extent) (coloured by Weigert’s hematoxylin). This fgare shows
how clearly the islet of sclerosis (A) is distinguished from the surrounding
healthy tisene ; tho white colour of this islet indicates that all its fibres have
completely lost their sheath of myelin.

form exists, and should, on the contrary, be considered as a type
of sclerosis cecurring in foci.

As respects the inner part of the islet, it is composed of a
more of less dense network of neuroglis, which is thickened,
and of which the meshes are more pressed together. Its ap-
pearance has thus a special character analogous to that which
would result from elongated crystals being grouped together
in the direction of different axes, which cat each other at acute
angles (vide fig. 105). It should be remarked that of all
forms of sclerosis in the spinal cord insular sclerosis is, in the
opinion of Weigert, that in which the proliferation of the
neuroglia is most pronounced, so that if the forms of sclerosis

144 LECTURES ON DISEASES OF THE SPINAL CORD.

Fig. 104.—Longitudinal section of the spinal cord from a case in which the islet
of eclerosie wae seated at the surface of the cord (elightly magnified), (Damas-
chino collection.)

Fig. 105—Section of an islet of sclerosis in the white substance of the brain,
A, sound tissue, B, islet of sclerosis, the tissuo adjoining which is composed
of fibres disposed in columns, and which eross each other at the most different
angles, A blood vessel is seen at the centre of the islet of sclerosis

INSULAR SCLEROSIS. 146

were classified according to the extent to which the neuroglia is
involved it is insular sclerosis, and not Friedreich's disease, which
would occupy the first place.

Within the meshes of the network, in a section produced by
congelation, brilliant, refractive, or black and granular bodies,
according to their distance from the objective, are seen to bé
sprinkled in large number, hese are the granular bodies of
which the signification will be discussed presently, I would
only mention their existence at the present time, and point out
that abundant and irregularly scattered as they are in islets of
recent formation, they are to be found also in islets of old date,
though almost solely at the surface in that case, and little, if at
all, in the central parts.

Tn conjunction with these granular bodies within the islets of
sclerosis, and as an explanation of their existence the destruction
of the sheaths of myelin should be mentioned, such destruction
being complete within the islets, whilst, as already observed, the
sheaths outside these lesions are absolutely sound. It is to this
destruction that the clear colour contrasting with a black ground
which is produced by Weigert’s coloration by hamatoxylin in
these sections is due. It will be remarked that not a single
sheath of myelin remains at the centre of the islets, whilst at
their peripheral part, where the patch adjoins the sound tissue,
some of these sheaths are seen to be disappearing, and the
process of their destruction can be studied at that part in its
different stages,

As you know, gentlemen, the nerve fibres contained in the
central organs differ from those in the peripheral nerves in the
fact that they do not contain a sheath of Schwann, being only
composed of an axis cylinder and sheath of myelin. You have
heard what happens to the sheath of myelin, and what becomes
of the cylinder axis? In a gencral way it must be admitted that
the axis cylinders are retained in insular sclerosis. This fact, as
you will see, is of great importance, and affects the whole clinical
history and pathological anatomy of the disease. ‘The honour of
having first shown and proved this to be the case, and of haying
indicated all the consequences which resulted from this fact,
must be given to Charcot,

‘This condition of the axis cylinders has been verified by a very
lange number of observers, and Babinski has given in his thesis

ais)

146 LECTURES ON DISEASES OF THE SPINAL CORD.

very exact representations of it, You can easily perceive this by
observing the preparations coloured either by means of carmine
or by the method of Pal; in the midst of the meshes of indurated
tissue a number of coloured points will be seen which represent
the section of the axis cylinders. In places you will also observe
that some of these points are much larger than the others; these
consist of swollen axis cylinders, which are not infrequently
seen in islets of insular sclerosis, and also it may be said in
some other varieties of sclerosis affecting the spinal cord
(Hayem).

In the islets of ancient date, however, in which sclerosis exists
in a pronounced degree, a certain number of the axis cylinders
disappears ; this, however, is merely an accessory fact, and would
not be in opposition to the axiom that “in insular sclerosis the
axis cylinders are retained in the midst of the affected: islets,
although their sheaths of myelin may have been

Certain deductions may be drawn from this persistence © the
axis cylinders :—

a. One of the principal of these is the absence of secondary
degeneration in the path of the nerve fibres connected with the
diseased part. This again is a fact which was indicated by
Charcot from the time that he first studied the pathological
anatomy of this disease. The absence of secondary degeneration
ean be easily recognized in sections of the parts seated above
or below the affected islets. The same fact may be observed
if longitudinal sections are made ia a direction parallel to that
of the nerve fibres, and the morbid changes will be seen to be

limited in the same precise way above and below, as at the sides.
From a clinical point of view it will now be understood how
it is that the paralysis or paresis which exists, though of the
spasmodic type, is not accompanied by permanent contraction,
namely, that owing to the absence of secondary degeneration
there is no reason for its existence.

In the rare cases of which I haye already spoken, in which
‘the axis cylinders are also finally destroyed, the existence of

“slight secondary degeneration is observed ; this, however, shows
tendency to extend, and should be considered as purely
i I.
. The remission, improvement, and even cure which may
occur, can be olso easily understood, since the axis

INSULAR £CLEROSIS. uz

cylinders not being destroyed the most important, in fact the
only part of the nerve which is absolutely necessary for trans~
mission of the nervons current is retained. When the inflamma-
tory process diminishes, even to a slight extent, it is intelligible
that the function of the nerve becomes almost completely re-
established. In some cases indeed when recovery occurs, Charcot
is of opinion that the sheaths of myelin, which had disappeared
in the islets of sclerosis, may be restored, so that the nerve
fibres return to their normal condition, not only from an anato=
mical, but also from a physiological point of view.

+. According to many authors the persistence of the axis
cylinders with disappearance of their sheaths of myelin throws o
great light upon the pathology of the tremor. Comparing in
fact the nerve fibres to electrical wires, their sheath of myelin to

* “the igolating material which surrounds them, and the nerve force
to a current, one is naturally led to think that on account of the
destruction of the sheaths the fibres are no longer sufficiently
“isolated,” and that the “current may escape in places,” this
being physiologically indicated by the tremor. Tt should, how-
ever, be added that, although generally admitted, this explanation
of the tremor is not accepted by every author. Some attribute
it to the existence of islets in the thalamus opticus, while others
look upon it as purely and simply an excessive degree of reflex
action in the spinal cord.

Such, then, is the condition of the nerve fibres in the islets ;
as regards the nerve cells, on account of what has been already
‘observed by me as to the relatively sound condition of the grey
matter in this complaint, you would not expect them to be very
frequently involved, which in fact is the case. At the same
time, when contained in one of the islets, they are found to be
diminished in size, to lose their prolongations, to present clear
indications of atrophy, and even to almost completely dis-

When islets oceur in the optic nerve they present characters
in every respect analogous to those of the islets in the brain or
spinal cord. Uhthoff describes them as follows :—“ The process
of proliferation seems to take place to a very pronounced degree ;
in the first place, in the part occupied by the finest elements of
connective tissue, seated between the nerve fibres contained
within the lange meshes; in the second place, in proliferation

of the meri: se pre oy ake eed & the
septa, and to the Goer Seat of te eptie ere,

atrophy of the nerve tiseue should, therefore, be considered as
secondary. The dissppearance and destroction of the sheaths
occur in 3 relatively rapid and complete manner, while the axis
cylinders entirely, or almost entirely stripped of myelin, com-
monly persist. ‘The islets of sclerosis do not usually produce
secondary degeneration of the fibres in the optic nerve, or if
they do so to a very slight extent, as is shown by the condition
of the optic disc, either se seen by the ophthalmoscope ot the
microscope. Even though very pronounced atrophic changes
may exist behind the globe of the eye, the results of ophthalmo-
scopic examinations remain completely negative, and the atrophic
deecoloration of the optic disc is usually quite incomplete and
jal, even though very extensive alterations may exist behind
the globe of the eye, involving the whole thickness of the optic
nerve, and having produced the most decided atrophy of this
nerve,” According to Ubthoff the conditions required to pro-
duce the phenomena of optic neuritis in insular sclerosis are the
existence of recently formed and extensive islets in the optic
immediately behind the globe of the eye.
to finish the study which is now being made of the

INSULAR SCLEROSIS, M49

elements contained in the islet of sclerosis the blood vessels must
be mentioned. The alterations which they present are most
clear, the thickening of their walls having been pointed
out by Charcot from the time that he first wrote about this
disease. This thickening specially involves the external coat,
and it is on account of this that the vessels remain open, and
apparently dilated in section when the sclerosis is very pro-
nounced, At times dilatation appears really to exist and to be
accompanied by increase in the diameter of the perivascular
lymphatic sheaths ; these in the islets which are recently formed,
or in process of being formed, are absolutely full of granular
bodies. Lastly, it is not useless to remark, as you will soon
understand, that vessels are often found at the centre of the
islet, and that the most serious changes in sclerosis occur close
to these vessels.

So far, gentlemen, I have purposely omitted to speak of the
PATHOLOGY of insular sclerosis, and of the place which it should
hold in a classification of diseases, becanse I wished to put first
before you every step of the process. After what has been said
of the wtiology and pathological anatomy of the affection this
study may be now undertaken by us.

In the first, place one fact is generally admitted, namely,
that the morbid process is inflammatory and of interstitial origin.
Charcot was again the first to express this opinion, which was
afterwards almost unanimously adopted. A few years ago, how-
ever (1886), Adamkiewie®, on account of results obtained from
staining by safranin, thought himself justified in stating that
the affection commenced in the nerve elements, and that the
interstitial change was but secondary, Unfortunately the effect
of staining by safranin, which was the sole origin of this idea,
has found so few supporters and so many adversaries that it is
unnecessary to say more about this theory, and the morbid
process which exists in insular sclerosis may be safely considered
as belonging to the group of inflammations which are primarily

The origin of this primary interstitial process connected with
the islets of sclerosis is in the blood vessels. After what has been
said with respect to the infectious nature of insular sclerosis
this will not surprise you, but it is an interesting fact that even
without adopting this view the seat of the lesions is definite

INSULAR SCLEROSIS. 151

onset dates from several years ago islets of small dimensions
containing granular bodies are still found, both at the centre
and periphery, having, in a word, all the characters of yellow
islets, whilst in other parts of the nerve centres islets are found
of undoubted sclerosis of large size, and only presenting granular
bodies in any abundance at their periphery, that is to say, having
every appearance of islets of long duration. If the former fact
is interpreted in a strictly logical way we should say that the
morbid agent must, remain in each islet, since it seems to
continue its action for an almost indefinite time. As regards
the second fact it will bring us to the following conclusions :
the morbid agent remaining in the organism is capable of
reproducing itself even at the end of a greater or less length
of time and of undergoing a totally fresh dissemination. The
course of the disease confirms to a certain point this supposition,
since not only may it be continuously progressive but aleo

sudden aggravations which seem to indicate a fresh development:
of the lesions. I thought it my duty, gentlemen, to mention
these conclusions since they emanate directly from the facts
which I have described to you, at the same time I should be
the first to advise you only to accept them as suppositions ; the
evolution of most lesions in the nervous centres is still enveloped
in s0 much obscurity that it would be impradent to discuss them
in any other way. The equation contains too many unknown
quantities, and at the present time, as I have already stated, we
can only establish the terms, and clearly state the questions to
be answered.

‘Thoogh however, gentlemen, we are still in doubt as regards
certain points the following facts seem to be certain, viz., that
insular sclerosis is of vascular origin, and apparently due to
some morbid process similar to that which attends embolism ;
that it is liable to be prodaced by the infinence of several
ae Sees Probab by the mechanism of combined

a, wovane, must still be discussed, 1 cannot in fact
admit, as most authors do, that all the cases in which foci of
sclerosis, in greater or less number and more or less disseminated,
‘are found in the spinal cord should on this account be regarded
as examples of “insular sclerosis.” ‘Two forms of sclerosis may
in my opinion be quite clearly distinguished. In some autopsies

INSULAR SCLEROSIS. 158

cases of “ diffuse multilocular sclerosis,” and T hope that you are
convinced of the necessity of doing this.

We will now consider the TREATMENT of insular sclerosis.
From what you know, gentlemen, of the nature and lesions of
this affection you would naturally doubt the efficacy of such
therapeutic agents as are now employed. These doubts are by
bo means unreasonable, and when you read of cases in which the
administration of an alkaloid or hypnotism have been followed
ty Sproat in insular sclerosis, you may unhesitatingly state

the diagnosis was incorrect, and that the patient was merely

from hysteria. At the eame time, you are now aware
that thts affection tends of itself to diminish and even to improve,
and this tendency can, perhaps, be to a certain extent increased
by the administration of drags suitable on the one hand to the
“sclerotic,” on the other to the “infective” element of the
disease. You know how much good may be done by iodide of
potassium or sodium in vascular sclerosis and these should there-
fore be administered in small doses, but for some length of time.
As regards the second indication, which it is more difficult to
fulfil, perhaps the best drug to employ is mercury; this you
would therefore be justified in administering for some length of
time in a moderate dose and in the form which seems most suit-
able to the condition of the patient. It must be well understood
that this medicine is not given as an antisyphilitic since syphilis
appears to play but the slightest or no part in the mtiology of
true insular sclerosis. It is only on account of its disinfecting
properties that I should recommend its administration, and the
other disinfectants used internally may be also administered. 1
have little doubt in fact, gentlemen, that by the employment of
such a substance as the vaccine matter of Pasteur or lymph
of Koch the evolution of insular sclerosis will some day be
rendered absolutely impossible.

i

TABES DORSUALIS, 155

chronic affections of the spinal cord, and no doubt: of other parts
of the nervous system were called by the name of tabes dorsualis
(dorsal consumption). Attempts were then gradually made to
class the different cases. A progressive elimination was made,
and under the term “ tabes dorsualis” Romberg described in
1851 many symptoms belonging to the type of which Duchenne
of Boulogne, several years later, viz., in 1858, traced so masterly
& picture under the name progressive locomotor atazy.

Many other names have been proposed for this disease, some
of which have been temporarily adopted. Grey degeneration of
the posterior columns (this was at the time when the opinion of

was followed, and too much importance was attached
to the colour of the lesions in the nervous system, softening of
the nervous tissue being regarded as red, yellow, white, &e.).
Posterior lencomyelitie, the designation proposed by Vulpian,
has never been at all generally adopted. Sclerosis of the posterior
columns is a term which was somewhat favourably received by
those authors whose faith in pathological anatomy was sufficiently
strong to induce them to make this the basis of their. classifi-
cations. The name, however, appears tome bad for two reasons,
firstly, because many other affections of the spinal cord are
accompanied by sclerosis of the posterior columns, secondly,
becanse the lesions of tabes are by no means limited to sclerosis
in this part of the cord.

‘The reasons on account of which the term progressive loco-
motor alary, after receiving general approval, has now fallen
into disuse are the following: in the first place the claims made
by German authors in favour of Romberg, whose description of
“tabes dorsualis,” although very incomplete, was certainly
anterior to that of Duchenne; in the second place the greater
knowledge which we now possess about the nature of the disease
enables us to know that inco-ordination and ataxy of movement
do not necessarily occur as symptoms.

‘The term tahes has thus prevailed. On the pretext that the
word “dorsualis” is bad Latin, as Erb represents, should this

‘ epithet be replaced by “dorsalis”? I have little inclination to do
this, and since the word “ tabes ” is itself an anachronism prefer
preserving this anachronism in its complete state, and sball
therefore continue to use the term ‘tates dorsualis” as the
authors did inthe old time, and as my master Charcot does now,

em

156 LECTURES ON DISEASES OF THE SPINAL CORD.

However this may be, gentlemen, the claims of Duchenne
are incontestable. It is true that Hutin, Monod, Cruveilhier,
Horn, Steinthal and others had previously made some observa-
tions upon pathological anatomy connected with cases of tabes,
and that Romberg had for the first time sketched out the disease.
‘The merit of Duchenne is undiminished by these facts, since not
only is his description very much more complete, but he also
discussed ineo-ordination as a separate symptom. He was the
first to show, as he did by means of the dynamometer, that this
inco-ordination was due, not to paralysis but to loss of the
muscular sense, In a word he at the same time introduced
into science the notion of atezy as a special phenomenon.
After the name of Duchenne of Boulogne, the French names
which should in all justice be recalled are those of Trousseau,
Jaccoud, Topinard, Marius Carre, Charcot, and Vulpian. It is
my intention to give a more circumstantial history as regards
each of the symptoms which will be separately studied. But
this enumeration cannot be closed without the name of Westphal
being also mentioned, who contributed so much to the diagnosis
of this affection by the symptoms which he discovered.

SYMPTOMS OF TABES.

The symptoms of tabes are so numerous that in order to study
them methodically it will be necessary to arrange them in
systems or functions ; however artificial this plan may be you
will in my opinion prefer it. The general account of the
symptoms of this disease has been so often given, and by such
celebrated authors, that it would be presumptuous on my part
to do the same; I shall on the other hand carefully discuss each
symptom separately.

A. Motor Symptoms.

‘The motor symptoms which may exist are (1) disorders of the
muscular sense; (2) involuntary movements ; (3) paralysis.

1, Disoxpers or THE MUSCULAR SENSE. These will be inves-
tigated, gentlemen, in the patient who is now before you for
that purpose. After taking the foot, which for some years it
has been impossible to use in walking, and moving it in different
directions as upwards, downwards, and to the right or left side

158 LECTURES ON DISEASES OF THE SPINAL CORD,

weights, and thus ascertain their amount, and the weights being
varied to indicate whatever difference he can then perceive,

‘The disorders of the muscular sense are seen in these cases in
their most simple manner; this, however, is by no means the
case with respect to other muscular actions which we have still
to examine.

Difficulty in standing is one of the symptoms which was first
recognized, and even to-day is one of the best diagnostic indica-
tions of tabes, being termed the sign of Romberg.

We will ask this patient to stand ond to place the feet near
each other, even to join them should he be able to do so; this,
as you see, he can only do with difficulty, and after much sway-
ing from side to side. I then ask him to close his eyes; the
effect of his doing so is immediate, and if, knowing what must
inevitably happen, I had not placed myself by his side for the
purpose of supporting him he would undoubtedly have fallen to
the ground as a mass, after swaying once or twice from side to
side.

You will observe how much the difficulty in standing is
increased when the eyes are closed, and the movements cannot
be controlled by sight. This control by means of sight can be
to some extent estimated; thus, for example, if instead of
asking him to close the eyes the feet are merely concealed, by
placing a screen or sheet of cardboard horizontally directed in
front of the sternum, there is less swaying than when the eyes
are closed, but more than when no screen is interposed. It is
often quite by chance that patients suffering from tabes become
aware of the existence of these difficulties. When obliged to
rise at night, there being no light in the room, they find to
their great surprise that it is most difficult to maintain an
upright position.

Another patient will tell you that he observed, whilst dressing
in the morning, that when he placed anything over the eyes,
4s a sponge or wet towel, he commenced at once to lose his
balance, and was obliged to take hold of the toilet table with one
hand to prevent himself from falling; in this case again it was
the closure of the eyes which produced the difficulty in standing.
The same is again the case with some patients who aré in
danger of losing their balance at the moment when, in order to
put on their shirt they pass it over their heads, and thus cover

TARES DORSUALIS. 159

the eyes during a few moments, The most numerous examples
of this kind might be given, but I only wish to mention their
existence, since they will enable you to fix in a retrospective
manner the date at which the inco-ordination commenced.

T have used the word “inco-ordination,” gentlemen, in speak-
ing of the maintenance of the upright position, since this is
only possible when the different muscles of the foot, leg, and
pekeae nee ris raid wal actaca; hence the celebrated aphorism,

immobility is the finest movement of the soldier," is from a
point of view merely an expression of the pure
truth. On account of the disordered co-ordination the contraction
of the different muscles does not take place with the necessary
precision, either as regards time or degree; equilibrium in the
upright position is not unconsciously maintained, but becomes
the object of special attention on the part of the patient. In
this condition sight is of great service to the patient, by enabling
him to rectify any bad position which loss of the muscular sense
prevents him from otherwise perceiving, and hence occurs the
difficulty or even impossibility of remaining in the upright
position when the eyes are closed. As to the swaying move-
ments which occur when a screen is interposed between the eyes
and the feet, this is obviously due to a similar cause.

‘The motor disorders in this disease are due to analogous
causes. They are the more interesting on account of the fact
that in the first description of Duchenne of Boulogne, as you
know, these held the leading place.

Before describing the motor disorders connected with the
Lower limbs 1 must first show you the gait of some of the patients
whom I have caused to be brought to this amphitheatre. ‘The
ataxic gait* is well known to you all, and. twenty times already
the teachers in the hospital with which you are connected will
have pointed out its characters. I will not, therefore, dwell
at any length upon this point. You see with what diffi-
culty this unfortunate patient takes the few steps which
he bas to make. He has just risen from his chair, and
it is not without some difficulty that he maintains his equilib-
rium in the upright position. You will have observed that he

* MM. Demeny and Quonu have studied the ataxic gait in an interesting way

‘by means of the photo-chronographic procestes of M. Morey (Academie des
Sciences, May, 1289),

D. The patient is then asked to hop, the eyes being open or
closed. ae

E. He is then directed to descend a staircase and place him-
self at its foot, so that the least disorder in the gait may be
perceived, while the awkwardness of the patient is increased in
consequence of his being observed.

Such, gentlemen, are the ingenious methods which will some-
times be found of real service, and in doubtful cases 1 most
strongly'recommend your having recourse to what may be termed
* Exercice 4 la Fournier.”

Disorders of gait are not the only symptoms connected with
the lower extremities. Charcot dwells in his lectures upon a
singular phenomenon which not infrequently occurs in tabes.
‘The patient, who had never experienced any difficulty in walking,
suddenly feels his legs give way beneath him, and falls quite un-
expectedly, perhaps in the middle of the road at the risk of being
ron over; bis foot has not struck anything, nor has he stumbled,
but the legs bent without being influenced by the will, or rather
withdrew themselves entirely from its control. A moment later
the will regained its power, and the patient rises and continues
to walk as before. This is the “giving way of the legs" of
English authors (Buzzard). It may occur early in the disease
before the period of inco-ordination or they may both exist at
the same time,

As regards the upper extremitios, inco-ordination may be as

in them as in the lower, but on account of the
difference of their functions the symptoms produced by it are
also different.

Speaking in a general way, extreme awhiardness is found to
exist in the different movements, I have asked some of the
patients now before us to button their jackets, and you perceive
that they have great difficulty if they are able to do so at all.
‘The same difficulty occurs in tying a knot, shaving, &e., and
holding any small object between the fingers is in their case a
real task, and requires all their attention. If the eyes are closed,
or they are in any way prevented from seeing the hand, the
object is almost instantly dropped, and usually without their
perceiving it,

One motor disorder in insular sclerosis to which Charcot
never fails to draw attention in his lectures is the mode in which

ays

ri

162 LECTURES ON DIGRASES OF THE’ SPINAL CORD.

these patients take up an object. Tf you ask one of them to
take up an object of small size, as, for instance, a pencil, you

ante Fete cage ten toe

wa” yy le SL

Fig. 107.—Example of the effect which the controlling influence of Bessette
the inco-ordination of movements in tabes. The upper line was written:
patient whowe eyes were open. The eyes were then closed and he was, Se
see meare era gee wee as ‘The difference between these two
lines shows how much the inco-ordination had incrensod in the second case.
_ (Damaschino collection.)

will see that he does so in a peculiar way. Whilst you or I
reach the object almost insensibly and without abruptness by:
moving towards it one hand, of which the thumb is opposed
to the fingers, and only separated from them by a distance
equal to 2 or 3 times the diameter of the pencil, the patient
opens the hand, completely separates the thumb from the
fingers, and then, moving the hand close to the object to be
taken, causes it to “hover,” as it were, for a moment above it,
and then falling rapidly to seize it unawares os if it were capable
of taking flight.

2. InvoLuNrary MOVEMENTS resembling thore which are termed
“athetoid.” Such, gentlemen, are the principal manifestations
of inco-ordination ; but these ave not the only disorders con=
nected with the muscular system which may be observed in
tabes; I must mention a symptom which is less known, but
which may undoubtedly exist. I mean the movements resembling
those which are termed “athetoid,” and muscular shaking which
occur in certain patients suffering from this disease. Rosenbach*
had already mentioned them in 1876, and in his Revue of 1877,
upon Athetosis, Grasset + called attention to this observation,
In a memoir specially devoted to this subject, Audryt studied
these movements, which he attributes to lesions in the lateral
columns of the cord, corresponding with those in the posterior

* Rosenbach, Virchor's Archie. LXVII.

+ Grasset, Montpellier médical, 1877.

I Audry, Revwe de médecine, 1887. During the publication of these lectures a
new work appeared by Andry upon double athetosis, in which the subject of
thoso movements in tibos was very completely invostigated.

=

TABES DORSUALIS. 163

column. - Lastly, at a more recent date, in 1890, Laquer*
published the account of two other cases in which these move-
ments occurred, Other cases have been noted by B. Stern,+
and by Oppenheim.t I myself had the opportanity of observing
a very clear case, in which these movements occurred, (not
published) in 1885, since which time I have seen it twice,
though not to such a pronounced extent as in the former case.
From the works to which I have just alluded, and the knowledge
obtained from these few cases, I feelsustified in telling you that
objectively the condition is not one of true athetosis, but that a
series of inyoluntary movements occurs somewhat frequently
repeated, and usually more or less identical with each other in
the same patient. Thus whilst in one patient, for instance,
a finger will be raised, and almost immediately fall again, in
another, some degree of pronation or supination of the hand will
oceur. In reality these movements do not present in any way
the obligatory, and, to a certain extent, spasmodic character of
those which occur in true athetosis. Nothing in their form
specially characterises the movements, wrongly called athetoid,
occurring in insular sclerosis; the only fact about them which
specially attracts attention is that they are involuntary, and most
often unperceived by the patients. On account of the absence
of spasm I am not inclined to attribute thes: movements to
extension of the lesions to the lateral columns; I am more
inclined to connect them with loss of the muscular sense, the
amount of nerve force influencing the muscles being regulated
in a very imperfect manner,

5. Panarysts. Lastly paralysis, a symptom of quite a
different character, is one of the motor disorders occurring in
tabes which must be studied. Until the present time, gentle~
men, in order to fix more completely in your mind the true
character of the motor disorders in tabes, you have been told
that these were solely due to inco-ordination, and that even
when they were most pronounced the muscular power was in no
way diminished. To such an extent was this the case that
in an examination with the dynamometer the numbers often
indicate considerable muscular strength. This, gentlemen, was,

* Laquer, Wanderversumml. der 8, W. destecten Newrviogen, 180,
+ B. Stern. Arch. /, Paychiatrie, XVIL :
$ Oppenheim, Aer! Klin, Woekensehrif?, 1850, pp! 985.

You will notice that in this list no allusion
paralysis of the ocular muscles, which will be discu
‘quent lecture,
With snch a number of cases before you it will be
understand, gentlemen, that paralysis in tabes
recorded by numerous authors, of whom I need only
Marius Carre, Pierret, Grasset, Debove, &c.

Tt is usually benign in character, and should often be
paresis rather than paralysis.

Its duration is short, varying from a few days to a few v
» after which time the paralysis often entirely ceases to exist
spontaneously and without treatment.
Exceptions, however, may occur, and it must not be e:
that in every case the paralysis which exists in tabes w
completely disappear. The subject of paralysis in tabes
‘usually treated with few details in medical works, I shall
ce eee gentlemen, to say a few words about hemipl
paraplegia with sudden onset, and paralysis affecting but
nerve, or a somewhat limited region.
Hemiplegia has been specially studied by Debove* and |

* Debora, Pregrés médical, 1881,

TAMES DORSUALIS. 165
Blanche Edwards,” an te ccna A. Perma-
nent hemiplegia which may be accompanied by secondary

contracture; B. pierre hemiplegia unassociated with he=
minnimetheaia; C. Transient or permanent hemiplegia with
amesthesia of the skin and special senses. As regards the
pathological anatomy a focal lesion may be found in the cerebral
hemigpheres or pons varolii (haemorrhage, softening), or there
may be no appreciable lesion; it is curtain that in the latter
case, specially after hemianmsthesia, as Charcot observed, the
patients were merely suffering from hysterical hemiplegia ass0-
ciated with tabes, and analogous to that already observed to be
associated with insular sclerosis.

However this may be, gentlemen, one general rule is observed
in both varieties; the tendon reflexes, if lost before the onset of
hemiplegia, remain absent after its occurrence; the spasmodic
tendency, in short, which usually accompanies hemiplegia is
unable to counteract the atonic tendency which exists in tabes.
Tn some cases, however, the patellar tendon reflex has been
found to return, and even to be excessivet after hemiplegia.

Paraplegia with sulden onset is a symptom which should be
known to occur or serious errors in diagnosis may be made. 1
myself well remember the surprise which I felt in 1879 when,
coming into the service of Charcot with the knowledge which
was then current about this disease, I found myself in the
presence of such a case. Yes, gentlemen, notwithstanding the
classical opinion that paralysis only occurs in the later stages
of tabes, in the period which has been arbitrarily termed the
third period; notwithstanding this opinion, I say, you will
sometimes find that paraplegia is the first symptom which
attracts the attention of the patient. In his lectures npon the
preataxic period of tabes, Fournier, who has carefully studied
the different cases, qaotes a very instructive observation made by
Albert Robin. The case is that of a man who, “ whilst hunting
on foot, when strong and in good health, attempts to leap over a
diteb, and in doing so falls heavily into it. At first he attributes
this to clumsiness, or to taking off badly, but soon feels his legs
to become weak and benumbed. He limps, and has no longer

* Blanche Edwards, Tivse de Paris, 1880.

+ Goldfiam, Ueber das Wirdererscheinen von Sehnenrefiexen bei Taber, Je, Berliner
Klin, Woehsh/?, 1891, No. 3.

no maintain the upright position.

This form of paraplegia often obeys the general rales
T have just mentioned, that is to say, it is slight in
after a certain time more or leas completely ceases to exis |)
this, however, not being always the case as the paralysie is im
some cases found to persist.

‘To what is this symptom due? Scarcely anything has been” ;
written upon the subject, and one can only make suppositions,
Is there a focal lesion in the grey matter of the spinal cond
associated with changes in the posterior columns? This is
possible but has not by any means been proved to be the case.

As regards paralysis affecting but one nerce or a limited region,
the most frequent form, as you will see, is that affecting the
ocular muscles. This will be considered at a future time; at
the present moment I will only allude to unilateral paralysis of
the facial nerve, which sometimes occurs and may affect all the
branches of the nerve, as when due toa peripheral cause. Tt
must be observed, however, that this so-called facial a val
tabes is often only the glosso-labial hemispasm of hysteria,

nerve (E, Remak, Mibius), unilateral paralysis of the muscles of
mastication (Schultz) must also be mentioned. ‘These forms of

i
i!
aE

‘TABES DORSUALIS, 167

paralysis are also, as in the first mentioned varieties, benign in
character, and liable to diminish or cease of their own accord.
The electrical reactions are very variable, being at times normal,
while in some cases the reaction of degeneration exists in a
mire or less complete form, even though the paralysis is of but
temporary duration.

With respect to these cases of local paralysis I must repeat,
gentlemen, the observations already made in connection with
the hemiplegia which occurs in tabes. It is very probable that
a certain number of these cases also are due to hysteria alone,
and you must always be on your guard, and consider this
alternative in forming your diagnosis.

TABES DORSUALIS. 169

‘The shooting pains are also rapid and transient, but they are
more locally seated, occurring and ceasing in one spot, and being
often compared by the patients to the prick of a pin or even the
stab of a dagger. This, gentlemen, is not merely a mode of
speech, and, in fact, patients are not rarely met with who tell
you thst at first they often turned sharply round to see who had
assaulted them, so much did the sensation resemble that of being
stabbed by a knife.

The wrenching pains are also localised but distinguished from
the preceding by the fact of their occurring less rapidly. They
can be, to a certain extent, analysed, the patient having the
“sensation of something being screwed into the tissues,” the
penetration seeming to be accompanied by a twisting motion.

‘The turning pains consist of sensations resembling those of
Pasi tees aeoaliare.0h tions. ware ernoratieacl "These vary in
the same manner as the forms of pain already mentioned.

‘The seat of pain may be, as already stated, the limbs, specially
the lower limbs, the pain being felt either in the thigh, or below
the knees. In the upper extremity the seat of election is usually
the inner side of the forearm and hand; pain is liable to occur
also in the trunk, and at times is felt in the face.

According to the description of the greater number of patients,
they appear to arise less in the skin itself than underneath it,
and even in the muscles, bones, or articulations.

‘The pains are at times isolated, and arise one by one; at other
times they arise at the same time in many points, and then

between which the patient is at times completely free from
pain; then when the paroxysm commences, the pains occur in
more or less compact groups; each paroxysm varies in duration
from half an hour to several days. They are usually of spon-
taneous origin, sometimes occurring so regularly that the
patients expect them at a definite period; sometimes, however,
they may be due to exposure to cold, change of temperature,
fatigue, or emotion,

The intensity of the pain varies much, and corresponds in no
absolate degree with the severity or duration of the disease;
usually, however, the pain occurs early in the complaint, and
often more or less completely ceases when the period of inco-

2 apbiehnpelidememepaticrtit :
by the name of—

y- Paroysmes of muscular exhaustion, 'Thes
tioned by Pitres* in 1884, and have been n
number of cases since that time. According
specially exist at the onset of the disease;
and without obvious cause, persisting during so
days, and ceasing without being followed by |
sensation of fatigue. ‘These paroxysms occur in
as those already described at varying intervals,
avmost painful sensation of fatigue and muscular
analogous to that felt by healthy persons after too 1
prolonged exercise., The seat of the sensation is in
erectores spins muscles, and it may be so severe that en
and vigorous persons are obliged to take to their
middle of the day, and rest in the dorsal position
paroxysin has ended.

b. Persistent vaix. The character of these pains vay
but they often tend to exist in a circular form; such is
famous “ girdle-pain,” a feeling of constriction as if a be
iron was tied tightly round the chest or waist of the p
and which is sometimes of extreme severity. Fournier q
a case of this kind, in which the girdle-pain was severe en
to produce dyspnoea, resembling that which occurs in
affections of the heart or Jungs. In the limbs the fee
constriction may resemble that produced by a bracelet
upper,and bya gaifer in the lower extremities. Not infreqn
areas exist in the limbs, or more often in the trunk, of
or less size, in which a persistent and burning sensation

e. ANORMAL SENSATIONS. The most usual of these sensations
are those of tingling and numbness which are felt in ce
* Pitres, Progris médical, 1984.

TARES DORSUALIS. 171

parts, specially in the limbs, and most often along the ulnar
border of the forearm and hand. Sometimes, again, the patients
feel a strange sensation of cold between the skin and subjacent
tissues, “aa if water was passing under the skin”; lastly, but
inore rarely, an itching sensation is felt in different parts of the
limbs, £

None of this class of symptoms, except the feeling of numb-
ness opon the ulnar side of the forearm, is of any great importance
as far as the diagnosis of the disease is concerned. In some
cases, however, the exiatence of an area of numbness, such as
the sensation of a veil, or spider's web, upon’ the face or penis,
has indicated the existence of tabes ab the most early period of
the complaint (Charcot).

IL—SENSORY SYMPTOMS AMENABLE TO OWECTIVE CONTROL,

(a) Anmathesia (analgesia). Anwsthesia occurs not only at
the surface of the skin but also in the deeper parts, viz., the
muscles, bones, and articulations. ‘Thus it is possible to make
the muscles contract in some patients by means of a strong
electric current without producing pain, and, as I shall have
oceasion to remind you, fractures and dislocations are- most
often painless in patients suffering from tabes. Lastly it
will be seen that this analgesia favours the occurrence of
certain trophic disorders (callosities, perforating ulcers, arthro-
pathy, &e,).

When it is very pronounced and its existence is accidentally
revealed to the patient he may know that this analgesia exists,
but most often it has to be sought, and in the most careful
manner,

Although the seat of the anmsthesia varies and may oceur in
any part of the body, and in different forms, it is most often
found clinically to prefer certain parts and to be disposed in a
special way. Oulmont has published some interesting remarks
upon this subject from which, though absolute rules cannot be
Jaid down, useful information may be gained. In a few words
these are the conclusions at which he has arrived :-—

Of 20 women suffering from tabes under the care of Charcot,
taken recently by hazard at the Salpétriére Hospital, he found
anesthesia to be totally absent only in 3 patients.

é

‘TABES DORSUALIS. 173

whilst the back of the foot is seldom affected. Very frequently
there is some rypermatheria upon the arch of the foot. In the
legs, the parts most often affected are the Imecs and madleoli,
‘The thighs are less liable to be affected by anesthesia than the
legs below the knees, their inner surface being the part which
remains longest unaffected in the whole zone which corresponds
to the adductor muscles.

You will observe, gentlemen, in the figures which I now
place before you, the areas of anesthesia found in different
patients suffering from tabes, that the seat of these areas
is not over the course of a nerve or nerye branch, nor is it
strictly limited to the area of distribution of the latter;
most often, on the contrary, the affected part extends over
areas supplied by different cutaneous nerves, and you will find
that in some cases the area of anwsthesia has the shape of a
leg of mutton, but there is nothing else in its appearance
analogous to that described by Charcot as existing in hysterical
paralysis.

b. Hypercesthesia, or rather hyperalgesia, since, as Leyden traly
observes, the tactile sensibility never becomes more acute in
tabes than in the normal state, the sensibility to pain being alone
increased.

‘The hypermesthesia usually exists in areas, which are of mach
smaller size than those of anesthesia. At the end of a certain
time again the hyperesthesia may be followed by anesthesia,
the duration of the former condition being usually far less than
that of the latter,

Tn these areas the hypermsthesia may exist in a moat excessive
degree, the gentlest friction with the hand or clothes, even a
current of air may produce sensations of acute pain. In some
cases this condition is one of torture to the patient, and the
symptom of which he specially complains,

Sometimes again these areas of hyperalgesia constitute a
centre from which the lightning pains start; a reservoir, as it
were, of pain, which is but too often inexhaustible.

According to Erb, when hyperwesthesia exists to somewhat
pronounced degree in tabes, the meninges probably participate
in the morbid process.

Under the name of relative hyperesthesia Leyden, who has
carefully studied the sensory disorders which exist in tabes, has

pronounced after the influence of cold.
c. Paresthesia. Under this term I shall i
disorders of sensibility which I am about to enu
myself, however, to the few observations which it
make in order that you may understand the su
being considered. These disorders have been
by Leyden, Berger, Binswanger, and the different
have devoted their attention to tabes.

‘The retardation of sensations is frequent in tabes, sp
the lower limbs. It is easily discerned by directing
to call out as soon as the pain produced by a prick |
measuring the length of time which passes between the m
at which the prick was made, and that at wh
announcing its sensation occurred. The length of |
is usually 2 or 3 seconds, but in some cases it amo
10 seconds, or even more, Richet states that in tl
patient the interval is of greater length when the foot #l
the thigh is pricked. Is this difference due to the g e
of the path through the nerves in the one case than in
or, as seems more probable, to the fact that the sensory
are very much more pronounced in the foot than in ti
It must be observed that this difference is liable to ver
yariation in the same patient from one moment to another,

The sensations produced by an impression may be all reta
or this may perhaps occur in only one or more forms of
tion. Thus, for example, a patient suffering from
closed eyes is pricked, and he states that he experi

‘TABES DORSALIS. 175.

senation of contact at once, but it is only after 3 or 4 secorids or
more that he shows by the contraction of his features, and’ by
his cry that a sensation of pain is now felt. In this case the
sensibility to touch was well preserved, while the sensibility to
pain was much retarded. ‘The same fact may be observed in
connection with sensibility to temperature ; when a fragment of
ice is placed upon the skin the patient feels at once a sensation
of contact, and it is only after an interval that the ice is
perceived to be cold.

Sensory metamorphosis is said to exist when patients suffering
from insular sclerosis cannot clearly recognise the nature of a
sensation perceived, ‘Thus, for example, a prick or pinch may
seem to them a more or leas severe burn,

The inadility to recognise the seat of sensations is not an infre-
quent symptom, and patients suffering from tabes may feel the
sensation of a prick in the foot when in reality a pin has been
passed into the calf. It may, again, happen that impressions
are not peresived over the whole part where they exist. Thus
# scratch some centimetres (1 centimetre = ‘394 in.) in length
may only be felt as a simple prick.

The following symptom is called by the name of “ dissociated
ancesthesia.”

Tn a patient suffering from tabes who cannot feel a prick the
sensibility to temperature is preserved, or vice verad (M, Par-
tnentier has recently published such a ease), or while the
sensation of a prick is preserved compression is not perceived.
Tactile sensibility is usually lost later and in a loss degree than
sensibility to pain, though it is also liable to be affected. This
notably contributes to increase the awkwardness in using the
upper or lower limbs (the ground is felt by the patient as a
carpet, cotton-wool, indisrubber, &e.).

The recurrence of sensations should be specially mentioned,
since, from a clinical point of view, a knowledge of its occurrence
is indispensable in order that a complete examination of the
sensibility may be made in these patients. When, in fact,
daring the examination of a patient suffering from tabes a
certain number of pricks has been made, a moment often
arrives when the patient will feel the sensation of a prick

none bas been made, in a word he feels that he is
pricked when this is not the case. The previous impressions

preesling

‘TAKES DORSUALIS. 177

are clearly perceived by the patient. This symptom is of the
same nature as those in which impulses, which are of insufficient
strength when isolated to produce the effect desired (sensation,
muscular contraction) join their effect together, and experiencing
that which physiologists term a “summation,” finally exert
upon the nerve centres safficient influence to determine the
effect desired.

The loss of power to respond to impressions is also a symptom,
the existence of which should be known, since it may com-
pletely vitiate the results of an examination ; it is exactly the
reverse of the “summation of impressions” "; in this case in
fact the sensibility is diminished by continuation of the same
exciting force. E. Remak, B. Stern, have studied these facts,
and the first of these authors showed that in some patients
suffering from tabes, a feeble electric current which was at
first well perceived soon ceases to be so; if the strength of the
current is then increased, it is again perceived, soon however
ceasing to be felt, the same occurring again and again as the
strength of the current is changed. At times this diminution
in the power of appreciating impulses occurs in eclipses ;
the temperature of a hot object applied to the skin is at first
clearly felt, but after some moments is no longer perceived ; if,
however, the object remains in contact with the skin it is again
felt, although no change in the temperature has occurred, then,
again, it is not perceived after some moments, the same changes

ing as time goes on.

Tn all these examinations, which require the most careful
attention, it must not be forgotten that, owing to the effect of
certain purely physical influences, the sensibility may be much
modified ; it is thus that anwsthesia may be increased by cold,
whilst, on the contrary, heat, friction of the skin, the passage of
the electric current may cause it to cease for a certain time, or
atany rate diminish its intensity,

Such, gentlemen, are the different sensory symptoms which
occur in tabes, to which I wished to call your attention; if 1
have described a few of these at some length, considering the
importance which they assame in the course of this disease,
and the frequency with which they occur, it is because they are,
asa rule, but little known, and special interest is thus attached
to their description. Nor do I doubt that rare as some of these

F

178 LECTURES ON DISEASES OF THE SPINAL CORD.

symptoms seem to be, they are found to exist more frequently
if carefully songht in all the patients suffering from tabes who
come before you.

I must ask you to remember, gentlemen, in every case, that in
discussing the sensory symptoms of tabes, my object has been
of a didactic rather than clinical character. It is little probable
that you will ever find these different symptoms associated
together in the same patient, and one cannot too much insist
upon the irregularity and variety of the sensory symptoms in this
disease. In some patients the symptoms are extremely pro-
nounced, while in others they scarcely exist; in a certain number
after having been very pronounced they finally almost completely
disappear. Unfortunately, whilst we can recognize the various
forms in different patients, we have to confess our ignorance as
regards the cause of these varieties; we are actually unable to
indicate the anatomical cause on account of which one patient
suffers intensely whilst another is almost entirely free from
pain.

LECTURE XVI.
TABES.

SYMPTOMS (continned).

C, Dtsonprms oF merunx action. Putellar tendon refer. Procastions to ho

GeyrLeMEN,—The disorders connected with reflex action are
amongst the most important which occur in tabes from a
nostic point of view; the symptoms which are thus in fact
produced occur at the moment, and in the conditions which we
ourselyes choose ; such production is the cause of their exist-
ence, and on that account a degree of precision is acquired
which the different ‘‘ symptoms” developing spontaneously, and
cecurring, so to speak, when they please, cannot pretend to have.

You are aware, gentlemen, that reflex movements may be
obtained throughout the whole length of the cord, and even by
means of the brain; some of these, again, resemble each other
so much that they may be classed together. ‘This, in fact, is the
ease with the fendon rojlexes, and it has been ugual to i i
specially those of the knee, the tendo-Achillis, that of the wrist,
elbow, and not the tendon reflexes of all the different muscles,
In the same way, as regards the cutaneous reflexes, the planter
and abdominal rejlex are specially tested, without much attention
being paid to the reflex movements which may be produced by
irritation of the skin in the intermediate region. Such is the
case in studying tabes, in which disease some of the reflexes are
of special interest, and it is these only, gentlemen, which will be
now considered. As you know the reflexes connected with the

» SaCTOREE YAS a ES CO

wet ee amps Ge nus Inger sel shweld perhaps be
weeeeeert oius =~ 0 arr ere. op apenk of then
ag. et ee Ue re Cet mee Spee occurring 2
pes wail Ste

Tyesccaa SsOesus=sx «6 Gr saperiemee beth af the
femain enit ac ums ie cuntied mr to know thet
somes Se ne mennecs If ie dca

ees scnient Wau fs 7 sami sr poe Qemdon-refiex; ,
were dee weve ik =: mectudionily: a wry smell mambo
ame ee ee ova cally iene and ine tree siguili-
wee. Ib wil we Stems Je ceive, ie mv egimiom, to review
wegeli. So speaior -f ies. <r rimcipel fees whack are mow

aes prog 2 Se ceo
ee
Geese smi i: ae a wher thet it may be
eaten. ene. =m 2 pow ame ft waew, certain pre-

the bollow 7
nage nen its
of the patient. and piacing ene kare below the calf of the
p be tested raises it t» sach an extent that the foot does
gach the ground.

The observer raixs the thigh by means of the hand
below the tendons of the bicep: and semi-membranosns,
he patient is dir-cted to rest the leg upen it: the

TARES DORSUALIS. 181

extension of the thigh upon the pelvis which the patient thas
produces has often for its consequence to relax the quadriceps
femoris which is itself a flexor of the thigh upon the pelvis.

When the patient, instead of being seated, is in bed, he is
enjoined to leave the limb relaxed, and raising the thigh the
lower limb is placed in such a position that the hip and knee
joint are both in a state of flexion; percussion is then effected.
Tf the patient cannot relax the limbs sufficiently he is made to eit
npon the edge of the bed, care being taken that he is so entirely
seated that the edge of the bed touches the hollow of the knee.

When the relaxation of the quadriceps is obtained by one of
the means which I have just mentioned, the second part of the

must be effected, viz., percussion of the Ligamentun
Patella, This percussion should as far as possible be made upon
the middle part of the tendon at the same distance from each of
its insertions ; if by simple inspection of the region this point
is not accurately indicated it can be detected by means of the
finger. The blow may be given by means of the ulnar border
of the hand or the extremity of one or more fingers, or come
instrament; that which is most often used is the percussion
hammer of Skoda; in England a stethoscope is often used, of
which the ear piece is hollowed out in such a way as to contain
an indiarubber band. Whichever plan is adopted the blow on
the tendon should be slight at first but always “sharp,” becoming
gradually stronger if necessary; the strength of the blow neces-
sary to produce muscular contraction is in fact an important
consideration. You will be able to see that when the reflexes
are excessive a much feebler blow is required than when they
are diminished.

The percussion having been effected, it is necessary to detect
the muscular contraction iehich ie thus produced (patellar tendon-
reflex). Most often this is easily done since the contraction of
the quadriceps prodaces a more or less pronounced movement
of extension in the leg, and, as on account of the relaxation
which follows in all the muscles of the lower limb, the leg
almost immediately falls again, an oscillating movement of the
whole lower extremity is clearly perceived. Sometimes, how-
ever, no such movement is produced; it must not be inferred
from this that the reflex is absent; the contraction of the
quadriceps may in fact: occur, but be too weak to produce a

, however, suppose that this is always an

irst sight it appears to be, and I must now men’
of error.
| some cases, even after taking all the pree
ned, no reflex movement can be obtained on
been impossible to place the muscles of
afliciently relaxed condition, and you must

nsider these cases as examples of abolition o
‘a general rule, whenever you are not

the observation that the fibres of the
itly relaxed, before coming to a definite conclu
i ition of the reflex, repeat the examination
sys until you are certain that the muscles of the
acc only when this is the case that you are
itive opinion,
is often liable to mistake for a contraction of the
. the movement of the leg, which is produced in a
nical way by simple communication from the bl

attention, and by varying the strength of
can be easily avoided.
ly Westphal has given the name of pseudo-t

h a blow upon the skin of the knee p

‘TABES’ DORSTALIS, 183

reflex movement of the leg analogous to that which oveurs in
the patellar tendon reflex. If this is thonght to be possibly the:
case, it would suffice, in order to solve the question, to make a
somewhat thick fold of the skin with the fingers of the left hand
in front of the patellar tendon, and to make the blow fall, not
upon the tendon, but upon the fold of the skin ; should a move-
ment of the leg be thus produced, it is certainly due to the
of Westphal.

Such are the principal points to which attention must be paid
in making this investigation, Let us now attempt to determine
the nature of what occurs, and to ascertain what signification
should be attributed to it,

Tt is said to be a reflex movement. What then is a reflex
movement? a movement due to the motor portion of the cere-
brospinal axis being caused to act by irritation of a sensory
organ transferred to that axis by afferent nerve fibres. A tendon
reflex is « reflex of which the origin is a special form of irritation
of the sensory organs contained in the tendon.

Let us consider what in this special case (patellar reflex) will
be the seat of the irritation and consequent reaction.

‘The first part of the process, as you have seen, is the blow on
the patellar tendon ; this blow constitutes the primary irritation.
That this irritation may be transferred to the nerve centres it
mast so influence the peripheral receptive organs that the vibra-
tion will react upon the cord, through intermediate nerves.

Do the tendons then contain nerves* and nerve endings,
which can act as organs of peripheral reception? ‘The
anatomical knowledge obtained during the last twenty years
enables us to answer this question with certainty.

A certain number of authors have already noted the presence
of nerves in tendons; in 1875 Sachs stated that these nerves

free extremities seated in the thickness of the tendinous
lamin, and at their surface; a short time afterwards T'schiriew
noted the existence of swellings in these extremities. On the
other hand Golgi* discovered in 1878 the existence of special
organs in the tendons (musculotendinous bodies), analogous to,
and in close relation with the Pasinian corpuscles, which are also
found in large number in the tendinous lamina.

* Sachs, Die Nerven der Schnen.—Reiekert's und Du Boys Regwond’s Arch., 1875.
“+ Techiriow, Arch. f. Paych., V, p. 303.

a

faces parallel to the surface of el
(from 80 to 100 w in length, and 5
can consequently be detected by mear
They are usually seated upon that surfac
the muscular fibres are ‘Toma, oa

Be Ouepsils te Galgl wait toeodan ramifoations of she rmaeng

left extremity E with the muscular fibres D, whisk oan to be i

it. Aisa Pacinian corpusclo, which receive one of the branches of

of tho nervous filamont C, of which the other branch passes into tl

of Golgi.
becomes gradually confused with the tendinous fib
the other somewhat thicker and longer, gives inser
different levels to a few muscular fibres. As regards
the corpuscles of Golgi are formed of fibrillar connect
containing scattered nuclei.

These fibrils are parallel to the axis of the corpusel

* Golgi, Zatorno alle distribuzione © terminazione sei nerci nei tendini «
@altri vertebrali (Gaz, méd. itala-lombarda, 1878) et Mémoire de f Acad,
Turin, série I, t. XXXII.

+ Cattaneo, Sugli organi nervosi terminali muscolo-tendinel in condiziont
-@ sul loro modo di comportarsi in sequito, &c. Accad. Reale dele Science di
9 Tanvier, 1887. ‘

TARES DORSUALIS. 185

abi: casing coated with endothelium, somewhat
© that described. by Ranvier upon the sheath of

the interior of the corpuscle, the nerve fibres
trated either into its middle portion, or more
of its oxtremities, divide either dichotomously, or
ions which spread over the whole corpuscle ;
go to its surface haying lost their sheath of
forming a fine reticulum. ‘The nervous filaments

of the spinal cord (at the anion of the dorsal and lambar
‘case of tabes in which the patellar tenilon reflexes could still

‘case of tabos in which the patellar tendon reflexes had catirely
for some years, In thin case tho lesions extend considerably
Pointed line und penetrate into the territory of the external

Ry dwelt upon this delicate anatomical point
of my belief that you did not all perfectly know
At the same time I have by no means the pre-
that amongst the different nerve endings in the
tendons it is the function of one form rather

the question of the different
rk by M. Pilliet, im the Journal ale I

which the fibres pass before joining the g
terior horn; it is in the zone of entrance of th

‘The seat of these fibres is external to a Tine |
the posterior median fissure, and passes from
posterior horn to the posterior surface of the
mere onal bain: ae ee
Every lesion seated in this part* would p
them, and may, in poe ecmith tod
diminution or absolute loss of the patellar

It must not however be thought that the fibres
tute the afferent path of this reflex exist
length of the cord; they are only found in a ¢
Westphal has also determined their seat both in
and transverse direction, According to him th
the cord exclusively at the union of the dorsal and
of the covd.

You will therefore understand, gentlemen, that
reflex will not be caused by every lesion of the post
of the cord, but only in those in which the seat of

* The importance of this Jocalisation by Weatphal
It amounts, in fact, to w statement that tho afferent
tendon-reflex enter the cord through the posterior roots of the 1
part of the lumbar rogion, and that thoy are abort fibres, We
most fibres of tho posterior roots enter the cord by means of the tr
“tho external bandicts.” ‘Tho short fibres divide here while the Tong
those of modium longth pass on in order to join the column of B
of Goll. A losion in the external bandlets alone being able to
of this roflex, it ix vory probablo thut the afferent fibres by:

do not leave those bandlots, and are in consequence short |

TARES DORSUALIS. 187

in the region of the external bandlets and quite at the upper
part of the lumbar region. This localisation is in the case of
tabes of considerable importance. Let us return to the study of
the patellar tendon reflex considered in a general way. We
have traced the path of the primary impulse whose origin was
irritation of the patellar tendon as far as the grey substance of
the posterior horns. When the grey matter has been reached
what is then its path?

Tn all probability the nerve fibres by which the impulse passes
transmit it to one or several cells of the posterior horns, and on
account of the means of communication existing between these
and the cells of the anterior horns it passes into the latter, or
very possibly into the posterior root fibres which place themselves
directly in contact with the cells of the anterior horns. The
effect of the impulse is to cause the anterior horns to act: accord-
ing to their function, that is to say, by producing contraction
of those muscular fibres over the action of which they preside.
‘The primary afferent impulse is thus transformed into an efferent
inotor act ; the motor impulse passes on its part by the following
path: the anterior roots, sacral plexus, motor fibres of the erural
nerve supplied to the quadriceps femoris whose contraction pro-
duces the movement of the leg which you perceive.

‘This, gentlemen, is a somewhat long and complicated path,
and you will easily understand that if at any point some
disorder occurs connected with the working of the organs
through which the impulse passes, a diminution or even
cessation of the reflex may occur. You will also understand
that causes which affect either the circulation in the cord, or
the museular tone, may have considerable effect upon it.

Let us first examine (@) the Local conprrions which may
affect the production of the patellar tendon reflex when seated
in one or other of the segments of its path.

As regards the patellar tendon itself, adhesions or displace-
ment of the ligamentum patella or condyles: may occur after
arthritis, dislocation or some other cause which prevent the
necessary impulse being produced by percussion, and hence the
reflex cannot occur.

Tn the afferent peripheral nerve any disorder whatever of the
nerve trunk or its branches (peripheral neuttis) may produce
diminution or cessation of the reflex.

iy eas

‘Fig. 112.—Dingram of the path taken by the patellar t

cells an afferent motor impulse is transmitted to the qx
lettor B indicates the patella.

the nerve fibres which connect them, are destroyed, fl
diminished or ceases; this occurs in the course of acut
poliomyelitis, and specially in infantile spinal p
Tn the efferent motor nerve. Any lesion of this
traumatic or due to peripheral neuritis from
prevent the motor impulse from being transm
muscle and consequently produce cessation of the
In the muscle, Atrophic muscular, or other cl
diminish their contractility produce first diminut
cessation of the patellar tendon reflex. This speci
in idiopathic muscular atrophy.
Under the name of GENERAL coNprTIUNS '(b),
u the production of the patellar tendon
all those which do not immediately pass along.
path by which the reflex passes.

‘TAMES DORSUALIS. 189

Of these conditions some tend to produce piminuTiON, others
CkssaTion of the patellar tendon reflex.

Age has a striking effect, and thus P. J. Mébius observed that
in 56 persons free from disease of the nervous system, who
were more than 80 years old, this reflex was lost in 9
cases, and very slight in 7 others, On the other hand,
Pelizas, who tried to obtain the reflex in 2,400 children, never
failed to do so, It ig usually more pronounced in children than
in adults.

Fatigue has a similar effect. Orchanski having showed that
when muscular exertion is commenced the knee reflex increases
whilst on the occurrence of fatigue it diminishes, and even ceases
when the fatigue is such as to cause trembling of the limb.

Sleep has also the effect of diminishing the reflex,

Anemia of the spinal cord (?) from any cause would have the
same effect.

Different infectious diseases (diphtheria).

Some forms of acwle intoxication, notably that by clilorofirm,
may totally prevent the appearance of the reflex, thia effect,
though complete, being transient.

Certain forms of transverse lesion of the spinal cord seated at
some distance from the lumbar region of the cord (in the dorsal
or cervical region) are associated with paraplegia, in which the
sapere degree tei bed ies es 9
reflex. Ch. Bastian,* then Bowlby,t recently called attention to
these facts, and Babinski has just published an observation of
the same kind. According to Bastian, this cessation of the
reflexes would only occur in cases in which both motion and
sensation (notably with respect to pain) are absolutely lost, and
the transverse lesion is consequently complete.

‘The general conditions which may cause the patellar tendon
reflex to be EXCESSIVE are of different kinds.

Certain forms of strong emotion, such as that produced by
loud music, would have this effect, according to Lombard, who
has investigated the condition of the reflex in himself in 239
different conditions.

* Bastian, Royo! Medical and Surgical Society, 23th February and 17th May
“Soin Ss a lo 18M 5 9408/10

Babinski, Parapligie flasque par comprossion de la moollo, Arch. de wéd,
expin 11, 208

L)

emcee

belongs. In these cases there is a very different

ibility the inhibiting effect which is normally
the fibres of the pyramidal tract upon the centres
__ Matter of the cord ceases to exist, and these oe
_ restrained in action become more irritable, this co
7 increase of the patellar tendon reflex.
are the principal facts upon which the patholo
cis based. I must, however, say a few words in
which exist or have existed upon this

‘TANES DORSUALIS. 191

at the same time shall avail myself of this opportunity to mention
the history of this interesting question.

In the first place allow me to remind you that from very early
times children have played the following practical joke upon
their companions : ee
tion their companions amuse themselves by sharply
aera peer enor aoe Naar ico Pe
hand, the immediate result of which is that they fall suddenly and
unexpectedly to the ground; or when their friends are seated
they lightly strike the knee of some one near them, whose legs
are crossed, in the same sudden way, being amused by the abrupt
movement which is thus produced in the foot. These are but
the games of children, of which physiologists, being seriously
inclined, were careful to know nothing, absolutely ignoring, in
fact, all phenomena of this kind. It was necessary for the medical
profession to take the matter in hand, this not being the first
time that such a thing has occurred, and it is to be hoped in the
interest of physiology that it will not be the last.

In 1875, Erb, in Archiv fir Psychiatrie, devoted some pages
to the description of certain reflex movements, which he terms
tendon reflexes, special attention being paid to that which takes
place in connection with the knee, to which he gives the name
of pateilar tendon reflex (patellarsghnenreflexe),

By a singular coincidence in the same number of this treatise,
and immediately after the article of Erb, was one by Westphal
upon the knee phenomenon, which is nothing in reality bat the
patellar tendon reflex. The explanation of its how-
ever is quite different in the eyes of Westphal. In his opinion
the contraction of the quadriceps is due to the irritation produced
in this muscle by irritation of the tendon which is struck. As
it is in this tendon that all the fibres of the muscle end, it is
not surprising that its irritation should specially affect the
muscle. That the reflex action however may occur, the muscle
must be in acertain state of tone, upon which Westphal much
insists, although be does not clearly explain its nature. At the
same time, it is the loss of this tone which causes abolition of
the patellar tendon reflex to ocour.

‘The comparison of these two memoirs is on the whole to the
advantage of the former, It is certain that Erb was the first to
describe the phenomenon, and it is equally true that he was

Hospital
the oe lcton of the works which Ih
was also interested in this symptom,
his remarkable thesis for the doctorate, d 0
of pages to it which T cannot too strongly
read. °

‘The opinion that this reflex had a tendinous
become more and more generally adopted, an
at the instigation of Westphal had studied ite fi
logical point of view, specially maintained it, alll
just said, it was contrary to that of the profe

In England a certain number of authors,
the least distinguished, amongst whom I
and Waller, are of opinion that this is an
muscular contraction. In support of thii
allude to the fact that the interval for the p
(that is to say, the time which passes between p
tendon and contraction of the quadriceps) is only from
thousandths (yy to Js) of a second (Tsirjew), it
admitted that a reflex act can possibly be ef j
time, since according to physiologists the rapidity:
current is only 30 metres (1 metre = 89-87 in) 7
More recently these authors have also recognized th
cord has some influence, and state that in order
tendon reflex may be produced, the integrity of #
absolutely necessary. Jackson and Bastian be
action of the cerebellum is indispensable in order |
may be maintained, which enables the tendon
produced.

It may be replied to these objections that on the one]

rapidity with which the nervous impulse passes is not
known, while on the other hand the interval for the
tendon reflex is longer than 30 to 35 thousandths (5 to gts) of
a second, measuring in reality from 48 to 52 thousandth (¥ to

becomes anzemic (as from compression of the aorta).

4. The loss of the same reflex in diseases during which the
direct irritability of the muscles is scarcely modified at all (tabes,
general paralysis of the insane).

¢. Its production by the sammation of irritations (Jarisch and
Schiff)* upon condition that these very small shocks take place
at the rapidity of at least one in each second.

d. Its production by percussion of the periosteum (periosteal
reflex) in certain regions, notably that of the wrist. In these
parts, in fact, it cannot be said that traction of the muscle by
the tendon causes its irritation; and idiopathic contraction,
since the blow is nob meds upon a tendon) bat pasa dire ody,
incapable of being moved by traction, namely, the bone itself.

We have passed in review, gentlemen, the principal theories
which have been suggested in connection with the patellar tendon
reflex, and. almost exactly the same ideas have been formed as
regards the other tendon reflexes (that of the Achilles tendon, of
the triceps in the arm, of the flexors of the foot, of the wrist,
of the masseter, &e.), These notions will now be applied to
the study of the patellar tendon reflex in the course of tabes.

Usually there is loss of this rejlex in tabes from the very
beginning of the affection.

To this rule, however, there are some exceptions.

In some patients suffering from tabes the reflex persists
although the signs of tabes are already quite characteristic, In
these cases the lesions most often occupy almost exclusively the
upper part of the spinal cord (upper form of tabes). You must
know, however, gentlemen, that in these cases its preservation

* Inrisch et Schiff, Cutersuchwegen aher das Kniephanimen, (S32. a

196 LECTURES ON DISEASES OF THE SPINAL CORD.

form of paralysis has occurred at some period of the disease the
number would, in my opinion, be much higher.

As regards the greater frequency of paralysis in one or other
of the musclesa difference of opinion exists. According to Berger
and Woinow the external rectus is most often involved ; accord-
ing to de Watteville and Landolt it is also the external rectus.
Then come the superior rectus, inferior rectus, inferior oblique,
and Instly the superior oblique, ‘In some cases the paralysis,

Fig. 113,—A man suffering from tabos with double ptosis, This photograph wae
taken while the pationt was in the attitude which he assumed when not wishing
to look atany object. (Collection of Charcot.)

instead of being confined to one muscle, affects them progressively
one after the other, until the eye cannot make the al ightest
movement ; gressive eelernal ophthalmoplegia is then mines
exist.

Of the nerves of the eye it is the third nerve which is most
often affected. There will usually be no difficulty, gentlemen,
in recognizing the existence of these forms of paralysis on
account of the deviation of the ocular axes which they produce.
Tn the cases in which no deviation exists when the eye is in the

:

TAKES DORSUALIS. 187
ordinary position, it must be sought by causing the patient to
follow the finger with his eye whilst it is moved in different
directions. In addition to this the inquiry must be made
whether diplopia has existed, and one of the first questions
which must be asked is the following: do you see two objects
instead of one? have you done so ati any time?

Such o form of paralysis may be either unilateral or bilateral.
Fournier, who has given an excellent description of this symptom,

Fig. 114.—A man wuffering from tabss with double ptosis, This photograph was
taken when the patiest was in the attitude which he assumed when looking
at an object; he was then obliged to throw his head back until the object
corresponded with the narrow opening which existed between tho two eyelids:

rightly insists upon some of its characters, namely, that it is
dissociated, partial, perhaps very limited in degree, transient,
st instantaneous duration, that
nsly after such a

ephemeral, and at times of alm
is to say, the paralysis asually ceases epontane
short time as a few weeks or days, or even a still shorter time;
it is however Malle to recur,
Of these forms of paralysis
are due to a periphe

some, in the opinion of oculists,

al caw others to a central cause of

is |

1% LECTURES ON DISEASES OF THE SPINAL OUREL

These facts, however, have not been clearly

proved

Ptosis is a symptom which is freqaently observed at the oust
ef tabes. In most caces it is unilateral, bot may exist on both

Fig. 115.—Asymm
tates. (Damas

of the axes of the cyes on account of ocular paralysie in

no collection.)

sides ; it is usually associated with deviation of the eye outwands,
this being the

of paralysis of the thinl nerve; some
ed and unassociated with any paralysis of the
. Sometimes it is incomplete, but at times is eo
pronounced that even when bend the head backwards the
patient is unable to with the aff ; he is obliged to
aise the € ctly with the finger, or if his work requires
the use of the two hands by means of a bandage or eye-speeulam.

Sometimes watering of the eyes or epiphora are associated wath
peular paralysis, In certain -s these symptoms are not due
to paraly sis, but to a disordered condition of the secretion, of
which we shal

Lastly, other sympt arved to which attention
hae only been recently drawn, and which are consequently but
Tittle known

Beoplitha!inos, of which we shall speak at greater length when

¥ the cardio-vascular complications of tabes.

times it is isol:

ocular mo

ley

lid di

again speak.

h

TABES DORSUALIS, 199

A slight narrowing of the padpelral opening (Jacobson and
Berger), due to paralysis of the smooth fibres of the: i
nerve supplying the eyelid, and analogous to that which is
found to occur after some lesions of the cervico-brachial nerve
roots. This symptom is, in fact, somewhat frequent.

Diminution of the orular tone (Berger) may exist, on account
of a diminution of the intra-ocular tension in consequence of
paralysis of the sympathetic nerve.

IL Ivrernat OrGaNs oF tHe Eye.

The iris ia liable to many disorders which are of grent im-
portance in the diagnosis of tabes.

The size of the pupils is very often either relatively or
absolutely altered, Sometimes there is only a slight inequality

Pig. snag acaoeslig ts ais ot a pepe? ‘The black fgare
in the middie represents the pupil of a healthy man moderately illuminated.
‘Tho black Sgure on the left side represents the pupil when mydriasis exists
{it may be of even larger size). Tho black figure on the right side represonts:
‘the pupil when myosis exists.

between the two pupils, without its being possible to say
that one is too Isrge or the other too small; sometimes the
papil is much reduced in size, the condition to which the term
myvsis is applied. The pupil may in these cases be no larger in
diameter than that of the head of a small pin, notwithstanding
which fact the patients make no complaint whatever of weak-
ness of sight.

In mydriasis the reverse is the ease. It consists of dilatation
of the pupil, which may be carried to on extreme degree
in tabes. It is rarely equally pronounced in the two eyes;
mydriasis may in fact be present in one eye. while myosis exists in
the other, The latter condition is more frequent than the former,
and it is by means of its existence that the disease hitherto un-
recognised may sometimes be known to exist.

The form of the pupil may again be changed, and it may
become of a somewhat oblizuely oval shape, that is to say, instead
of being round, it becomes more or less oval (Berger) with the

close to the eye, that is to say, when the
Pocreniiornle 1b, i619 866 FOP OE

light reflex constitutes an excellent sign of tabes a
although this fact had been already mentioned
Coingt, many authors continue to call it by the
of Argyll-Rabertson. In connection with this
you one piece of advice which may be of pi u
the condition of the light reflex is investigated, be es
the patient to look at some distant object pe b
which is close to his eyes; otherwise, on aceoun
being fixed on a near object the pupil is seen iis °
you are liable to mistake for light reflex the chan
which is really due to accommodation.
The accommodation reflex is, as I have just obse
retained during the first stages of tabes, but after.
it usually also ceases to exist.
(e) Pain vejlew. This reflex consists in the fact
irritation is produced by pain in a healthy person (b

aad —[—

TAHES DORSUALIS. 201

a pinch, &e.), and the pupil is examined at the same time it is
seen to dilate momentarily. As Erb has shown, this reflex is
very often lost: in tabes, ceasing to occur in certain cases at an
earlier period of the disease than the light or accommodation
reflex,

The optic nerve is often affected in tabes, and its lesions are
specially formidable inasmuch as they may induce complete loss
of sight, Statistics differ somewhat as to the frequency with
which optic neuritis occurs ; at the same time the number cannot
be said to vary more than between 10 and 20 per cent. of the
cases. The eye seems to be the part specially involved in some
patients with tabes, since optic neuritis is more frequent in those
who have previously suffered from paralysis of the ocular muscles.

Optic neuritis is rare in cases which are already of some years
duration, and is most usually found during the preataxic period.
At the same time Gowers states that he has never observed it as
the first evidence of tabes : all te Bet ee
been preceded by loss of the tendon reflexes or lightning pains.
Optic neuritis is almost always, if not always, bilateral in tabea ;
at the same time its onset may not be absolutely simultaneous
in the two eyes, and in that case the left eye is usually first
affected.

The length of time which passes between the onset of this
affection of the optic nerve and the occurrence of Lose of sight is
variable; the mean time was found by M, Benger to be 3 years,
but it varies from 2 months to 17 years. In some cases the
affection of the optic nerve remains in a stationary condition;
loss of sight is not, therefore, constant, and at times the con-
dition actually improves.

Lastly, some authors, notably Gowers, have remarked that a
kind of antagonism exists between optic nearitis and inco-
ordination, It is certain that in the cases in which optic neuritis
occurs at an early period of the disease amaurosis rapidly follows,
but there is little or no inco-ordination; on the contrary in the
cases in which optic neuritis only exists, after inco-ordination
there is usually but slight amblyopia, and very rarely amauros’s-
It has even been said that when optic neuritis occurs the inco-
ordination diminishes. The latter assertion seems to me some-
what ungrounded. For my part I am quite ready to admit the
first part of the assertion, namely, that the cases of tabes in

TAVES DORSUALIS. 203

Berger, who has specially studied the ocular disorders which
cecur in the course of tabes,

Quite at the onset the papilla is at times of a uniform red
colour, Most frequently the first change observed is that the

@

Fig. 117.—Extreme concentric con Fig, 118.—Contrction of the Gold of
tetion of the field of vision in a vision in tho left eye, in » eave of
aso of tabes (Damaschino col- tabes with atrophy of the optic
lection.) nerve. (Danisschino collection.)

Fig. 119.—Scotoma simulating contraction of the visual field with the hemiopie
form in s esse of tabes. (Damaachino eollection.)

nasal portion of the optic papilla becomes of a somewhat grey
colour, so that instead of being, as in its normal condition, more
red than the temporal portion it becomes of almost the same
hue, his grey colouration may have a bluish or pearl-grey
tint, Afterwards, when the lesions are very advanced, it becomes
white, like mother-of-pearl, but even at this period its edges are

and presents neither the irregularity nor the va
#0 spt to occur when it is due to disease of

the auditory nerve being involved; the

analogous to the optic neuritis of the same diseas
‘This explains the fact that when the external

is examined it is found to be free from disease.
time, since the middle ear may be affected in

TABES DORSALIS, 205
Weber,* which 28 pros contesting te are aaa elo ee
information.

Sule wande ne funy bend in taes, being of varied
character: at times very violent and painful buzzing sounds
are heard, or it may be the sound of hissing, or different musical
noises,

Awval vertigo (Méntive’s disease) is by no means rare, if one
includes under this name all the cases of tabes in which ver-
tiginous sensations exist, but do not occur in trae paroxysms
as in Mémiare's disease properly so called. This fact was first
mentioned by Pierret,+ and by Charcot; I myself studied this
subject from a special point of view in conjunction with Walton
in 1883.{ Since that time many works have been written on
this subject, one of the last and most important being that of
A. Marina. §

The cases of aural vertigo which occur in tabes may in my
opinion be classed in two groups.

A. Tn some cases there is considerable diminution in the
acuity of hearing, and different lesions may exist in the trans-
mitting portion of the ear. In these the same happens as in
cases of ordinary aural vertigo when tabes does not exist. This
disease has in fact but little if any effect upon it, though perhaps
it may produce increased irritability in the nerves supplied to
the labyrinth. :

B. In other cases there are neither diminution in the acuity
of hearing, nor lesions in the transmitting portion of the ear.
It is in connection with these cases that Walton and I proposed
the hypothesis that a lesion probably existed in a special portion
of the fibres of the auditory nerve, which is termed by some
anatomists and physiologists the nerve of space, a lesion in all
appearance directly due to the existence of tabes.

The excessive irritability of the auditory nerve to electric currents

* The method of Weber cousinta in placing a vibrating tuning fork in contact
with the vertex, which is usually more distinctly heard upon the side which i
the sent of the diseased ear, when the midile ot external ear are affected.

+ Pierret, Contribution a l'étude des phénombnes ofphaliques de tabes dorsualis,

‘sone la dependance du nerf uuditif, Rev. menavelle de méd, at de chir.,
are auras gt -Weltea, Des troubles vertiginens dans le tabos (Vortige de
Méniére tabstiqae), Herwe de Médecine, 1883, p. 42.

§ Al. Marina, Zur Symptomatologie cer Tiches dorsalis mit, otc., Archiv f. Paych.
t. XXI, p. 156.

206 LECTURES ON DISEASES OF THE SPINAL CORD.

in tabes has been specially studied by Marina. That author has
shown that this condition is by no means rare in tabes, since he
found that in 8 cases out of 11 electrical reactions were produced
in the auditory nerve by means of currents whose intensity was
less than 15 milliampéres, an intensity at which they only com-
mence to occur in healthy persons. This excessive irritability to
the electric current may be looked upon possibly, to some degree,
as explaining the relative frequency of vertiginous sensations in
some patients suffering from tabes in whom the ear is but very
slightly affected.

OLFacTorY OrGaxs.

OLFACTORY ORGANS.—The patients are at times troubled by
more or less disagreeable odours of purely subjective origin. In
rare cases true anosmia exists which is probably due to a lesion
of the olfactory nerves which is special to tabes.

GvsTaTORY ORGANS.

GrstaToRY ORGANS.—Some patients experience strange sensa-
tions of taste, notably a more or less persistent taste of sugar,
for the existence of which there is no reason. Sometimes, again,
there is complete loss of tuste, this being also probably due to
some change in the nerves of taste.

In conclusion, it may be said that these sensory disorders are
rare, slightly pronounced, and but little known.

ill, perhaps,
samptive tendency of tabes is due to thea
extra or intra-medullary nerve fibres anal
Bee ees br Sen ee

a “Spontaneous fractwres, These fractures
frequently in patients suffering from tabes,
fact, than one would be at first inclined to fl
for the first time by Weir Mitchell, their ¢
generally recognised until 1873, after they
Charcot. The works of Forestier, Richet and
tributed to increase our knowledge upon this
excellent critical survey by Talamon generali
in these lectures review the works of Regnard
speaking also of the opinion expressed by Vo
the effect which it had upon the development of
of tabid fractures in Germany ; I shall also have mo
to quote the conclusions formed in some of the
recently made, to which I shall call your
lectures progress.

These fractures present in most cases, without ¢
their ieticlogical conditions, certain objective chai
are sufficiently pronounced to distinguish them,
their being separately described.

One of the principal amongst these characters is |
absence uf pain due to the fracture; this absence of
complet ny of the patients do not even

TARES DORSUALIS. 209

the fracture has occurred, and stili continue to move the limb as
if nothing had happened ; you know, gentlemen, how painful
fractures are in healthy individuals, so much so in fact that the
least shake, the slightest movement, are quite intolerable.

In some cases the patients state that during a certain time
before the fracture pain was felt in the place where this subse-
quently occurred; such would therefore be a premonitory pain.
Tn other cases the fracture occurs during a paroxysm of lightning
pains; in all these cases, however, the pain is never due to the
fracture itself. This, as 1 have already said, remains painless in
most cases, in some, however, pain exceptionally occurs, but it
rarely becomes as pronounced as in healthy individuals. ‘The
absence of pain is in all probability due to the analgesia which
exists in the deep parts, and specially in the periosteum.

When the fracture has taken place the swelling of the part is
considerable, often greater than in an ordinary case, and of longer
duration.

The course of these fractures also presents certain special
characters ; the fendency to consolidation is somewhat rapid, but
it must not be thought, as some authors have said, that in the
spontaneous fractures occurring in tabes it takes place more
rapidly than in persons in good health. The consolidation may
in fact take place at quite a late date, and even in some cases’a
false articulation. (pseudarthrose) is formed.

As regards the tendency to produce much callus, this is not
constant, only occurring in the fractures in which the immo-
bility has not been complete. In such cases, in fact, on the one
hand the adaptation of the fragments is very incomplete, and
the size of the bone consequently increases at this part ; on the
ather hand the following incident occurs upon which Kredel
rightly insists: on account of the absence of pain the patient
allows the extremities of the bone to be rubbed one against the
other without being troubled by their being so; a process which
is employed by surgeons for the purpose of accelerating union
when the callus is slowly produced. ‘The effect of such reciprocal
friction is that the process of bone formation is much hastened,
and hence the fragments become of larger size as well as the
callus which joins them together.

It has heen also stated that the spontaneous fractures which
oceur in tabes are specially liable to be associated with shortening

VW

™

210 LECTURES ON DISEASES OF THE SPINAL CORD.

of the limb, ‘his fact is generally true, but may easily be
explained without any special property existing in the callus,
These fractures not being attended by pain are liable to be
unperceived both by the patient and his medical attendant, and
even when recognized to exist may be left more or less untreated ;
the patient, suffering no pain, does not trouble himself to keep
the limb quiet, and the fragments are liable to be displaced even
after having been put in a good position. Owing to the frag-
ments passing one over the other, the callus forms abnormally

Fig. 120.—Fracture of tho lower ex- Fig. 121.—Fracture of the two bones
tremity of the humerus in tabes, of the forearm in a case of tabes,
displacement of the fragments, large largo quantity of callus. (Collection
quantity of callns, (Collection of of Charopt.)

Charcot.)

(in excess), and the limb is shortened to a more or less consider
able extent, There is also another cause of the shortening
observed in fractured limbs, not only in ordinary cases, but when
the arthropathy which occurs in tabes is associated with fracture
of the head of the femur or humerus ; on account of the tendency
to absorption of the bony extremities which exists in arthropathy
so that the head of the bone may completely disappear. The
result of this would be a considerable reduction in the length of
the leg or arm.

Before commencing to study the lesions to which these
symptoms are due, we must briefly consider their etiological
conditions ; you will again see that in this case one important
fact confirms what is said by those who maintain, as Chareot
was the first to do, that the spontancous fractures which oceur
in tabes constitute a separate group. This fact is the disproportion
which exists between the slightness of the injury and the fracture
of the bone, which is so pronounced that these were consequently
termed “ spontaneous fractures.”

i ail

TABES DORSUALIS. 2n1

The history of tabes contains numerous observations in which
one or several fractures have been produced by mere traction or

Fig. 122.- A woman enffering from tabos with fracture of the left femur (the
figure having been turned round it is represented aa the right femur): aftor
this fracture so much absorption took place at the extremity of the femur that
the length of the thigh was considerably shortened. (Collection of Charcot.)

Fig, 123—A, A normal femur. B, femor in a case of arthropathy in tabos. ©.
fomur in a case of fracture in tabes. Such absorption of fragmenta
that the bone scarcely attained half tho length of that of a normal fomur. The
‘bone belonged to the patient represented in fig. 122 (Collection of Charcot.)

a trifling shock. Upon some occasions the patient was merely
crossing his legs, or drawing off his boots, or perhays lying in

TAHES DORSUALIS. 218

Tt will be observed, gentlemen, that fractures of the lower
limb are incomparably more frequent than those of the upper.
In my opinion there are two reasons for this: 1, Tabes being an
affection which specially involves the lower half of the spinal cord,
it is not surprising that the lower limbs should be more affected
in every respect than the upper. 2. It is in the same bones of the
lower limb, specially the femur, that fractures are most liable to
occur in those whose bones have been changed in their anatomical
or chemical composition, as for example from the effect of age.
J must also observe, and this is the objection which occurs most
rendily to the mind, that these are the bones which are most
exposed to injury, though, when everything is considered, the
latter fact does not seem sufficient to explain the predominance
with which the spontaneous fractures of tabes occur in the lower
limb.

The different statistics do not seem to show that the fractures
specially occur on either side of the body. It is possible, how-
ever, that they are more likely to take place upon the side in
which the symptoms of tabes are most pronounced whenever
some asymmetry in the symptoms can be recognized to exist.

It not infrequently happens that mulliple fractures exist in
the same patient, either from many bones being simultaneously
fractured, which in some cases has happened jn os many as 5,
or even 6, or from more than one fracture occurring in the same
bone (as many as 3 in one ense). This also shows, in my
opinion, that the fractures are due to a special condition of the
bone.

In connection with the long bones a special variety of fractures
should also be mentioned, which differs from those which have
been hitherto considered; it is that which ocurs when a bony
prominence or apophysis is torn off by the muscles inserted upon
it. On account of the absence of immobility and the friction
which is facilitated by the analgesia of which I have spoken, an
excessive amount of callus is formed, whence, according to Volk-
mann (quoted by Kredel), the hony prominences are produced
which penetrate into the muscles and tendons. I am ready to
admit that this may occasionally happen, but in some cases [
have seen the intra-muscular osseous prolongations attain such 4
length that I cannot help attributing them to ossifying myositis.
However this may be, gentlemen, I call your attention to.

L

‘TAKES DORSUALIS. 215

themselves to the observer. At times the analgesia is of such a
character, as I have already stated, that the patient is not aware
of its existence, and if the fracture interferes but little with his
movements, as when he is bedridden, it may be completely
unperceived. Or again, the mdema, which in the fractures of
those suffering from tabes is usually more pronounced than in

Fig, 124—Fracture of the spine. On scconnt of the sinking of the vertebra
which has occurred there in considerable lateral curvature of the spine. (Col-
Ieotion of Charcot.)

ordinary fractures, may cansé 80 much swelling as to make any
examination of the part impossible.

The diagnosis will lastly be found very difficult or even
impossible when the fracture involves an epiphysis of which the
articular portion is affected by arthropathy ; in that case, again,
the ewelling of the adjoining parts and mobility of the bones
prevent a proper examination from being made, You will not
therefore be astonished, gentlemen, that at the autopsy of those
who have suffered from tabes, fractures and bony consolidation
are often observed to exist, of which there was not the least
suspicion during life; it is especially in conection with the flat
bones which are situated somewhat deeply (sternum, pelvis) that
these surprises are wont to occur.

. HISTOLOGICAI

TAKES DORSUALIS. 217

Changes ia the osteoplasts, These suffer more or less from
atrophy in certain points, owing to granular and fatty degenera-
tion, presenting also a tendency to lose their angles and to
present rounded outlines.

Embryonic transformation of the medulla in bones (Richet),
This exists in great abundance, filling all the cavities produced by
dilatation of the Haversian canale. These changes would indicate,
according to Richet, somewhat pronounced osteitis,

Lastly, to these histological alterations an enumeration of
CHEMICAL CHANGES found to exist in the bones in tabes by
P. Reynard should be added.

Diminution of inorganic materials, Instead of the normal
proportion of 66 per cent. of the total weight, the inorganic

|

!

Fig. 125.—Longitndinal section of the upper extremity of along bone (humerus)
affected by the disease which existe in the arthropathy of tabes, showing the
redaoed size of the compact tissue and cartilage, where the arthropathy (A)
exists, (Damaschino collection.)

portion of the bone only forms 24 per cent., a lange deficiency in
phosphates being specially found to exist (10 per cent, instead
of 50 per cent.),

Tnerease of organic materials. These normally form 33 per
cent. of the total weight; in tabes they form 76 per cent.; the
proportion of fatty matter is specially great (37 per cent.),

None of these chemical or histological changes, however, is
exclusively characteristic of tabes, they show at most the existence
of rarefying osteitis, an affection which may exist in many chronic
affections of the bones.

The only lesion special to tabes is that which Pitres and
Vaillard found to exist in a case of spontaneous fracture of the
tibia. The nerve filament which enters the nutritive orifice of
that bone, according to these authors, presents obvious changes.
Siemerling afterwards observed the same lesion in an analogous

219 be

oblongata,
(traumatic, syphilitic, rheumatic arthritis, arthritis doformans). Conclusions,

GexTLemen,—Tabid arthropathy is called by another name in
England, that of “Charcot’s joint disease.” ‘The fact is that
Charcot not only discovered the existence of that affection, but
described it from the very first in so complete a manner that
this name is in every way appropriate. His first works upon
this subject were written in 1868. In the following year
Ball already mentioned 18 cases. In the same year (1869)
Clifford Albutt published an account of the first case described
in England, the second being recorded by Buzzard at a some-
what later date (1873), In Germany the idea of tabid arthro-
pathy developed but slowly. Professor y. Volkmann having
stated that the joint affections in tabes were purely and simply
due to injury or twisting of the joints in the irregular move-
ments of the disease, his authority was sufficient to prevent any
other explanation from being admitted. Since that time a
great change has occurred, and I shall more than onee have to
quote from important works written by German authors.
The following numbers, taken from a memoir by Weizsiicker,

TABES DORSUALIS. 221

has made the very probable suggestion that there is not only
an effusion into the articular cavity but also rupture of the
capsule, which allows the liquid to penetrate throughout the
entire limb.

‘The subsequent history of the swelling is as follows:—After
some days, or rather weeks, or sometimes many months, it
diminishes in size and becomes confined to the joint and
adjoining stractures. It may remain in this part for some time,
after which it completely disappears, although the strangest forms
of dislocation may be subsequently found to exist in its place, of
which I shall speak presently.

Orepitation often, but not always, oceurs in the diseased
joints; usually occurring on the onset, and subsequently ceasing
to exist at the time when effusion into the joint occurs.

The movements of the joint, strange to say, are little if at
all affected, specially at the onset; the patient merely com-
plains that the limb seems to be heavy and that be is soon
fati 5

But of all these symptoms, that which combined with the
swelling is most characteristic of tabid arthropathy, is the
whence of pain, however pronounced the articular lesions may
be, a fact which is quite in opposition to that which occurs in
most joint affections. We have also seen, gentlemen, that the
same occurs in the spontaneous fractures of tabes, of which it
was the special character to be unaccompanied by pain.

As to the course of tabid arthropathy, as Charcot pointed out,
two forms may be distinguished :

Tn the mild form the effusion disappears in a short time, and
the joint returns to its normal condition, slight crepitation
being the only trace of the affection, At the same time, how-
ever mild the affection may be, it must be known that relapses
are liable to occur, relapses which may be far more serious than
the first attack; so that a mild form may be subsequently
followed by a serious attack.

Tn the serious form the oedema of the limb does not disappear
as rapidly as in the preceding form; it remains over the joint
and adjoining parts ; at the same time the lesions in the bones
and ligaments occur, and soon become so much developed as to
interfere more or less completely with all the functions of the
limb.

223 LECTURES ON DISEASES OF THE SPINAL CORD.

It is from this form that the patients who are assembled her:
suffer, though it has not the same form in the different cases.

Tn this woman, for example, the arthropathy has only existed
for a few months in the left hip joint; the @dema, as you
will observe, still occupies the whole thigh, although as the
patient tells us, it has much diminished; the movements are
somewhat difficult, but still possible, and there is little if any
crepitation.

In the second woman one of the knees is affected; in this

Fig. 126—Recont tabid arthropathy in the left hip joint. (Datmaschino collection.)
‘The cnormour increase in the sizo of the Jeft thigh will be observed, The
lesion having existed but a short timo, the upright position can be maintained
withont pronounced deviation of the lower limb,

case there is but little swelling, but on account of the lesions in
the bone, some deviation is already beginning to occur im the
direction of the limb, and upon my attempting to produce
lateral movements in the joint, I can do so, as you se6, without
difficulty, whereas when the jomt is in a healthy condition
the lateral movements are barely perceptible; henee there is
apparently much difficulty in walking.

Lastly, in the third patient, the woman who is lying upon

al

TARES DORSUALIS. 223

‘the litter, the affection has, so to speak, reached its most serious
point. It has lasted certainly for many years, but the difference
of its appearance from that of the other cases is great; in this
patient there is no swelling, but, on the contrary, fattening in
the region of the hip joint so that the condition of the articu-
lation can be easily examined, Its abnormal mobility is first

Fig. 127.—Tabid arthropathy in the left knee. (Damaschino collection.) The
knev and thigh aro swollen, the kneo presents prominences which canse it to

appear of a square form. The lateral movements were very prononneed, and

ooald be produoed by alight pressure of tho hand upon tho foot

observed; the most impossible movements can be given to
the thigh, in comparison with which the well-known straddling
position of the most famous acrobats would cut but s poor
figure; at the same time, however extensive these movements

TARES DOMSUALIS. 225,

Fig. 129.—Reeont tabid arthropathy in the shoulder (from o patient named
Berthel). (Collection of M. Charcot,) Much oulargement of the shoulder,
The condition of tho shoulder joint a few years later is een in the following
Sgare,

Fig. 140.—Ascient tabid arthropathy in the shoulder (from a patient named
Berthel), (Collection of M. Charcot.) The head of the humerus, which is no
longer in relation with the aeromion, forms a prominonce beneath the skin in
front of the angle of tho inferior masilla.

15

|

224 LECTURES ON DISEASES OF THE SPINAL CORD.
may be, no resistance is felt in the joint. The reason of thie
is that the joint can be distended to an extreme degree, the
articular surfaces in which a great change has occurred being
no longer in close relation with each other. You yourselre:
can again easily understand what occurs, even at a distance,
since the subentaneous prominence which you perceive in the
hip is really the upper extremity of the femur, which change
its place whenever the thigh is moved, and to an extent whieh
corresponds with the amplitude of the movement. Owing to its

Fig. 128.—Tabid arthropathy in the knee, (Damasohino collection.) ‘The mest
extraordinary dislocations can be produced in the lower limb of this patlet
without the slightest pain being produced.

being possible in this way to bring the upper extremity of the
femur, so to spenk, underneath the skin, it is easy to feel its
different parts, and to recognize that both the head itself anda
large part of the neck have almost completely disappeared, ani
that this extremity is almost solely represented by the gmat
trochanter. It is unnecessary to add, gentlemen, that with such
lesions the patient is quite unable to walk; but the fact which
Lam anxious to repeat and impress upon you is, that however
much I move or drag the limb no pain whatever is fele by the
patient; if she does not walk it is solely because the mechanic
conditions of the joint do not enable it to support the weight of

the body

_———

TABES DORSUALIS. 225

Fig. 129.—Reoent tabid arthropathy in the shonlder (from o patient named
Berthel). (Collection of M. Charcot.) Mech enlargement of the shoulder,
‘The condition of the shoulder joint a few years later is seen in the following
figure.

Fig. 199. —Ancient tabid arthropathy in the shoulder (from « patient named
Beri). Collection of Charo.) The bead of. the humeres, which ia no
longer in relation neromion, form: prominence ber
front of the angle of the inferior matilla. = ea

Vo

|

226 LECTURES ON DISEASES OF THE SPINAL CORD.

What may be called the pathognomonic characters of con-
firmed tabid arthropathy are thus, as you have just seen,
abnormal mobility, in excess of any that could be imagined, and

Fig. 131—Tabid arthropathy in both knees, (Collection of M. Charcot.) In this
caso tho deformity which existed was lateral; though pronounced in degroe it
did not prevent the patient from walking a few steps with the help of two
aticks.

Fig 132.—Tabid arthropathy in the two knees. (After M. Dresehfeld.) Thaw
patient presente in the most pronounced way the deformity which is known by
tho name of “ genu rocurvatum.”

considerable change in the articular surfaces, neither of yhich
conditions produces the s'ightest pain.

- : _ :

ARES DORSUALIS. 227

Such are the ordinary features of this affection; in some cases
other symptoms occur which should perhaps be regarded as
complications. Thug—

A spontaneous fracture may occur either in the diaphysis or
more often at the extremity of a bone giving rise to the
formation of a foreign body in the joint (specially the head of
the femur).

Pain, which, as already observed, is usnally almost entirely
absent, may occur in some cases and persist daring several days
or weeks, this being specially the case when arthropathy affects
the small joints (of the hand or foot).

‘The articwar extremities may pose through the skin, as has
been observed in some cases, and their mobility enables one to
understand that this may be the case; in reality, however, it
very rarely happens,

Suppuration, again, is a complication which is 80 rare that its
oczurrence need not be feared. At the same time, I might
mention cases in which it has happened either after an im-
pradent puncture, or during the course of a general affection
(pneumonia), or without obvious cause, but T would again say,
gentlemen, that its occurrence is quite exceptional,

Tn order to finish what is connected with the clinical aspect
of tabid arthropathy, gentlemen, its ario0LoGy will be now
discussed.

As regards the direct causes of its existence I shall have to
speak of the part which injury plays in its production at a later
period when the nature of the arthropathy is considered,

At the present time the conditions in which it occurs will be
specially considered. he time at which it takes place is very
variable, Kredel® found that in 132 cases it appeared—

21 times in the premonitory period of tubes.
38 cimes between the lst and 3rd year of tabes.

82 times between the Sth and 10th year of tabes.
41 times after the 10th year of tabea,

It is therefore very difficult to specify the time of its oceur-
renee, the truth being that it occurs at any period of the
disease.

* Krodlel, Die Arthropathien und Sponton Tacturen bei Tabes, Vollmman's Savum-
ung Kiciee’ er Vorindge, 1888.

TABES DORSUALIS, 229

maxillary joint were far more rarely affected, giving numbers
which vary between 6 and 4. Yon will have observed that all

Fig. 134—Tabid arthropathy in the thumb. The Inet phalanx of tho thumb ix
turned abruptly outwards, so as to be almost at right angles with the first
phalanx. The bones connected with the fingers are somewhat

wwollen at their lowor extremity.

Fig. 185 —Tabid arthropathy in the right elbow,

the joints may be affected, but that they are so with a varying
degree of frequency.

TABES DORSUALIS. 231

many hondred in number, so that the articulation may give to
the finger the sensation of a bag of walnuts.
As regards the extremities of the bones they present very
different aspects, which may be referred to two large types :—
A. The atrophic type, the most frequent, the most characteristic
as regards the pathological anatomy of the disease, in which there
is more or leas complete atrophy of the cartilage and articular

Fig, 137—Tabid arthropathy in the shoulder, A. Normal head of the humerus,
B. Hoad of the humerus upon the side in which arthropathy exists; the
articular surface of the head of the humerus has much diminished in aize upon
this «ide. (Darmaschino collection.)

Fig, 138.—Tabid arthropathy in the Fig, 139,—. thy in the shoulder.

hip-joint. (Collection of Charcot.)
‘The lowor part A of the articulating
portion of the head of the femur is
completely absorbed and has dis

1, 9 plane surface alone

(Collection of Chareot.) ‘Tho glenoid
cavity of the scapula can scarcely
now bo seon, and the head of the
Iumeres has almost completely dis-
appeared,

appeared
remaining in its place.

surfaces of the bone, the wasting being possibly so considerable
that a somewhat long portion of the epiphysis may entirely
dieappear. Thus, in the femur, for instance, not only the whole
head, but even the neck of the bone may be completely absorbed,
Tn this way, as in the patient whom I showed you just now, the
great trochanter, which is itself much eroded, may be the only

TABES DORSUALIS. 233

however, they are undoubtedly both examples of trne tabid
arthropathy, To what then is the difference in the appearance
due? Solely, in my opinion, to the character of the affected
joint. Some joints respond to the morbid process which exists
in tabid arthropathy of the type A, while others respond to that
of the type B. Kredel, whose works I have already had occasion
to mention in connection with the disease in which we are now
interested, has remarked that certain joints, notably the hip and
shoulder joints, tend specially to suffer from atrophy when they
are the seat of some morbid process, specially that of fuberewlar
urthritis, whereas others (above all the knee and elbow joints)
are more disposed to suffer in a similar case from hypertrophy.
This is precisely what happens in tabid arthropathy ; when it
is seated in the shoulder joint the atrophic type A is almost
invariably observed, whereas when the knee is affected the
hypertvophie type B is more frequently found to exist, And the
proof that the anatomical difference is more dependent upon the
character of the joint, than the special quality of the arthropathy,
is that the two types may be combined in the same patient.
If the hip and knee are simultaneously affected, the atrophic
form will occur in the former, the hypertrophic in the latter
joint.

Besides these two forms the variations produced by comlination
of the two types should be mentioned, in which by the side of
atrophic lesions bony outgrowths or osteo-cartilaginous pro-
jections of more or less considerable size are found to exist.
The appearance of the joint may again be changed in the cases
in which an intva-arlicular fracture co-exists; the fragments of
bone which are thus produced vary in dimensions from the size
of a pea to that of a bean or more ; they may either come from
the epiphysis, or from the condyle, or may even be due to the
whole condyle or epiphysis being torn away. The fragments
may either remain free and constitute a foreign body in the
joint, or be absorbed, or lastly, and specially in the hip joint,
may become closely adherent to the articular surface of the other
bone of the joint (the head of the femur to the acetabulum).

Such are the different anatomical conditions in tabid arthro-
pathy; they are only found, it must be understood, in the joints
in which this condition existed in a pronounced degree. At the
same time it must not be supposed that the other joints are

TARES DORSUALIS. 235

the nerve seated in the nutritious canal of the bones, are led
to believe that the symptoms connected with the joints are due
to some change in the peripheral werces,

B. The primary cause is not a nervous lesion.

Amongst those who have held this opinion the first whe must
be mentioned is Volkmann ; according to this author the arthro-
pathy is purely and simply a lesion of frawmatic origin, and the
possi oF ite. Chonan ts Aes eafucay ei tata Oa
because injuries to the joints are far more frequent in these
patients on account of the inco-ordination which exists in the
movements. Hither on account of falls, or because the joints
are being constantly irritated by the irregalar movements, they
are more liable to suffer than those in healthy persons; to these
unfayourable conditions the influence of analgesia must be also
added (Jonathan Hutchinson).

Striimpell, on account of tabes occurring frequently as a late
effect of syphilis, is of opinion that the arthropathy in tabes is
really syphilitic arthropathy, and he consequently connects this
affection of the joints with syphilis alone, and in no way with
the disease of the nervous system.

Lastly, there is an opinion, which exists widely in England,
that the arthropathy in tabes is merely chronic rheumatic
arthritis. With regard to the ideas which exist in England about
this affection I cannot help mentioning to you that expressed
by one of the most eminent surgeons of the United Kingdom,
namely, Sir James Paget.

According to this surgeon the arthropathy in tabes is not only
a recently discovered, but a really new affection ; it would only
have existed so to speak during the last 25 or 30 years. In
support of this opinion Sir J. Paget quotes the fact that in the
rich collections of the Royal College of Surgeons, where examples
of all the osseous or other lesions which may be met with have
accumulated during so many years, no specimen whatever is
found of tabid arthropathy. Although Ido not form the same
conclusion as Sir James Paget, this fact, at the same time,
appears to me interesting, and we find in it special confirmation
of the aphorism of Charcot, “one only sees that which one has

Aearnt to see.”

‘To return to the supposition that the arthropathy in tabes is
ofa purely rheumatic character I must also quote the opinion

i
is in very small quantity; (¢) th
one of hypertrophy, whereas in the art
first exists ; () the movements of
limited, on the contrary, we have
of tabes the mobility is much
deformity may occur in both affections,
different. Other points of pate

that tabid arthropathy and arthritis defor
distinct affections.

Such, gentlemen, are the principal theori
respect to the nature of tabid arthropath;
numerons, and differ much from each other, Ti
useless to increase the difficulty in which
places us, and though I cannot oblige you
opinion I must be allowed to add that it is sti
that of Charcot, which seems to me the best.
‘cannot. be simply and purely due to the
occasioned by the inco-ordinate movements
as we have already seen, it often occurs in the
when no inco-ordination of movement exists. 1
simply the result of syphilis since unfortunately
persons affected by that complaint is legion, and
also exists they never present this singular
pathy,

With regard to the connection of tabid at

TABES DORSUALIS, 237
peripheral neuritis as its initial lesion, I must confess, gentlemen,
that I cannot believe this to exist, and for a reason analogous
to the one which I have just given with respect to its purely
ayphilitic origin. We see, in fact, gentlemen, a large number
of cases of so-called peripheral neuritis, and yet never, even
in those who present the most characteristic symptoms of that
affection, does any symptom oecur which resembles tabid arthro-
psthy, On the contrary an affection is known, due to a special

Fig. 141 —The innominate bose and
femur in a case of tabid arthro-
pathy of the hip joint. (Collection
of Charcot.) The acetabulum has
almost eatirely disappeared, and its
margin barely remains, the bottom
of the cavity being continuous with
tho adjoining surface of the bone.
‘The head and neck of the femur have
also completely disappeared, and the
great trochanter alone remains.

Fig. 142—Tho innominate bone and
femur in a case of chronic rhoumatiom
(arthritis deformana). The margin
of the acetabulum is mnch increased
in alze, and the cotylold cavity much
deeper than in its normal condition,
The head of the femur is also in-
creased in size.

lesion in the spinal cord, syringo-myelia, which is sometimes
accompanied by arthropathy very similar to that which ocenrs
in tabes.

For all these reasons I would refer the initial lesion of this
affection of the joints in tabes to change in the spinal cord, and,

238 LECTURES ON DISEASES OF THE SPINAL CORD.

as I have already observed, certain autopsies seem to confirm
the opinion of Charcot, who places this lesion in the grey sub
stance. This being well established I find no difficulty it
supposing that in some cases traumatic injury, either directly
inflicted cr due to the irregular movements, is an occasiona
cause of tabid arthropathy, and determines its seat.

LECTURE XX,
TABES.
SYMPTOMS (continued).

The foot ia tubes: Wlstory.—Characters. Somewhat sudden onset, swelling of the
foot, thicknons of ite innor margin, flattening of tho arch of the foot, deviation
of the mctatarmas, shortening of the foot, enlargement of the malleoli, ankylosis
of the joints of the foot, absenoe of pain.

Saeenvbe Serene spongy appearance of the tarsal and metatarsal bones, their

Trophic dsarders inthe Rorous tone; articular eapenles ; intra-articular ligaments;
tendons, their rupture.

Trophic disorders of the akin; different eruption»— Perforating ulver, its characters;
Gangrene, Spontaneous ecchymoses; Loss of the nails and teeth, Hyperidrosis ;
anidrosis. Nuture of these trophic disorders.

Gextiemex,—A separate place must be made by the side of
tabid arthropathy and spontaneous fractures for an affection
which, as I have already said with regard to the vertebral
lesions, proceeds with almost the same frequency from either of
these two morbid states—I mean the tabid foot.

The tabid foot was first described by Charcot and Féré.
Immediately afterwards numerous observations made by Boyer,
A. Chauffard, &c., showed the accuracy of this description ; in
England, one of the first cases published was that of Page; in
Germany, that of Bernhardt. In all these cases the result of
the examination of the parta was almost identically the same.

This condition usually exists at a comparatively carly period,
occurring most often at the end of the first or commencement
of the second period of tabes, in other words in the prmataxic
stage. The onset is usually sudden, resembling that already
described in connection with arthropathy; within twenty-four
hours an abnormal swelling appears upon the back of the foot,
after which the other morbid conditions gradually occur.

'The principal characters of this affection are as follows :-—

A somewhat considerable swelling forms, whose chief seat is the
back of the foot, specially pronounced near the tarso-metatarsal

TABES DORSUALIS. oa

Pronounced shortening of the foot exists in some cases, and
this contributes to make the foot have a thickened, cubical, and
in all respects singular appearance.

‘The malleolé are often enlarged and swollen, and may be
the seat of co-existing arthropathy; the same may be said of
the toes, which at times suffer also from arthropathy in those
in whom the “ tabid foot” exists.

The mobility of the different segments of the foot is diminished,
and at times complete ankylosis occurs.

Cropitation, whether spontancous or during passive movement
of the joint, occurs but little if at all.

The ahsence of pain on pressure is as complete as in other
forme of spontaneous fracture and arthropathy. Sometimes
anesthesia also exists when the back of the foot is pricked,
while sensation exists in the lower part, the sole of the foot
retaining its sensibility both as regards contact and temperature.

‘The result of the pathological anatomy of the condition is really
singular, although the lesions found to exist are analogous to
those with which we have already become acquainted in connec-
tion with other forms of tabid arthropathy. What are most
surprising are the intensity of the lesions, and the amount of
destruction which is produced by the morbid process in this
form of arthropathy. In order to give an idea of this T now
put before you the photograph of a specimen in the collection of
Charcot, regretting that I cannot show you the specimen itself
(since it is a most: instructive one), on account of its having been
recently stolen from the museum at the Salpétriére Hospital.
Charcot described the osseous lesions found in this case in the
following way: “The articular facets upon the inferior surface of
the astragalus, and os calcis are eroded and worn away, their
mangins being covered by granulations; the astragalus is frac-
tured transversely at its neck; the scaphoid and cuboid bones
are worn away, and can scarcely be recognized. Numerous
separate fragments of bone exist, amongst which the external
eaneiform bone can be with difficulty recognized. All the bones
of the metatarsas and tarsus present & spongy appearance, and
are unusually fragile and light.” The fact is that in the tabid
foot not only do wasting and atrophy of the different bones of
the tarsus and metatarsus occur, but their absolute disintegra-
tion, to such an extent that at certain points a gruel-like mass is

16

242 LECTURES ON DISEASES OF THE SPINAL CORD.

alone found, containing small fragments of bone; one ean ther
fore understand and agree with the following statement of

Fig. 144.—Bones of the foot in tabes showing tho os caleia to be formed of
extremely loose cancellous tissue, while the bones of the tarsus are almost
completely destroyed. Fragments of bone which have passed beneath the foot
are of loose cancellous titane and jagged, coming from the bones of the tarves,
and found at the autopsy to be scparated from each other. (Collection of

Chareot.)

Fig. 145.—Tuabid arthropathy in the foot. The os calcis forme, as it wore, a thin
Gsseous cup at ite lower and anterior part, into which the astragalue hax
doxoonded. (After Tnfficr and Chipault.)

A, Chauffard, made in his excellent laconic expression, “Ty the
tabid foot there is not more osteopathy than arthropathy.”

= | a

TARES DORSUALIS. 43

What is finally the condition of the tabid foot? Much
more simple than would have been expected when the intensity
of the symptoms and the osseous lesions are considered. The
swelling gradually subsides after a somewhat long time, but
the deformities persist, and the almost absolute ankylosis which
exists in the foot does not prevent the patient from using it,
if not to a full, at any rate to a sufficient extent. Sup-
puration does not oceur more in the tabid foot than in other
forms of arthropathy, or in spontaneous fractures, Tt is un-
necessary to say moré about these pointe, which have been
already sufficiently discussed, and I have spoken separately of
the tabid foot in order that I might mention in detail the
different objective characters which cause it to have such o
peculiar appearance.

Tropaic DisoRvERs 1X ‘THE Fisrous Tissvr.

Whilst: considering these changes in the bones and articula-
tions I should say a few words about the trophic disordera in the
fibrous tiseue,

In speaking of the different forms of arthropathy the lesions
which occur in the articular capswles have already been enumer-
ated; we have seen that they become relaxed, distended, and at
times perforated, while atrophy may occur in certain parts they
have invaded, in others newly formed bone exists. It is useless
to dwell more upon this subject.

Tn a similar way we know that the intra-erticudar ligaments
may be much altered, and even completely disappear.

What oceurs in the tendons? In arthropathy more or less
extensive lesions may occur in them in connection with the
joints over the mobility of which they preside.

There is nothing surprising in this, which might have been
foreseen. It is, however, another example of trophic disorder
which should be mentioned, namely, the rupture of the tendons
which occurs in tabes.

This symptom has not yet been observed in very many cases,
but I have no doubt that it will be more frequently seen to
occur when attention is drawn to this point,

T must quote the case of Hoffmann, in which rapture of the
tendo Achillis occurring when the patient made the simple

*TABES DORSUALIS. 25

time ulcerates in its centeal part, and this ulceration may either
remain superficial and heal, owing to the effect of rest, after a
time which is of no great length, by the formation of fresh
layers of epidermis; or on the contrary, extend more deeply to
the subjacent articulations and bones, producing such lesions
that the surgeon may be obliged to amputate the affected parts.
This second form is certainly rare, being the form which I would
term surgical, and which Chipault* had doubtless alone in view
when, in his interesting Heview upon the perforating ulcer, he
stated that it was of rare occurrence. As regards the first form,
on account of which the patients never requive surgical treat-
ment and of which the symptoms are often too mild even to
attract attention, it is undoubtedly frequent, and may even
be said to be very frequent, It is undoubtedly true that an
ulcer which only consists of slight ulceration is not really a
perforating ulcer; this, however, simply applies to the name,
and an abortive form of so many affections exists that we
should not be justified in refusing to look upon this as an
example of such a kind. In some cases Taffier and Chipault
have shown that arthropathy of the joint immediately adjoining
the perforating ulcer is already in existence when the onset of
this complication occurs (specially in the metatarso~phalangeal
joint). The two lesions are independent of each other and the
primary arthropathy still exists when the perforating ulcer has
been cured.

The perforating ulcer specially affects certain parts. Some
cases have been mentioned as occurring in the hand (Peraire),
bat this was before the clinical symptoms of syringomyelia
were well known, and the observations might perhaps now be
revised as to this point, As regards the supposed perforating
ulcer in the viscera, I mast confess, gentlemen, that I am more
inclined to consider it an erroneous expression than a reality.
However this may be, the perforating ulcer, as a general rule, is
seated in the foot, usually below the head of the Ist, and often
below that of the 5th metatarsal bone, or sometimes below the
heel. In some cases again it is found st other points in the sole
of the foot. Since the researches of Duplay and Morat most
authors attribute the perforating ulcer to some change in the

* Chipault, Lo mal perforant General Review in the Oazette des Hipitwwr, 1801,
No. 83, p. 765.

~

‘TAGES DOUSEALTS. 247

with such rapidity would not occur without the intervention and
presence of more or less special septic micro-organisms. My
opinion therefore is, gentlemen, that in the gangrene of tabes as,
in fact, when it occurs in other affections of the nervous system,
each of these two pathogenic elements has its special effect. At
the same time the symptom is itself rare.

Spontaneous ecchymoses form an interesting episode in the
history of the trophic and vasomotor disorders of tabes. They
were first mentioned and studied by I. Straus.* It was shown
by this author that they are in most cases obviously connected.
with the paroxysms of lightning pain, oceurring when these
cease and at the moment of their cessation. They are not, how-
ever, seated in the same place as the pains, but somewhat above
the point at which these occur. This symptom, though not
extremely frequent, is less uncommon than might be supposed
to be the case; at the same time, from its requiring investi-
gation, you will not be surprised that it is very frequently quite
unperceived.

By the side of these trophic disorders of the skin you will
allow me, gentlemen, to place others which may be logically
olasged with them ; these are shelding of the nails, and lose of
the teeth, ag alzo the existence of hyperidrosie and anidrosia,

The shedding of the nails bas been often observed in tabes, and
1 would specially quote the observations and memoirs of Joffroy,
Fournier, Domecq-Taron, who have studied the conditions in
which this complication occurs.

The nails of the toes are usually involved, and often in a
symmetrical manner; this often happens more than once. The
nail is generally shed without the association of other decided
changes, becoming gradually detached from the free part towards
‘the base, and shed without the occurrence of pain, “separating”
in the same way “as an ecthymatous scab” (Fournier). Some-
times, however, tingling or slight pain is felt by the patient;
more rarely, true lightning pains precede the shedding of the
nails. Lastly, in some cases a patch of ecchymosis appears
beneath the nail before it is shed, usually without the occurrence
of pain, By the side of this shedding of the nails must be
placed their dyerophy which sometimes occurs. They then

Ver opiebate pespragery ebvoen hs 10 pst den esene Simla

TABES DORSUALIS. mo

principally due to periostitis and osteitis affecting the alveoli
and teeth, a3 well as to the loss of the latter, and destraction of
a portion of the alveolar arch, with which the presence of micro-
organisms in the teeth is associated.

‘The last symptoms of which I would now speak are properly
speaking disorders of secretion rather than of nutrition; but
since we have specially considered in this lecture the symptoms
eonnected with the skin which exist in tabes, it does not seem
to me in any way illogical to speak at this time of the disorders
which affect the #oriparous glands.

Hyperidrosis, or an increase of their function, is not extremely
rare in tabes; it may be unilateral or exist on both sides, and
in the latter case may occur to such an extent as to be most
inconvenient; thus, quite recently, I had the opportunity of
seeing a patient suffering from tabes, who told me that he was
obliged to change his flannel vest almost every half hour on
account of the abundance of the perspiration. It should also be
stated that in some patients this increase of function occurs in
paroxysms which yary in duration, and are more or less com-
pletely absent during the intervals.

The opposite condition, anidrosis, or absence of perspiration,
is also observed in tabes, and may also be unilateral, or exist
upon both sides; the skin is then dry, and at times more or less
covered by scales; even after a fatiguing exertion not a drop
of sweat: is seersted. It should be stated that in some patients
who suffer during a certain period from hyperidrosis, more or
less complete anidrosis subsequently occurs.

These are by no means the only disorders affecting secretion
which occur in tabes, and in connection with such troubles I
might mention different changes occurring in the composition
of the urine, specially glycosuria which sometimes exists, the
excretion of an exaggerated quantity of urine, a strange form of
tabid diarrhoea, the hypersecretion of acid in the stomach in the
gastric crises, &c., but I prefer deferring the study of these
disorders until we speak of the different viscera, and of the com-
plications and symptoms which occur in connection with them.

Tastly, an enquiry should be made as to the pathology of
these trophic disorders in the skin. Are the nervons changes,
upon which these disorders more or less directly depend, seated

in the spinal cord or peripheral nerves? This is a question |

251

LECTURE XXI.

‘TABES,

SYMPTOMS (continued).

Muscular atrophy: Distinction from the conditions of muscular emaciation, which
aro due to consumption. Is specially seated in the lower limbs, and most
often bilateral; occars also in the upper limbs and in the muscles supplied by

Onset. Extension. Fibrillar

Electrical fs
Pathological Anatomy of these forms of amyotrophy ; museular lesions ; opinions
‘as to the nature of the nervous lesion (in tho spinal cord, in the peripheral
nerves). Hemiatrophy of the longue : ita symptoms. Appearance of the tongue,
paralysis of the soft palate, and inferior yooal cord upon the same aide, Lesions
of the medulla oblongata in casos of hemiatrophy of the tongue, Conclusions
which may be drawn owing to the co-existence of paralysis of the soft palate
and vocal cord, Attempt to range the forms of amyotrophy occurring in tabea
in two distinct gronps.

GENTLEMEN,—In the preceding lectures we have reviewed the
trophic disorders which occur in tabes in connection with the
bones, joints, and skin; nor are the muscles exempt from them,
and the muscular afrephy which occurs deserves special con-
sideration, This symptom was some years ago studied in the
most complete way by Condoléon,* the pupil of Joffroy. I shall
have occasion to quote more than once from his remarkable
inaugural dissertation,

Condoléon. was preceded in the atndy of thia subject by soveral

authors, amongst whom I would quote Charcot and Pierret, who
were the first to call attention to this symptom, the occurrence
of which they explained, Leyden, Westphal, Dejerine, Eulenburg,
Jofiroy, &c., who published interesting or important observa-
tions upon the theories which will be brought before your notice
by me.

Before commencing to describe the muscular atrophy which
occurs in tabes, it must be understood, gentlemen, that by this

Bee ek Tone pathogvnique de l'amyotrophie tabétique
Thine ¢ Paris, 1887.

TABES DORSUALIS. 253

when they exist it is usually when the amyotrophy occurs early
in the disease.

Fig. 147.—Atrophy of the muscles of the right shoulder and of the hands in a case
of tabes. (Collection of Charcot.)

The electrical examination, according to Condoléon, usually
shows considerable diminution of electrical irritability. The
response of the muscle to the electric currents is otherwise un-
changed ; Joffroy, however, in a case of club-foot found that the
reaction of degeneration partially existed in the peronei and
extensor muscles of the toes (retention of faradic and galvanic
irritability in the peroneal nerve, diminution of faradic irrita-
bility in the muscles, slight increase of galvanic irritability with
stronger contraction at the positive pole).

The cowrse of these forms of amyotrophy is but relatively pro-
gressive; that is to say, the atrophy may gradually increase in

TABES DOKSUALIS. 205

however, that in one case Joffroy found partial reaction of
degeneration to exist.)

Fig. 148.—Tabid olub-foot of Joffroy. (Collection of Charest.)

*'The tabid club-foot neither results from contracture, for the
reason already stated, nor from such atrophy as exists in infantile
spinal paralysis, nor are the bones concerned with its production,
but it is a club-foot produced by flaccidity—an atonic club-foot.
In these conditions the foot, owing to the weight of bedclothes,
being constantly in the position of flexion, elongation of the
anterior ligament of the tibio-tarsal articulation, or rather of the
fibrous sheaths enclosing the tendons and taking the place of
ligaments, occurs; this elongation is facilitated by the flaccidity
and atony of the muscles of the leg; on the other hand, the
contrary oceurs in the region of the tendo Achillis, which is
shortened either on account of adhesions, fibrous bands, or
muscular contraction ; in no case does contracture of the gastroc-
nemii muscles occur, which are as flaccid as the muscles in the
anterior region of the leg.

“The prophylactic treatment is to prevent the bedelothes
from weighing down the foot, or if necessary, to apply a simple
orthopaedic apparatus.”

Such is the talid cluh-foot, gentlemen, which, notwithstanding
the similarity of the names, you must not confuse with the tabid
foot; the latter is, as you have seen, the consequence of an osteo-
arthropathy, whereas the former is due, as we have just said,
to atrophy of the muscles of the leg. I should add, that besides

‘TABES DORSUALIS. 257

‘matter of the anterior horn ; this lesion according to Charcot and
Pierrot is oonsecutive ta, erbeasiot. oF testa iespe eye Comes
the posterior columns,

ioc wigne bts ee ell Py SES ee
suffering from rery pronounced amyotrophy. ‘8 preparation taken {rem
epee hehe ten Se sist 11) hy AA Marla Ses na eng

condition; BB. degenorating muscular fibres; C. band of connective tissue
between the fasciouli. It will be observed that the faacioali of wasted fbres
alternate with those which are sound, and that the degenerated fibres are dis-
eeminated in the midst of those which are sound.

On account of this difference of opinion the researches of
Condoléon are of great interest, since the special object of these
was to ascertain which of these two theories was most in
accordance with the existing facts. The results to which they
led Condoléon were as follows: lesions were found to exist in
the cells of the anterior cornua with very slight changes in
the anterior roots and large nerve trunks, and on the other
hand pronounced lesions in the intra-muscular nerves. Thus
Condoléon is of opinion that the hiatus in the seat of the
lesions fully explains the fact that in certain cases the change
in the cord was ignored, and the peripheral neuritis alone
— 17

TARES DORSUALIS. 259

seldom know that any diminution has occurred in the size of
their tongue, It is the medical examination which reveals it to
them.

In every, or almost every, case the clinical aspect is identically
the same. The appearance of the tongue when protraded is
found to be that of a curved surface, in the form of a small
crescent enclosed in one which is larger, The side affected by
atrophy (the small crescent) is shrivelled, reduced in size, placed

Fig. 15),—Hemiatrophy of the tongue in caso of tabes (in this dingram the
hemiatrophy is seated on the right side, the figuro having been torned round;
in reality it waa.on the loft side). (Collection of M. Charcot.)

at a somewhat lower level than that of the sound side. A series

of furrows, which are more or less twisted, exist in it, which

give to it the appearance of convolutions affected by atrophy.
The tip of the tongue is distinctly turned towards the side of
the atrophy. ;
When the tongue is taken between the fingers (J. Hatchinson)
it ix found that when that organ moves the half in which atrophy
has occurred does not harden, whilst, on the other hand, the
ominary change occurs in the consistence of the other half.

Observe, gentlemen, that however considerable the deviation
and atrophy of the tongue may be, all its movements are still
possible, except that of giving itself a grooved surface. Hence
no appreciable functional disorder exists. The speech, mastica-
tion, and deglutition are unaffected, and, as I have just said,
the patients remain ignorant during an indefinite time of the
existence of hemiatrophy in this organ,

TARES DORSUALIS. 261

Progressive it remains limited to the side affected and never
changes to labio-glosso-pharyngeal paralysis; that is to say
severe, and posssibly fatal bulbar paralysis does not occur.

tongue occurs with the same characters in other affections than
tabes ; it has been observed in general paralysis of the insane,
which is not surprising when the close analogy which exists
between these two affections is remembered, which causes them
to have more than one symptom in common, It has been also
observed, and these perhaps are the most frequent cases, in
syphilis, affecting the pons varolii and medulla oblongata. This
is a fact upon which I specially insisted in my work written in
collaboration with PD. Koch. Gouguenheim and Lendet had
also mentioned it some weeks previously. This wtiology is, in
my opinion, of very great interest, since, it being allowed, as
we shall see in a subsequent lecture, that the greater number of
those who suffer from tabes or general paralysis of the insane
have been affected by syphilis, it may be asked whether the
hemiatrophy of the tongue is due to syphilis or to tabes. In
other words the question is whether hemiatrophy of the tongue
and tubes are connected affections, and the former depends
directly upon the latter, or the two are merely associated dis-
orders which should be considered as different manifestations or
one and the same general disease, syphilis.
However this may be, the lesions of the bulb in cases of
hemiatrophy of the tongue are clear. The large nucleus of the
hypoglossal nerve on the side corresponding to that of the lingual
hbemintrophy is much smaller in size than that on the opposite
side, a certain number of nerve cells having disappeared, as seen
in the disgram which I now place before you. In addition to this
the accessory nucleus upon the came side shows analogous changes

TABES DORSUALIS. — 263

(Gravx-Féréol, Mathias Duval), When change has occurred in
the nucleus of the lntter nerve this muscle is found to be

we

Fig. 15b—Seotion of the left hypo- Fig. 155—Section of tho right hypo-
glosal nerve (normal) from mcase of © gleaxal nerve (ilitensed) from m cano
bemiatrophy of the tongue in tabee, of hemiatrophy of the right side of
{After P. D. Koch and P. Marie.) the tongue in D.

Koch and P.

fibres which are in a norma! con

dition and havo rotained their sheath

of myolin (black points) are very few.
paralysed to a certain extent, viz., in respect to certain positions
of the eye, whilst its functions as regards other positions are
totally unaffected. The same is the case as regards paralysis of
the soft palate and larynx in tabes, which result from this change
in the hypoglossal nuclens, Considerable disorder is seen to exist
in these two organs, although there is, 80 to speak, no functional
trouble. The fibres coming from the hypoglossal nucleus act
therefore in a special way which differs from the action of the
fibres issuing from the nuclei of the 10th and 11th pair of
nerves. I cannot give you more information as to this point
since these facts are as yet but little known; all 1 can do at
present is to call your attention to their existence.

This is what I wished to say about lingaal hemiatrophy. In
connection with the disease which we are now studying, its
consideration cannot be looked upon as a mere digression, since
these cases show us in the most undeniable way that one form
at least of the amyotrophy which occurs in tabes is accompanied
by obvious change in the central grey nuclei.

T said “one form at least of the amyotrophy which occurs in
tabes,” since I must confess, gentlemen, that T am doubtfal

LECTURE XXU.

‘TABES,

SYMPTOMS (continied).

‘Viscrran Disonnsne.—I. Digestive o,stem: (@) Stomach.—a. Gastric orives, their ,

GextLemes,—Until the present time we have specially con-
sidered the symptoms in tabes which are connected with the
limbs or organs of the senses; other symptoms have still to be
discussed, which occur almost as frequently, are as important,
and also form part of the ordinary clinical aspect of the disease;
these are the derangements which occur in the viscera.

‘THE VISCERAL DISORDERS in tabes are numerous, and scarcely
any system is free from them; they will be reviewed as they
occur in the different organs,

I—Dicesrive System.

A. Sromacn,—Amongst the symptoms which occur in tabes
in connection with this organ, the most interesting is undoubt~
edly the gastric crisis,

a. Gastric crisis, Although examples of its occurrence are
found in the work of Topinard, and in that of Delamarre (1861),
it is again to Charcot (1872) that we owe the knowledge of
this singular symptom; the description which he gives in his
first lectures remains always classical; more recently in his
“ Legons da Mardi”* the eminent professor of the Salpétriére

* J. M. Charcot, Lecons du Mardi, 188889, p. 333.

266 LECTURES ON DISEASES OF THE SPU
Hospital again discussed this symptom,
extending his classification ; it is this descrip
guide us in the study which we are about to
mention also to you certain facts which were b
Fournier,
Many of you have without doubt been already
of the dramatic episodes of tabes which are t
since these are far from rare; in whatever
may prosecute your studies, one ease at least is al
to come before your notice.
‘The two symptoms which specially characterise
crises in tabes are: (a) the pain, (b) the uneontre
() The pain corresponds to that of gastralgia 5
the epigastrinm, being at times limited to that
often extending in different directions, towards
side or the back ; very severe in the great majority of ca
at times absolutely agonising, seeming to exceed wh
being can bear in the degree of suffering.
(Gin naccinecRlablesstostars 38) SI
but soon afterwards exclusively of slimy —_
consist, of a clear liquid, mucons in character and
abundant; at times the vomited matter is ©
or more rarely by blood; it may quite
appearance of “coffee grounds” (a case
Vulpian, one under that of Charcot), Usu
occurs very frequently, but varies in abundai
few grammes (1 grm. = 15'432 grs. Tray) of lig
on each occasion, but sometimes a great deal ;
characters vary according to the patient and
from day to day.
During the last few years different wo
the composition of the liquid vomited in.
conclusions formed are certainly not as
but that is no reason whatever, gentl
to be interested in the researches wh
In 1885 M. Sahli* stated that an
the acidity of the gastric juice
quantitative analysis of the
(1886), upon examining the liquid
*H Sabli, Correspondbl. f

TABES DORSUALIS. 267

of the gastric crisis, Rosenthal found during the first days 030
per cent, of hydrochloric acid, and during the last days only 0-10
per cent,: now according to Ch. Richet the mean quantity of
hydrochloric acid in normal gastric juice is 0174 per cent.
‘Thus the figure obtained by Rosenthal clearly indicates, as you
see, that there is an excess of acid in the liquid vomited. A
similar excess of acid in the vomit during the existence of the
gastric crisis in tabes was also independently mentioned by
Simonin (of Lyons) in 1886.

More recently, Hoffmann, a distinguished pupil of Erb, again
studied this subject; in one of his patients he also found that
the gastric juice was secreted in excess, and contained temporarily
an excess of acid ; the quantity of hydrochloric acid varied, often
amounting to twice the ordinary amount, and being rarely less
than the normal quantity, and this only when owing to the
abundance of the matter vomited the acid was somewhat largely
dilated.

On the other hand Hoffmann was not able to prove the fact
atuted by Rosenthal, that the amount of hydrochloric acid
diminished or increased according to the intensity of the crisis. A
cause of error in this respect was very aptly suggested by Hotf-
mann, consisting in the fact that the more severe the vomiting
the greater is the thirst, and the amount of liquid injected
consequently more considerable, the gastric juice being diluted,
and therefore Jess acid in reaction.

Such great technical difficulties are associated with the study
of these facts, and so few attempts have been made to elucidate
ene ee Stelle that ne Defntso results have yet
though it seems most probable that such will

The gastric crises are usually early 83
occurring even in the prodromie period.
‘They are rarely isolated, that is to say, tl
suffers from but one crisis of this kind; usually,
once occurred, they tend to reeur more or
at times indeed taking place somewhat
example every year, every 6 months, or every
regards what ultimately happens, either of
sequel# are possible: not infrequently the gi
few years gradually diminish in number and i
completely cease; or they may persist during the '
of the disease, death at times occurring during o

ro

‘TABES DORSUALIS. 269

whilst the patient is in the collapsed condition, or after the
onset of coma.

Such is usually the aspect which the gastrio orlais presonts,
but you should know, gentlemen, that it does not always occur
in this manner, tas heeds ree Cano Cid ba aso Tia
abnormal forms.

‘A. The gastric crisis retains all the fundamental characters of
the type, but the patn is so agonising as to simulate that which
occurs in hepatic or nephritic colic, or from poisoning.

B. The crisis is accompanied by such a degree of collapse that
the symptoms are attributed to severe malarial fever, cholera, or
even to an organic cerebral affection,

C. There is no vomiting, the only symptom being pain, often
very severe, and occurring in paroxysms, or at any rate pre-
senting exacerbations (the cramp-like pain of Fournier).

D. On the contrary there is 10 pain, but vomiting exists, pre-
senting the characters which have been already mentioned
(Vulpinn, Pitres, Fournier).

E, Fournier has described a jlatulont variety of gastric crisis,
was alii a ranpeagse crm ape

ar ‘The crisis is sometimes of very and exceptionally short
duration, lasting at the most but o few hours, On the other
hand Blocq, who observed facts of this nature, remarks that
the crises may occur daily, and continue during a somewhat
considerable length of time.

The rluration of the gastric crisis may again be prolonged, and
continue during from 15 or 20 days to a month, or even more.
A patient observed by Buzzard informed that author that during
9 months consecutively he had suffered from gastric crises. It
should be added that in such cases the symptoms are not
equally severe during the whole period, but present times of
exacerbation or remission.

8. Another interesting derangement of the stomach which
occurs in the course of tabes is that which Fournier has
described under the name of tabid anorexia, This symptom,
however, is very rare, even in the opinion of this author. It
consists in the fact that the patient has lost the sensation of
hunger; at the same time slight vomiting occurs at times with-
out any appreciable cause. The patient, so to speak, censes to

TANES DORSUALIS. 271

irregularity in the diet, exposure to cold or other cause, being,
as Fournier expresses it, a form of “essential diarrhaa.” On
the other hand it persists almost indefinitely, and may continue
during 2. 3, or 4 years, certainly with intermissions and
exacerbations. In addition to this, and which is also a new
character, treatment is absolutely useless as regards this form
of diarrhoea, which you will understand, although not specially
serious as far as the general health is concerned, is a very great
inconvenience to the patients who sniffer from it.

Ti.—Vascunar Sysres.

Lesions and derangements frequently exist in this system,
much more often, in fact, than is usually believed to be the case,
since, a8 must be confessed, the patients suffering from tabes
are too seldom examined with regard to the occurrence of such
symptoms. If this was more often done their existence would
be more frequently recognized. The lesions and
are numerous, and affect the heart and blood vessels in different
manners. In connection with the vascular derangements I
shall bave to speak to you of a complication very recently
observed, viz., exophthalmic goitre.

(a) Heart. A somewhat large number of works have been
devoted to the study of the lesions which may occur in the
heart during the course of tabes, but I shall not enumerate
them, and the more so as the conclusions formed in them are
far from being the same ; according to some, in fact, lesions of
the mifral valve are by far the most frequent; according to
others lesions of the aortic valve ; I must add, gentlemen, that
in my opinion there is no doubt about this point. An exam-
ination made in numerous casea of tabes has shown me that
lesions of the aortic are infinitely more frequent than those of
the mitral valve. With regard to the latter, stenosis is specially
found to exist, whilst in the former incontinence is the most
common.

Although statistics do not enable me to state the frequency of
cardiac lesions in tabes, they may certainly be supposed to exist
in a somewhat lange number of cases; I would even say that if
four or five patients be taken fortuitously, who are suffering
from tabes at an advanced period of the disease. more or less

‘TAMES DORSUALIS. 273

symptoms of that complaint; the sense of constriction and pain
behind the sternum, often agonising, the extension to the left
arm, the feeling of approaching death, &, According to
Leyden, this “angor pectoris” is due to the fact that the cardiac
branches of the vagus are involved in the morbid process of tabes,
and the professor of Berlin remarks in support of this idea that
the symptom is at times associated with gastric crises (case of
Vulpian) ; on the other hand Oppenheim has observed in certain
cases of gastric crisis a premonitory sensation of agonising pain
and numbness in the left arm; these different characters would
indicate, according to Leyden, that the branches of the vagus
participate in the affection, This is quite possible when the
multiplicity of the nervous lesions which exist daring the course
of tabes is borne in mind, but in my opinion the question may be
asked whether this is always the case, and whether the lesions of
the aorta, of which I have recently spoken, are not in themselves
sufficient to produce the symptoms of angina pectoris without its
being necessary to associate it with the morbid process of tabes,

With the different cardio-vascular symptoms I will connect,
in order to facilitate the classification, but without wishing to
dogmatise, another singular phenomenon which has been recently
observed—I mean the coincidence of the disease of Graves and
tabes.

Attention was drawn to these facts in the same session of the
“Société des Hépitaux,”* by Barié and Joffroy. The latter
communicated six observations of their occurrence at the same
time. Much surprise was caused, but there was no doubt about
the fact; the patients were undonbtedly suffering from tabes,
since the lightning pains, the symptom of Westphal, the symptom
of Romberg, the inco-ordination of movement, the plantar
anesthesia, and the loss of sexual power were present; again,
the disease of Graves existed since the exophthalmos, the rapidity
of the heart’s action with hypertrophy of this organ, the
paroxysmal enlargement of the thyroid, the tremor and polyuria,
ke, were all recognized to exist.

This coincidence of the disease of Graves and tabes, is it of
extremely rare occurrence ? Certainly not, gentlemen, and since
we have “ learnt to see” cases of this kind fresh ones are being
continually found, and for my part I have very recently observed

* Bari¢, Joffroy, sold ser Pir pee ls one
1

TARES DORSUALIS, 275

exist. Either this is simple “ instigation,” as, for example, in
the cases in which another form of neurosis, hysteria, coincides
with tabes, or perhaps the symptoms of exophthalmic goitre are
directly consecutive to the tabid lesions in the medulla oblongata
as Barié believes.

Tt must also be observed that, if the coincidence of tabes and
the disease of Graves is not very frequent, hasten are
occasionally observed in connection with one of these diseases
when it exists in an isolated form, which recall the other
affection. Thus, in his first works upon tabes, Charcot remarked
the fact that tachycardia was frequent, the heart being often
found in tabes to contract 100 or 120 times in the minute.
Kabler was able to verify this assertion, and in 50 per cent.
of his cases of tabes the frequency of the pulse was found to vary
from 80 to 120 times in the minute; one of the cardinal symp-
toms of exophthalmic goitre, tachycardia, is thus frequent in
tabes. On the other hand, in the course of studies connected
with the abortive forms of exophthalmic goitre I twice found the
patellar tendon refler to be absent. Tn these cases one of the
symptoms of tabes was found to exist in the disease of Graves
(possibly these were cases of unrecognized tabes, since at that
time my attention was in no way directed to cases of this kind),
‘These facts are interesting, and if this connection does not settle
the question it should at any rate be noted.

Whatever explanation is given of the coincidence, it should
be remembered, gentlemen, that the fact: itself is indisputable,
and we should be grateful to those who first recognized its
existence and made it known, since every fresh discovery is a
step towards the victory of scientific truth.

TABES DORSUALIS, 277

Fano found hypermsthesia or anesthesia of the soft palate to exist
in 30; in 14 cases the sensibility of the pharyne waa diminished,
and in 9 cases that of the laryna. In 4 patients this author
found the pharyngo-laryngeal reflexes to be excessive and associated
with hyperesthesia of the pharynz. In 5 casea the epiglitie was
much flower in position. It would be interesting to know
whether all these changes are really due to tabes, or would be
also found to occur in patients enflering from different consump-
tive affections, and if so with what frequency, this being a
question which unfortunately I cannot answer,

On the other hand, Oppenheim® has described by the name of
pharyngeal crisis a symptom consisting of a series of deglutition
very frequently repeated (24 to 32 in the minute) and accom
panied by more or less sonorous indications of its occurrence,
These crises in the case observed by Oppenheim continued
during from 10 minutes to half an hour with short intervals:
they were accompanied by much pain, and associated with
congestion of the face and profuse perspiration.

B. Lanyyx.—The derangements of the larynx which may
occur during the course of tabes are numerous, of varying
intensity, transitory or permanent, and present considerable
modifications in their aspect ; Charcot includes them all under
the name of “ tabid laryngismus,” which is most convenient in

joe.

1, Acule symptoms of tabid laryngismus: Laryngeal crisis,
The first author who mentioned these was Fereol (1868). Jean
specially called attention to their existence, and they were then
carefully studied by Charcot and Krishaber, and it is under the
influence of these two authors that the monograph of Cherchewsky
was written, which presents a fall account of this subject, Since
that time it has been observed that these symptoms occur fre-
quently in a more or less pronounced form, and numerous works
or observations have been published about this condition,

The laryngeal crisis consists specially in dyspnoea of varying
intensity and existing in an isolated form, or in association with
fits of coughing, In the former case the respirations are short,
superficial, rapid, and during their occurrence one inspiration
takes place which is deeper and accompanied by a sound whieh

* Oppenheim, News Beiinige swe Path. der Tades dorealis (Arch. f. Paysh.
XX, 1888).

‘TAHES DORSUALIS. 279

the patient rises of his own accord and im!

senses without the occurrence of any condition reser

* confusion," which is liable to occur after an attack of true
epilepsy.

You have seen, gentlemen, how severe the attacks of dyspnea
may be in the laryngeal crisis. Fortunately this is not always
the case, and the symptoms may be limited to those which I
first described, It must also be stated that the duration of
these crises varies from a few minutes to several hours, How=-
ever severe the attacks may be they usually end favourably, the
dyspnea gradually diminishes and then ceases to exist, some-
times, however, in a much more abrupt manner. It must not,
however, be supposed that the crises always terminate in this
favourable manner. In some cases death has been known to
occur within a short time. This fact, gentlemen, should not be
forgotten, and if the complication becomes really serious pre-
parations should be made which enable the operation of
tracheotomy to be performed if

‘As regards tha frequenoy and course of laryngeal erisen it is
difficult to make any precise statement, on account of the
variations which occur; frequent in some patients, they ocear
seldom in others; constantly increasing in frequence and
severity in some patients they diminish in others, and are even
found at times to cease

Nor are authors agreed as to the: patheloy of this wymiptomt
Some believe it to be due to paralysis of the muscles which
dilate the vocal chords, On the other hand, Charcot and
Krishaber have shown that the crises are due to reflex con-
tracture of the muscles adjoining the glottis, a contracture
which is due to hyperwsthesia of the mucous membrane, these
authors having been able to reproduce it by touching that
mucous membrane with a soft body. This explains the fact. of
these crises being at times produced by a current of air, by the
contact of a cold body, by the act of speaking, by that of walking
quickly, é&c. Oppenheim has recently indicated another means
of artificially producing the laryngeal crises. This consists of
pressing upon a sensitive point situated between the sterno-
mastoid muscle and larynx, at the level of the cricoid cartilage.
I cannot give any personal opinion upon the value of this
mode of procedure, which I have never adopted, but according

: connected wit

TARES DORSUALIS. 281

The coincidence of the Jaryngeal crises with paralysis of one
or more laryngeal muscles is not quite constant, though frequently
observed

As regards the ataxy of the vocal cords mentioned by some
authors, it seems to be rare, and not to have been very clearly
observed.

The lesions to which these different symptoms are due will
now be considered,

As regards the muscles, secondary lesions exist in them which

Fig, 156,—Right half of a section of the medulla oblongata in a sound condition.
‘The upper transverse line represents the floor of the 4th ventricle; the vertical
line which limits the figure om the left aide ie the septum. A, nuclous of the

ic nerre; B, asconding root of the glosso-pharyngeal nerve, in
which mre fibres of tho pnoumogastric and spinal accessory nerres—the
“ slender column"; C, ascending root of the 5th puir of nerves; D, root Stree
of the pneumogastric nerve. (After Oppenheim.)

differ in no material degree from those already studied in con-
nection with the muscular atrophy which occurs in tabes.

Tn the nerves, changes have been fonnd to exist in certain
nerye trunks, either in an isolated condition, or conjointly with

bas

282 LECTURES ON DISEASES'OR THE SPINAL CORD.
lesions of their roots or their nuclei

BB0ry ypenthi
that the electrical irritability of the recurrent nerve is lost.
Tn the medulla oblongata, atrophy of the nuclet of these same
nerves (the vagus, glosso-pharyngeal, and spinal
been mentioned by a somewhat large number of

Fig. 157.—Right half of « section of the medulla oblongata, from a case of tabes
with laryngeal crises. (To be compared with the preceding figure.) A, nucleus
of the pnoumogastric nerve, which is altered and no longor contains herr
fibres; B, ascending root of the glosso-pharyngeal nerve, in whieh are fibres of
the pneuniogastric and spinal accostory nerves—the “slender column”; thir
teaot is very much altered, its nerve fibres having completely disappeared ; ©,
ascending root of the 5th pair of nerves which is affected and of a white
colour; D, the root fibres of the pnoumogastric nerre have almost disappeared.
(After Oppenheim. )

Demange, Landouzy and Dejerine, J. Ross, Kahler and Oppen-
heim),

To these facts I must add those which I havealready men-
tioned, in which hemiatrophy of the tongue comeides with
laryngeal paralysis, or rather with atrophy of the vocal cord on
the same side, In these cases the laryngeal symptom would, in

TABES DORSUALIS. 283

the opinion of P. D. Koch and myself, be solely due to the
lesion in the nucleus of the hypoglossal nerve, and the groups:
of cells connected with it,

Lastly, another lesion has been mentioned, and in a special
way by Oppenheim, namely, that which occurs in the ascend~
ing root of the lateral mixed system, or slender column.

These different lesions may either occur conjointly, or be
isolated from each other; in some cases the nuclei, in others
the nerve roots, in others again the peripheral nerves are.
affected; in this case, as when muscular atrophy of the limbs
exists, we meet with the same irregularity, the same opposite
conditions,

IV.—Uniwany Sysrem.

A. Derangements of the urinary secretion. These may be
qualitative or quantitative.

Amongst the former I must mention glycosuria,® which is
perbaps due to the existence of tabid lesions in the floor of the
Ath ventricle. Other derangements are undoubtedly due to the
consumptive tendency, of the existence of which in these
patients I have spoken more than once, Such are a diminution
in the amount of urea, and in the total quantity of phosphoric
acid, with 9 proportionate increase in the earthy phosphates,
Lastly there is a great variety in the quantity of chlorine
éliminated. with a tendency to the prodaction of hypochlorites,
after the researches of Livon and Alezais,

Albert Robin (verbal communication) has remarked that in
certain patients suffering from tabes the quantity of phosphorus
eliminated by the urine in an incompletely owudised state is
more considerable in the early morning than at bedtime. This
difference would be due to the nervous irritation produced by
the lightning pains during the day, and the sedative influence
which is due to sleep at night.

As regards the quantitative changes, the excessive secretion
of urine (many pints in the 24 hours) must be mentioned, which
occurs in certain patients, and often in a paroxysmal manner,

*G. Guinon and Souquos, in the Archives de Nevrologie, 1391, maintain the
interesting opinion that in some patients suffering from tabes glycosuria might
“be the expression of « hereditary diathesis, since as Charcot has shown, tabes in
frequently met with x families which suffer from diabetes,

‘TARES DORSUALIS. : 285

turn, were provoked on account of the long time which passed
before the place was unoccupied.

Complete retention of urine sometimes occurs, though it is rare,
and most often of temporary duration; IT have however known
it require the daily introduction of a catheter,

Absolute incontinence of urine may also be observed, specially
in the advanced period of the disease, but is also rave; when it
occurs it is often like retention of temporary daration, On the
other hand relative incontinence is frequent; under the influence
of emotion, effort, or even during sleep, and without apparent
cause, a few drops of urine dribble upon the shirt or into the
trousers, the patient in some cases, on account of more or less
anwethesia of the mucous membrane of the urethra, only perceiv-
ing that this accident has happened owing to the sensation of
moisture which is thus produced,

Tn some cases mictorition is abnormally frequent, and “ pol-
lakiuria” exists, or, on the contrary, it may be infrequent, so
infrequent in fact that some patients suffering from tabes no
longer feel the desire to urinate, and only pass water, as Fournier
says, from “a sense of duty.”

T have just spoken, gentlemen, of the anesthesia which some-
times exists in the mucous membrane of the urethra, and probably
also in that of the bladder; it must not be supposed that this
excludes the occurrence of pain in these regions. Pain, in fact,
is observed to occur either of a lightning character, or of the
persistent type (sensation of a foreign body); these are seated
either in the urethra, or at the neck of the bladder, or in the
bladder itself, and in certain patients, according to Fournier,
trae vesical colic may exist. This colic according to that author
may be compared with the gastric“ colic” or crisis. Lastly some
authors have mentioned cases in which the character and seat of
the pain were very analogous to that which occurs in nephritic
colic, which explains the name of nephritic crises which has been
applied to them.

V.—GENERATIVE SYSTEM.

‘The derangements of this system should be studied separately

in the two sexes, not because there is any essential difference,

bat on account of the special role of the male or female sex in
the sexual act causing them to have peculiar characters.

we are already acquainted.
Go fr all the information whisk it Se necessary to have with
respect to the different must be obtained

Gechidsa sogara or ts extnaoes of shinveca ms mec Ote

I have caused the pubes of the healthy man to be exposed, and
the thighs as far as the knees in order that you may see the
scrotum clearly, as it is this part which you must closely watch,
with the object of observing what takes place,

I rapidly rub the inner surface of the thigh from above down-
wards by means of the nail or some soft body, gentle pressure
being made upon the skin; this is only done once, and the
immediate effect is that the.testis upon the same side rises
towards the inguinal canal ; ad often as I repeat this action the
same elevation of the testis océeurs; this is what is termed the
eremaateric reflex. We will ndw examine the condition of the
same reflex in the tabid patient suffering from impotence; I
may perform the same action again and again along the inner
surface of both thighs without ‘producing the slightest result; no
movement in the testis occurs, and the cremasteric reflex is com-
pletely absent. There is in fact; gentlemen, an intimate, I dare
not say absolutely constant connection (these facts not haying as
yet been s0 thoroughly tested as ‘one would wish) between the
absence of cremasteric reflex and impotence, when the latter is
due to an organic lesion of the. necvous.system, as in tabes; on
the other hand, when impotence exists in other nervous diseases
and is not dué to an organic lesion Of the’ nerve centres, the

‘TABES DORSUALIS. ‘2389

havé been mentioned and studied “by Pitres and Rividre.* The
atrophy of the testis was found by them to exist in 3 out of
20 cases ; with regard to the anesthesia of this organ when

i pronounced degree.

‘These, gentlemen, are symptoms of tabes connected with the
genital organs, of which there is direct evidence, and which
give yaluable information as to the condition of their functions
in the male sex,
| 8. Tn the remate. In the female sex, as I have already
observed, the derangements connected with the genital organs
sre almost analogous to those occurring in males, but the differ-
ence of the organs causes them to have special characters.

(a) Genital depression. This may exist in a greater or less
degree; the previous disposition of the patient must naturally
be considered, a somewhat large number of women in healthy
condition haying no sexual desire whatever. In females suffers
ing from tabes this desire may either be diminished or perhaps
completely lost, as in the male sex. In this case again the
symptom is at times preceded by indications of genital excite-
ment,
~ (1) Genital excitement. This sometimes occurs to & very pro=
nounced degree, as for example, when the condition occurs
which is termed by Pitres} the clitoridean crisis, The best
means of making you understand What occurs in these crises
‘is for me to quote verbally one of the observations of Pitres:
“The yolaptuons sensations occurred, according to the account .
‘of this woman, at any moment in the day, specially when
‘she was inactive, and without being preceded by any pro-
voking cause or lascivious thought. It began by a sensation
of vibration within the vagina. The sensation extended to the
clitoris, which entered into a state of erection, this being soon
followed by a true erotic spasm accompanied by ejaculation, in

the same way as during ordinary sexual connection.
is spontaneous crisis was almost always repeated three or
(oer times during the same day, after which one or two weeks

* Bivitro, De Tancsthcsie ot de Matrophie testiculaires dans Mataxie locomo
trio, Thése de Bordeauz, 1885,

+ Piteos, Dos crises clitoridiennes au début on dans Je cours de l'ataxie locomo*
teice. Progris médical, 1834, No. 37, p. 729,

ke :

291

LECTURE XXIV.

'TABES,
SYMPTOMS (continued).
‘VI. Ceresmat sverew.—It Epileptiform
attacks. Acute symptoms connected with the medulla oblongata, Psychical

Counse o¥ Tanxs.- —Prodromal period. Second period, period of inco-ordination.
‘Third period, confinement to bed. Forms of tabos: upper or cervical, corcbral,
mild, and severe forms.

‘VI-—Cerresrat System.

The hemiplegia which somewhat frequently occara in the
course of tabes will not be again considered, this complication
having been discussed with the other motor disorders. The
cerebral disorders which will be now discussed are the
apoplectiform or epileptiform attasks which sometimes oceur,
and the peychioal derangements, I would also remind you that
many authors (Berger, cc.) have observed migraine to indicate,
80 to speak, tho onset in certain cases of tabes.

The apoplectiform attacks in tabes have been specially studied
and described by Lecoq and Giraudeau. Kahler has also con-
sidered these complications, and terms them collectively cerebral
tabes; the symptoms vary from simple vertigo to complete loss
of consciousnéss, and may be accompanied by aphasir, or, as
we have said, by hemiplegia of the face or limbs.

The epileptiform attacks may be of the severe or mild form,
and Jacksonian épilepsy at times occurs.

By what mechanism are these complications produced? It
is probably multiple; in some cases foci of real hemorrhage or
softening exist, or some morbid process of long duration in the
meninges or ependymia, or vasomotor disorders due to some
change in the medulla oblongata or pons varolii. My opinion;
however, is that such lesions should not be considered due to
the existence of tabid le:ions alone, and it se2ms far more

_ TARES DORSUALIS. an 293

disease, whatever Some duthors may say to the contrary, Con-
sider facts which have occurred, gentlemen, and decide this
question for yourselves; there is no liberal profession to which
tabid patients are nob known to belong in some numbers, and
to be in full possession of their intellectual powers: the one is a
famed musician, the other a learned scholar, the third a states~
man, the fourth a man of business, I mean with the management
of important matters. In mentioning bat those who are dead ;
the artist’ Manet suffered from tabes, as did Henri Heine, and_
remember, gentlemen, that at Bapaume Faidherbe already did
so. After this enumeration, truly martyrological, it must be
allowed, Teese that Stel eoveal) Bieter anno assay
associated with locomotor ataxy.

Coursk AND FoRMS oF THE DISEASE,

T have been careful, gentlemen, when speaking of a symptom,
to indicate as far as possible the period of the disease in which
it occurs; and I have no wish to repeat what has already been
said; at the same time we must necessarily consider, from a
fetrospective point of view, how the principal symptoms occar,
that is to say, what is the course of the disease,

In ordinary cnses the progress of the disease is very slow.
and we are more or less justified in distinguishing certain
periods: the prodromic period, the ataxic period, the period of
confinement, and that of paralysis, These stages, gentlemen,
must not be considered an article of faith, their real merit being
that of being classical. In reality this is not of great
importance, and is often in complete disaccord with the facts
of the case, at the same time it somewhat facilitates the descrip-
tion of the disease, and on that account I shall ask for permission
to adopt it.

Prodvomie period.—This is pre-eminently the time at which
the lightning pains occur, which are most often referred by the
patient to some totally different cause, and be voluntarily terms
them “rheumatic.” Thus reassured as to their nature he troables
himself but little about their existence, except at the time
when they occur, and does not consult a medical man unless the
pain is very severe. It is thus but rarely that the ocourrence of
these lightning pains enables the existence of tabes to be recog~
nized. In most cases something more is required, some things

TABES DORSUALIS. 205

control over thé movements of thé lower limbs, The patient is
then obliged to remain either in bed or upon a chair; he has
become totally powerless. ‘To this should be added an aggrava-
tion of the urinary disorders, the existence of cystitis with
pyuria, at times sloughing of the skin in the gluteal region or
heel, a more or less pronounced change in the general nutrition
of the patient, which also diminishes his power of resisting the
different sources of infection (pneumonia, erysipelas, phthisis)
which may occur, and you will at ones understand to what great
dangers the patients suffering from tabes are exposed. ‘There
‘ire some patients, however, who can still withstand the effects
of the disease during many years, however bad the conditions
may be in which they are placed. Thus, the duration of tabes
with its. three classical periods may certainly be 10, 15, 20
years, or even more. For a still stronger reason the duration
may in some cases be longer, viz., when the first or second
period of the disease alone exist, since the causes of death are
notably fewer at these times than in the third period.

A few words should be also said, gentlemen, about the difs
ferent forms of tabes. Of these forms some are connected with
the seat of the morbid process, others with its mode of derelop=
ment. Although the abnormal cannot be separated from the
ordinary form by any definite character it is obviously better to
distinguish them from a clinical point of view. The principal
forms will alone be mentioned by me.

The superior or cervical form of tabes is characterized by its
onset, which consists of pains in the upper extremities; the
lower limbs are but little affected and ataxy rarely exists, or if
80, 13 but little pronounced ; in this form muscular atrophy often
occurs (Leyden). As to the persistence of the patellar tendon
reflex, Weir-Mitchell, Martins, Bernhardt, Eichhorst, who have
published cases of this form, are far from giving the same
necount of what occurred, since according to some the patellar
tendon reflex existed, while according to others it was absent ;
thus it is difficult to make a definite statement with regard to
this fact. In my opinion the persistence of this reflex does not
seem to me at all impossible. In every case you must be very
careful, gentlemen, to avoid the mistake which consists in re-
garding cases which belong exclusively to syringo-myelin a3
examples of cervical tabes.

TAHES DORSUALIS. 297

the tabid symptoms, and of their generalization ; in some cases
the patients seem to suffer from every symptom which can
occur during the course of this affection, These were the cases
described by Duchenne of Boulogne; they are truly patients
who are most suitable for the study of the disease, but at the
same time they deserve our fullest sympathy, since they may
be considered the most unhappy of men.

In other cases the form is severe on account of the rapid
course of the disease, These cases have been already mentioned
under the name of acute tabes. Within some months
ataxy of movement occurs in these patients or they may be
confined to bed after a short lapse of time.

It is sometimes the consumptive tendency which develops to
fn unusual degree and constitutes the danger; the patients are
then found to be extremely emaciated, with hollow eyes,
pinched features, and an earthy complexion; should an
epidemic oecur, or the patients be placed in contact with the
tubercle-bacilli (which is not infrequent in the wards of our
hospitals) they are already destined to be victims of this disenze.

Lastly, the severity of the disease may be due to the coinci-
dence of general paralysis of the inane with tabes, of which you
already know; the head and lower limbs then suffer simal-
taneously, the unfortunate patient is entirely helpless, and death
inevitably occurs,

‘TARES DORSUALIS. 299

excess have been much exaggerated, as also of onanism, sexual
connection in an upright position, or too frequent venereal
congress, That such exeess may have the effect of producing
such pronounced organic lesions of the nervous system is a fact
which I absolutely deny. The only concession which I can
make is by allowing that a condition of more or less accentuated
nervous instability may be thus produced, and the more so since
it is only those whose nervous system is in a weak condition
who give way, at any rate habitually, to such sexual excess as
to suffer from disease. In this opinion, however, namely that
tabes may be produced by sexual excess, as in all popular
beliefs, there is some basis of truth. It is a fact that a large
number of tabid patients have been somewhat profligate. At
the same time, by the simple application of the law of proba-
bilities, they have also been more liable than others to contract
syphilis, as I shall presently be able to show you. “Tabes
venerea” is possibly the cause, but upon the condition that
larger place is made for an impure than for an immodest Venus.
The effect of injury bas been invoked by many authors,
amongst whom I would name Verneuil, Spillmann, Parisot, and
Klemperer, who has: specially studied this mode of causation;
traumatic causes in general are considered in this case, Guelliot,
Bernhardt, and some other authors have specially, and typically,
‘incriminated the trepidation produced in the whole body by tho
use of the sewing machine. his, however, but little accords
with the greater frequency of tabes in the male sex, and to me
this mechanical action seems of too slight a nature to produce
such considerable lesions os those which occur in tabes. I
should be more inclined to attribute the origin of the disease in
these cases to the fact that those who work the machines are
not: usually of spotless virtue. It ia by no means impossible
that the patients whose history has been recorded were simply
suffering from syphilis,
» With much the same idea as to the causes of the disease
Hoffmann states that he bas seen tabes occur in a nail-making
workman who worked upon a machine, where he was exposed
to somewhat violent shocks varying in number from 6,000 to
10,000 daily; symptoms of tabea occurred after 3 months of
this work. In this case also, gentlemen, I can scarcely believe
that these shocks were the real cause of the disease.

'TARES DORSUALIS, 301

to Althaus 90 percent, By the side of these numbers I ought to
fnention, in fairness, those of Gowers and Seguin, which vary
from 70 to 53 per cent, With regard to the statistic of
Westphal, the number, which only reaches 14 per cent., differs
so much from those which precede, and from my own observa-
tions, that I cannot but believe it to be vitiated by some
important cause of error, and to be of no value as a guide.

Quite recently Erb* has published a second statistical account,
including 369 new cases of tabes observed by him between 1883
and 1891, ‘This work contains information which is so important,
and in my opinion so convincing as regards the opinion main-
tained by me, that I shall claim your permission to borrow
largely from it.

Of the 369 cases 300 refer to patients belonging to the higher
class of society, 50 to the lower class, and 19 to women,

‘The 300 first cases are composed as follows :—

A. Cases in which it was impossible to find any indication
of syphilitic infection a, SL,

B. Cases in which syphilitic infection had oceurred’ = 89°,

‘The latter included :—
«. Cases in which symptons of segniory syphilis had

existed en = 63:3*/,
A. Cases in which the chancre had alone existed, ho
secondary syphilide having occurred . aa oo ape em SBS

The i realninhin poracioarnenat ane
A. Cases in which it was impossible to find any indication

of syphilitic infection .... a == Qf,
B. Cases in which syphilitic infection bad occurred we = 76],
‘The latter included :—
e Cases in which symptoms of secondary syphilis had
existed = R/,
a Casts ia "which the chasers hed alone ecearrel 9
syphilide having existed... ou we oe = My

Thus, in the first 300 sibs cat de ies Wa Ba
of 89 per cent. who had suffered from syphilis agrees completely
with the figures of Fournier and Erb in his first statistical
record, It is true that in the lower classes the number is not

= W, Brb, Zur Htiolegie der Tobes, Berliner hlisische Wockenschrift, 1891, No,
29,p. 713. This work was published after the time at which the lecture way
Lise Lowey dy to rears nore wd thw paragraph of

‘the importance will be evident to all,

TAKES PORSUALIS, 303

other viscera when syphilis often produces in them lesions which
are quite ineffaceable ?

3. Lastly, a great argument specially directed against the
statistics themselves is the following statement: Syphilis is so
common in the class from which the hospital patients come,
that this, eo to speak, is a common élement, and cannot be
invoked as a couse of the disease more than any other chronic
diseass might be. This argument is easily answered, and in
this respect it suffices to ascertain how many persons suffering
from syphilis are found amongst those who are free from tabes.
Many authors have made statistical observations of this nature,

Erb, in a first statistical record, found that in the collection of
500 hospital patients who were free from tabes, 77 per cent.
were certainly wnaffected by syphilis, whilst it must not be
forgotten, gentlemen, that 88 per cent. of the patients suffering
from tabes were ascertained to be thus affected.

Levinsky found that in 620 hospital patients who were free
from tabes, an analogous number, 80 to 85 per cent., were free
from syphilis.

Neegeli in 1,450 patients arrives at an even higher per-

Quite recently (1891), Erb, in the work from which I have
already quoted, returns to this subject ; he has examined 5,500
patients who passed through hia hands with respect to the
existence of syphilis, taking care to eliminate those suffering
from tabes, and all the patients who cam» to consult him
specially on account of syphilitic affections to the exclusion of
every other affection. From this large number he obtained the
following results: 224 per cent. of these patients were syphi-
litic, in the remaining 77°5 per cent. there neither was nor
had been any indication whatever of syphilis. Compare there
fwo numbers: 225 per cent. of syphilitic patients amongst
those who did-not suffer from tabes, and 89 per cent. amongst
those who did so, and you yourselves will easily form an opinion
as to the connection of tabes with syphilis.

Such are the principal arguments invoked by the authors who
will not admit the connections which so evidently exist, in my
opinion, between these two affections, I hope that I have
shown you, gentlemen, that the refutation of these arguments is
not really very difficult.

—

‘TARES DORSALIS, 305

tabid fathers have not necessarily tabid children, but may
be indirect, the most different diseases of the nervous system
possibly existing in the ancestry of these patients. Epilepsy,
hysteria, hemiplegia, chorea, mental alienation, and psychical
degeneration, in all their forms are found, according to Charcot,
in the near relations of tabid patients. Another affection,
diabetes, the pathology of which is also intimately allied to that
of nervous disorders, is also one of the most frequent hereditary
antecedents of locomotor ataxy (Charcot),

Heredity must also be invoked in the etiology of tabes with
regard to hereditary syphilis, Fournier considering that this
affection may be the sole cause of the existence of tabes. I
cannot say that this fact has been definitely proved ; the futare
will show what should be thought as regards this connection,
and enable us to know more certainly whether the affections of
the spinal cord which are due to hereditary syphilis belong or
not to the ordinary forms of tabes. It is possible that they may
present special clinical features and lesions, /as, for example, the
disease of Friedreich (272).

Leaving, gentlemen, the question of the causes of tabes, which
are now I hope understood by you as far as the great majority
of eases is concerned, the other wtiological conditions of tabes
will now be discussed.

The age at which the onset of the affection occurs is variable ;
it most often commences at a certain time of life (between 30 and
45 years). It is rarely observed before the age of 25 years,
although cases have been recorded at the ages of 16 cr 17 years
(22); it is rare also after the age of 5) years.

Tn connection with its onset a most interesting question is
that relating to the period of time which elapses between the
existence of the chancre and the onset of tabes. Erb has given
a statistical table of 300 patients in connection with this point,
and arrived at the following results—

The onset of tabes occurred—
lto 5 years after the syphilitic infection in 12-3 per cent. of the eases.
9 to 10 1" ” " 37 ” ”
Utols ” ” “ Wee «
16 to 20 " ” 42 » ”
21 to 25 ” " ” 48 ow ”
26 to 30 - ” 19 ” ”
31 to 35 ” ” OF» ”

2

TABES DORSUALIS. 807

female sex be referred to the fact that, other things being the
same, this sex possesses a special immunity with respect to this
affection of the spinal cord? This opinion does not seem very
probable ; it is more likely that the females being less often
affected by syphilis than the males are on that account much
less exposed to the danger of contracting tabes, ,

Perhaps also the greater frequency with which the nervous
system in females is over-wrought should be taken into con-
sideration. Syphilis, as Mobius has shown, playa the same
part in the wtiology of tabes in females as in males; thus of
the women suffering from tabes observed by Erb syphilis was
indicated or its existence was extremely probable in 89-5 per
cent.

Does race exercise any influence upon the frequency of tabes?
Tt has been said to be more frequent in persons with blue eyes;
for my part I believe that I have been able to recognize the
truth of that assertion, but my observations have not been
sufficiently numerous to enable me to form a definite conclusion,
No works have been written upon this question of race, and I
can only mention one statistical table of Minor which again is,
as you will see, susceptible of another interpretation. This
distinguished physician; who. practises in a region of Russia
where the Jews are somewhat numerons, examined how many
tabid patients were found in an equal number of Jews and
Russians; he found that in the latter there were 2:9; in the
former only 0:8 per cent. ; it thus appears that the Jewish race,
in which from so many points of view the power of resistance
and vitality are so remarkable, is gifted with a special immunity
as regards tabes. Such, however, is not the opinion of Minor ;
this author has in fact reasonably observed that the Jews on the
other hand are extremely liable to suffer from every affection of
the nervous system; while he attributes the infrequency of tabes
among them solely to the fact that on account of their special
mode of life they are rarely affected by syphilis. This, then,
would be another important, though indirect, proof of the part
played by syphilis in the wtiology of tabea. This fact will be
brought before us in another way during the study which we are
about to make in connection with the effect of the professions
upon tabes.

The different professions suffer most unequally from loco-

LECTURE XXVI.
TABES,
DIAGNOSIS,

Drsorosis: DINGS Lo EEE irae ee ee ey Differ-
ential character as regarda: Cerebellar affections, q

arsenic; B. Diabetic prendo-tabes; C. Neurasthenic pseudo-tabes, Taxnarncrice:
A, Taternat remedies; matenle of siivor, strychnine, aconitine, atropine, ergot
pechay anti-syphilitic agents: moreury, iodides; B. External remedice:
Socoe: (rites appted oa ua alin, close, Seoeea penacearertien
, hydrothorapy ; general summary of the treatment adopted.

‘The p1aGNosis of tabes, if completely considered, would require
scarcely less than a volume to expound it; the whole of path-
ology would have to be reviewed. The most different disorders,
either connected with the limbs and skin or with the different
organs, may open the scene, and owing to their intensity oceupy
it exclusively, in such a way that the other symptoms due to the
affection of the spinal cord are entirely masked by them.

Thus, for example, the gastric disorders of tabes may, as we
have seen, stimulate the most severe organic affections of the
stomach, or even cause an attack of cholera to be suspected.

‘Thus again the genifo-urinary derangements of these patients
often cause mistakes to be made, and lead to an organic affection
of the urethra or bladder being wrongly suspected to exist,
whence the designation of “false urinary patients” which
F. Guyon rightly gave to them.

The same may be said of the larnygeal disorders, and of the
lightning pains, x0 often termed rheumatoid, as also of the ceular
or auricular troubles.

T have already, gentlemen, spoken to you of these different
facts, and shall not therefore return to them.

Nor shall I now consider the diagnosis as regards certain
affections of the spinal cord, with which you are not yet supposed:
to be acquainted, and which will be described in the course of

TABES DORSUALIS. 3

to advance either by crawling or leaping or moving his chair ;
that which he cannot do is to walk. In tabes, on the contrary,
inco-ordination, when it. exists, affects every. movement of the.
lower limbs whatever it may be.

Syringo-myelia, in which the symptoms are often numerous
and, so to speak, unforseen, so varied is the deat of the lesions:
which constitute that affection, is often attended by phenomena,
which are more or less analogous to those occurring in tabes:
these consist of trophic derangements, sensory disorders, pains,
and at times the loss of the knee-jerk. Usually the. existence
of the sensory disassociation which occurs in yelia,
will enable that affection to be distinguished from tabes; at the
same time the latter disease may in some cases (Parmentier) be
attended by this disassociation ; the intensity and nature of the:
trophic disorders will be also a guide (the whitlows, the loss
of one or several phalanges), as will the frequency and forms
of amyotrophy. No exaggeration however must. be made, the
diagnosis ceericrenlpeoisadscleae fos = usually un-
attended by diften!

Ro ah oto cans ieee aes ey sale
te which, owing to their resemblance to tabes, the name of
Pseuilo-tabes bas been given. These psendotabid affections,
depending upon morbid conditions which are yery different
from each other, have been specially studied in France, from the
special point of view which we are now considering, by Leval-
Picquechef; this group is classified as follows:—

A. Toxic psewdo-tabes—These are produced by certain forms
of intoxication, notably those which are due to alcohol and —
arsenic, It is to this class that the cases described under the
name of neuro-tabes belong, which are in reality but cases of
aleoholie psendo-tabes.

In these cases, gentlemen, there is often most severe pain,
very obvious disorder of the gait, and considerable diminution
or lose of the knee-jerk; ocular disturbances consisting in
impairment of vision and at times, but more rarely, ocular
paralysis and ptosis exist.

When these symptoms occur the difficulty is at times very
great, How may these cases of pseudo-tabes be
from the real disease? A careful pe a of the symptoms will
enable this to be done.

TABES DORSUALIS, 313.

occurs, Iam referring, gentlemen, to an affection with which
you often meet, to whatever class of society your patients may
belong. I would even say whatever the ‘class of diseases may
be, which you specially treat; I mean, in one word, Newras-
thenia.

In order to give you an idea of neurasthenie pseudo-tabes L
shall not have to tax my memory to any great extent, so numer=
ous and clear are the cases which I can recall. You scarcely
know, gentlemen, how many of your colleagues are thus affected.
Those in the medical profession, in fact, on account of their
knowing the dangerous character of the disease, are the more
ready to exaggerate the importance of the symptoms which they
foel, or at least to interpret them in an erroneous manner.

Cases of neurasthenic pseudo-tabes present symptoms which
are essentially tabid: pains having the “lightning” character
at times, in some cases with a girdle-like sensation ; a difficulty
in the gait, which is clumsy, often staggering and resembling
that due to intoxication, while at times there is more or less
pronounced vertigo; in most cases there is also a more or less
pronounced diminution in the genital power; lastly the knee
reflex may be diminished or lost. Almost invariably, a fact
which should be well noted, gentlemen, the patients come to
you with the absolute conviction, boldly stated by them, that
they are suffering from disease of the spinal cord. As you
perceive, the condition of things is an embarrassing one for
the physician, and those who are most distinguished have been
led into error. If you would avoid mistaking neurasthenic
psendo-tabes forthe true disease, this is how I would recommend
you to act :—

In the first place, before anything else is done, put to your
patients this searching question: “ Have you ever suffered from:
syphilis?” If it is a man of education, whose sincerity you
can trust, and the response is negative, consider that in all
probability it is parely and simply a case of neurasthenic
pseudo-tabes, and endeavour to show that this is the case by a
careful examination of the following characters :—

(a) The pains, however acute they may be, never have the
terrible severity which is so distressing in those due to tabes.

() The wenkness of the genital functions, even when im-
potence exists, is not associated with loas of the cremasteric ©

and should be forewarned in order not to be astonished at the
dusky hue which the skin of these patients presents, This tint
is in no way due to the trophic change of the skin which occurs
in tabes, as one might at first suppose, but solely to the
employment of silver as a medicine which the patient has
taken during a long period of time. orwit

Chloride of gold, arsenic, the salts of zinc and other metals
have been recommended by a large number of authors... =

Of the alkaloids, strychnine, aconitine, and atropine must
be specially mentioned. nay
' Another remedy drawn from the vegetable kingdom deserves:
special consideration, namely ergot of rye: this is almost: the
only medicine which has any perceptible effect, at any rate upon
certain symptoms. It is to Charcot that we owe the knowledge
of its influence, and of the cases in which it should be employed.
The urinary disorders of tabes in fact are very successfully”
opposed by ergot. of rye, and it is when they exist that this
drag should be specially used, though at times it has also a
favourable effect upon some of the other symptoms which occur
in tabes,

Tn the employment of this remedy, however, certain se
cautions must be taken, and, as-you are aware, gentlemen, the
ingestion of ergot of rye in too large doses, or continued for too.
long a time, may produce the most unhappy effects; gangrene
of a limb has been actually known to be produced by such
administration of the drug. Ergot of rye should therefore be
given as Charcot recommends, in moderate doses, and the
patient should only be exposed to its effects daring a short
period of time ; thus, for example, 2 or 3 doses of 30 centigrams
(4 grs.) of the powder of ergot of rye should be given upon each
of the first 8 days of the week during a month or 6 weeks. The
action of the ergot will be thus obtained without fear of the
dangers which I have just mentioned.

‘The second or recent therapeutic phase is that in which, under
the impulse of Fournier's discovery, a form of treatment based
upon the etiology of the disease was instituted, a form in which,
in one word, the anti-syphilitic remedies were employed. I
cannot certainly, gentlemen, be suspected of treachery after
haying declared myself as T have done, an absolute believer in
the syphilitic nature of tabes; but at the same time it mast be

‘TARES DORSUALIS. 317

patients suffering from tabes in whom the specific treatment, far
from being useful or inactive, is absolutely injurious.

B. External vemedies comprise a large number of agents,
among which I should mention the following :—

(a) Counter irritation through the skin,—Setons, the oaulery
and most forms of counter irritation have been almost entirely
abandoned. One form, however, must be excepted which has
justly been and is still held in great esteem. I mean the
‘application of heated points which Charcot has always strongly
recommended. These points should be applied, not over the
spinal processes, but over the lamin of the vertebre; they
should be numerous, yery superficially placed, and re-applicd
about once a week.

(b) Electricity will consist either in the form of the firadic
ewrrent applied to the skin, a plan which Rumpf states that: he
employed with real success, or of the voltaic current applied to
the vertebral column either in the stable, weak or combined
form, or at first stable and then weak, associating with it, if need
he, as Erb recommends, galvanism of the peripheral nerves.

(c) Massage and electro-massage may produce’some effect, but
only upon certain special symptoms,

(d) Nerve-stretching—If I speak of this process it is only
from a historical point of view, since it is now entirely abandoned ;
the process consisted in dividing the skin and soft parts of the
gluteal region or thigh until the trank of the sciatic nerve was
‘reached; more or less violent efferent traction (stretching) was
then exercised upon this nerve. This plan, if Tam not mis-
taken, had been previously employed by Niissbaum in a patient
suffering from obstinate neuralgia (and who in short was hysteri-
eal, and hence the brilliant success), Langenbuch applied the
game process to tabes; if, however, some success was obtained in
connection with the lightning pains, such complications were
produced (paralysis of the lower extremity, death from menin-
gitis, hemsto-myelia, shock, &c.) that it was soon abandoned.

(e) Suspension —It is not necessary, gentlemen, that I should
speak at much length about this mode of treatment; you all
know it, and I need merely refer those amongst you who wish
for a detailed account to the numerous reviews and articles
which have been published on this subject in the Progrés Médical
in 1889 and 1890. Ineed merely remind you that suspension

TAKES DORSUALIS. : 319

cases of tabes? Considering the terrible influence which they
have upon these patients, I should recommend you, gentlemen,
to resist the entreaties of your patients as long as possible,
otherwise you will undoubtedly cause them to be inveterate
morphine maniacs. The injection of morphine is only excusable
in cases in which the pain is really terrible, or only occurs in
paroxysms (the gastric crises for example), it being then possible
only to give the subcutaneous injections in quite a transitory
manner,

The lightning pains may also be treated by numerous other
external means than suspension: prolonged hot baths, douches,
friction with ether, chloroform, the different anodyne balsams,
the spray of ether or chloride of methyl, &e.

As regards the different visceral crises, they should be treated,
as I have already said, by injections of morphine or other nar-
coties, by ice, by the different counter-irritants, and in some
cases (the gastric crises) by the application of heated points or a
Blister to the seat of pain (Charcot),

When once the best treatment to be applied to the symptom .
has been settled, the disease and the patient may be considered.
As regards the disease, you know my opinion as to the necessity
of anti-syphilitie treatment. Should the state of health permit
it, a trial of specific remedies is, as I think, fully authorized ;
you will then prescribe mercury, administered by means of fric-
tion, during three weeks, and as regards iodides, you may con-
tinue their administration during a longer period. It is fully
understood that you watch carefully the effects of this treatment,
and that should the least complication, or even indication of one,
occur these remedies are at once discontinued.

As to the patient, you will already have done much for him in
avoiding the injections of morphine. You will continue to help
him by administering tonics “and strengthening remedies in
order to delay as long as possible the “consumption.” of which
Thave already spoken upon several occasions. Is it advisable,
as some authors, and specially Weir-Mitchell, recommend, to
give complete rest to patients suffering from tabes, and even to
keep them in bed? This is not my opinion, and, on the con-
trary, they should not, as I think, forget how to walk. If
required, artificial means should Le used, and a go-cart, used for
teveral years at the Hospital of Biodtre in order to enable

321

LECTURE XXVIL
TABES (continued),

PATHOLOGICAL ANATOMY,

spongy zone of the golatinoas substance, and (#) the typical gelatinous aub-

stance of Rolando. Spongy substance with its two zones, the anterior and

the posterior. C. ANATOMY OF THE COLUMN OF CLARKE. Its seat, limits,

ay Within this tract are found: (a) noreo cola; (5) a eotwork
nerve fibres,

whose opinion is formed upon this point, and who recognize in
the “primary sclerosis of the posterior columns” a condition
which is necessary and sufficient to produce the lesions of tabes.
If some of you gentlemen are incloded in this number, I hope
that the sight of our unceasing hesitation, and doubt, will not
trouble a placidity which one cannot too much envy.

The changes in the spinal cord specially affecting the tracts
in the posterior colunms and posterior cornu of the spinal cord
should, on account of my engagements, be described to you at
the commencement of this discussion, and the principal know
ledge which has been acquired as to the anatomy of these
different, tracts,

A. ANATOMY OF THE POSTERIOR COLUMN.—We have already
considered the anatomy of this part as respects the origin of
the fibres which compose it, while discussing the ascending

21

TABES DORSUALIS. 328

developed, are those marked by the letters A, D, and G, in
figare 164; on the contrary, in the other parts the colour was
much more marked, and consequently the myelin in far larger
quantity.

Tn G, the fibres of the auenes Goll, as you already know,
will be developed, and which as you are aware, after a very long
conrse along the posterior fissure, join the grey substance of the
nuclei called “ those of the column of Goll.”

The lettters A, D, represent the postaro-internal root zone ;
you will observe, gentlemen, that in the lambar region this
zone is divided into two parts by, as it were, a spur coming
from the part marked B, of which the most internal and largest
seems to be a projection from Goll’s column; according to
Flechsig, this is by no means the cass, the segment being in
no way connected with the fibres of that colamn.

As regards the other regions, the myelin does not develop
simultaneously in them, so that the tracts can be distinguished
from each other by means of the difference in the time of their
development.

A number of fibres first appears close to the posterior com-
missure (specially in its lateral part) and the posterior cornu
almost throughout its whole extent; it is to these fibres that
Flechsig gives the name of anterior root sone of the
columns. We have elsewhere had oceasion to speak of the fibres
contained in this region, at the edge of the posterior commissure,
while discussing secondary degeneration, and I have shown you,
gentlemen, that a certain number of these seemed to be com-
missural fibres, which join the grey substance existing at-
different heights; hence their name of cornw-commissural zone.

The part seated between the anterior root zone of the
posterior columns and the postero-internal root-zone has re-
ceived the name of middle root-zone (B, E). Flechsig observes
that all the fibres contained in this zone are not developed at
the same moment; from this point of view two systems can
be distinguished = "the Gibres of tho first syslem of the midille
voot-zone develop cotemporaneously with those of the middle
zone ; while the fibres of the second system of the middle root-
zone develop cotemporaneously with those of the column of Goll.

At the inner part of the posterior column, immediately
adjoining the posterior fissure, is the middle zone F, C, which

‘TABES DORSUALIS. 325

If we recapitulate and consider the series of these different
tracts in connection with the time of their development we
find that the sheath of myelin first appears in the anterior root-
zone, then in the first system of the middle root-zone R, at the
same time as in the middle zone; the colamns of Goll come
next in order with the second system of the middle root-zone, a8
also with the postero-internal root-zone.

Such is the anatomy of these different tracts. However
interesting the study may be, it would not be complete without
inquiry being made as to the origin and termination of the
fibres by which the tracts are constituted. In regard to these
points the opinion of Flechsig will be given.

‘The anterior root-zone receives a large number of its fibres
dircetly from the posterior roots. After a course which varies
in length these fibres pass into the posterior cornua, becoming
lost in their anterior portion. These fibres do not join Clarke's
column, with which they are totally unconnected. On the other
hand, as I have just reminded you, there are reasons for thinking
that this zone contains a certain number of commissural fibres,
which connect the grey substance which exists at different levels
in the cord.

‘The fibres of the jirst system of the middle root-zone all issue
from fibres of the posterior roots, and after a course, which is
usually somewhat short, in the posterior columns, pass into the
reticulum of Clarke's column; in the regions in which the latter
have no clear existence these fibres pass with the posterior cornua
between the edges of the anterior cornua.

‘The fibres of the second system of the middle root-zone in the
lumbar region of the spinal cord are the origin of the fasciculi of
fibres which at a higher level join together to form the columns
of Goll.

As regards the middle zone no information seems to have been
given in connection with them, and Flechsig himself states that
he can give no positive opinion upon this point.

Such are the different tracts which constitute the posterior
column. It must be well understood, gentlemen, that their
limits and differentiation are not always so clear in reality asa
description such as the one which I have jast given you would
indicate.

(b) Zone of Lissaver—Marginal zone (Lissauer), postero-ex-

TARES DORSUALIS. : 327

may not be more than a fifth or even a tenth part of that of the
external segment, has, on the other hand, a triangular form and
penetrates like a wedge between the horizontal fibres of the

Fig, 165.—Seheme of the different tracts and fibres of which the posterior cornu
is composed (lumbar region), after Lissauer, A.—Antorior zone of the «pongy
substance. B.—Posterior zone of the spongy enbstance. C.—Fascieuli of
lange fibres ascending in columns, D.—Golatinous substance, E,—Pibres
insinuating themselves between the spongy sone of the gelatinous substance
and the fibrea of the Jateral column. F.—Spongy zone of the gelatinous
snbatance, G.—Fibres of the lateral column adjoining the external margin of
‘the posterior cornu. H.—Externa! marginal zone (ezternal segment of the sone
of Lissauer), 1.—Small root-fibres passing into the zone of Lissauer. K.—Pos-
terior root-fibres. L,,—Internal marginal zone (internal segment of the cone af
Jisauer, M.—Fibres of the posterior column. N.—Pascienli of transverse
fibres becoming mixed with the posterior root-fibres at the moment of their

with a direct passage into the gelatinous substance. P.—Eadiating fibres of
‘the posterior column.

7

'TARES DORSUALIS, 829

it is in the lumbar region that it is most developed; its smallest
size is in the dorsal region, and it becomes slightly more
developed in the cervical region.

From this deseription, gentlemen, you will be able to under-
stand how it is that the small fibres which constitute the zone of
Lissauer, although having the same origin (the posterior roots),
as most of the fibres forming the posterior column are clearly
distinguished from them by a certain number of characters.
The study of the development of the fibres which constitute
this zone also accentuates this dissimilarity ; Flechsig has, in
fact, shown that these which in his nomenclature formed the
posterior and external root-zone receive their sheath of myelin at
a much later time than the other fibres of the posterior columns,
namely quite at the end of foetal life,

B. Axatomy or rae Posterton Horx.—Under this name
we will study the posterior horn, properly so-eclled, and the
columns of Clarke, these two parts of the grey matter in the
cord being apt to present changes in the course of tabes.

(@) Posterior horn properly so-called : the normal anatomy of
the posterior horn has been the subject of many works; it is
again from that of Lissauer that I shall borrow the principal
facts in the description which I am about to give. (In con~
nection with the whole of this description of the posterior horn
consult fig. 165).

‘The extremity or “ apex” of the posterior horn does not reach
the surface in any way as you would be tempted to think when
looking at a greater number of schematic figures which represent
the spinal cord. It is separated from it by a suhjiamaterion
layer, which in fact occupies the whole surface of the cord (vide
thé very beautiful figures of Waldeyer in his memoir upon
the spinal cord of the gorilla).

This extremity of the posterior horn is covered by the
gelatinous substance of Rolando, which is itself coated exter-
nally by a special layer termed the sfratum zonale, of which the
tissue is analogous to that of the subpiamaterian Iayer with
which in fact it is continuous.

‘The fibres of the zone of Lissauer or marginal zone are found
to be entirely contained in the layer beneath the pia-mater
through which they must consequently pass in order to reach
the posterior horn.

|

‘TABES DONSUALIS, ASL
‘This layer is the spongy sulstance properly so-called in which
Lissauer also distinguishes two zones :

a. Posterior cone; this zone consists chiefly of the longi-
tudinal fibres, some of which are small and belong in great part
to the tract of the posterior root-fibres, while others are large
and form a small number of fasciculi, seaming to be the direct
continuation of the posterior roots,

B. Anterior cone; this differs from the preceding zone in
consisting specially of fibres placed in a transverse direction.

According to the recent works of anatomists who have made
use of the colouring process of Golgi: a larye number of fibres
and nervous collaterals terminate in the spongy substance,
specially between it and the gelatinous substance, some ending
in the latter part ; it is the passage of these fibres and collaterals
“through the gelatinous substance which causes this to have a
striated aspect ; lastly, some of these fibres pass through the
posterior commissure and end in the gelatinous substance of the
posterior horn on the opposite side.

QO. Asaromy or ‘THE CoLuMN oF CLarKe—These columns,
notwithstanding some claims as to their having been first
described in Germany (Waldeyer), would more rightly be called
columns of Stilling-Clarke. Stilling, in fact, mentioned these
groups of cells as early as in 1843, whilst the description of
Lockhart-Clarke was only written in 1851; it should be added,
however, that the latter is far the most complete both as regards
the columns themeelves, their seat, and their path.

These columns are, as you know, gentlemen, seated in the
neck of the posterior horn, at its internal part, distinctly behind
4 transverse line passing through the posterior commissure, at
any rate in the lower regions of the spinal cord, since at
a slightly higher level these columns tend to pass more
anteriorly.

They are not fouud in any other part of the spinal cord, but,
according to the generally received opinion, only between the
upper portion of the lumbar and that of the dorsal region. On
the other hand Tooth, and I am much inclined to share his
opinion, believes that the columns of Clarke only extend from
the second lumbar to the eighth dorsal pair of nerves; at this
level they cease to exist as columns, and between this point and
the level of the second or first, dorsal pair are only represented

Clarke. Waldeyer also thinks that the columns of C)
exist in the dorsal region of the cord alone, but throngho
whole length of the cord without exception ; Dat that in
parts than the dorsal region it is not a large mass orn
that is found, but simply a rou 0€ tw, three OR
cells which so strongly resemble those of the columns of
in thets form and seat that they must be considered! cf thimaaias
nature. |

In these columns we must, while considering how tha peal |
logical anatomy can be applied, specially consider two elements:
(a) the cells, (b) the nervous reticulum.

(a) Ganglion cells of the columns of Clarke.—These are large
cells which are consequently visible by means of a small magni-
fying power somewhat resembling in this respect the large cells
of the anterior horns. They differ, however, considerably from
the latter, since they are of smaller size, and present a much
smaller number of processes ; only one or two lateral processes:
can in fact be recognized to exist. ‘According to te isa
of Obersteiner, both the upper and lower poles of these cells (in
form they are elongated in the vertical sense) have a process
which constantly exists; as, however, these processes form
suddenly they are not fusiform in shape, and the body of the
cell preserving its rounded outline they contain a large and
well-marked nucleus and much pigment. In all probability it
is from these cells that the fibres which constitute the direct
cerebellar tract take their origin, to which they probably act as
4 trophic centre, having the same office perhaps in regard to
some fibres of the column of Gowers. |

(8) Reticulum of the columns of Clavke.—The knowledge of
this reticulum is as you will see, gentlemen, very i
the study of the lesions which occur in tabes, In the normal
cord coloured by the hematoxylin of Weigert the general tint
of the section through the columns of Clarke is almost analogous
exception being made of the brownish- yellow points repre~
‘the nerve-cells) to that of the remaining grey substance

= a

TABES DORSUALIS. 333

of the anterior and posterior horns. It is somewhat dark on
account of the presence of a thick network of nerve-fibres con-
taining myelin, and it is only through this bluish network that
some yellowish points or lines can be seen by transparence
indicating the cells and position of the neuroglia. The nerve-
fibres of this reticulum are chiefly afferent fibres coming from
the posterior roots. At the present time I merely indicate these
facts, to which I shall return when speaking of the pathological
anatomy of tabes.

TALES DORSUALIS, ‘ 335,

Pierret that in this form of sclerosis the external bandlets were
Jirst agfected, and that the morbid process was first seated in the
region of these bandlets. This, in my opinion, is an important,
discovery, not only because it has met with general agreement
but because it algo indicates the way in which researches should
be made in future with regard to the nature of tabid lesions.

I will commence, gentlemen, as I have already said, by stating
the facts, after which the opinions which exist will be con-
sidered. ‘

A. Spixat conp.—A certain number of changes in this part
can be perceived even with the naked eye. Often, in fact. the
cord is diminished somewhat in size, specially when the lesions
are very pronounced, It is sometimes also slightly thickened,
and the pia-mater is more or less opaque at the level of the
posterior columns. Sometimes, again, when the membranes
have been removed, these columns will be specially observed on
account of their grey hue, which has the appearance of a long
ribbon which extends over the whole length of the cord. In a
section it is equally observed that this grey band encroaches
upon the posterior columns, into the interior of which it extends
more or less deeply, This, gentlemen, is what is observed, as I
have just said, when the lesions are very pronounced ; when
they are but slightly marked the diagnoses as connected with
the pathological anatomy of the disease can only be made by
means of the microscope.

In order to understand thoroughly the microscopical appear-
ance of the spinal cord in tabes it should be studied in two
different stages of the affection ; that of the onset, in fact, alone
enables the seat of the fundamental lesions to be explained,
since at a later period, when the whole posterior column is
affected, no anatomical dissociation can possibly he made.

(a) Av THE onsET—When by means of a moderate mag-
nifying power one observes the posterior columns, it is remarked
that at certain points in them the nerve-fibres are far less
numerous, while the tissue by which they are supported is much
more abundant ; a short examination is sufficient to show that
these changes ave really due to the formation of trae zones of
sclerosis,

Posrerton CoLumMN.—lIts different segments should be con-
sidered separately : S j ame

336 LECTURES ON DISEASES OF THE SPINAL CORD.

a. The external bandlets, that is to say the part of t
posterior column which adjoins the posterior horn upon each
side, not throughout its whole extent but specially in its middle |

Fig. 166 —Section of the spinal cord in the lumbar region from a caso of tabos at
the onset. (The white parte are affected by sclerosis.) Tho change in the
zones of Lissauer at the extremity of the posterior horns, and the seat of the
selorosis in tho posterior column will be observed ; thia extends from the part
immediately adjoining tho posterior horne (external bandlets properly so-
called), and spreading transversely an far as the posterior fissure, occupies the
whole thicknoes of the posterior column,

and posterior thirds, and at which level numerous expansions of
the nervous tissue are seen to pass into the middle part of the
posterior horn. The form and size of these external bandlets
varies somewhat according to the part of the cord at which they
are observed, and I cannot now enumerate the varieties which
exist; one thing, however, is certain, namely, that in all or
almost all the autopsies of tabid patients, even at the onset of
the disease, the external bandlets are affected. It should be
observed, gentlemen, that they are not necessarily involved
throughout the whole length of the cord, but may be almost or
wholly unaffected in the cervical, though much changed im the
lumbar region. Sometimes, however, they are affected not only
in the lumbar, but also in the dorsal region, as well as in the
cervical enlargement ; above this point they are usually in a
healthy condition:

8, The column of Goll—This column is in most cases already

‘TABES DORSUALIS. 337

affected, but often to quite a slight degree, both as regards the
intensity and extent of the lesions. Why they are seated in

onset. ‘The intensity of the lesion is proportionate to the light colour of the
diagram. The column of Goll is affected: the column of Burdach on the other
hand is free from disease except at its posterior part; the lesions are most
Pronounced in tho external bandlets, properly so-called.

this part is not yet understood, and future researches will be
required to explain the fact.

ry. The vest of the column of Burdaeh—The extent to which
sclerosis occurs in this part increases proportionately to the
duration of the disease; in addition to this the islets become
more apparent when the external bandlets of the subjacent
segment of the spinal cord are much altered. The exact position
of the lesions in this tract are, however, quite inexplicable to us
in the present state of our knowledge; thas I prefer merely
patting before you sketches which faithfully represent the
appearance of the posterior columns in cases of tabes which are
not yet in an advanced stage.

6. Zone of Lissaver—As you know, gentlemen, this zone is
seated between the apex of the posterior horn and the surface of
the cord, and is composed of a mass of narrow nerve-fibres. In
tabes this zone, as Lissauer has shown, is much altered; all or
almost all the small nerve-fibres which constitute it entirely
disappear. Thus, in preparations coloured by the hematoxylin

of Weigert, this region is found to have a special appearance,
BB)

|

338 LECTURES ON DISEASES OF THE SPINAL CORD.

seeming to be quite denuded, Tt is this appearance which has
induced some authors to deseribe sclerosis of the postero-
internal part of the lateral column as frequently co-existing: in

Fig. 168,—Soction of the spinal cord (corvies! enlargement) from & case of tabes:
at the onact. The column of Goll ix affected throughout ite whole Tength.
The middle zone of the column of Burdach is also involved, a» well as the
external bandlet properly so-called, The parts are affected in proportion to
the lightness of their colour.

tabes. In reality the lateral column is in no way affected in
these cases, the zone of Lissauer is alone changed, which, as I
have already aid, belongs entirely to the posterior root-zone. 1
should add that degeneration of the zone of Lissauer is one of
the early changes in tabes; the examination of the lesions at
this point, which is by no means difficult, being thus interesting
in every respect.

(b) Ar AN ADVANCED STAGE.—At this period again the appear-
ance of the lesions presents great variations since the extent of
the disease differs to an unlimited extent; I shall therefore but
roughly indicate the changes which exist, feeling sure that the
figures which I shall place before you will explain more than
the most detailed description.

1, Posrerton coLuMN.—In very advanced cases nearly all
this column may be affected. It then presents a singular
Appearance, specially in the dorsal region, where, on account
of participation of the zone of Lissauer, the part affected by
Sclerosis extends beyond the posterior horn and, assuming the
form of a “crescent,” appears to extend at the expense of the
lateral column. The external bundlets ave, as you would expect,

TABES DORSUALIS.. 339

completely degenerated, at any rate at the level ofthe lumbar and —
cervical enlargements, and in a large part of the dorsal region,

A

Fig. 169.—Section of the spinal cord in n case of tabox. The postorior column ix
affected throughout its whole extent, with the exception of a few healthy
fibres which are completely ixolated, and of which the number in «lightly
greater in tho anterior portion adjoining the posterior commissure. The oxten-
sion of the islet of sclerosis in the form of a fan will be specially observed,
This appearance is due to the fact that the marginal zone of Lissauer is
involved. It will be understood that on account of the occurrence of such
cases as these the lesion has been supposed to extend from the posterior to the
\nteral columns,

The column of Goll is usually much affected. In some cases the
lesions are found to be more pronounced in the cervical region
than in other parts of the posterior column. In these cases the

Fig. 170.—Spinal cord from the cervical region in a case of taben (the parts light
in colour are affected by sclerosis). The fibres B (commissnral?) should be
observed seated in the anterior part of the posterior column ; extension of the
lesions in the posterior column in the form of # fan on account of the zone of
Lissauer being involved.

whole of the column of Goll is affected; in others, a part only of
this column is involved.

oh LECTCEES ON DISEASES OF THE SPINAL CORD.

Lastly, Strampell has shown that s small fusiform tract of
fibres is not rarely seen in a healthy condition at the junction of

Fig. 171.—Spinal cord in the lumbar region from = caso of tabes (the parts of =
light colour are affected by acleroris). The (commissural f) Sbres (C) seated
the anterior part of the posterior column are sound. A small part (A)
posterior column upon eseh side of the posterior Sasuro is aleo sound. The
unaffected condition of thie «mall part mentioned by Strimpell is mot infre-
quent in the lumbar region of the mpinal cord it: tabes.

g
E

Fig. 172.—Section of the spinal cord in the lumbar region from a case of tabes
(the parte of a light colour are affected by sclerosis). The (commierural
flores (B) seated in the anterior part of the posterior column are wound, It will
be remarked that the sclerosis extends into the white columns at the extremity
‘of the posterior horn, the «mall quadrilatoral which ix thus formed, and which
‘one »ees to be marked by «mall points, is the marginal zone of Liseauer affected
by the degeneration; its colour abould be lexs dark.

the posterior with the middle third of the posterior fissure. This
small band of fibres corresponds to the middle zone of Plechsig.

TABES DORSUALIS. Sil

As regards the rest of the column of Burdach it is in the
middle part of thie tract that the lesions are usually most pro-
nounced, while certam parts of the column, as Striimpell has
very truly observed, are only affected by degeneration at quite a
late period of the disease. These are, on the one hand, the
anterior part of the posterior colamn which adjoins the posterior

'3.— Section of the cord in the cervical region from a case of tabes, Almost
the whole of the posterior column is affected with the exception of a fow Sbrex
at its posterior and external angle, Which aro sound, as are also the fibres
reated behind the posterior commissure and along the base of tho posterior
cornu, the cornu-commissural zone. It will be also observed that lines of
inolated fibros extend from the posterior commicaure along the posterior fissure
which they then quit in order to pase to the surface of the cord. Those ixolated
fibres aro the last traces of tho healthy tracts which are found at this point in
aloes advanced stage of the diseaxo. Compare fig. 101 with the above figure,

commissure, and the most anterior part of the base of the pos-
terior horn"; on the other hand, the postero-external angle of
the posterior column adjoining the posterior cornu. The healthy
condition of the latter part is often very marked in sections of
the cervical region. It should be observed, however, that this
condition does not persist so long in that part as in the anterior
portion.

* We have already seen, in connection with the secondary degeneration asso-
ciated with transverse lesions of tho cord, that a certain nambor of commissaral
fibres exist in this portion of the posterior column. It is probably these fibres
whieh remain for a timo wnaffected in tabes, the so-called corna-commissural
zone,

7

When almost the whole of the posterior column is affected by
the sclerotic lesions the posterior fissure not infrequently dis-
appears as if the two halves of the cord were completely joined
together.

The changes which I have just mentioned are usually identical
in the posterior columns upon both sides. At the same time,
there is in some cases obvious asymmetry in the islets of sclerosis
as in the example which I now place before you.

From a histological point of view the lesions of the posterior
columns are as follows: the meshes of connective tissue are much
more extensive and contain a larger number of nuclei than in
the normal condition. The number of nerve-/ihres surrounded

342 LECTURES ON DISEASES OF THE SPINAL CORD.

Fig, 174.—Section of the cervical region in the cervical cord from a case of taber,
‘This figure shows that some asymmotry may exist im the lesions of the
torior columns. Upon the right side, in fact in tho angle formed by the janetion
of the posterior and oxtornal margins of the posterior column, a patch of sound
tissao in found which does not exist upon the loft wide,

by this tissue is much diminished, and this diminution seems to
me to occur specially and primarily in the fibres of small size
which normally exist in the posterior columns. We have our
selves noticed, in fact, that in very pronounced cases the nerve-
fibres of the posterior column may almost entirely disappear, It
fis uausl to describe an abundance of granular bodies in the pos
terior columns. 1 must confess that for my part, althongh they
Leon most carefully sought, I have been rarely able to find
bodies in the posterior columns of those who have
‘from tabes. On the other hand, I have recognized the
Sof these elements both in cases in which the lesion was

yi

‘ FARES DORSALIS. Bas

commencing to exist and in those in which it was in an advanced
condition. In this point I therefors completely disagree

most of those who study pathological anatomy. As regards |
blood-vessels, their walls are much thickened at the spots in
which they are surrounded by a fibrous zone, but only at these
points.

Thave already said a few words about the meningeal changes
and the opacity of the pin-mater in the posterior columns. It
seems that the degree of meningeal change corresponds more or
less with that of the sclerosis in the cord, but the cause of this
thickening of the membrane or, as it may be said, of this chronic
meningitis is unknown.

2. Grey substance-—The changes which occur in the grey
matter during the course of tabes have been frequently studied,
and are the subject of some special works, specially by Pierret.
The knowledge which we have upon this subject, however, is still
by no means complete.

a. Anterior hora.—I spoke of the lesions which may be found
in this part in a previous lectary, when discussing the different,
forms of amyotrophy which occur in tabes; it is unnecessary to
say more about them, and other parts of the grey matter will be
now considered.

8. Columns of Clarke,—The changes in this part, though
mentioned by several authors, have only been really described
in 1885 by Lissauer. Their character is as follows:—As you
know, gentlemen, ganglion-cells on the one hand are distin-
guished in the columns of Clarke, and on the other a reticulum
of narrow nerve-fibres, The reticulam is alone affected in
tabes, the cells remaining free from disease, in such a way that
the latter, in sections coloured by the hematoxylin of Weigert,
instead of being placed, as in the normal condition, upon a blue
ground, are found to be contained within a yellowish disc, which
contrasts strikingly with the rest of the grey matter. This
yellowish dise is merely the part occupied by the column of
Clarke, of which the nerve-fibres which form the reticulum have
lost their myelin and degenerated. Lissauer has remarked that
at the time of the onset of tabes this degeneration of the fibres
of the reticulum is much more pronounced upon the inner than
upon the outer side of the colunms of Clarke; he explains this
difference in the intensity of the lesion by the fact that the

TABES DORSUALIS, 345

which come from the cord at a higher level join the external
portion. This healthy condition of the cells in Clarke’s column
in tabes should be joined with the corresponding state of the
direct cerebellar tracts, of which these cells are, as you know,
the trophic centres. In the cases, which, however, are very rare,

Fig. 177.—Section of the spinal cord (lowor dorsal region) from a caso of tabes.
‘The whole of the posterior column is affected by sclerosis, ‘Tho appoarance of
the columns of Clarke will be observed: their aspect is quite clear on account
of the disppearance of tho reticulum of norvo-fibres which exists in tho
healthy cord ; the number of ganglion-cells hus undergone no diminution.

in which the direct cerebellar tracts are affected (Jendrassik),
their degeneration would perhaps be due to the fact that the
cells in the column of Clarke are, by rare exception, involved.

ry. Posterior horn.—One would, 4 priori, expect that the grey
matter of the posterior horn must inevitably be involved in the
more or less pronounced lesions which occur in tabes, so intimate
are the connections which exist between this part and the tracts
of the posterior columns. Such is the opinion maintained by
Lockhart, Clarke, Leyden, Pierret, &c., whilst Vulpian is more
doubtful, and even considers that the lesions in the posterior
horn are of exceptional occurrence,

a

346 LECTURES ON DISEASES OF THE STINAL CORD,

Tn his work upon the condition of the posterior horn in tabes
Lissauer analyses in a more exhaustive manner than
been done the lesions which may ba met with in tabes. Amongst
other things he mentions the disappearance of a large number of
the small fibres seated in the posterior cone of the spongy substance,
as alzo of the radiating jibres which come from the posterior column.
As regards the cells which are special to the posterior horn, no
precise information has been given upon this point.

6. Centval canal.—Its condition varies; not infrequently the
cells of the ependyma in it are in such abundance as to obstruct
the canal. In some cases considerable dilatation of the central
canal coincides with pronounced lesions in the posterior horns.
Whether this is a fortuitous occurrence in the course of tabes
or an exceptional form of syringomyelia I am unable to
say.

4 ef tho medulla oblongata ina case of
ithe white colour in the posterior columns indicates the parts affected by
» Lesions in the column of Goll, B. Losions in the columns of
wpa pyramids, D. Traces of the anterior horn.

La l*

my remarks upon the pathological anatomy
with the spinal cord, I would observe that

he

. —

‘TABES DORSUALIS. ‘B47

the latter may be affected throughout its whole length, from. and
inclusive of the filum terminale to the medulla oblongata and
even higher, The study of the lesions which oceur in the bulb
in tabes is not within the scope of these lectures, besides which
I would remind you that in another lecture I stated the facts
which it was necessary to know in connection with the laryn-
geal crises and lingual hemiatrophy. I would only add, in order
to terminate what is directly connected with the pathology of
the spinal cord, that the changes in the colamn of Goll can be
very clearly traced almost as far as the nucleus of that tract,
while thoxe of the column of Bardach can be similarly traced
almost as far as the nucleus of the same name.

B. Posterion noots.—The atrophy of the posterior roots
which oceurs in tabes is 20 prominent a symptom that it has
been recognized to occur from the very earliest time at which
the disease was studied. Leyden is one of the authors who have
most specially insisted upon the existence of this lesion in the
roots of the spinal nerves, and is one of the few who have
believed it to explain the origin of the changes in the spinal
cond which ocenr in tabes. The roots of the canda equina and
lumbar enlargement specially suffer from this atrophy, which is
also observed at ‘the upper part of the spinal cord in which
the roots connected with the cervical enlargement are chiefly
affected.

Under the microscope the number of nerve-fibres in the roots
which thus suffer from atrophy is found to be very consider-
ably diminished, On the other hand, the number of small
nerve-fibres appears to be sometimes increased, though T am
unable to say whether this increase really exists or is simply
relative,

Tt should be observed, gentlemen, that the cranial nerves,
which for the most part (with the exception, certainly, of
the optic ond olfactory nerves) are, after leaving the medulla
oblongata, but posterior roots, act in precisely the same way
as the roots of the spinal nerves and are linble to analogous
changes.

C. GaxGLia or THE sPIvAL NERVES.—The changes in these
ganglia have been mentioned by a large number of authors who
are by no means agreed as to their nature; no methodical de-
scription whatever of the lesions which occur has been written.

TABES DORSUALIS. 349

general paralysis of the insane, was recognized by Jendrassik in
two cases of tabes. In these the posterior and inferior convolu-
tions, however, were affected, whereas in general paralysis it is
usually the convolutions of the frontal lobe which are specially
involved. I shall return to this subject in my next lecture,
when I shall put fresh details before you.

i

TABES DORSUALIS. ~ 3a

symptoms of that affectiow are directly due to these cerebral
lesions ; this author in fact is much inclined to consider the

Fig. 179.—Scction of the second frontal convolution (normal) made at right
angles to its surface, (After Jondrinsik.) The free surface of this convo.
Intion is at the upper part, while the lower part of tho aootion (E) is continuona
with the corona radiata, Tho vertical lines B represent the fasciculi of fibres
which, in the healthy convolution», contribute to form the rays of the corena
radiata.

changes which occur in the spinal cord to be due to secondary
degeneration caused by the lesions of the brain.

It is not unreasonable, gentlemen, that you hear this opinion
for the first time with o feeling of surprise, so much does it
differ from the views which are generally held. At the same
time it should not be rejected without examination, since though
even an observer of Jendrassik’s importance may make a
mistake, his error will certainly be associated with a large
amount of truth. As I had occasion to remark in the preceding
lecture, these cerebral lesions certainly exist in a large number
of patients snffering from tabes, even when there is no indi-
cation of general paralysis. I am unable to say that they

cil maple line represents the surface of the convo
bodies im somewhat large number. Tho fascicnli of n
affected convolution are far less numerous, Pe

‘The fact, however, is in itself true; as to its in
cannot accept that which is proposed by Ji
is specially based upon physiology, a frail science.
lesions which he regards as the cause of scle
have in my opinion no direct connection with
volations suffer of their own accord in the same way
cases a portion of the crus cerebri, pons

TABES DORSU ALIS.

independently of any destructive action it may have upon the
posterior colamns of the spinal cord.

If the symptoms due to changes in the cortex of the brain
occur after the onset of tabes, general paralysis is said to have
supervened in the course of tabes, if not, the reverse is said to
have occurred, Tt is unknown at the same time why in certain
eases general paralysis or tabes develop, either separately or
conjointly, and why it is sometimes one and sometimes the
other of these affections which first occurs; we are quite as
ignorant of the reason why optic. neuritis exists in some
patients suffering from this disease, whereas in others it never
occurs.

Certain alterations in the SYMPATHETIC nerve have been sup-
posed by some authors to be the cause of the disease ; Duchenne
of Boulogne thought that the derangements were chiefly fanc-
tional. MM, Raymond and Arthaud have been able to show,
however, that organic lesions undoubtedly exist in that nerve.

In the opinion of Ordofiez, Adamkiewicz,", Buzzard, &e., the
sclerosis which exists in the posterior columns of the spinal
cord is of vascular origin; the blood vessels contained in these
columns, which supply them with blood, become the seat of
more or less prononneed changes, and it is, in their opinion,
consecutively to these alterations that sclerosis occurs in that
part. This theory was for a certain time somewhat generally
accepted; it seems reasonable, and, as I shall have occasion to
show you, is well adapted to explain the lesions which exist:
in cases of diffuse combined sclerosis. When, however, an
affection so clearly “systematic” as tabes is concerned, such
an explanation seems to me impossible. How can it be sup-
posed, in fact, that a disease in which the lesions are so small,
and have such a definite seat, can be due to such a widely
spread, I would almost say blind process, as that of perivascular
inflammation ?

The same arguments may be used in connection with those
authors who, on account of the fact that the pia mater is found
to be thickened in some cases of tabes, believe posterior meningitis
to be responsible for the existence of the lesions which occur in
tabes,

We will now consider the theory according to which tabes is
* This author, howover, admits that sclocosis may be dae to other cannes,
23

> ry
Fig. 182.

TABES DORSUALIS. 855

learnt to know by the names of middle root zone and median
zone, the development of these two zones in the embryo being
synchronous; the next lesions which occur in tabes are in the
zone of Lissauer and columns of Goll, then in the pocterior and
infernal root zone, and only at the last period of the disease in
the anterior root zone.

I believe, gentlemen, . Shaluasinuaaaeces eawlodaiat ae
present time it is impossible lo admit that prinury aclerosia of ane
or other column of the cord can possibly exist. In order that a
nerve fibre may degenerate, it mast either be separated from
the cell from which it takes origin. and which is its trophie
centre, or the cell from which it comes must itself disappear.
It would be paying far too much honour to these organs, which
are simply condactors (nerves or columns of the cord), to attri-
bute to them such an independent existence that they can suffer
from primary lesions. No, gentlemen, every systematic altera-
tion in the columns of the cord is but a secondary change; in
all degenerations of a nervous tract, whether it is in the cord or
has a peripheral seat, the cell in which disense exists must be
specially sought.

Let us endeavour to apply these facts to a case of tabes.
What, in that affection, is the system of fibres in the spinal cord
in which the changes specially occur? It is the posterior column.
Which, then, are the cells from which the fibres of the posterior
column take origin, and do any reasone exist for supposing that
these cells are diseased ?

I have already, gentlemen, had occasion to discuss in some
detail, in connection with secondary ascending degeneration,
the origin of the fibres which constitute the posterior column.
Allow me to remind you, in a few words, of the principal facts
observed.

The posterior columns, we observed, do not at first form a
definite part of the cord, at least during the earliest period of
embryonic life, It originates in the lateral neural ae and
only penetrates in a secondary manner into the cord by the
medium of the posterior roots, which, budding from without
inwards, penetrate into the eikconioh tha ayeeal Gola
fibres during this course are disposed in a direction parallel with

TABES DORSUALIS.

much more clear; these animals are certainly far removed from
us: the amphioxus, lumbricus,” &c.

that if a nerve is divided below its spinal ganglion degeneration
occurs in a certain number of fibres in that ganglion, and in the
posterior root connected with it. If the results of these experi-
ments were conceded by all those interested in pathological
anatomy the truth of my supposition might be looked upon as
at once demonstrated. I must admit, however, gentlemen, that
these conclusions have met with

In the third place, and I feel that this reason has a more
solid foundation, since it concemms human pathology, it has
been shown that after amputations of the thigh, as I have
explained to you in a detailed manner, and division of a mixed
nerve such as the sciatic ascending degeneration takes place in
that nerve; this is followed by obvious atrophy of the corre
sponding posterior column. We must, therefore, necessarily,
and upon that occasion I reminded you of how Friedliinder and
Krauss had made the same observation, we must necessarily, as
T said, admit that fibres exist in that nerve of which the trophic
centre is seated in some part of the amputated limb, and of
which the degeneration is consequently ascending. I would
also observe, gentlemen, that in the case of amputation of the
thigh, which I have taken as the basis of my description, the
lesions due to the degeneration of the nerve extended as far as
the spinal cord, and that in this part they were seated in the
same points as those which exist in tabes (the external bandlets,
the column of Burdach, the column of Goll).

I have, however, allowed this digression to carry me away
from the subject of the lecture, nothing being so complicated as
the explanation of a hypothesis.

We were saying, gentlemen, that the nerve fibres contained
in the posterior roots come from two distinct trophic centres;
one constitated by the cells of the corresponding spinal ganglion,
the other by the peripheral ganglion cells.

I have just mentioned to you the argument which I thought
important as indicating change in the peripheral ganglion cells

* M. Von Lenhostek. Ursprang, Verlauf und Endigung der sensibeln Ner-

venfasern bei Lambrious, Archiv. {ir Mikreskopische Anatomie, t. XXXIX.
1892,

'

358 LECTURES ON DISEASES OF THE SPINAL CORD.

in tabes, The condition of the spinal ganglia in that disease
must now be considered,
As T have already observed to you, gentlemen, the recognition

Fig. 184. Fig, 185,
Fig. 186,

‘ig. 184—Section of the cord in the lumbar region. Fig. 185.—Seotion of the
cord im the doreal region. Fig. 186.—Section of the cord in the corvieal region.
From n caso of amputation of the left thigh It will be observed. 1, That in
the lumbar region into which the norve fibres coming from the divided perrous
parts the external bandlet the column of Goll and that of Bardach are affected:
by degonoration, on the contrary in the dorsal and cervical regions, which no

Teceive the affected fibres, the colums of Goll alone show signs of

tion. 2. On the left tide the degeneration of the nerve fibres
in tho posterior column not only of the left but also of the right

} this fact will explain the symmetry of tho lesions in tabes notwith>

the number and indopendonoe of the centres in which the degenor-

Sottnences (spinal ganglia and posterior roots).

TALES DOKSUALIS, 359

of the lesions in these organs is attended by some dil

and as, when an autopsy is made it is not habitual, to

these parts, in order that a subsequent examination may: be
made, the knowledge with respect to their lesions is but slight.
At the same time the writings of Oppenheim and Siemerling, to
speak only of recent works (observations of this kind published
more than fifteen years ago have much less value on account of
the progress which has been made in the mode of carrying on
the examination), deseribe and figure considerable lesions in
cells and fibres of the spinal ganglia in tabes. For my part
could not speak so definitely in the affirmative, or say that in
the one case in which I specially searched for them lesions
clearly existed in these nerve cells. At the same time I quite
believe that in this case the cells were less numerous than should
be the case in an unaffected ganglion, and some of them seemed to
present signs of degenerative atrophy. In the same case atrophy
clearly existed in a somewhat large number of the nerve fibres
contained in the fasciculi which pass within the spinal ganglia,
I am, therefore, convinced that changes do exist in the spinal
ganglia in all cases of tabes, and that these changes* are of the
greatest importance as regards the origin of the process of
degeneration in tabes.

It is somewhat probable, gentlemen, that these lesions in the
cells of the spinal ganglia are also the cause of the disorders
known by the name of peripheral neuritic, You know, in fact,
that these cells have a trophic effect both upon the fibres of the
posterior roots and upon those of the peripheral nerve tranks.
‘The lesions of the ganglion cells would therefore be accompanied
by degeneration in the peripheral nerve trunks. This, it seems,
should occur in tabes, and such, in my opinion, is in part the
cause of some of the changes which occur in the peripheral
nerves. This, however, would not explain all the changes
which exist, specially the fact that the small peripheral branches
are more involved than the nerve tranks themselves.

T am convinced, for my part, that the degeneration of these

* During the publication of these leetares I learn from an artisla by Detier
(Gas, bebdom de Méd., 90th Jantary, 1802) that M. Babinski also
cin hath iin tron aon hl linc th
spinal ganglia, Babinski is of opinion that a functioval derongement in the

Tesotions of the calla in those ganglis wouldbe sufficient to produes the moctia
process which occurs in tabes.

TABES DORSUALIS. 361

oecur in the fibres of the posterior roots, 1 must show you how
it is that the degeneration of these fibres which penetrate into
the cord in order to form the posterior column, produces sclerosis

Figs 188 -190,—Tke meaning will be explained directly.

TABES DORSUALIS. 363

for a certain distance in the cord, being inclined towards the
posterior fissure and then eurving outwards and passing into
the posterior horn nearly at its middle part; a certain number
of these fibres pass into the columns of Clarke. his group of
fibres is contained in the column of Burdach.

3. The group of long fibres; these fibres come specially from
the roots of the cauda equina and pass through the whole length
of the cord to join certain nuclei in the medulla oblongata;
they constitute the columns of Goll,

We will now consider how all these anatomical data accord
with the conclusions furnished by the autopsies of those who
have suffered from tabes.

In the spinal cord the initial seat of the lesions as we have
said is in the external bandlets and zone of Lissauer; this is the
more easy to understand on account of these being the points at
which the posterior roots (the short fibrés and those of moderate
length) enter the cord and pass into the grey matter of the
posterior horns. As regards the sclerosis which occurs in the
columns of Burdach, it is specially due to degeneration of the
fibres of moderate length, the disappearance of the reticulum
of nerve fibres in the columns of Clarke being also the cause,
Lastly, the sclerosis of the columns of Goll is directly due to the
degeneration which occurs in the long root fibres.

You will thus be able to completely understand, gentlemen,
how the well-known zones of sclerosis of the posterior columns’
are formed in tabes. Tt should be observed also that degenera-—
tion may occur not in the fibres connected with one or two of
the spinal ganglia alone, but perhaps in those connected with a
large number, or possibly them all. You will thus understand
why in some cases lesions occur in the external bandlets
out almost the whole length of the cord, while all the root fibres
in the upper part of the cord, the short fibres being comprised,
are affected similarly to those in the lower regions.

In other cases, on the contrary, the zone of the external
bandlets is not affected in the cervical region or even in the
upper part of the dorsal region. In these cases the seat of the
lesions is limited to the column of Goll and the middle portion
of the column of Burdach (wide fig. 191). It may then be sup-
posed, gentlemen, that the lesions of the posterior root fibres
and consequently of the corresponding spinal ganglia only

7

304 LECTURES ON DISEASES OF 'THE SPINAL CORD.

existed in the lower part of the cord (the cauda equina, lumbar,
and lower part of the dorsal region); the degeneration has
therefore only involved the upper part of the cord. the fibres of
moderate length and long fibres coming from the roots at a
lower level, that is to say, the fibres which are exclusively seated
in the column of Goll and the colanm of Burdach.

Tt may again be supposed that the groups of fibres in the
different posterior roots are not simultaneously affected, but that
the lesions commence in the group of long fibres, and in that of
the fibres of moderate length ; it will then be understood how i
sections of the cord which are made in parts where the lesioms
are by no means in an advanced stage (the dorsal or cervical
region), the changes are seated almost exclusively in the column
of Burdach, and to a slight extent in the colunm of Goll; the
external bandlets properly so-called are found to be almost

are of u lighter colour in proportion as th2y are more affected by sclerosis), If
the figure {» turned round in such a way that the posterior horus are at the
uppo: part, it is seen that the part which ix chiefly affected by eclerosis hax
clearly the form of the letter M. The band of healthy tisxne which separates
the onter from the inner stroke of the letter seoms to occupy precisely the samo
Togion ws that in which the comma-shaped degeneration of Schultze is found to
coour in ome cases of descending degeneration. The posterior part of the
column of Goll and the middle portion of the colamn of Burdach are disensod
case; the external bandlet, properly so-called, in unaffected.

- |

'TARES DORSUALIS. 865

exclusively unaffected. It is probable that the same occurs in
the cases of tabes which complicate general paralysis of the
insane, since in the diagrams which illustrate such cases and
are furnished by M. Westphal and Flechsig (figs. 181, 182, 193),
it will be observed, gentlemen, that in the sections of the cord
above the lumbar region the lesions are not seated in the
external bandlets but in the colamn of Burdach and in the
column of Goll.

‘There is then, in my opinion, one theory without which the
pathological anatomy of tabes cannot be deseribed ; this is the
important faet that the lesions of the spina! cord in tabes osewr by

=
Fig, 192.—Soction of the spinal cord (upper part of the cervical region) in tabes.
‘Ths anterior half of the column of Goll is alono affected by xolerosia in this
case; the general form of the region affected is that of a Y roversod.

segments, each posterior root’ bringing into the posterior column
a fresh contingent of degenerated fibres.* The great difference
which exists, gentlemen, between two patients suffering from
tabes indiscriminately selected both as regards the clinical his-
tory and pathological anatomy of the disease, can be thus easily

© As regards the difforont segments of the cord, it ia specially in those cousti~
tuting the lumbar and cervical enlargements that the lesions of tabes occur.

=a

TABES DORSUALIS. 867

This change in the cells which is the primum movens of the
lesions which occur in tabes, how and why are they produced?
You will remember, gentlemen, what I have already said with
regard to the etiology of tabes as to the réle played by syphilis.
It is to the syphilitic infection that the cellular changes must
be attributed, But by what means? This is no longer o
case of new syphilitic growths, presenting or not the appearance
of circumacribed or diffuse gummata; nor is it the interstitial
tissue of the nervous system which is affected, but the nervous
substance itself, the cell.* This mode of action differs consider-
ably from that which you have been accustomed to find occur
in syphilis. The progress which has been recently made in
the knowledge of infectious diseases enables us, however, if
not to solve, af any rate to propose a rational solution of
the problem. ‘his solution I shall borrow from Striimpell.
In my opinion, we cannot help admitting, in accordance
with that distinguished physician, that syphilis acts as 3
real organic poison, a fomine, It is only in this way that
we can explain the fact that cells, as distant from each other
as are those of the spinal and peripheral ganglia, are simul-
taneously affected by the same morbid process, and in the
same special elective manner.

This is certainly a hypothesis, but, as I observed when
commencing this series of lectures upon the pathological
anatomy of tabes, everything connected with it is still in a
condition of uncertainty and doubt. Fortunate are those who
believe in the existence of medical questions which are
“completely understood,” and that tabes is one of these.

change (some forms of amyotrophy, hemiatrophy of the tongue, 4c). Tho
cdlls of the drain may also be affected, aa is shown by the dogonaration of the
verve Bbres of the convolutions which occurs in some cases (general paralysis
of the insane, cases observed by M. Jendrasaik).

FRIEDREICH'S DISEASE.

gait resembles that due to cerebellar disease and to tabes, in
addition to which chorelform instability exists, The articulation
of words is difficult and nystagmus exists, but none of the pupil
symptoms which are observed in tabes or insular sclerosis.
The patellar tendon-reflex is completely lost, but no sensory
disorders or lightning pains exist.

The discovery of this affection is undoubtedly due to one
of the most eminent clinics] observers of German origin,
Friedreich, who, as you know, was at the head of the School
at Heidelberg, from which so many distinguished physicians
have come. In 1861, at the Spire Congress, Friedveich presented
several patients who were suffering from a singular form of
ataxy, of which the character was not at all in accordance with
the masterly description which Duchenne of Boulogne had just
given (1858—1859) of locomotor ataxy, The German observer,
after observing these cases, came to the conclusion that the
description of Duchenne was inexact, and, above all, incom-
plete, since it did not apply to such cases as these, In a word
Friedreich believed this to be purely and simply a form of
locomotor ataxy.

In France, during a long period, these facta were ignored,
and, owing to the influence of works devoted by Charcot,
Vulpian, Bourneville, and Guerard to the study of insular
sclerosis, the cases of hereditary ataxy were believed to belong
entirely to that disease.

In England, Carpenter in 1871, and Gowers in 1880, had
published similar casea; the disease was always “hereditary
ataxy,” this, however, being but a form of locomotor ataxy.

The condition of the question remained thus, and I have a
special reason for remembering it, since, in a memoir which T
wrote in August, 1882, for the competition in connection with
resident hospital appointments, I endeavoured to draw up,
according to the description given by authors, a clinical picture
of this affection, with which I was not myself acquainted,
which no one in France had as yet observed, and which T
also considered as a hereditary form of tabes. In the same
year (1882) an excellent thesis was written by Brousse, a
thesis composed under the influence of Grasset, and in which
the author clearly expressed his opinion that this was an
independent affection ; considerable progress was thus made,

24

>>”

870 LECTURES ON DISEASES OF THE SPINAL CORD.

and Brousse proposed to call the affeetion by the
* Friedreich's disease.”

The first case observed, or at any rate recognized, in
was that of a young lad who had been admitted into the
of Charcot in 1884 at a time when I had the honour of
his clinical assistant. I still remember the circumstances
nected with his admission, which I shall mention in order
show you what was known at that time about the disense.
boy was brought to Charcot by a physician attached to one
the hospitals, who was most interested in medical
well-informed, and, if T may use the expression, quite on th
watch for any new discovery. This distinguished physician hed
intended to exhibit the patient at the meeting of a learned
society in order to prove a fact, which was denied by some
authors, that tabes might occur in children. Before doing this,
and owing to a feeling of scientific scrupulousness which ean-
not be too highly praised, my distinguished colleague wished to
have the advice of some one who was well authorized to give an
opinion upon this disease, and he consequently bronght the Ind
to the Salpétriére Hospital. As was naturally the case, Charcot
at once recognized from what disease the boy was really suffer-
ing, and a few days subsequently devoted a lecture to the study
of this patient, the permission to do eo being readily given by
the physician who had asked for his advice, and who was but
too pleased to escape from the error into which he had so nearly
fallen; this lecture definitely established the separate existence
of Freidreich’s disease,

Since that time many works and reviews (Riltimeyer, Musso,
Massalongo, &c.) have been written about this disease, numerous
cases have been published, and the disease of Friedreich now
constitutes a well-defined malady. Amongst these works I must
specially name those written in the French language by Gilles
de la Tourette, Blocq and Huet, the very remarkable thesis of
Soea (1888), and a critical review by Ladame (of Geneva); and
I shall have in fact to borrow frequently from these different

authors.
When the pathological anatomy of this affection is discussed

I shall have to mention other works to you.
The symproms of the disease of Friedreich affecting several
different systems should be described in somewhat methodical

__—

f FRIEDREICH'S DISEASE. 871

| order, and I shall follow that whieh is usually employed, it being
at the same time understood that this classification is purely
artificial.

A. Motor disovdera —Those may be said to constitute the chief

Fig, 199.—Boy aged six years, rother of the patieat who forms the mubject of the
next figure, suffering from the disease of Friedreich. (After 2. Marenlongo.)

Jnr0-ordination very pronounced, the abtitade of the logs, arms, and head will
be observed.

group of symptoms, and to be those to which the attention of
the patient and that of the physician are specially called.
(a) Disorders of the gail—In confirmed cases these troubles are

==

are neither so violent, so sudden, or so unreasonable us in tabes,
while at the same time the unsteadiness somewhat exceeds that
which is due to cerebellar disease alone; with this fact is aaso-
ciated a certain degree of inco-ordination which does not exist in
the latter case; thus the disorder of the gait occurring in Fried~
reich’s disease is termed tabid-cerebellar. ‘The head during this
time presents a series of unsteady movements, whieh are very
analogous to those which occur in insular sclerosis.

(h) Difficulty in maintaining the upright position —This difficulty
was well perceived by Friedreich, and described by him as static
atany. It is not easy for the patient to maintain the upright
position, even when the limbs are separated; he is obliged to
alter the position of his feet from time to time in order to
preserve his equilibrium, the whole body being subject to
irregular movements, while his head is affected by « somewhat
long series of slightly pronounced movements of salutation.
The symptom of Romberg is usually, but not always, absent.

(c) Atactic tremor—This tremor does not occar by any
means in every case, but when it exists exactly resembles that
of insular sclerosis. Having discussed this symptom at some
length, and while speaking of that disease, it seems to me
unnecessary to repeat what was said upon that occasion.

(2) Choreiform movements —These movements are “ abrupt,”
somewhat resembling thore which occur im the chorea of
Sydenham. Usually, however, they are not so long, and occur
not only in the limbs, but also in the face and neck, contri-
buting to give to the patients an appearance of instability
which is really characteristic of the disease.

(e) Paralytic symptoms.—Is there in Friedreich's disease a
more or less pronounced degree of muscular paralysis? A slight
difference of opinion exists upon this point, though for my part
I certainly agree with Musso and Soca in thinking that such
is the case, There is no difficulty in recognizing the fact that
the affected muscles are absolutely inactive, but it seems to me
indisputable that the strength of certain muscles is aleo much
diminished, and that these are the seat of somewhat pronounced
paresis. Such is the case, though it must be well understood,
in some patients, only as regards certain muscles of the legs,
trunk, shoulders, and probably of the upper limbs.
| B. Sensory disorders.—These, differently from what occurs in

372 LECTURES ON DISEASES OF THE SPINAL CORD.

very pronounced, and the patient reels in walking, the legs
being wide apart, the steps irregular, awkward, and often re-

Fig. 194 —Girl aged ten yoara affected hy tho disease of Priedruich, (After R,
Masaalongo.) ‘The attitude of the patient clearly indicates the inco-ordination
‘and difficulty in maintaining the upright position which existe,

sembling those of an intoxicated person ; it does not resemble
the gait which exists in locomotor ataxy, since the movements

=

FRIEDREICH'S DISEASE. 373

are neither so violent, so sudden, or so unreasonable as in tabes,
while at the same time the unsteadiness somewhat exceeds that
which is due to cerebellar disease alone; with this fact is aszo-
ciated a certain degree of inco-ordination which does not exist in
the latter case; thus the disorder of the gait occurring in Fried-
reich’s disease is termed tabid-erebellar. ‘The head during this
time presents 4 series of unsteady movements, which are very
analogous to those which occur in insular sclerosis.

(bh) Digieully in maintaining the upright position. —This difficulty
was well perceived by Friedreich, and described by him, as etatic
atazy. It is not easy for the patient to maintain the upright
position, even when the limbs are separated; he is obliged to
alter the position of his feet from time to time in order to
preserve his equilibrium, the whole body being subject to
irregular movements, while his head is affected by a somewhat
long series of slightly pronounced movements of salutation.
‘The symptom of Romberg is usually, but not always, absent.

(ec) Atactic tremor—This tremor does not occur by any
means in every case, but when it exists exactly resembles that
of insular sclerosis. Having discussed this symptom at some
length, and while speaking of that disease, it seems to me

to repeat what was said upon that occasion.

(d) Choreiform movements.—These movements are “ abrapt,”
somewhat resembling those which occur in the chorea of
Sydenham. Usually, however, they are not so long, and occur
not only in the limbs, but also in the face and neck, contri-
buting to give to the patients an appearance of instability
which is really characteristic of the disease.

(e) Paralytic symptoms.—Is there in Friedreich's disease a
more or less pronounced degree of muscular paralysis? A slight
difference of opinion exists upon this point, though for my part
I certainly agree with Musso and Soca in thinking that euch
is the case, There is no difficulty in recognising the fact that
the affected muscles are absolutely inactive, but it seems to me
indisputable that the strength of certain muscles is also much
diminished, and that these are the seat of somewhat pronounced
paresis. Such is the case, though it must be well understood,
in some patients, only as regards certain muscles of the legs,
‘trunk, shoulders, and probably of the upper limbs.

B. Sensory disorders. —These, differently from what occuve dn

vernal DISEASE. d 375

not really examples of Friedreich's disease ? or should this excess
be attributed to the fact that the lateral columns of the cord are
more affected by the morbid process than is nsually the case?
‘The first suggestion seems to me by far the most probable, and I
should recommend you to have some doubt with regard to a
supposed case of Friedreich's disease in which the tendon-reflexes
are excessive.

D. Disorders in the organs conneeted’ with the senses.—These,
properly speaking, do not affect the organs themselyes, bat their
appendages, specially the muscles if any are present, This is
another point of difference between this disease and tabes,
analogous to that which consists in the reper of sensory
disorders during the course of Friedreich's disease.

(a) Ocular disorders.—The most frequent and interesting dis-
order is :—

Nystvgmus.—This in fact exists in most cases, but it must be
known that as a general rule it occurs at a late period of the
disease: you will only meet with it in fact two, three, or even
more years after the onset of the disense. As in the case of
insular sclerosis, this nystagmus is not accentuated, and may
even disappear during the time of rest, but from the moment
that the patient fixes an object, and specially when he is obliged
to make an effort to do this (the object being placed laterally),
the nystagmus at once occurs or is considerably increased,

Paralysis of the ocular muacles with or without diplopia may
exist, and Joffroy has published such a case; you must not
expect however, gentlemen, to meet with disorders of this nature
in Friedreich's disease in the same way as in tabes; they are as
frequent in the latter as they are rare in the former.

The optic neree and accuity of vision which are so often and
so severely involved in tabes, are on the contrary unaffected in
Friedreich's disease.

No pupil symptoms exist; the size of the pupil undergoes
its normal changes, and the symptom of Argyll Robertson is
absent.

(b) No special observation need be made as to the power of
tasting, hearing, or smelling.

E, Cerelval disorders,—Vertigo occurs somewhat frequently,
existing at times almost permanently, while in other cases it
comes on in well-marked paroxyama,

is never observed either in eqn aaee
of the sexual instinct in the Baler

a

YRIEDREICH’S DISEASE. 377 |

G. Trophic and vasomotor disorders.—There is no trophic
disorder of the skin which I need mention to you, and I may
specially state that nothing exists in the foot resembling the
perforating ulcer which is so frequent in tabes. I should,
however, call your attention to certain singular symptoms
which may be as well ranged under the head of the trophic
disorders as by any other term.

A special form of club-foot is often observed in patients
suflering from confirmed disease of Friedreich, This symptom
consists in a deformity which recalls talipes equinus, the foot
being shorter than when in a healthy condition, the metatarsus
large, while the whole organ seems to be compressed in the
antero-posterior direction; upon observing the foot from the
side it is seen to be much curved on the plantar surface, whilst
the prominence of the dorsal surface is excessive. In addition
to this the foot is “ claw-like,” on account of the forced exten-
sion of the toes; notwithstanding this, voluntary extension is
possible to a pronounced degree, the toes being then in o
position of extreme extension, which causes them, as has been
observed, to have a similar appearance to that which they
present in cases of athetosis, These deformities are bilateral,
and partly disappear when the patient is in the upright
position. It must be observed, gentlemen, that this occurs at
4 comparatively early period in the disease, the first indication
of its occurrence being retraction of the great toe, and sub-
sequently of the others; thus in those families of which
Friedreich's disease affects many members, the sign which is

eat hy the parents to indicate that one or other of their
children suffers from it is precisely this elevation of the great
toe. To what are these deformities due? It is extremely
difficult to express an opinion in this respect; one thing is
certain, namely, that not only the muscles, but also the bones
of the foot participate in the changes which produce this
condition.

Muscular atrophy sometimes occurs, and affects different
muscles ; in the cases which I have had the opportunity of
seeing, the muscles of the shoulder girdle and pelvis were
specially affected; I cannot, however, describe what occurred
in detail. Joffroy and Dejerine have observed patients
similarly affected, and in those named by the latter author

FRIEDREICH'S DISEASE. 379

the atrophy was seated in the peripheral segments of the
limbs.

Considerable lateral curvature has been observed in most
cases of confirmed Friedreich's disease, this being a symptom
which sometimes oecurs at an early period. It consists nsually
of scoliosis, which is most often found in the dorsal region, and
inay be accompanied by more or less pronounced lordosis in
the lumbar region.

Such are the symptoms which cause Friedreich's disease to
have a special character. As regards the order in which they
occur, and the CouRSE of the disease, the following observations
have been made by different authors ;—

The first symptom which attracts the attention of the patient
or his acquaintance is almost always the disorder in the gait,
being sometimes, however, that of the speech or the scoliosis.
In those families in which it is feared that each fresh child may
he affected by disease of the spinal cord, and in which the child
is constantly watched for the occurrence of the different symp-
toms, extreme extension of the great toe, or the absence of the
patellar tendon reflex ore most often sufficient to indicate the
onset of the disease in any of the children in which these symp-
toms occur,

However this may be at the end of a few, usually from three
to five years, the ataxy which exists in the lower as well as the
upper limbs is quite characteristic; this again continues to in-
crease both in the hands and feet, so that not only is the patient
unable to walk any longer, but also has great difficulty in using
his fingers ; he is then confined to his bed, this being a second
period of the disease,

‘The condition now remains the same, and the patient is on-
able to leave his bed or arm-chair during fifteen, twenty years,
or even more. Death occurs from the effect of some intercurrent
disease and not on account of the progress of the disease in the
spinal cord.

Recovery is a termination which is absolutely unknown, what-
the treatment adopted or age of the patient may be. At the
same time, remissions, which may be of somewhat long duration,
sometimes occur. On the other hand, in some cases sudden and
serious aggravations occur.

I do not believe that the affection Las ever been known to be

gai ;

the tabid-cerebellar type, that is to say,

which exists in tabes, the tottering oc

to that which is due to cerebellar

disease again, as you know, sensory
with

the organs of the senses, 1

FRIEDREICH'S DISEASE. 38r

observed before the age of twenty-five years. The first of
these two diseases is essentially a family affection, which is by
no means the case with respect to the second.

As regards insular sclerosis, I have already
insisted upon the numerous ways in which it resembles
Friedreich's disease ; I must now show you that the points
of difference are even more numerous. In the former the
disorder of the gait is specially of the spasmodic cerebellar, in
the latter of the ataxic cerebellar type; in the latter again
choreiform irregularity exists, which does not occur in the
former.

Lastly, as you have seen, neither ocular paralysis nor
optic neuritis, which are so frequent in insular sclerosis,
exist. in Friedreich’s disease, wherens the loss of the tendon
reflexes, the scoliosis, and club-foot, which are almost con-
stant in the first of these two affections, do not occur in the
second.

The chorea of Sydenham has been known, as I have already
said, to give rise to erroneous diagnosis in some cases; certain
features are in fact common to these two affections, euch oa the
so-called choreiform unstableness, and the alteration in the
voluntary movements both of the limbs and face, while bath
affections are, so to speak, the appanage of childhood.

With some attention, however, nothing is more easy than to
distinguish these two diseases, since in chorea the movements
are more extensive, and usually more pronounced in the upper
than in the lower limbs; neither club-foot nor nystagmus occur,
the patellar tendon reflex is retained, and the symptom of
Romberg is absent.

I may perhaps be allowed to mention upon this occasion the
cases recently described by Nonne,*" in which inco-ordination of
movement and disorders of speech analogous to those which
oxist in Friedreich’s disease were observed to occur in many
members of the same family; this affection only occurred at
puberty. At the antopsy of one of these eases Nonne did not
find the sclerosis to exist, which is characteristic of Friedreich's
disease, but only pronounced tenuity (gracilité) of the spinal
cord and some other parts of the nervous system. What is the

* Nonno. Uebor eine cigonthimliche familidre Erkrankangsform, &o., Arch.
f. Faye®,, XXIL, p. 253,

882 LECTURES ON DISEASES OF THE SPINAL CORD.

nature of this affection? We ‘are completely ignorant, but
whilst waiting for more information upon this subject I thought it
my duty not to leave unnoticed facts recorded by so distinguished
an observer, this being perhaps the indication of some fresh
neuropathic condition.

LECTURE XXXI.
FRIEDREICH'S DISEASE (continued ).

ETIoLoay: It is a family affection —What is the rile of the patbological ante
cedents in the paronts or ancestry? What is that of syphilis? ‘Tho onsot of

peripheral nerses in the enbjoct of very divergent opinions on the
ent observers, Nature of Friedreich's disease. Opinion of
Lotulle, Pitt and Grassot. Therapoutics : its want of anocess.

GextLemeN,—You will have noticed, owing to what has
already been said, that from a clinical point of view Friedreich's
disease is clearly distinct from the affections with which one is
at first sight inclined to connect it, It has also its own special
wtiology, as you will soon perceive.

It is in fact a family disease, that is to say, a disease which
affects many members of the same family. If you read the
account of the observations published by different authors you
will find that three, four, or five brothers and sisters often
suffer consecutively from this disease, It also appears that in
some cases the disease is found to have occurred in the
and a father, mother, and several of their children may be
found to present the symptoms of this malady; at times the
morbid tendency skips a generation and s person apparently
healthy, but in whose father or brother this form of inco-ordination
existed, is the father of children who are thus affected. These
cases, however, are very rare, if they exist at all, and most
usually, as I have just said, its co-existence in many brothers
and sisters alone stamps it as a “family” disease. This notion

384 LECTURES ON DISEASES OF THE SPINAL COR

importance, as shown by its prominent place in
atazy proposed by him. ;

‘Tt mast not be thought, however, gentlemen, that t
character is absolutely necessary ; you will im fact m
isolated cases, one child may be alone affected in |
whilst its brothers and sisters remain in perfect health.
observes in connection with this fact that such e
specially occur in small families, and suggests
number of children was greater in them some would
have suffered from this disease.

This is very possible, but ab any rate the fact is ¢
Friedreich's disease does not necessarily occur in all thy
of the family when one of them is affected.

As regards the pathological antecedents and their p
the wtiology of the disease nothing definite is kn
connection with the parents more or less
the nervous system are somewhat frequently found to exi
without any definite signification, and varying in nature
some kind of neurosis to the most various organic diseases,

Owing to what you now know of the wtiology of tabes 3
will naturally ask, gentlemen, whether syphilis does not ;
play a part in that of Friedreich's disease. No long
passed since I in the same way asked myself that qu
when any opportunity occurred I endeavoured to ase
whether it might not be simply and purely due to here
syphilis. I questioned the parents carefully, but came into
session of no facts which indicated such a connection; in {
observations, again, which have been published by
authors you will rarely find that any specific infection
parents is known to have existed. We are not, th
the present time authorized to suppose that this disease
nected with syphilis.

‘There is little to be said as regards the affections from
the patients are liable to suffer previously, except that in «
cases Friedreich's disease has seemingly occurred af
infections disorder such as measles, scarlatina, or sm:
There is but little probability that they are directly con
with each other as cause and effect on account of the hered
nature of the disease ; at the same time it is quite probab

FRIEDREICH'S DISEASE.

the disease is ao influenced by the infectious disorder that its
symptoms appear at an earlier date in consequence of its exist
ence, he fact in itself is by no means extraordinary, and the
same occurs in another affection which is essentially

myopathic atrophy. of which’ he onset seems nt-times to ba alee
connected with one of the infectious diseases of childhood.

At what age does the onset of Priedreich’s disease occur? T
have already had occasion several times to say that this is
specially a disease of childhood, not only of puberty, as Fried-
reich supposed, but also of childhood, and in 76 cases of
which the age was known in not less than two-thirds, the
disease had begun before the age of fourteen years; in some, in
fact, the first symptoms had been observed during the first
months of life. On the other hand the onset very rarely occurs
after the age of sixteen J.

As regards the age at which the onset occurs Soca has made
a remark which he has enunciated in the form of a law in the
following terms: “In the same family the onset of Fried-
reich’s disense occurs at the same age in the case of each
member of the family who is affected by it”; each family is
thus attached by Friedreich's disease im a special manner, not
only as regards the age of the onset, but also it appears owing
to certain clinical features being observed in some families and
not in others,

The male is affected somewhat more frequently than the
female sex (in the proportion of 68 to 47—Soca), the contrary
opinion having been held at first by Friedreich.

Patuotoaica. Axatomy.—This branch of the study of
Friedreich's disease is, I must admit, gentlemen, by far the
least advanced ; few autopsies have been made as yet, and the
results have not been always in accordance with each other, T
will endeavour, however, to describe the different lesions which
have been found in this disease to you, but as I would again
say, this description should be considered as simply an attempt
which recently published works will assist me to make.

A feature which should be first mentioned is the fenwity
(yracilité) of the spinal cord ; this change can be at once recog-
nized to exist by the naked eye, since its diameter does not
exceed the } or even of that which normally exists; it seems
to be specially pronounced in the dorsal region. At the same

Bw

ipa Vota lances uunibet of, flmestin ae
retraction of the part affected by sclerosis, or.
in the development of the different tracts.

An examination by the microscope enables a
of lesions to be recognized, notably in connectic
tracts of the columns, viz.,the existence of i
‘Let us first examine the seats of this affection:
A. In the posterior column. The columns of G
the seat of most pronounced degeneration which
from the lower part of the cord as far as the nib of

e¢ &

Fig. 196.-Section of the spinal cord in the middle part of the dorsal n
ncaso of Friedreich's disease. (After Blocq and Marineso.) A,
portion much degonerated, seated in front of the direct cerobellar t
which according to Blocq and Marinesco, perhaps, represents the
Gowers; B, lateral much degenerated ; C, direct cerebellar tract ; D, ex
of Burdach, degenerated ; G, band of sound fibres which adjoins the
horn. ‘

scriptorius; these tracts are thus affected throughout
whole length. The columuis of Burdach are also i
throughout their whole extent, but in varying inten
conling to the region of the cord, the affected part not
senting the same appearance at different heights; their e
zone is usually unchanged. The lesion of these ¢
diminishes progressively as the cervical region is left
appear altogether in the lower part of the medulla oblo

FRIEDREICH'S DISEASE.

a,

B. Direct cerelellar tract. This column is affected from
origin, that is to say, the lower part of the pester rad the
lesion being more pronounced in the upper part of the dorsal
region, while on the other hand it diminishes considerably in
the lower part of the medulla oblongata, where but a fe:
degenerated fibres are found to exist in the seat of these tracts,
In some observations (Pitt, Riitimeyer, Blocq, and Marinesco)
it is clearly indicated that the lesions extend anteriorly beyond
the seat of the direct cerebellar tract, and that the tract of
Gowers was also affected; I believe that this change may be

Ae @
a @ W

Sections of the cord from a case of Friedreich's disease. (Case I. of Ratimeyer.)
Pig. 197.—Upper part of the cervieal enlargement. Fig, 198.—Region seated
between the cervical and dorsal portion of the cord. Pig. 199.—Middle part of
the dorsal region, Fig. 209.—Region seated between the dorsal and lumbar
regions of the cord,

considered as constant in Friedreich’s disease when somewhat
advanced in degree.

C. Lateral colwsane.—Most authors regard the lesions of the
lateral columns as seated in the crossed pyramidal tracts; I
confess, gentlemen, that I have some difficulty in sharing their
opinion. I allow that the lesions are seated in the region of the
crossed pyramidal tracts, but I cannot readily admit that the
affected fibres belong to those tracts. You know, in fact,
gentlemen, that different varieties of fibres exist in these tracts
(large fibres, narrow fibres) there is, therefore, nothing sure
prising in the fact of these fibres having a different origin, and

888° LECTURES ON DISEASES OF THE SPINAL CORD.

‘the proof that such is the ease is the fact that in enses
sive lesion in the pyramidal system a certain numbe

Fig. 202.

Sections of the spinal cord from a case of Friedroich’s disease, (Case IL. of |
Ratimeyer,) Fig. 202,—Lower part of the cervical enlargement. 203, —
Upper part of the dorsal cord. Fig. 204.—Middle part of the dorsal cord.
Fig. 205.—Lower part of the lumbar cord. Fig. 206.—Sacral cord.

are found to be quite unaffected in the middle of the diseased
zone. In the disease of Friedreich the following are the:

upon which I based my opinion that the affected fibres do not
belong to the crossed pyramidal tract (FPyC): 1. The | 1
in the lateral column diminishes considerably from w
upwards as far as the lower part of the medulla oblongata, which
would scarcely be the case if the pyramidal tract was affected.
2. Ina transverse section of the cord the seat of the lesion in
the lateral column does not exactly correspond to that which
a lesion in the pyramidal tract would have, occupying a
more external portion of the cord. 3, Nothing in the clinical
symptoms recalls those which constantly occur when the
pyramidal tract is affected. If, then, the pyramidal tract is not
involved, which are the fibres, you will ask, whose disease
produces this sclerosis in the lateral column ? It is difficult to
answer this question in a positive manner, but we may, I think,
presume that these fibres are connected with the direct cere
bellar and anterolateral ascending tracts, which they

together. ‘

&

FRIEDREICH'S DISEASE. 2 389

D. The marginal zone of Lisaoner.—Chonges in the nérvo
fibres of this region are not admitted to exist by all the different,
authors. Riitimeyer and Ladame state that they did not find
them, while Letalle and Vaquez, Blocy and Marineseo observed
them to exist; the latter authors remark that in the cases seen
by them the lesions in the zone of Lissauer only existed in the
lumbar region, this part being unaffected in the dorsal and
cervical region, In the presence of these diverging opinions it
is difficult to form a conclusion; I should at the same time be
very inclined to think that this lesion of the Glirea in.thaaone af
Lissauer exists very frequently, if not in all cases of Friedreich's
disease.

‘The grey matter is also the seat of different changes :—

(@) Clarke's columns.—The nerve fibres which constitute the
nerve-network of these columns not only disappear to a more
or less pronounced degree, as.occurs in tabes, but the cells
exist in smaller number, are of smaller size, and have usually
lost their prolongations.

(8) Posterior horas.—These in the same way as all other parts
of the cord are of diminished size, in addition to which the
number of cells which they contain is appreciably less.

(e) Anterior horns.—Changes have been also found in these
by some authors (Eriedreich, Ratimeyer). Atrophy of the cells
having been sometimes found to exist, these lesions will perhaps
explain how it is that in some cases, as I haye already said,
muscular atrophy is a clinical symptom of the disease.

The cenfrad canal of the cord is sometimes altered, the signifi-
cation of this change not being as yet understood; thus it may
be bifid, or lateral ectopia, or “ peri-ependymal” lesions which
may occur. ;

The meninges of the spinal cord do not seem to present,
always the same appearance ; thus some authors assert that they
have been clearly thickened in the cases observed by them,
while others affirm that such a change exists bat slightly if at all.
In any case, if such thickening occurs, it is most. pronounced.

The posterior roots have been also the cause of much difference
in the opinions expressed as to their condition; thus, for
example, Letulle and Vaquez state that they are affected in an
irregular manner, whilst Bloeq and Marinesco consider them to
be ag much changed as in ordinary tabes.

regards their seat; with ae te alk ee
some explanations which will put you on your guard

which are seen microscopically, that in cortain
cord the part affected by sclerosis is found to be clear
of long fibrils, plaited in the transverse direction, and
of this plaiting more or less obliquely placed with
each other. Recently, in 1890, Dejerine and Letu
the existence of these “ eddies,” making them the b
actual theory as to the nature of the anatomical
occurs in Friedreich’s disease. Comparing these

exist in sclerosis with the lesions described by
cerebral cortex of those affected by epilepsy, lesions

neuroglia, Dejerine and Letulle thought that the ¢
occurs in hereditary ataxy was special to the disease ;
fore believed themselves authorized to assert “that |
discovered a form of sclerosis affecting merely the neuro
only sclerosis of this nature hitherto known.” In
this view they relied not only upon the “eddies
but also upon the sound condition of the septa of c
tissue and blood vessels in the posterior columns. ‘Thus, a

ing to these authors, Friedreich's disease ix con
‘sclerosis of the neuroglia alone, being as it were
(sclerosis of ectodermal origin) of the posterior eo!

FRIEDREICH'S DISEASE. ‘SOL

the other hand. sclerosis of the crossed pyramidal and direct
cerebellar tracts would be always, in the opinion of Dejerine
and Letulle, a “ vascular form of sclerosis,” since in these con-
ditions the eddies of the neuroglia are absent, while sclerosis of
the septa of connective tissue, and more or less pronounced
vascular changes exist.

This thesis,which was zealously maintained by the above authors,
was for a time favourably received by the medical profession, but
the neurologists on the one hand and pathologists on the other
became united against it. Amongst the first opponents we find
Blocq and Marineseo. Achard and Weigert, authors who joined
together in order to prove in different ways that there was no
foundation for the statement of Dejerine and Letalle.

The following is a brief summary of the arguments which
may be considered more or less conclusive against the opinion of
Dejevine and Letulle: («) The vascular changes in the posterior
columns are not only present, but are at times so pronounced
that Pitt was of opinion that Friedreich's disease should be
rogarded as a vascular form of sclerosis; Blocq and Marinesco
again describe numerous changes in the vessels, proliferation of
the nuclei, dilatation of the call spaces, and even obliteration,
(b) As regards the septa of connective tissue, they are far from
being always unaffected, and changes have been recognized in
them by some observers. Lastly (c), which is a more serious
objection, the so-called specific nature of this form of sclerosis,
owing to the nenroglia being specially affected in Friedreich's
disease, has no true foundation. On the one hand, the “ eddies”
which exist seem, in my opinion, to be merely an indication that
the sclerosis in the nervous centres fulfils the two following con~
ditions ; 1. That of being of very long duration. 2. Of having
commenced in childhood at the time when the nervous centres
were still being developed, (I for my part, in 1884, in a work
written conjointly with Jendrassik, showed that the condition of
the parts affected by sclerosis in the cerebral convolutions was
very analogous to that of the eddies which exist in the cord in
Friedreich's disease, and I maintain that this condition is en-
tirely due to the two causes which [ have just mentioned.)
Certain special methods of staining the sections (Malassez,
Weigert) have also shown, as Achard* very truly observes, Sus

* Achard, Bulletin de la Soctet? Anatomiyue, 1390.

Tab tin aclorpets,svhich exiata in tie tadtac T
be regarded as a form of glioma,
‘This prejudicial question having been thus

anatomy of hereditary ataxy ? Friedreich lool
the result of arrest in the development of the
believed the lesion in the lateral columns to be
of ‘the change in the pia-mater, which was itself
the alteration in the posterior columns. Pitt,
opinion that the disease was due to some defeet in
ment of the cord, states that owing to this fact the
develop at a late period are specially affected in-
disease, the diseased fasciculi, none of them :

the opinion of Grasset is again diferent;
Friedreich’s disease is a form of combined aclorcalage
considers, is a term applied to a special group of
shall haye occasion to express my opinion
of disease, and you will see that it is very diff
expressed by the distinguished profeasor of Mont
At the end of this chapter upon the pat c
the disease, I should add that T have only spoken of |
in the spinal cord which occur in Friedreich's dise:
the fact that they are the only ones as yet known.
ever, probable that as the study of neurology
lesion will be found in the medulla oblongata, bn
bellum, since Friedreich's disease seems to me
cerebro-spinal disease. At the same time, you

* ©. Weigert, Zor Pathologischen Anatomie dea Neurogliafaserg
Contralblit far Allgemeine Pathologie, 1690), p, 729 et seq.

FRIEDREICH'S DISEASE. 393

gentlemen, to be absolutely silent in other respects as regards
the nature of the disease, since I have been so far totally unable
to form even an approximate opinion upon this subject.

How should Friedreich's disease be treated? Some symptoms,
especially the disorder of the gait and inco-ordination, have in
xyme cases been improved by means of suspension and electrivity ;
autipyria, recommended by some authors, has no beneficial effect.
After what you have learnt as regards the origin of the disease,
and the way it occurs in families, you will not be surprised to
hear, gentlemen, that it ix absolutely incurable.

COMBINED LATERAL AND POSTERIOR SCLEROSIS. 395,

specially mentioned. Lastly, that of Grasset should not be
forgotten in this enumeration, one of the most complete works
which have appeared upon this subject; I am the more anxious
to allude to this work, owing to the fact that, as you will see in
the course of these lectures, I am far from agreeing with the
opinions of that author.

Its pathological anatomy having, as I have already observed,
been the special cause of this form of combined sclerosis being
recognized, it would be best, in my opinion, to commence by
studying what has been written upon this subject.

Tn order to have a typical example of the disease, though I
would not by any means say that this type exists as often as is
said to be the case, we will imagine, gentlemen, a spinal cord
in which the miecroseopic examination reveals the simultaneous
existence of the following lesions :

A. In the posterior cohanns :—

(a) The columns of Goll have degenerated throughout almost
the whole length of the spinal cord, as far as their termination
in the medalla oblongata.

>

a

Fig. 207.—Section of the spinal cord from a case of combined Lateral and
posterior sclerosis (lumbar cord). ‘The cloar portions indicate the parts
affected by sclerosix; they mre of lighter colour in proportion as the sclerosis
is more pronounced. A. Column of Goll} B,C, Posterior part af the Interal
column near the pyramidal tract; D. Column of Burdach,

(b) The colemna of Burdach are also involved, but usually to a
slighter, less complete, and in all eases a more variable extent.
Aveceniiig te Brig uate the lesion in these columns is almost
in the dorsal than in the lumbar cord,

q - in this column in tabes

Sections of the spinal cord from a case of combined lateral
of the cervical region. Pig

B. In the lateral columns :—

(a) The crossed pyramidal tract is not usw:
say, involved throughout its whole extent; its p
part is usually unaffected: in the same way as re
of the lesions in these tracts, it is observed that st «
can scarcely be said to exist in the upper part of
region, being much more pronounced in the dorsal
cord.

(2) "The direct pyramidal tract is usually un

cases, however, an islet of sclerosis has been f 1

which compose it, specially at the internal part of thi
©. In the divect cerebellar truct :— 9
This tract is always affected, and to a somewhat pro

degree.
D. Gowers’ ascending antero-lateral tract :—
‘The lesions are usually less pronounced ii
preceding tract; of times, in fact, they are but
marked.

COMBINED LATERAL AND POSTERIOR SCLEROSIS. 397

B. In the grey matter : =
(a) The cells of the anterior horas may be involved in the
morhid process, though this rarely occurs.

Fig. 211,

Fig. 213.

according to their greater or loss intensity. Fig. 211.—Lesions in the column
of Goll, column of Burdach, tho direct corebellar tract and the crossed pyramidal
tract (uppor part of the cervieal cord). Fig. 212 —The same lesions as in the
preceding figure (the middle part of the dorsal cord). Fig. 213.—The lesion
in the posterior columns is loss pronounced, and tends to rocede from the
modian line (upper part of the Tumbar cord). Fig, 214—Tho lesion of the
posterior columns can scarcely be soon (lower part of the Iumbar cord), It will
be observed that all those lesions diminish in intensity from above downwaris.

(b) The cells of the posterior horns apparently may or may not
participate in the changes which occur in the posterior columns.

(c) The cells of Clarke’s columns suffer in some cases from
more or less pronounced atrophy, bat the knowledge which
exists about this point is not explicit enough to enable me to
» make any definite statement.

Such, gentlemen, is a summary of the changes which may
possibly be found to exist in the same spinal cord. You will
understand what a great variety of symptoms may be produced
by such a combination of lesions.

A. There are symptoms of tabid nature which consist in—

Inco-ordination, which is indicated by the awkward move-
ments, ataxic gait, and symptom of Romberg. The loss of the
patellar tendon reflex ; the lightning or other pains; anwsthesia
and paresthesia; the ocular derangements; the disorders con=
nected with micturition; the genital derangements, &.

You observe what a singular combination of sym
exist. Do not, however, suppose that these sy
ciated together quite indescriminately and without fi
cause. For from this they tend to exist together im gros
that different clinical forms can be sa
time this does not imply that the combined form of sclero

a distinct morbid condition which may be com
“system diseases” of the spinal cord.
I will now mention to you the clinical groups |
often observed.

The following is what usually occurs :—inco
exists, after which spasmodic rigidity of the lower 6
cecurs ; carly and rapid diminution of genital power; di
inthe badder are soon observed, and a certain deg
numimess in the lower extremities. As regards pains,
already observed that they are usually of slight inte
short duration. Gowers observes that while these
specially seated in the lower limbs, in tabes they ch
in the lumbar or sacral region in the combined
sclerosis.
The ocular disorders ave vare, and the gastric, in
laryngeal crises scarcely ever exist.

Tn those cases in which spasmodic rigidity occu
limbs, the patellar fendon-rejlew is naturally @3
much more rarely absent, and only when the cli
of the case more closely resemble th
The disorder of the gait may

COMINED LATERAL AND POSTERIOR SCLEROSIS, 399.

tabid-spasmodic; that is to say, it resembles both that which
exists in tabes, and that associated with spastic paraplegia.

‘The upper limbs are rarely affected, and if so to a less degroe
than the lower limbs.

It is in order to recall the principal characters of the affection,
which have just been mentioned, that Dana proposes that the
name “ progressive spastic ataxia” be conferred upon it, and this
from a descriptive point of view seems to me preferable to that
of “ ataxic paraplegia” proposed by Gowers.

The diagnosis must be specially made as regards (a) tabes, (b)
the principal forms of spastic paraplegia,

(a) Tabes.—In this disease the complete absence of any spas~
modic symptom, the frequence and intensity, as also the seat of
the pains (which specially occur in the lower limba) associated
with the loss of the knee-jerk, and the existence of ocular dis-
orders usually enable the diagnosis to be made. In combined
lateral and posterior sclerosis again paralysis of the lower limbs
exists to a somewhat pronounced degree far more often and
always at a much earlier period of the disease than in tabes,

(6) Spastic paraplegia —The forms of this disorder, which are
found to be distinguished with the greatest difficulty from the
combined form of sclerosis, are the following :—

(a) That which oceurs in insular sclerosis. In the lnttar dis-
ease the absence of symmetry, the irregularity in the symptoms,
the tremor which occurs, and the disorders of speech will prevent
its being mistaken for the combined form of sclerosis.

(8) That which is due to diffuse transverse myelitis. In this
case the grey matter is specially affected ; there is more or less
extensive muscular atrophy, and disorders often occur in con-
nection with the action of the sphincters; in addition to this it
can be recognized that the symptoms correspond to a transverse
lesion in the spinal cord, owing to the fact that all the parts
above the point at which this exists are totally unaffected.

As regards the 2r10LoGy of combined lateral and post
"0 hitherto been written upon this subject does

with a diffuse lateral sclerosis; it
myelitis.”
According to Ballet and Minor the di
diffuse pseudo-systemic sclerosis, which is y
nature, and often again due to a process
(the latter opinion is also that maintained by
In the first place, gentlemen, one thing:
namely, that it is absolutely impossible to eo
of combined lateral and posterior sclerosis as ¢
morbid process. It is necessary to class th
groups, which may be, as you will see, very di
other,
The cLassitcation is almost the same
Ballet and Minor in their memoir, and contain:
T. There is a combination of different forme of tr
sclerosis, which, for example, exists in the f
(a) In certain cases of general paralysis of
the lesions in the posterior columns, which ar
tabes, are found to co-exist with yery evident!
the lateral tracts, containing the pyramidal th
(5) Systemic sclerosis of the columns of Goll
Burdach, and the direct cerebellar tracts are
combination. Cases in which this association
been rarely observed, and have not been
iS = remark, however, gentlemen,
very analogous to that which I hi
as pte in sin Friedreich's disease,

. oe

COMBINED LATERAL AND POSTERIOR SCLEROSIS. 401

should like, gentlemen, to draw your attention to an observation
in pathological physiology which is most interesting. No one,
I believe, has as yet applied it to the study of the lesions which
we are considering, and yet it throws muck light upon their
origin.

You undoubtedly know, gentlemen, the celebrated experi-
mont of Stenson, which consists in temporarily arresting the
course of the blood in the abdominal aorta and then re-
establishing the current, and observing the disorders in the
fanctions of the spinal cord which result from such’ momentary
arrest of the circulation in the lower part of the spinal axis.
Ehrlich and Bri
of this observation in order to examine fron: a microscopic point
of view the lesions which are thas produced in the cord. They
noted the fact that extensive degeneration exists at that time in
the grey matter and antero-lateral columns, whereas the posterior
columns are almost unaffected, except at their anterior border
in the part which adjoins the posterior commissure. To this
very important fact another may be added, furnished by Singer
and Miinzer, namely, that a marginal zone which completely
corresponds to the regions of the direct cerebellar and Gowers’

© conceived the ingenious idea of making uze -

COMBINED LATERAL AND POSTERIOR SCLEROSIS. 405

traced as far as the conus medullaris of the cord. The two
above branches may join at a lower point, or the anterior spinal
artery may again divide at certain points into branches which
afterwards join to form a single trank.

‘The anterior spinal artery is reinforced by many tributary
branches from the lateral spinal arteries which pass through the
orifices in the laminw of the vertebre and accompany the
anterior roots in order to join the anterior spinal artery. These

branches are usually of very sinall size, and according to

Adamkiewicz there are only two or three of those vessels
throughout the whole length of the cord which are large
enough to be of much importance; the tributary vessels seated
in the lower part of the cord are of larger size than those in its
upper part,

A series of somewhat large branches pass from the anterior
spinal artery at right angles to that vessel from before backwards
into the anterior median fissure, constituting the exterior median
arteries, of which I shall presently have to speak. The anterior
radicular arteries and some anastomotic branches which join the
system of the posterior spinal arteries, and contribute to supply
the lateral parts of the cord with blood, come from the same
vessel.

Il. Losterior system.—The posterior spinal arteries are two in
number upon each side; they also come from the two vertebral
arteries, and are formed upon each side by two branches, of
which one descends behind the posterior roots, namely, the
iuernil posterior spinal arlery, while the other, the external
posterior spinal artery, descends in front of them.

These arteries have a large number of branches which pass
into the interior of the cord or anastomose with those from the
three other arteries ; lastly, some branches communicate with
those of the anterior system.

The anterior and posterior systems are therefore very distinct
from an anatomical point of view, although of common origin;
from a pathological point of view, as I shall show you, gentlemen,
they are no less distinct ; in some affections the anterior while
in others the posterior system is involved. ‘This localization of
the lesions ina part of the cord supplied by one set of vessels
alone should not surprise you, since examples of this kind are
by no means rare, Thus, in the pathology of the heart the

What is knowen a4 to the ak of
co norm aus

:

Brrore entering more deeply into the
aystemic forms oo combined sclerosis of ¥
ask your permission to state in a few words:
which are now known about the CIRCULATION
srinat corp. For this knowledge we are c
you know, to the studies of Adamkiewicz,
devoted to this subject having, as you know, bee
inspired by him; Duret has also made importa
in connection with this subject.

‘The arteries of the spinal cord are derived on
from the vertebral, on the other from the
sacral arteries, and pass into the spinal
intervertebral foramina with the anterior or p
facilitate the description we will study se
extramedullary. B. The intramedullary bran

A. Exrra-MEDULLARY BRancaes, T. Anterior
anterior spinal artery is formed by the union of
branches, each of which is supplied by one
arteries; these two branches usually join at the
the cervical cord. The anterior spinal artery
formed corresponds to the anterior median fissi

COMBINED LATERAL AND POSTERIOR SCLEROSIS. 405

traced as far as the conus medullaris of the cord. The two
above branches may join at a lower point, or the anterior spinal
artery may again divide at certain points into branches which
afterwards join to form a single trunk.

The anterior spinal artery is reinforced by many tributary
branches from the lateral spinal arteries which pass through the
orifices in the lamime of the vertebre and accompany the
anterior roots in order to join the anterior spinal artery. ‘These

branches are usually of very small size, and according to
Adamkiewicz there are only two or three of those vessels
throughout the whole length of the cord which are lange
enough to be of much importance; the tributary vessels seated
in the lower part of the cord are of larger size than those in its
upper part,

A series of somewhat large branches pass from the anterior
spinal artery at right angles to that vessel from before backwards
into the anterior median fissure, constituting the anterior median
arteries, of which I shall presently have to speak. The anterior
radicular arteries and some civistomotic branches which join the
aystem of the posterior spinal arteries, and contribute to supply
the lateral parts of the cord with blood, come from the same
vessel,

IL. Losterior system.—The posterior spinal arteries ave two in
number upon each side; they also come from the two vertebral
arteries, and are formed upon each side by two branches, of
which one descends behind the posterior roots, namely, the
iuterndl posterior spinal artery, while the other, the external
posterior spinal artery, descends in front of them.

These arteries have a large number of branches which pass
into the interior of the cord or anastomose with those from the
three other arteries; lastly, some branches communicate with
those of the anterior system.

The anterior and posterior systems are therefore yery distinct
from an anatomical point of view, although of common origin;
from a pathological point of view, as I shall show you, gentlemen,
they are no less distinct ; in some affections the anterior while
in others the posterior system is involved. This localization of
the lesions ins part of the cord supplied by one set of vessels
alone should not surprise you, since examples of this kind are
by no means rare. Thus, in the pathology of the heart the

COMBINED LATERAL AND POSTERIOR SCLEROSIS, 407

anterior horns into which they penetrate either anteriorly or
laterally,

(c) ANTERIOR RADICULAR BRANCHES (coming in great part, as
we have seen, from the lateral spinal arteries); they accompany
the roots into the interior of the cord, giving off twigs both to
the white substance which immediately adjoins them and to the
grey matter in which they terminate.

IL, System of the posterior spinal arlery. (a) Posrerton
MEDIAN ARTERY.—This artery passes to the end of the posterior
median fissure; in its course it gives off small branches to the
adjoinmg parts. This artery is usually of somewhat large size,
and is very easily seen in most sections of the spinal cord.

()) Post-rxrermepiare artery.—This artery passes between
the column of Goll and that of Bardach in the intermediate
groove; it is contained in a somewhat large septum as indeed
are most of the intra-medallary arteries of a certain size.

(c) Posrenton RaprcULAR arTERY.—Each of these arteries
accompanies the corresponding posterior root, and terminates
with it in the grey matter of the posterior horn.

(@) Posrerton connvat antery.—This vessel passes through
the column of Burdach on the inner side of the posterior roots,
and terminates in the base of the posterior horn.

(e) PosveRIOR LATERAL ARTERY,—This branch, which is not
quite constant, is distributed to the posterior part of the lateral
column.

(f) Mippie Larerat arrery.—This vessel, usually of some
size, supplies the middle part of the lateral colamn.

(gy) ANTERIOR LATERAL aRTERY.—The anterior part of the
lateval column is supplied by this branch.

‘The 3 arteries e, f, g, specially the latter, are partly farnished
by the anastomotic branches, between the posterior and anterior
systems, bat ave connected with the posterior system of vessels,

Now that we have a general idea, gentlemen, of the blood
supply in the spinal cord, we will sgain consider combined lateral
and posterior selerosis, for which purpose this digression was
made.

1 observed to you, gentlemen, that a large number of cases of
this affection are of vascular origin, and this I shall now attempt
to prove.

Itwe place th dingram of a section of the normal spinal cord

408 LECTURES ON DISEASES OF THE SPINAL CORD.

in which the course of the vessels is indicated over «
senting o section of the cord affected by combined
posterior sclerosis, it will be clearly seen that the parts

Fig. 216,—Dingram made for the purpose of showing the part taken by vasct
lesions in the production of certain forms of combined sclerosis, E,
anterior, middle, and posterior lateral mrteries ; H, posterior z
1, posterior cornual artery; J, post-intormediate artery; K,

ide of the figure the existence of sclerosis ik
(by means of points) round each of the arteries in the posterior
‘the peripheral and posterior part of the lnteral column, the blood
chiefly supplied by the posterior spinal artery; the seat of
in thus indicated is a good representation of what ix really

com form of «cl srosis of vaseu"ar ovigin,

by sclerosis correspond to the p
their blood from the system of
Let us consider, for mp
observe, gentlemen, that the
Goll’s columns around the p
way in the colamns of Burd
post-intermediate artery, and
vascular changes sclerosis exist
recalls in the most exact way by
characteristic of tabes in an ad
This, however, is not all that may
seen, that. which characterises the
is the fact that Ht condition.
columns but also in the di

COMBINED LATERAL AND POSTERIOR SCLEROSIS. 409

columas almost at the points occupied by the pyramidal tract.
It can very eas ly be shown, gentlemen, that these lesions also

M, Ballet and Minor). ‘The parts of the white
of ablack or grey colour according to the
cord. Fig. 218—Middle part of

hee said, one of the best !
oppouition! to\thel teary Ast cil Geena
systemic nature, The system of the
however, share in the morbid process :
then exists in the cord which can si
appearance either systemic or pseudo
case which IT have put before you, the
spinal artery is but partly affected. In thi
exist round the anterior median artery in th
the cord. This artery, however, scarcely
sclerosis, is found to be unilateral, as in
now put before you, so that its pset
becomes even more manifest.
You see therefore, gentlemen, that the

d this opinion, that the vascular orig
by far the most frequent." ‘The meaning of tl
that that affection is essentially secondary, and

rhb Aa might be mentio

COMBINED LATERAL AND POSTERIOR SCLEROSIS. ALL

to this, far from being a disease of the spinal cord, it is at most
an example of a vascular inflammatory process of which the
effects are localised in certain regions of the cord. These
conclusions should take the first place in connection with the
cetiology of that form of combined sclerosis, and I have no doubt
that certain general diseases in which the blood vessels are
specially affected, as for example syphilis, would be often found,
if more carefully examined, to be cases of this kind.

Fig. 222. =Section of the spinal cord in a case of combined lateral and postericr
aclorosis (cervical cord). The white parta aro those in which sclorosis cxists.
‘Tho two posterior columns A. and the anterior column B, upon one side are
affected by sclerosix, Thix combination, which specially indicates a vascular
origin, is not rare. Westphal, Strampell, Sioli, Kabler, and Pick, &e., have
observed and given illustrations of cases in which it existed, but an islet of
dogenoration was asually found to exist also in the lateral eolamns,

|

INFANTILE SPINAL PARALYSIS. AIS

known since the memoirs of Duchenne of Boulogne (the father
and son) and the thesis of Laborde were written.

Such is the history of the disease as regards its clinical
aspect. The history as regards the pathological anatomy is no
less interesting, in which the works of Prevost and Vulpian,
Charcot and Joffroy, of Roger and Damnschino, hold a pre-
ponderating place.

I shall return, gentlemen, to most of these works, and speak
also of others. I wished, however, to commence by enumerating
the works of which T have just spoken, since the names just
mentioned are connected nob only with the history of infantile
paralysis but with that of disenses of the nervous system in
general. It was, in fact, by their observations that the first
ideas of medullary pathology were established, and the researches
of Prevost and Vulpian, Clarke, Charcot, and Joffroy, proved
undoubtedly the connections which exist between lesions in the
anterior cornua of the spinal cord and muscular atrophy.

Before, however, considering the pathological anatomy of
infantile spinal paralysis, interesting as it is, I would first,
gentlemen, make you thoroughly acquainted with the symptoms.

The mode of onset is of great importance, a fact upon which
I shall insist when discussing the pathology of the affection.
In a general way it resembles in every respect the onset of
acute febrile diseases ; the little patient is found, in fact, to be
suffering from high fever, the temperature rising, perhaps, to
103° or 104° F. (39° to 40° C.), or higher. The duration of
the febrile symptoms varies, and while sometimes this does
not exceed 36 or 48 hours, it may extend over several days.

At this time the gastro-intestinal derangements which usually
exist in acute diseases occur, namely, loss of appetite, a coated
tongue, diarrhoea, yomiting, &e., of which the intensity varies in
different patients. These are merely the ordinary accompani-
ments of the febrile condition and have no special connection
with this disease,

Symptoms associated with the nercous system often occur,
which may to a certain extent be qualified as “ general,” con=
sisting in a more or less pronounced condition of somnolence,
sometimes amounting to coma, which terminates in death.
Lastly, convulsions at times exist, but nob in my opinion as

often a3 come authors have stated to be the ease.

or one of the upper and both of
paralyzed, or perhaps the upper ex!
the lower upon the other. Lastly,
one side of the body is paralyzed
unafiected (hemiplegic form), ,
At this time the whole limb is often |
being possible to localize the affection in or
muscles. Tn a short time, however, this it
At the end of a few days, in fact, some of
to regain their power, and the patient is
termed the period of regression or sieation
This period usually lasts for about one or
day to day, so to speak, the extent of the
and instead of the whole extremity being :
of the limb are soon found to be alone aff
the first time, possible to ascertain the ex:
and to observe which muscles are involved,
interesting as reyards the seat of the lesion
Certain observations have been made as
muscles which should be mentioned, alth
applied to every case.
Tn the deltoid muscle the fasciculi may
by the paralysis; Sie demienlanserten b
same time as the upper part of the set

INFANTILE SPINAL PARALYSIS.

the tayra spinatus and rhomboid. The clavicular portion of the
trapezius, which receives its nerve supply from the spinal
accessory, is not paralyzed in conjunction with the other fascicali
of that muscle (Kirmisson).

In the lower extremity, the libialie anticus may be paralyzed
quite separately from the peronei and exleisor muscles of the toes.
At the same time, it must be remarked, that as one would be
far from expecting in a disease which is so definitely localized
in the cord, the dissociation of the muscular districts affected is
by no means so definite as might have been supposed.

The motor nuclei in the bull may also be paralyzed. Médin,
in the epidemic which he observed, found that the muscles
supplied by the 6th nerve, the portio dura, and the hypoglossal
nerve were affected; this fact should perhaps be accepted with
some reserve, although there is nothing improbable in its
occurrence,

The sphincters are usually unaffected, at the same time
incontinence may exist, but this rarely occurs except at the
onset and during the period of invasion. —

The period of regression continues, as I have already said,
during 1 or 2 weeks, the muscles of which the motor nuclei are
not or but little affected regain power, the paralysis which
existed in them having been due, #0 to speak, to the shock pro-
duced upon them by the vicinity of the focus in the spinal cord
now gradually ceasing, It is quite different, however, with
respect to the muscles, whose nuclei have been more or leas
completely destroyed by the lesion: these not only remain
paralyzed, but a new symptom, atrophy, occurs, indicating the
degeneration which exists in them.

A new period in the disease now commences, the period of
deformities. These may be of various kinds, and are due to
causes which we shall have to stady in a few moments.

‘The most frequent and characteristic of these deformities are
the following :-—

The elub-foot which usually exists, is the form eguinc-rurus,
vither alone or combined to a varying extent with flat foot.

Club-kand, of which the form varies in different patients, may
exist, and is often complicated by more or less pronounced
deformity of the fingers.

Various deformities occur in the drunk, from simple scoliosis

|

M16 LECTURES ON DISEASES OF THE SPINAL CORD,

to the more complicated position which is termed by the popular,
but at the same time expressive name of cul-dejatie, In the

Fig, 283,—A patient of tho male sex affected for a long time by infantile paralysis
of the left arm and shoulder, The atrophy is very pronounced. (Damaschine

collection.

latter case, the inferior part of the trunk and lower extremities
have been simultaneously affected, and cannot be used by the
patients, either to maintain the upright position or for locomo-
tion.

This deformity is somewhat rare, but in most persons in whom
it exists, is due to infantile paralysis. ‘The same is not the case
scoliosis, which may be due to a large number of

as regards
eases,

In order to understand the existence of these deformities and
the degree to which they extend, a certain number of elements
must be borne in mind :—

The long duration of the paralysis has evidently some effect,
ainly be considered a chief agent, aince in adults
who suffer from atrophic muscular lesions, the limbs are never
‘ound, even er 10, 15, 20 years or more, to be as much dis

trted as in infantile paraly:

The early age of the patients must be specially regarded as

but cannot cer

INFANTILE SPINAL PARALYSIS. AIT

the true cause of the pronounced changes which occur; the
patients in fact are children whose development is still taking

Fig. 224.— Infantile paralysis affecting the limbs and muscles of the
pelvis, and having produced the infirmity which is termed “cul-de-jatte.”
(Damasckiino collection.)

place, and every derangement in the growth of the limbs is
indicated by some deformity which increases that which is due
to the paralysis, and at times becomes exceedingly pronounced.

It must also be observed, that not only are the muscles
affected by paralysis and atrophy, but the latter condition also
exists in the bones, probably for the same reason as in the case
of the muscles, namely, the destruction of certain trophic centres
in the spinal cord. As a corollary to these muscular and osseous
lesions, dislocation occurs in one or several joints of the paralyzed
limb. These dislocations are as much due to predominance of
tone in the unaffected muscles whose antagonists have ceased to
exist, as to change im the direction of the articular surfaces.

‘The paralysis which is associated with atrophy in this disease
has also a certain number of features which contribute to give it
a special character.

‘The paralysis is essentially flaccid, both as regards the con-
sistence of the mascles, the tendon reflexes, and the movement:
of the different segments of the limb with respect to each other.

The tendon reflexes in the affected muscles are usually lost,
both in the upper and lower extremities.

As regards the movement of the different segments of the
limbs with respect to each other, this is in a most abnormal
condition ; if, for example, the leg is grasped in a case of paralysis
of the lower limb, and shaken, the foot is found to swing in
every direction as if it did not belong to a living person, but
to a doll; this condition has heen tertaed the deg of a puppet
(jambe de polichinelle),

ire

INFANTILE SPINAL PARALYSIS. AID

found to be changed, bat usually to such a slight and limited an
extent that it does puesta Co wc
tion of infantile spinal paralysis,

Rejlex action is also unaltered, both as regards the pntanecne
and tendon reflexes in the muscles, which are not affected by
paralysis,

Certain trophia disowlers occur with varying frequence and
intensity. The following are those which most often exist :—

The growth of the limbs is arrested, to which fact I drew
your attention just now, and which explains why it is that the
deformities are so considerable in s

The subcutaneous adiposis (local obesity of Landouzy) which
exists sometimes to a considerable degree in the wasted muscles,
so as even to entirely conceal the atrophy or even simulate
hypertrophy of the limb, ‘This subcutaneous adiposis, however,
is by no means constant.

The coldness of the paralyzed limbs. This coldness may be
very pronounced, and the parents try in vain to prevent it by
covering the limbs with the warmest clothing; the children
persist, however, in having, as it were, * frozen feet.”

Cywnosts, redness, marbling of the skin are the usual accom-

Fig. 226,—Fig, A the ecapnlum and humerus on the healthy side, Fig. B
seapulum and humeras on the side affected by infantile paralysis. The benes
are much smaller than on the sound side,

paniments of coldness of the limbs. An eruption of purpura
has been also known to occur im the wasted limb,

The thinness of the slin is at times 80 pronounced that ex-
coriations or ulcers are produced by the slightest injury, speci-
ally owing to the pressure of the orthopedic apparatus which
the patient hss to wear, or simply from the effect of walking in
boots which press slightly against the foot. When subsequently

Bea hymen enn 421

and is named on that account, acute adult spinal paralysis. Tis
symptoms, wtiology, and pathological anatomy are so completely
identical with those of acute infantile spinal paralysis that it
seemed to me that these two affections should be considered, so
to speak, simultaneously. We shall thus have the advantage of
avoiding repetition and gaining time.

As if to accentuate still more the identity of these two forms
of spinal amyotrophy, one finds the names of Duchenne and
Charcot connected with the origin of acute adult spinal paraly-
sis, Amongst many other works I would also name those of
Moritz Meyer, Bernhardt, Bourneville and Teinturier, B. C.
Seguin, &. ...

The only difference, as I said, gentlemen, is in the age at
which this form of acute spinal paralysis occurs, since it is
entirely the same disease: thus the difference which exists in
its clinical features are, as you would expect, but slightly
pronounced,

The onset is the same. being sudden, aceompanied by fever,
pain in the loins, cervical region, and at times in the limbs, with
or without the sensation of tingling. Gastro-intestinal disorders
may also occur more or less resembling those due to gastric
trouble. Symptoms connected with the nervous system are
often also observed at this time, These, however, do not consist:
of convulsions, as in children (you know that in early life con-
vulsions are, so to speak, the common mode of response on the
part of the nerve centres to different forms of irritation, whereas
in the adalt this is no longer the case). At this time of life
cephalalgia, somnolence, and more or less pronounced delirium,
are more liable to exist.

Paralysis occurs either at the end of a few hours or after
a day or two; the limbs are specially affected and rarely the
sphincters; the same parts are liable to be affected as in cases
of infantile paralysis,

In the same way again, as in the latter affection, after an
interval of a few days the period of regressim exists, in which the
paralysis only persists in the muscles which will be permanently
paralyzed.

The second period is followed by a third, “ that of regression,”
during which the size of the muscles diminishes and the
muscular fibres, remaining paralyzed, are destroyed, ft

422 LECTURES ON DISEASES OF THE SPINAL CORD.

Tt is useless to insist upon the character of the amyotrophy
since it is almost identical with that already described with
respect to acute infantile paralysis; the electrical reactions, the
flaccidity, the loss of tendon reflex in the affected muscles, are
the same. With respect to the deformities, the same changes do
not occur as in children. In the adult, deformities are less fre-
quent, and invariably far less pronounced in degree, as will be
easily understood from the fact that growth having ceased, less
extensive deformities are liable to occur in consequence of the
paralysis.

The truth is that acute adult spinal paralysis rarely occurs,
far more rare, in fact, than the infantile form, though I can
give no reason for this fact. It is perhaps on account of the
grey matter being more vulnerable in early life. This is merely
a supposition, but is one which appears to me somewhat probable,
on account of the clinical symptoms and pathological anatomy
of the disease.

125,

LECTURE XXXY.
INFANTILE PARALYSIS (confinwed).

AS KOmRAL Forms. Insidious onset ; onset daring convalescence after an

which it bas boon known to occur. Epidemics of infantile paralysi«: Cordior,
Leegard, Medin, Bergenholtz, Colmer, Briegleb. Hereditary influence. The
age at which infantile paralysis usually occurs is between one year and cightcon
months. ‘The sexes are equally affected.

GextLemen,—We considered in the preceding lesson the
symptoms of infantile paralysis and the course which it followed
in ordinary cases. I have insisted sufficiently, I think, upon
the character of the onset, which is accompanied by the usual
symptoms of a febrile affection, the occurrence of paralysis to a
somewhat extensive degree, to the subsequent period of
regression, and lastly, to the atrophic period, or rather condition,
which may be considered the result of the preceding stages. T
do not wish to repeat these facts, but would remind you that if
medical pathology is based upon general facts, clinical practice
is concerned with individual cases alone; the course which I
have described is that which usually occurs in this disease; but
exceptions occur, and the ordinary type of the disease is often
modified. It is of these abnormal cases that [ would speak
to-day,

The onset, instead of causing great distress, may on the
contrary oceur so insidiously that those living with the child
observe nothing extraordinary in its condition, I am not
alluding, gentlemen, to the cases in which a child is entrusted
to a nurse, and returns after being under her care in a paralyzed

INFANTILE PARALYSIS, 425 7

upper part of the spinal cord or medulla oblongata, and T au:
convinced that some children in whom death has occurred, as it
was supposed, from meningitis really suffered from.
infantile paralysis, in which the lesions so rapidly produced «
fatal result that the clinical symptoms of tha etarean: could not
occur.

As regards the course and termination of infantile paralysis,
T must dwell upon a most singular symptom, which is not
extremely rare, and the knowledge of which we specially owe
to Ballet and Dutil,* although some authors had previously
published examples of its occurrence (Charcot, Vulpian,
Seeligmiiller). The following is what occurred: a patient,
who at the age of 2 or 3 years for example had suffered from
infantile paralysis affecting either the upper or lower extremity,
and who has attained the age of 15, 20, 30 years or more with-
out being troubled by the disease in any additional way, finds
that he is suffering in a somewhat serious way, Movements.
either of the segment of the limb affected by paralysis, or of the
parts which adjoin it, become more difficult; the power of the
muscles, which is already much diminished in this region,
becomes progressively decreased, and what is worse, this
diminution of the inuscular power is observed to exist not only
in the muscles, which are affected by atrophy, but in those in
which there is no sign of this condition. Progressive amyotrophy
exists, of which the progress occurs more or less tardily, but: is
continuous with a pronounced tendency to generalization. It
must also be remarked that in some cases this second onset of
amyotrophy may be found (Rémond of Metly) to commence in
the same limbs as were paralyzed in the initial stage of the
disease, and which recovered power during the period of re-
gression. ‘These facts seem at least to show that at the time of
the invasion of the disease a lesion had existed in the part of
the spinal cord which corresponds to these limbs, and that at
a later period the morbid process, which seemed previously to
be dormant, or to have ceased, became again active. How
general the affection may become I cannot say, on account of
the small number of observations made; but it is certain thet
not only the muscles of the limbs, but also those of the trank
may be involved in this new amyotrophic process. Thus a

* Ballot aad Datsl Revue de M idécine, 18%,

INFANTILE PARALYSIS, 427

Syphilitie psondo-paralysis is also an affection which occurs
in early life; the works of Parrot, Troisier, and Dreyfons have
shown that this affection may involve one or more limbs; as for
example, one of the upper and both of the lower extremities,
and that the attitude of the affected limbs which hangs down in
4 motionless condition mach resembles that which is due to
paralysis, specially infantile paralysis. At the same time these
authors have shown us by what means a mistake of this kind
may be avoided ; owing to their observations we know that in
these cases true paralysis does not exist, but only a loss of
power in the limbs, a false paralysis due to a lesion in the bones
at the junction of the epiphysial cartilage with the diaphysis.
Slight attention to the case soon enables the following symp-
toms to be recognized: somewhat pronounced swelling of the
limb specially near the extremities of the affeeted bones ; the
erepitation near the joints; the maintenance of the electrical
reaction; preservation of voluntary movements in the extremi-
ties (hands, feet, fingers); pain which is often very severe in
the paralyzed limbs, but without sensory disorder in the skin;
a tendency to progressive aggravation of the disease, the limbs
being affected one after the other; lastly an eruption, which is
clearly of syphilitic origin, often adds the Iast touch to a picture
which is already most characteristic.

Infantile hemiplegia of cerebral origin is sometimes confused
with infantile spinal paralysis, since it produces in the same way
paralysis of the limbs with atrophy, and under its influence
considerable disorders may occur in the muscles and bones; in
some cases monoplegia and not hemiplegia exists. In this
affection, however, the tendon reflexes are excessive, instead of
the reverse occurving as in infantile paralysis, attacks of epilepsy
often co-exist, and the most varied conditions of intellectual
defects amounting perhaps to complete idiocy are not unfre-
quently observed, whilst, as we have already observed, nothing
analogous is found to occur in infantile paralysis.

Myepathie atrophy, in whatever form it may occur, can
scarcely be confused with the disease which we are now con-
sidering; it never, in fact, produces atrophy of a limb or even
of the segment of a limb, but wasting, which is exactly confined
to certain muscles, and which is seated in the root of the limb
rather than in the limb itself,

426 LECTURES ON DMSEASES OF THE SPINAL CORD.

A form of muscular atrophy must be also mentioned whick
occors in early life and which is known, at any rate in France,

227.—A girl affected during the first year of hor life by infantile hemiplegia
of corbral origin affecting tho left side. ‘Tho loft arm is shorter and low
developed, pronounced club-hand existing. The eamo remarks may be made
aboot tho left lez, (Patient under the care of Charcot. Stereotype of Lone.)

by the name of the Charcot-Marie form, in which both the
lower and upper extremities are thus affected by pronounced
atropby. What distinguishes this form of wasting from the
atrophy which exists in infantile paralysis is its onset. which

| d

INFANTILE PARALYSIS.

is essential and progressive; the almost perfect symmetry in
the muscular changes which exist upon one side of the body
or the other, and the absence of, or slight degree in which
trophic disorders affect the bones is also characteristic.

Lastly, the observations which I am making about the
diagnosis must not be brought to a close without my reminding
you that in some eases, which though certainly rare, have un-
doubtedly been shown to exist (A. Chauflard), children who have
been long affected by Aysterical paralysis of a limb, present, on
account of absence of growth, such want of development in the
size and length of the limb, that one might be tempted at first
sight to believe in the existence of infantile paralysis. When
this happens, the history of the case, the electrical reactions, and
tendon reflexes which are retained, and the recognition of other
hysterical symptoms prevent the occurrence of any mistake.

The etiology of infantile paralysis must be fully considerea
since, as you all see, gentlemen, unless this is completely under-
stood, the true nature of this affection cannot be realised.

The effect of ‘cold, and of injury, have, as you may well
suppose, been invoked by numerous generations of authors. 1
have had upon several occasions to give you my opinion as to
the importance of these so-called ordinary causes; I will not
repeat my observations upon this point, and merely say that
at most they can only be looked upon as occasional causes.

The stiological consideration upon which Duchenne of
Boulogne for his part most willingly insisted, was dentition,
According to that author, by far the most cases occur at the
same period of infantile development, and often at the very
moment when one or more than one tooth is actually piercing
the gums. You know, gentlemen, how great a part “the period
of dentition” played in connection with the diseases of children
in old times; you also know that a reaction, certainly excessive
as every reaction is, has occorred during the last few years.
After being regarded as the keystone of pathology in childhood,
this period has come to be looked upon as of no importance
whatever. I must admit, and I believe that my opinion is
shared by a certain number of clinical observers, that dentition
seems to me a state which should not be disregarded. Not that
it can itself be the direct cause of any disease, but because it
constitutes a period during which the organism is fatigued,

i

430 LECTURES ON DISEASES OF THE SPINAL CORD.

overwrought, weakened, either by the pain
sleep, or the diarrhaa which is so often:
eruption of teeth. The organism which is thus fa
‘on that account i cl ne he ie a
infection. Such is the part which I be
in infantile paralysis. This is a time of
organism being depressed, in aie ee
eg liable to suffer from the different
which may be followed by infantile paralysis. Dentition
an actual cause, but a period during which this

My firm conviction, in fact, is, gentlemen, that
paralysis invariably, or almost invariably, depends upa
existence of some general affection, usually of an ‘infectious f
This has been my opinion for many years ; and though I de
believe that it is confirmed as yet by many adherents in
this is of little consequence, and certain presenti 0
to believe that ‘the time approaches” when this
case. I hope, in fact, that I shall be able, if
you all, at any rate to obtain some converts
reserving until another occasion a state
which are farnished by its patholog
place before you those which are f
features alone.

As regards the onset of infantile p
length upon the different general
time; it is unanimously agreed th
to those which exist in acute febrile
possible that the acute disease may hi
resulting from some “ unknown affects

theory, the nature of the
what may be called the fand
Tt is during the course of 1
pox or whooping cough, th
oceurs.

Lastly, one of the
have the most weight, is that
nature of infantile paralysi
observations upon this subjeq

INFANTILE PARALYSIS. 431

Cordier® in 4 communication made to the Société des Sciences
Médicales of Lyons in 1887 states that he saw in two months
(June and July 1885), at Sainte-Poye-l'Argentiére, in a popu-
lation of 1,500 persons, the occurrence of 13 cases of infantile
paralysis; whereas in other years nothing analogous to this
took place,

Leegard+ mentions on his part the fact that in the small town
of Mandal (Norway), Oxholm and his colleagues observed 8 cases
of infantile paralysis between the ond of July and beginning of
September, this having never happened previously.

Medin{ has also recognized cases of the same kind, but in far
greater number. In the spring of 1888 he had already seen 5
cases, and after August the number increased to such a degree,
that the author met with 44 cases before the month of Novem-
ber. It is true that amongst these cases there are some which,
as I have already said, cannot be regarded as true examples of
the disease in its ordinary form. It cannot, however, be said
that they were fundamentally different from the more typical
cases of this disease, and, when the whole statement is considered,
eases formed but a small minority which could not seriously

ie the comprehensive statistical observation made by

of 13 cases at Umea, and that G.
of the parents of a child suffering
f from them that in their district,
i nd in 3 or 4 months, from 8
affected.
by Briegleb || in his clinical
of June and July, it should
om places which were so
npied by the train in
| value in connection

hé preceding.

LECTURE XXXVI,
INFANTILE PARALYSIS (continued),

ParuoLooicaL axaromy.—History of the diacovory of changes in the anterior
horn#: Vulpian and Provost (1885), Lookhart Clarke, Charcot and Joffroy,
Parrot and Joffroy, Roger and Damnschino, (a) Character of the lesions when
the affected focus is of ald dote, lesions in tho anterior horas, nature of these
lesions many foci usually exist which are rarely quite symmetrical ; atrophy of

alterations in the blood vessels. a prucomaey cepa eet
focus ia of recent date: every appearance of a true inflammatory focus then
exists, this focus of acute myeliti« may extend to the adjoining white matter ;
the reason of this fact probably eonnectod with tho distribution of blood reasele
in that region. Noxwan Awatomx of the blood vessels suppticd to the anterior
horn. ANTERIOR SPINAL sysTRM.—Awerlor spinel arterg; anterior median

infantile paralysis.
Identity of infantile spinal paralysis with infantile cerebral hemiplegia; my
disagreoment with Visioli and Strimpell. Observation of P. J. Mobias, This
explanation af what occurs enables tho late “ renewal” of the clinical course
of infantile spinal paralysis, which has been considered to be understood.
Tuxrarxutics.— Electricity, massage, hydrotherapy, orthopmdic apparatuses,

Tue pathological anatomy of infantile paralysis deserves full
consideration, not only on account of its own merits, but also
becanse it is the subject of numerous works which explain the
nature of this affection, as you will see; these confirm what has
been already said when the etiology was considered,

The time is far distant at which it was generally believed, as
by Rilliet and Barthez, that the disease had no material cause,
and was a “ form of paralysis eesential to childhood.”

Atone of the first autopsies made by Cornil and Laborde, tlie
existence of very pronounced changes was recognized in the
anterior and lateral columns. I shall presently have occasion to
insist upon the importance of such alterations.

The work of Prevost and Vulpian was written in 1865; these

Ww

INFANTILE PARALYSIS, 435

strongly coloured masses of protoplasm without processes and
with a round or obtuse margin, perhaps the relics of these cells
being found in places. In the whole affected region a network

Pig. 223.—Lumbar cord in a case of infantile parnlysix. (A.) Focus of old date
reated in the antero-external part of the left anterior horn ; it will be obrerred
that the left half of the cord taken as a whole is tmaller in size than the right
half.

of fibrils of the same nature as the neuroglia and with somewhat.
small meshes is found to exist; some of the cells termed cells of
Deiters being also found in this part. The nerve fibres, which
are normally seen in every part of the grey matter of the
anterior horns, are completely destroyed throughout the affected
region, or at any rate in much smaller number. The blood
vessels are usually thickened, seeming at times to be dilated,
while in some cases they appear to be much increased in number
on account of the retraction of the affected part, owing to which
they are contained in a smaller space.

These changes do not at the same time affect the anterior
horns throughout the whole length of the cord, they are also
usually unilateral, and in the rare cases in whieh both sides are
involved the diseased parts are never quite symmetrical, the
changes oceur commonly in the form of foci, which extend over a
length of one or several centimetres (from a quarter of an inch
to one inch) or more; 2, 3, 4, or more of these exist, and may
be found upon both sides of the cord, so that, as you haye seen,
the left arm and right leg may be affected in the same patient ;
or if the cells connected with the muscles of the upper and
lower extremities are contained in the foci, the infantile paralysis
may assume the hemiplegic form; these foci of disease may be

INFANTILE PARALYSIS. 437

noted by certain authors (Rampf, Colella,* Fornariot), who
observed that the motor convolations of the brain, which

Fig. 299,.—Section of the spinal cord from & ease of old infantile paralysis. (After
Charcot.) It will be observed that the white substance and groy matter upon
the right side, which is the seat of the lesion, are uffocted by considerable
atrophy.

corresponded to the paralysed limbs, were also affected by a
certain degree of atrophy, this again resembling what occurs in
amputation of the limbs (7). Theoretically this is hy no means
improbable ; but if, gentlemen, you ever undertake a research of
this kind you will soon perceive how extremely difficult: it is to
recognize so small a difference between the two

The anterior roots are recognized by the naked eye to be clearly
diminished in size, though under the microscope scarcely any
lesion, according to Joflroy and Achard.t is found to exist, and
islets of sclerosis are nob present as would have been expected
to be the case, The authors, whom I have just named, are of
opinion that this is due to the fact that the lesions in the
anterior horns having occurred during the time of their develop-
ment the fibres affected by secondary degeneration have dis-
appeared, the sound fibres, which previously adjoined them,
having taken their place, so that no vacant place exists which
might be occupied by the islet of sclerosis. A somewhat large

* Colelia, La paralisi spinale atrofica infantile in rapporto con i centri cortical,
ke. La Paichiatria, anno VIL.

. Neropathelogia, May-J
J doffroy and Achard, Archives dz Médécine Experimentale, 189)

INFANTILE PARALYSIS. 439

different points of the cireomference of the transverse section
of a long bone (Joffroy and Achard).

Microscopical examination shows, again according to these
authors, that in a general way the Haversian canals have a
smaller diameter than in the normal state; this diminution in
their diameter is specially marked in the deeper parts, where
the diameter of the system may be only half of its normal size.

Lastly, the changes which occur in the blood vessels of the
paralyzed limbs must be mentioned, These are sometimes !
much diminished in size, their walls being much thinner, and
they are affected by true atrophy, or at any rate very pro- |
nounced disorder in their development, on account of the
derangement which exists in the trophic functions of the spinal
cord. This atrophy in the vascular system may also affect the |
growth of the whole limb, and its small should be partly i
ascribed to this cauze, !

Such, gentlemen, ara the different changes which are found |
to exist when death has occurred several years after the onset of |
the affection. However interesting these alterations may be, as
J have already said, they are of but slight yalne as an indication
of the nature of infantile paralysis, whilst the study of lesions
which have recently occurred has enabled the most important
observations to be made; it is these which we will now consider. i|

B. The autopey takes place at the end of a faw days, or at moat |
Jive or six weeks :

Tn these cases the induration and sclerosis, the cicatrix as it
may be termed, is not found to exist in the part; on the other
hand a focus of true injlammulory softening (Roger and Damas-
chino) is recognized in the anterior horn, A moderate magni-
fying power shows that the details of this portion of the grey
matter are not clear, numerous granular bodies being seen to
exist, which are either free in the spaces formed by dissociation,
as it were, of the tissue by inflammatory effusion, or enclosed in
the meshes of the nervous tissue, or blended together in the
perivascular lymphatic sheaths.

Within these foci the nerve cells have either completely
disappeared or granulo-fatty degeneration is found to exist
in them, or again, simple atrophy with a tendency on the part
of the processes to disappear, so that at a later period they have
the form of the more or less rounded masses to which I have

INFANTILE PARALYSIS,

the external portion of the anterior horn. These foci, due to
acute inflammatory softening, are not therefore, as most authors
state, essentially limited to the grey matter but may also involve
the white substance.

‘This lesion iz not therefore, really systemic in nature, as will
at first sight appear, since it passes beyond the, limits of the
grey matter. What is the cause of this pseudo-systemic cha-
racter? In my opinion, gentlemen, its cause must be sought
in the distribution of the blood vessels within the spinal cord.
I hope to be able to show you that by the knowledge of this
distribution we shall be able to understand the singular nature
which exists in the seat of the lesions in infantile paralysis.

When speaking of combined lateral and posterior sclerosis
T had occasion to consider the intramedullary blood supply.
but my principal object at that time was to discuss the arteries
of the posterior spinal system. To-day it is the ANTERIOR
system which I shall take as my subject.

This system is composed, as you know, of the anterior spinal
artery formed by the anion, at the upper level of the cervical
cord, of the two descending branches supplied by the vertebral
arteries: the anterior spinal artery is seated in front of the
anterior median fissare, and can be traced as far as the cornas
medullaris; in its course it is joined by a certain number of
branches of the lateral spinal arteries.

The anterior spinal artery gives off a series of somewhat large
branches at right angles to its course which pass backwards
in the anterior fissure and constitute the anterior sinedian
arteries.

Each of the anterior inedian uvteries passes into the anterior
horn upon one side of the cord which it supplies with blood ;
on that very account of this artery is the seat of a serions Iesion,
the corresponding anterior horn will be also affected. In should
be observed, gentlemen, that the anterior median artery passes
to the end of the anterior fissure, and as Kadyi has shown,
either to the right or to the left into one of the anterior horns,
and does not divide so a3 to supply these horns on both sides ;
this explains the fact that the lesions in infantile paralysis are
nearly always wnilateral, In addition to this the extension of
the lesions into the antero-lateral column is quite intelligible,
since one or several branches of this arteriole in the anterior

442 LECTURES ON DISEASES OF THE

wie)
adjoins the anterior horn. Sah

It may also be supposed that the primary lesion in infantile
paralysis, which is constituted by a focus of ceute polio;

Isteral artery; F, middle Isteral artery. Two
infantile paralysis aro represented, of which
(M) ix dee to primary lexion in the branch of the
passes into the anterior horn, it is entirely
other (L) is due to a primary lesion seated in one
arteries, it extends into the white substance of the

myelitis, does not result from an affection of the
median artery, but of one of the anterior radicular
The seat of these arteries enables the fact that the anter
column is affected, and the assymmetery of the
explained. It is possible that these arteries
to be the seat of the vascular lesion which
this difference of seat explains certain vari
the symptoms. What, however, is th
vascular origin? Tt is in the
men, that we shall find the "
not certainly have forgott
of the infections nai
the febrile symp

INFANTILE PARALYSIS.

account of these facts, solely from a clinical point of view, we
decided that the diseasé was of an infectious nature.

Owing to what we are taught by normal and pathological
anatomy, we can now push our conclusions still farther, and
state that it is through the medium of the vascular system that
this infectious disease involves the spinal cord. As to the
exact process, by means of which the lesion is produced, it is
difficult to observe it whilst this is actually taking place; at
the same time it seems to me most probable that in reality an
infectious form of embolism exists, or that thrombosis occurs in
one or several arteries of the anterior median fissure.

It may appear to you strange that these arteries are the
special seat of this morbid process in such a comparatively
large number of cases. The fact, I allow, is singular; but as I
previously observed to you in connection with combined lateral
and posterior sclerosis, it is far from being unique, and other
examples of its occurrence can be mentioned. Is it again quite
certain that the anterior median and anterior radicular arteries
are the only vessels affected by the morbid process? I believe,
for my part, that a certain number of the acute nervous symp-
toms of childhood, called for the most part by the mistaken
name of “ meningitis,” are only cases of arteritis, or infectious
embolism, it occurring in different parts of the nervous system.

In connection with this subject I should mention the analogy,
I would even say the identity, which exists, as regards its
pathology, between infantile spinal paralysis and infantile hemi-
plegia, which is due to a cerebral cause. I do not mention this
identity for the first time to-day; I have done so constantly
since 1885, and I hope to be able to convince you, gentlemen,
that this opinion is in conformity with the facts which really
occur, ‘Two eminent pathologists, Vizioli and Strimpell,* pre~

INFANTILE PARALYSIS.

is indicated, as it seems to me, by the fact that they can be
asked.

Upon examining the different elements of the problem, one
fact must be considered specially from this point of view. We
have seen, gentlemen, that in some cases, after 10, 15, or 20
years the amyotrophic process which had seemed to have

- completely ceased, recurred in a most unexpected manner,
and became general. To what is this recurrence due? One
might be tempted to suppose that this was an additional =
that the affection was due to the effect of mi
microbes existing in the cicatricial foci becoming erp hic
owing to some as yet unknown influence, and finding a soil
favourable to their production in the grey matter of the anterior
horns, determine by the pullulation this progressive form of
amyotrophy. Examples of such long periods of rest, followed by
sudden and pronounced activity of the micro-organisms contained
in the bones or skin, are undoubtedly known to exist, specially
in certain cases of osteo-myelitis or furanculosis; but whether
this justifies the above statement must be decided by future
observers.

While still basing my observations upon clinical facts, which
are of more definite value, in connection with the “recurrence "
of the amyotrophic process, I would direct your attention to
another analogy which exists between infantile spinal paralysis
and infantile hemiplegia of cerebral origin. In the latter
affection it mot unfrequently happens that 5, 8 or 10 years
after the occurrence of hemiplegia, when the lesions may be
believed to have passed into a cicatricial condition, attacks
of epilapsy occur, often accompanied by a recurrence of the
paralytic symptoms, a recurrence of paralysis which exactly
resembles that which occurs in the cases of infwntile paralysis,
to which I have made allusion. ‘The latter analysis should, in
my opinion, be noted, since it shows that the fact is not merely
a fortuitous incident, but due to a process which is the very
essence of these affections.

The treatment adopted in infantile spinal paralysis is
unfortunately far leas beneficial than might have been

Daring the period of invasion, the object of the treatment
should specially be to reduce the pyrexia, and in fact to combat
the infectious disease of which it is the index: sulphate of

447

LECTURE XXXVII.

AMYOTROPHIC LATERAL SCLEROSIS.

History ;-—Disease of Charcot. Stmvroms: A, Spasmodic aymplome: excess of
tendon reflexca in the lowor and upper limbs, foot-clonus, tendency of the
limbs to pass into « condition of contracture, or at any rate of spasmodic
rigidity. B. Paralysis, C, Musewlar atrophy, its seat, existence of fibrillary
contractions ; electrical reactions. D. Bulbyr symptoms; paralysisand atrophy
of the maxoles of the lips, tongue, and soft palate; difficulty in mastication ;

modos
of onset: A. By atrophy of the upper limba; B. by bulbar symptoms ; C. by
spastic paraplegia. Duration of the disease, Termination inevitably fatal.

GentLemEN,—It may be said of amyotrophic lateral sclerosis
that, like a certain goddess of antiquity, it issued from the brain
of its creator in a completely armed condition ; the history of
this disease may be simply comprised in these three words. As
early as 1865 our distinguished teacher remarked at the autopsy
of some patients, who had suffered from progressive amyotrophy,
that sclerosis existed in the lateral columns ; in 1869, in com-
bination with Joffroy, and in 1871 with Gombault, he observed
other cases of the same kind; lastly, in 1872 and 1874, in his
well-known lectures, Charcot established the existence of this
affection, which he distinguished clearly from the common form
of progressive muscular atrophy, In 1877 the thesis of Gom-
bault appeared, and in 1879 the work of Debove and Gombault.
From that time amyotrophic lateral sclerosis was a definite
morbid condition, both from an anatomical and clinical point of
view. As the lecture continues I shall name to you the princi-
pal works written about the disease since that time.

The symptoms of this disease are directly indicated by its
name, which has been formed ina logical manner. Thus, lateral
selerosis signifies the existence, not only of paralysis, but also of
spasmodic symptoms, while the term “ amyotrophic” indicates

AMYOTROPHIC LATERAL SCLEROSIS. 419

and any attempt to separate them is opposed by the, action
of the shoulder muscles,

Fig. 283,—Woman affected by amyotrophic lateral sclerosis. This figure shows
tho atrophy of the hands and the position in whieh they were usually placed
by the patient; the logs, in which the atrophy is much leas pronomneed, are
clowe to each other, the fect being slightly rotated inwards, in short the
charactors of slight spasmodic rigidity are observed in the lower imbs.
(Collection of Charcot.)

The fore-arm is semi-flexed and in pronation, nor can it be
placed in the position of supination without much force being
employed and pain produced,

“The same may be said with respect to the wrist, which is
also seini-flexed, whilst the fingers are also strongly flexed into
the palm of the hand.”

As an annex to these spastic symptoms tremor bas ‘been
mentioned in some cases to occurin the limbs when voluntary (7)
movements are made, which would be a mitigated form of the
spasmodic tremor which exists in insular sclerosis. In concluding
these remarks about the spastic symptoms I would call special
attention, gentlemen, to the great difference of intensity which
may exist in them; they exist sometimes in the form of con-
tracture, sometimes in the form of simple rigidity of the limbs,
while at other times nothing of this kind occurs, though in all
29

AMYOTROPHIC LATERAL SCLEROSIS, 451

this is due to the fact that tendinous retraction has occurred
during the occurrence of these different processes.

Fig. 234—A man affected by amyotrophic lateral sclerosis, in whom extreme
spastic rigidity existed, involving all the muscles of the body. i
condition is indicated in the figure by the attitude of the pationt, who,
the muscles of the log were in a sound condition, could not maintain his
equilibrium without assistance, ‘The atrophy of the muscles in the hands will

be also observed. (Collection of Charcot.)

The muscular atrophy which exists in amyotrophic lateral
sclerosis is a form of atrophy in which the jibrillary contractions
are extremely pronounced ; this is often observed to be the case
in muscles which, at first sight, appear to present no sign of
atrophy; this is an important sign, which I recommend you
always to investigate with great care, since it will indicate to you
the muscles which will be affected in the course of the disease.

The electrical irritability varies in different eases, probably on
account of the fact that, as I have already said, the fasciculi of
the muscles are attacked one after the other; usually there is
diminution in the electrical irritability. In some cases the
reaction of degeneration is found to exist, but usually in a few
muscles only at the same time, and without its being very
clearly defined.

i
450 LECTURES ON DISEASES OF THE SPINAL CORD, |

cases the tendon reflexes are found to be excessive in both the
upper and lower limbs.

In addition to this spasmodic condition it must be mentioned |
that a certain degree of paralysis exists often from the first,
and which is due to a lesion in the lateral columns, On —
this account the limbs, specially the lower limbs, are moved
with much more difficulty than would be expected to |
on account of the small degree to which atrophy exists im
muscles.
This muscular atrophy, which constitabes the seco p

the symptomatic history of amyotrophic Iateral scl
certain number of special characters.

As regards its seat, the upper!
hands, in which, in fact, it eon
thenar eminences are first
affected at an early
disorders is that the
singe ") in its most ch
flattened, with the first pl

the arms above the
usually to a less degree.

In the lower limbs th
a very late period, after b
long time, by spastic paraly

Briefly, gentlemen, in
deformities and attitudes wl
lateral sclerosis the followin
paralysis, contracture,
traction must also be
contracture ceases to exist
sufficiently pronounced to hat
imuscle, and yet the de

452 LECTURES ON DISEASES OF THE SPINAL CORD.

‘Until the present time, gentlemen, we have specially con- ,
sidered muscular atrophy as it occurs in the limbs, we must
now study its existence at a higher level, namely, in the parts
supplied by the bulbar nerves. :

The symploms connected with the bulb ave, in fact, of special
interest in the disease which we are now ing, constituting,
as they do, the special gravity of the

The muscles connected with the lips (the orbicularis ois,
quadratus menti, depressor labii inferioris, and levator menti)

Fig, 235,—A patient affected by amyotrophic lateral sclerosis, with most pro-
nounced bulbar symptoms. The lips can scarcely be moved, and the
always kept open; the pronounced character of the nasal furrow, the
in the frontal region, the expression of wonder in the face Will ba Seana
4s clearly seen in the figure that tho hands are affected by atrophy
(Collection of Charcot.)

are sometimes involved early in the disease; the
tractions are sufliciently pronounced in the
muscles to indicate the changes which have
specially as regards the chin, which
depressions and elevations, which
appearance which it presents in
to cry.

When the changes in

AMYOTROPHIC LATERAL SCLEROSIS, — 453

owing to the effects of the paralysis and atrophy by which they
are affected, the following symptoms are observed :—

‘The mouth is open; the lower lip droops, and seems to be
pushed forward, but without being at all curved back; the
upper lip is somewhat raised, so that the upper incisor teeth
become visible, the whole face has a weeping appearance, which
is partly due to the depth of the nasal farrow, which drags the
angle of the mouth downwards; the saliva dribbles constantly
from the mouth, and the patients “slaver.” When they langh

Fig. 236.—Woman affected by amyotrophic lateral sclerosis, The appearance
of the mouth and ohin, and the pronounced character of the nasal furrow will
bo observed. (Collection of Charcot.)

the lips remain at times separated from each other in such a
way that they have to be brought together by means of the
hand. Lastly, om account of the paralysis and atrophy of the
lips the patients can neither blow, whistle, pout, or ae
the sors eand i.

eo when it still retains its normal size, presents
first affected fibrillary movements,
of an experienced eye. When

454 LECTURES ON DISEASES OF THE SPINAL CORD,

and depressions, which resemble depressed cerebral conyola-
tions. Lastly, when the paralysis reaches the most
degree the tongue remains depressed and flattened behind the
dental arches without being able to execute any movement.
Thus, not only does the pronounciation of the Iabials i, r, s, |,
k, g, ch, t, d, n become very difficult, but again at certain times
the speech becomes quite indistinct,
The soft palate, in cases in which the bulbar symptoms are
very pronounced, hangs down in a relaxed condition, being often

Fig, 237.—Face of tho pationt affected by amyotrophic lnteral sclerosis, who ix
the subject of fig. 233. ‘The appearance of the mouth will be obsorved, which
is drawn outwards, as also the depth of the nasal farrows, the wrinkles in the
frontal region, and the expression of astoniehment in the face. (Collection of
Charoot.)

covered by dripping mucosity, The paralysis of this organ,
besides making it impossible to blow or whistle, produces
certain difficulties connected with speech. Thus the patients
find it difficult to pronounce the letters b or p; in addition to
this, and for the same reason, the tone of the voice is very much
altered,

The phrases when uttered are short, and, as it were, clipped;
they have lost the power of so regulating the current of expired
air that a long sentence can be spoken, ‘To this paralysis of
the soft, palate the troubles connected with deglutition must be |
partly referred, specially that which consists in the return of |

AMYOTROPHIC LATERAL SCLEROSIS, 455.

food through the nostrils, Other elements, such as paralysis
of the pharyngeal muscles or of those connected with the
esophagus, also take a part in increasing the troubles con-
nected with deglutition,

At a more advanced period of the disease mastication is only
effected with difficulty, and the lateral movements of the lower jaw
are lost, the motor nucleus of the 5th nerve, as was shown by
Duchenne (of Boulogne), being thus proved to be involved, and
the lesion to be consequently seated in the upper part of the
bulb. .

At this time the vagus nerve becomes also affected, and, in
consequence, troubles connected with the respiration (attacks of
suffocation, dyspneea) and circulation of the blood (accelerated
and irregular contractions of the heart, syncope, sudden death)
may occur. It is possibly on account of some alteration in the
functions of the vagus nerve that the passage of alimentary
particles into the respiratory tract and the pmewmonia of degluti-
tion, which is its consequence, are liable to occur,

We have seen, gentlemen, of how great importance the con-
dition of the tendon reflexes in the limbs is in this affeetion, and
what valuable information was derived from them as regards the
state of the different segments of the spinal cord. As regards
the medulle oblongata, similar information is given by the con-
dition of the masseteric tendow reflex.

As there may be some amongst you who do not quite under-
stand the occurrence of this reflex and the mode in which it is
produced, I shall enter into some details in connexion with it
from a practical point of view, since, as regards the theory of its
production, there is nothing to add to what has been already said
with respect to the patellar tendon reflex, This reflex is obtained
by stimulation of the tendon of the masseter muscle, which may
be produced in two ways. One of these consists in directing the
patient to keep the mouth half open, but free from rigidity,
whilst by means of a percussion hammer the tendinous origin of
the masseter muscle from the posterior portion of the malar pro-
cess of the superior maxilla is gently struck. Another method,
described by De Watteville and Beevor, is also to cause the
patient to keep the mouth half open while the medical attendant
places one extremity of a paper-knife, held with the left hand,
upon the teeth of the lower jaw; by means of the right hand a

=

AMYOTROPHIC LATERAL SCLEROSIS. ~ 457

simplicity of the patient, are at times quite astonishing, Lastly,
the symptoms of newrasthenia are sometimes observed, specially
at a beginning of the disease. Always mistrust, gentlemen,

the pronounced neurasthenia which occurs in a sudden and un-
expected manner; when it is due neither to real misfortune, nor
loss of money, nor excessive mental labour, it is almost’ always
the unpleasant indication of a serious disease which affects the
organism in its most important functions. The way in which
nutrition is affected should then be carefully observed, the urine
should be examined in case diabetes exists, and the different
organs of the body, the nervous centres being included, should
be examined, Amyotrophic lateral sclerosis is thus sometimes
found to be accompanied and, 50) Gaerels preceded by the
symptoms of neurastheniay —

The course and symptoms of the disease vary much, both as
regards its duration and mode of onset.

‘The onset, in fact, oceurs in three ways which are quite dif-
ferent from each other.

A. The first symptoms which occur are in the npper limbs ;
muscular atrophy exists, which first affects the small muscles of
the hands in exactly the same way as when other forms of
amyotrophy affect this part.

13, The first symptoms are connected with the medulla ob-
fongute; fibrillary contractions occur, the lips are not easily
moved, there is slight difficulty of deglutition, &c., and it is only
at a later period that the muscles of the limbs are in some way
affected,

C. The first evidence of the disease consists in the occurrence
of spustie paralysis; while muscular atrophy does not exist, or is
so slightly pronounced as not to deserve notice from an objective
point of view.

Thus, gentlemen, the onset of the disease occurs in three ways,
which are so different that an observer who is not warned of
their possible occurrence could not help believing them to be
three distinet diseases.

Has this difference in the mode of onset any effect upon the
course of the disease? To a certain extent it has. Thus, for
example, in the cases which begin by spastic paraplegia (variety
C) the amyotrophy which subsequently occurs is not usually as
pronounced as in the other varieties. This, however, is not

LECTURE XXXVI, ,
AMYOTROPHIC LATERAL SCLEROSIS (continued),

PATHOLOGICAL ANATOMY. I, SPINAL CORD. A, Changos in the grey matter of
‘he anterior horns; atrophy of the largo ganglion cells, inflammation of the whole
anterior born. Dt le eee eee neers
tract (direct and crossed) ; (b) lesions of the whole antero-lateral columns, while
at times the lesions are apparently seated in the portion of theso tracts which

the anterior horns: lesions in the part occupied by the colwnas of Goll,

TL MepuLia omtoncata. A. Changos in the grey matier affocting the nuclei of
the krnaglattal, Reeaeete base one B. Changes in the white matter: the
pyramids, the posterior longitudinal tract (faiscean longitudinal postericur),
the band of Reil (Muratof),

TI, Tax rons VAnoutt,

IV. THe onus cenepnt.

V~. THe BRAIN, ‘The presenoo of graatilar bodice in the internal capsule in the
motor convolutions. Atrophy of the lange pyramidal cells of those convola-
tions, These lesions at the same time aro not constant. Tho method of seeking
the granular bodies.

‘Thoories which exist as to the xaruRe of amyotrophio latoral sclerosis.

Lesions of the ANTERION moots, the moTOR sxRVE-TRUNKS, the INTHA-
MUSCULAR NERVES, and the MUeCLES.

Draawosis, A, Of the amynrophic form from: the muscular atrophy of
Duchenne-Aran (7); muscular atrophy due to pil et ol ‘the different forms
of myopathy ; syringo-myolia ; hypertrophic corvical pachy meningitis; arthri-
tic musenlar atrophy. B. Of the form with spastic paralysis from: transverro
myelitis; spnatic paraplegia; insular aclorosis. C. Of the form with balbrr
symptom: from: soute bulbar paralysis; chronic bulbar paralysis; peeudo-
balbar paralysis.

GextLemen,—I have already had occasion to remark that the
name amyotrophic lateral sclerosis itself indicated the chief
symptoms of that affection ; it also contains an enumeration of
the lesions which exist; lateral eclerosis—changes in the grey
matter of the anterior horns.

I—Srinat Corp.
A. Changes in the grey matter of the anterior horns.
(a) The principal change, or at least the one which is most
evident to the eye, consists in atrophy of the large ganglion cells

Fig. 233,—Normal antorior horn, with ite ganglion o#lls, and
fibrow which when coloured by Weigort's hamatoxyhin give:

horn (eemi-diagrammatic).

All the cells in the anterior cornua, at any rate
the cervical cord, seem equally liable to be af
atrophic process, both those of the anterior and
antero-lateral group. Possibly, by studying:
development it may be possible to recognize
groups are first affected. In the lumbar cord
as a rule remain in a healthy condition than in
region. ’

(H) Tt would be a mistake to think that the large
cells of the anterior horns are alone involved to the
the adjoining parts. The grey substance is, in fact,
affected throughout the whole extent of the anterior h
the change can be traced as far as the base of the pos
horns, and perhaps even in these parts, This alters
grey substance is characterized by increase in
nuclei, a proliferation of the interstitial , a

AMYOTROPHIC LATERAL SCLEROSIS. 461

nounced disappearance of the nerve fibres, which normally form
an abundant network in this part. ‘The lesions in the grey

Fig. 239.— Anterior horn in a case of amyotrophic lateral sclerosis, Tho absence
of most of the nerve cells, and the atrophy of some others will be observed; on
acconnt of the fact that a large proportion of the nerve fibres in this part have
disappeared tho deop coloar which Weigort’s hematoxylin gives normally to
the anterior horn is not produoed (vide fig, 288); on the other hand this deop
hue is produced ta the posterior horn (semi-diagrammatic).

matter seem also to be more marked in the middle of the
anterior horn than at its periphery,

In very pronounced cases the posterior commissure seems to
take some share in the morbid process, by which the grey matter
in front of it is affected. The columns of Clarke are unaffected,
both as regards their cells and reticulum.

B. Changes in the white substance —(a) The white column, in
which the lesion is by far the most pronounced, is the pyramidal
tract; both the parts of which it is composed, the direct and crossed
pyramidal tracts are affected by degeneration in amyotrophic
lateral sclerosis. In this part the sclerosis is extremely pro-
nounced, so much, in fact, that with the naked eye, and when
the part has been for a short time in a bichromate solution,
this lesion is the one which seems in its:!f, and without the uze

AMYOTROPHIC LATERAL SCLEROSIS. 463.

other less extensive than when degeneration occurs after a
transverse lesion as already mentioned; for all these reasons it

A

Pig. 241.—Soction of the spinal cont (the dorsal region) in = case of amyotrophic
lateral sclerosis. The parts affected by sclerosis are whiter in colour in propor:
tion to the degroa of sclerosis, B, Sclerosis in the region of the crossed
pyramidal tract; D. aclerosis in the intermediate regions of the autero-lateral
column ; C. sclerosis in the region of the direct pyramidal tract; A. sclerosis
affecting Goll's columns to a slight degree. 1. Lissauor’s tract, which,
althongh of grey colour in the the diagram, is quite free from discase,

is difficult to define the limit of the affected part as exactly as
would be desirable. In some cases the sclerosis is found not
even to confine itself to the part of the antero-lateral column
seated between the surface of the cord and the anterior horn,
but to be seated in closer proximity to that horn, which it may
almost completely surround, although always most pronounced
in the lateral column properly so-called,

The condition of the Lateral limiting layer (Seitliche Grenz~
schicht of Flechsig) varies; in two cases of amyotrophic lateral
sclerosis, in which this part was specially observed by me, 1
found it almost unaffected in one, while in the other cord it
seemed to be involved in the sclerotic process. The anterior
commissure is also slightly affected in some cases.

(c) The condition of Goll’s columns must be specially men-
tioned. These are obviously the seat of sclerosis, which is
easily recognized to exist, either by the darker hue, which it

know, gentlemen, the influence of some of the motor nuclei in-
the bulb is so necessary to the maintenance of life that in this
case death occurs before the lesions are sufficiently severe to be
recognized by any of the means which we

Amongst these nuclei it is that of the in
which change is most often found to have occurred frente
and disoppearance of the ganglion cells, lesions of the very
substance of the nucleus). The chief nucleus is alone affected,
the nucleus of Roller, and the large cells existing along
the root fibres of that nerve being in a sound condition
(Muratoff).

At times the motor nucleus of the fifth nerve is involved (loss
of the lateral movements of the jaws).

The nucleus of the portio dura and posterior nucleus of the
vagus may also be altered to a more or less pronounced extent.

‘The nuclei of the nerves connected with the movements of the
eyeball are unaffected.

B. Changes in the white substance.-—In the same way as in the
cord it is principally in the pyramidal tracts that the lesions
occur. It should be observed that although these changes very
clearly occur, their intensity cannot be compared with that
which exists in cases of secondary degeneration due to a focal
lesion in the brain. A much larger numbér of sound fibres
always remains, in fact, in this part in amyotrophic lateral
sclerosis than would be found in secondary degeneration asso-
ciated with hemiplegia.

Mauratoff also states that the posterior longitudinal tract of the
bulb is changed to some extent, and observes the analogy which
exists between this tract and the anterior column of the cord,
which we have seen to be also affected in amyotrophic lateral
sclerosis,

The same author mentions lesions of the same kind as
existing in the raphe of the bulb and in the band of Reil
(Roth).

It ehonld be observed that in some cases lesions in ths
white sabstance are found to diminish to a considerable degree
from below upwards, so that they seem to be exhausted in the
medulla oblongata, above which they are no longer found to
exist. In other cases, on the contrary, these lesions can very
clearly follow each other from below upwards beyond the

a

AMYOTROPHIC LATERAL SCLEROSIS,

AMYOTROPHIC LATERAL SCLEROSIS. 467

crus, The lesions of the grey matter in this part, if they exist,
are also unknown.

V.—Braiy.

M. Koschewnikof first noted the existence of changes in the
internal capsule and white substance of the motor

what large number in the two parts which I have just named.
A short time afterwards (December, 1883), ering had occasion
to make an autopsy of this kind I was careful to show the
existence of granular bodies in the brain and spinal cord by
means of processes which enable om elements to be seen in
the very seat which they occupy. The following is the result
of my investigations in connection with this point, the plan of
taking sections by freezing the part having been employed.*

a, In the internal capsule more or less numerous granular
bodies are scattered through the region which anatomy has
shown to be occupied by the fibres of the pyramidal tract, that
is to say the anterior two-thirds of the posterior segment of that
capsule.

4. In the motor convolutions two kinds of changes have been
noted (only it must be understood in those cases in which the

* The following is the plan which I adopt: The nervous centre is placed im
Muller's flaid at the temperature of the surrounding air during from 10 to 20
days. (During the last part of this period Miller's fluid will not be added but
the liquid in the beaker will be maintained at the same level by pure water
which is added in order to make up for the loas due to evaporation.)

When the part is sufficiently consistent (it mast not be really hard, since when
thix is the case disaggrogation of the sections is apt to occur) it {a placed during
from 15 to 30 minutes in « somewhat thick solution of gum arabic; after this
time it is placed upon a microtome adapted to frozen tissues, and sections are
made which aro zecoived into a vessel containing Muller's fluid, which is diluted
and bas been boiled some hours previously and then «llowed to cool.

A fow nections are then separated and placed in a vessel containing boiling
water, Muller's fluid and the gum with which they aro impregnated being thus
removed from thom-

The stctions are thon placed npon slides and may eithor bo examined at onco
or, what ix preferable, be stained by one of the following processes :—

A-—Mount the section in glycerine coloured beeper eh ein
dissolving the bleu de Quinoléine in a few drops of alcohol and
giyeorino, tho Piorre Marie and Huet process) ; asthe onlil'n feces
eovered by the corer glass, and under the microsoope the granular bodies are
found to be of = doep blue colour, which {a cleatly Gistinot from. the tunquélen

AMYOTROPHIC LATERAL SCLEROSIS. 469

exist in amyotrophic lateral sclerosis, How arphniddte
nected together and their existence be

fae ual’ ban ey Roabet teeta See
changes show for the whole length of the pyramidal tract this

Fig. 244.—Section of a convolution (motor region) in a case of amyotrophic
lateral sclerosis. ‘This section was made in « direction parallel to that of the
fibros of the white snbstance, Black granulationa are observed in the white
substance disposed in diverging linear series. The granulations represent the
grannlar bodies which resulted from degoneration of the nerve fibres, A, Grey
matter ; B, white substanco,

explanation is apparently quite simple; the first lesion is seated
in the large pyramidal cells of the cerebral cortex; the fibres of
the pyramidal tract subsequently degenerate, and this degene-
ration may be followed through the crura cerebri, pons yarolii,
and medulla oblongata as far as the spinal cord. At the same
time the motor cells of the cord are affected by atrophy of the
same nature as that which exists in the large motor cells of
the cerebral cortex.

Unfortunately, gentlemen, this seductive theory very imper-
fectly explains the morbid process which produces amyotrophic
lateral sclerosis, and serious objections may be made to its
adoption.

We know, in fact, that, at any rate in the cord, the lesions

believe hab he preter Eee
mediate segment of their path,

Th seense 00) iorposafble’ toreeeptaén.tbtevAlaalfyslieanezcrere
opinion it would be better to confess openly that we cannot
explain the morbid process which exists in amyotrophic lateral
sclerosis.

All that can be said is that we are no less powerless to
explain why it is that in general paralysis of the insane the intra-
medullary degeneration of the lateral column, when it exists, can
very rarely be traced as far as the crus cerebri. It appears,
therefore, that the pyramidal tract is both liable te complete
descending degeneration, and to ascending or descending
degeneration occurring in segments, which in the actual state
of our knowledge is quite absurd.

As to the admission, which would be less illogical, that the
pyramidal tract is composed of two columns, the fibres of which
are closely mixed together, the anatomical knowledge which we
possess does not permit us to believe that such is the ease.

Before ending these remarks upon the natare of amyotrophic
lateral sclerosis allow me. gentlemen, to insist once more upon
the fact that more than one point of contact seems to exist
between this disease and the forms of general paralysis of the
ingane, to which I have just alluded, and in which
of the pyramidal tracts, which’ is limited to the cord, is
observed to oceur (Westphal, Zacher, &c.), Analogies exist as
regards the pathological anatomy ; the clinical condition shows
(indisputable decrease of mental power in every case of amyo-
trophic lateral sclerosis, even when the indication of paresis and
atrophy seem confined to the limbs); these seem to me good
reasons for comparing these two morbid conditions with each
other. It must be well understood, gentlemen, that I do not
consider these two diseases as identical or even analogous from
a clinical point of view, but am convinced that they should be
placed close to each other if merely considered in connection
with general topographical nosography.”

Enough, however, has been said about the nature of amyo-
trophic lateral sclerosis, and our ignorance on this subject.
Pare ara nate nies icteric Hecci

AMYOTROPHIC LATERAL SCLEROSIS.

Trnportant
differences exiat between thom while very few analogies bring them together,

AMYOTROPHIC LATERAL SCLEROSIS. “a8

slight lipomatosis exists between the different fasciculi, this
tendency being far more pronounced in the tongue than in
other parts,

Diacnosis.—The clinical features of amyotrophic Isteral
sclerosis are so numerous, that it will be well to consider them
separately when considering the affections which simulate this
disease in making the

A. DraGxosts of amyotrophic lateral selerosis of the FORM IN
WHICH MUSCULAR ATROPHY occURS as regards other kinds of
AMYOTROPRY :—

(a) The amyotrophy is combined with diminution or lose of
the tendon reflexes :

Progressive muscular atrophy of Duchenne-Aran (?) ;

Maseular atrophy supposed to be due to peripheral neuritis;

Myopathies.

Tn these cases, the diminution in the tendon reflexes alone
suffices to distinguish them from amyotrophic lateral sclerosis,
and it is therefore useless to dwell upon other points of differ-
ence which enable these affections to be clinically distinguished
from esch other; the seat of the atrophy; the age at which the
onset occurs, the presence or absence of fibrillary contractions,
the electrical reactions, &c.

(b) The amyotrophy is combined with excessive tendon rajlexes.

It is really as regards these affections alone that the diagnosis
requires some attention.

Syringomyelia sometimes produces the same combination of
symptoms, namely, excessive tendon reflexes associated with
amyotrophy in the upper limbs; it is distinguished from amyo-
trophic lateral sclerosis by the special sensory derangements
(loss of sensibility to temperature), and the trophic disorders of
special nature (perforating ulcer, whitlows, dropping off of the
phalanges, &e.).

Cervical hypertrophic pachymeningitis is also often accompanied
by muscular atrophy in the upper limbs, and excess of the
tendon reflexes; but not only are bulbar symptoma usually
absent, but this affection is characterized by the existence of
pseudo neuralgic pains which are never observed in amyotrophic
lateral sclerosis.

As regards the more or less general amyotrophy which occurs
after certain forms of infectious poly-arthritis, it suffices, gentle-—

AMYOTROPHIC LATERAL SCLEROSIS. ath

As regards the cause of amyotrophic lateral sclerosis there is
nothing which indicates the reason of its existence. If inquiry
is made as to the antecedents of the patients with regard to
hereditary pre-disposition to affections of the nervous system
no answer is usually given, Neither is the influence of any
infectious disease found to be in any way connected with
this disease of the nervous centres, Nor does syphilis appear
to be in any way the cause. There at least remains,
if the empty sound of a word will satisfy ws, the power of stating
that amyotrophic lateral sclerosis is a disease of involution.

What shall I say of the ‘TrEaTwexT of amyotrophic Iateral
sclerosis ?

After the observations which I have just made about the
Etiology of this affection you will spare me, gentlemen, the
regret of confessing that we know nothing, absolutely nothing,
of the treatment which should be employed against it. How
in fact could it be otherwise when the two cardinal facts from
which all efficient treatment starts the cause and the nature of
this affection are equally unknown to us? Until our know-
ledge about these two points has increased, we must be
contented, gentlemen, to observe as powerless witnesses the
progress of a conflagration in the grey matter of the medulla
oblongata and spinal cord which we can neither extinguish nor
limit.

-—

ba)

INDEX.

——

Abnormal sensations in tabs; tingling and numbnoss, £¢., 170—178.
Abortive forms of spastic paraplegia, not rare, 4; of insular sclerosis, 125;
diagnosis of, 126; one symptom existe in an isolated manner, 126; or
predominates much over the other, 126,
[Atiamnce’ of knee/erk, posstble in’ Sasufas'wolescslay/ 153 tho cane Toate iat
difficult to distinguish from ono of tabes, 129,
Absence of pain, combined with swelling of the joint, is characteristic of tabid

arthropathy, 221.
Absence of symmetry in cord, specially in cases of amputation, 71, 72.
‘Abesrptlon cS the oasscuis ieterinl meee the Hivcsesias ote een
ACUTE ADULT APIXAL PARALYaIR, 420,
This affection is quite analogous to ‘infantile spinal paralysis previously
studied, and occurs when childhood is ended, 420; the «ymptoms, atiology,

Aoute intoxication, Tang chiorotcon, bay arovens te palaTac tesa Sala fae
occurring for s time, 189.

Adult spinal paralysis, quite resembles tho infantile form, and ooours when ohild~
hood is ended, 420; the aymptoms, xtiology and pathological anatomy are
identical with those of acute infantile paralysis, 421,

Advanced cases of tabos, the lesions in, 338.

Btiology of insular sclerosis, 131-134.

Etiology of tabes, 298—300; the only trae canto of tabes is ayphilis, a fact fret
stated by Fournier in 1876, 300; Erb confirmed this idea, 300; number of
tobid patients in whom syphilis exists, 900-903; wticlogy of combined
lateral and posterior sclerosis, 399 ; of infantile spinal 420,

Ago at which the onset of tabes occurs, 228; nge of onset of Friedreich's disease,
385; at which most casos of combined lateral and posterior sclerosis occur
in adults, 399; age at which adult spinal paralysis occurs, 420; age at
which infantile paralysis occurs ie the earliest infancy between 1 year and
18 months.

Age most affected by insular aclerosis, 132; age and eex specially affected in
amyotrophic lateral sclerosis, 474.

Alterations in spinal cord in amyotrophic Intoral sclerosis, 459 (in the grey
matter, 459 ; white «abetance, #61; lesions of the pyramidal tract, 461; and

obliquely oval shape, &o., 199,
in insular sclerosis noted by Charcot to be always more excessive than

Josions in the optic papilla, 115,

INDEX, 479

Anterior horn—reduoed in size in the lumbar region in amputation of lower
oxtremity, 72.

Antero-latoral colymn, 30, its epee 8; pect penj tepals
specially affected by atrophy, 71; scemed smaller than on same aide after
amputation of thigh, 74.

Antero-median artery passes into the anterior hora upon one side of the cord,
srplalnng why the lelcot Se paralysis are nearly always unilateral,

Ajeplesifoce te epeplitana iAiasisy in insular sclerosis, 123; in hysteria, 190;
spoplectiform attacks in tabes may be of a mild or severe form, 291; being
probably due to haemorrhage, some process of long doration in the
meninges or ependyma, or vasomotor bphtdlgren bth nto
oblongata or ponm varolii, 291; such losions aro probably connected with

hip and knoe-faint,
=o frequenoy of arthropathic cases, 228 ; nceording to Erb 2 in $4 cases,
Aitiantcted ob Rede imbals sa esjanision/itean ot

Astasia-abaaia, in the diagnosis of tabor, 310.

Axymmotrieal action of right and left pyramidal tracts, 10,

Atazic gait not procisely the samo as that due to cerebellar disoase in abortive
insular sclerosis, 129,

Ataxy of the vocal cords seems rare in tabes, 281.

Atary, the existence of, constitutes Friodreich’s disease when slowly progrossive,
383; occurring at an early age, 808; and sometimes in many of the same
family, 368; incroasing difficulty in walking follows, 968.

Atrophio changes, which are pronounced, may exist behind the globe of the vye,
133.

Atrophy of cerebral convolutions, ia not within the seope of these lectures, 77-

Atrophy of the optic nerve noted by Charcot, in some cases, 115.

Atrophy of the vocal cord may coincide with hemiatrophy of the tongne om the
same side, 232,

Anditory organs, frequency with which they are involved in tabes, often during
proatazic period, 204.

Aural vertigo (Meniere's disoase) in tabes ocours in two groups, 205. Tt differs
frou Ménidre's disease, properly so-called, in not occurring in true paroxyama,
205, as stated by Pierret, Charcot, &c., 205,

Autopsy, differeace in after one month or 5, 10, or 20 years, in infantile paralysis,
434.

Awkwardness of movemont greatly contributes to tho «pastic paresis existing in
insolar sclerosis, 106.

Axis cylinders are retained, which affects the whole history of inralar
145; thie many authors have verified, 145, 146; as may be dove by the micro-
scope, 146,

Rabinski, at an autopsy at which amyotrophy was associated with degoneration
of cord fousd no lesion in cornu, cord, or nerves, 23,

481
Cerebral rheumatiam seemed to Chareot to be followed by erry gr
Cerebral

snd. Goes Metin guts. foun, 005 ta Dae Sees ‘of tasular sclerocis,

(ines supposed to exist im the nerve filament which enters the nutritive
orifico-of the tibia which is to suffer from spontaneous fracture, 217;
Siemerling having observed an analogous case, 217,218.

Character in spastic paraplegia always eapricions and odd, 94.

Charcot and Bouchard, aso cf secondary dogoncration of pyramidal tract in its
description by them, 2.

Chareot, opinion of that bilateral degeneration of crossed pyramidal tracts may
oceur after lesion in one cerebral hemisphere, 26; opinion of regarding cause
of amyotrophy accompanying degeneration of pyramidal tracte, 28, 29.

Chemical changes in the bonos in tabea, 217.

Chorea, cannot be mistaken for insular sclerosis, 127.

Choreiform movemente in Friedreich's disease, 373,

Ciroulation in the cord, canses which affect it, 404; I. Extra-mednilary branches,
404; IL, Intra-modullary branches, 406.

Clarke's columns, effect of division of posterior roots in canda equina upon
Clarke's columns, 49; the origin of the dircot cerebellar tract, 55; thelr
structure, 4; how they end, 56; a lesion in the celle produces ascending
degeneration in the fibres of the direct cerebellar tract, 56; smaller after
amputation of a limb, 72; hoalthy condition of the colls in Clarke's columns
in tabes should be joined with the corresponding atate of the direct cerebellar
tracts of which these cells are the trophic centres, 365.

“ Claw-like " attitude of hands in tabos, 252.

Clab-foot, tabid clab-foot, not to be confused with tabid foot, the former being
duo to atrophy of the muscles of the log, the latter to ostoo-arthropathy,
255.

Collatorals, emergence of, 12; formation of, 35.
‘Columns of Clarke, cide Clarke's columns,
Commixty LaTeRAL AND PosTERIOR ScLEROSIs.

‘Tho «ymptoms of this disoase attract loss notion than its pathological
anatomy, 3¢; Westphal first mentioned it, 394; works written opon this
subject, 39, 35,

A. In the posterior columns: degeneration of columns of Goll and Burdach,
39.

B, In the luferal columes: the croased pyramidal tract not wholly involved,
396; direct pyramidal teact usually unaffected in combined lateral and
posterior acleroais, 396; direct cerebellar tract always affectod, 396; Gowers’
ascending antero-lateral tract wsuully less affected, 396,

In the grey matter cells of anterior horns may be affected, and the eame
may be said of the posterior horns, 397; certain cells at times suffer from
atrophy in this disease, 307; symptoms of tabid natare may cxist in this
discase, viz., inco-ordination (awkward movements, ataxic gait and «ymptem
of Romberg, &c.), 397 ; conjointly with which excess of the knee-jerk, foot
clonas, paresis or paralysis, cramps, &e., may exist, 308.

Diagnosin of disease a regards (e) tabor, (5) tho principal forma of spastio
paraplegia, 909 ; wtiology of the discase, 390; most cases occurring in adults
botween the ages of 20 and 40,

L. The classification is mach the same as that formed by Ballet and Minor,
and contains four groupe, 400; (a) some forma of general paralysis of the
‘ineano, (6) aystemio sclerosis of the columns of Goll, columns of Burdach, and
direct cerebellar tracts, 400,

31

Degeneration ofthe nerves nad spinal cond efter amputation of = Hb; ot bf
ey.

Descending degeneration consecutive to transverse lesions of the cord, 3) ef reg. 5
say ob eet Aeny i Sia ees OO eer
Descending system of sulco-marginal zono, effect of degeneration upon, |

Desirastis of tha shentha of rats; whhén tea Meee be lenee ieee
those outside being sound, 145, 0
Development of pyramidal tract in animals, 12; in the human embryo, 12,
Diagnosia ia not always of typical form in insular sclerosis, 130,
Diagnosis of amyotrophic Interal aclerosin, 473, 474.
Diagnosis of combined lateral and posterior sclerosis as regards («) tabos, (0)
the principal forms of spastic paraplegia, 399,
Diagnosis of infantile spinal paralysis as regards birth-palay, 426; syphilitic
i 429,

peor
Diagnosis of spastic paralysin only made later whon movements are not found to

Diagnosis of tabes, 900, &c.; of Friedreich’s dincase, 380.
Dingrum: if the diagram of a section of the normal spinal cord in which the

affocted by acleroais (combined lateral and posterior) aro found to correspond.
to the parts which receive blood from the system of the posterior spinal artery,

407, 408.

Diffuse sclerosis occurs at a later ago (40) than inanlar sclerosia, and dove not
present the classical symptoms of insular sclerosis, 152.

Dignat, affection of sound limbs in a hemiplegio patient enffering from contracture,
245 dimination of muscular foroe (Dignat), 24.

torminn-

5
tion, 53, 56; affected from its origin in Friedreich's dikoase, 387; ates
pelea etl pr tan cron chert goad El
combined lateral and posterior sclerovis, 296,

Diceot pysmanldel test neal) mania eae
aclorosis,

Dia reenact Gash} Tesion in, said to produce more pronounced

‘than one in the crotsed tract, 13; while according to Gowers the

function of the dirvet tract is specially connected with the movement of the
‘upper limbs, Lt.

Discovery of Friedreich's disease, 389.

Disease of Graves is not infrequently associated with tabes, 273.

Disordors of gencral nutrition in tabes, 207; most suffer from consumption or
modullary phthisis, ax it used to be termed, 207,

Disorders of sight in insular sclerosis, their difference in form and intensity, 117,

Dispeeetion atone Oe See ot Ses ae at ee ee

in the “ spontaneous fractures" of tabes, 210,
Disseminated : this word indicates that the islets are of «mall size in insular
sclerosis, 141,

Dissociated anmsthesia, 175,
Division of peripheral nerves, secondary degeneration after, G2.
Double rision ix the natural consequence of such ocular paresis, if pronounced,
cccurring in certain positions of the eye, and corresponding in fact to the
paresis producing it, 114,
Drink: ti: bow the patient le able to drink in Insular ecleroes, 108, 100, -

Fatigao has the same effect aa age, in tabes, upon the patellar tendon reflex,
causing it to coase when trembling of the limb occurs from. fatigue, 189.

Foot often cross each other in spastic paraplegia, 88,

Fibres of posterior root, short, 42; of medium length, 42, and long, +

Fibrillar contractions usually but not always absent in the museular atrophy of
tubor, 252; uenally existing when amyotrophy occurs early in tho disease
alone, 253; their presence in amyotrophic lateral eclerosix, 452.

Field of vision, normal in ineular solerosis, 117.

Final condition and death in Friedreich's disease, 379 ; recovery being unknown,

H snddon agyravations, 379.

Flochsig reopened question of eeeondary degeneration of pyramidal tract in
cord, 2; section of in brain, 5 ; mode of porforming, 4, 5; tract of, 53; middle
zone of—shown by Striimpell to correspond to a small fusiform tract of
fibres in a healthy condition, 340,

Foval lesion Aeron matter of the cord, may It possibly be associated with

in tho posterior columns, when paraplegia occurs suddenly in tabes,
465, 166.

Foci, insular sclerosis occurs in foci, 151, of which the origin is most clear, and is
‘not a diffuse form of sclerosis, 152,

Follin, belief of, as to deformities in hemiplegia, 19.

Foot-clonus nay occur upon sound side, when the tendency to spaam ix increased
in degeneration of the pyramidal tract, 26; when a child advances in spastio
paraplegia, 87; often se pronounced as to occur epontancously whenever the

presents:
Forme leghag hye tabes, 264; what varieties of amyotrophy are contained
in each,
Forins of taber, superior or corvien! form, 2%; cerebral form, and mild form of
Charcot, 296 ; tabes dolorosa, 206 ; severe form, 206 ; morbid processes, pro-
ducing the severe forms, 296; consumptive tendency often prodaces the

Feactare, all or nearly all the bones in tabes are liable to fracture, 218,
Henetares of Sis eeiien ey er ee
Frequency of insular sclerosis, 132.
Friedliinder, affect of upper limbs in a hemiplegic patient suffering from oon-
tracture, 25,
PAreDRRICH’s DISEASE, RisTORY, 309—370,
A. Motor disorders constitute the chief group of symptoms, 371; disorders
of the gait, 971, &e.
B. Sensory disorders may exist, but are raro, 874,
C. ‘The disorders of the reflexes, 374.
D. Disorders in the organs conmected with the sensor, 375-382; nature of
the disease, 382,
This iso disease which tends to affect many members of the same family,
983; Priedreich noticing thie proposed the uame of hereditary ataxy, 384;
wtiology, 333-385; age of onset, 385} sex most affectod, 385.
PaTHOLooical ANATOMY, 3855 thin branch is the least advanced, 335;
the tennity (gracilité) of the «pinal cord should be first mentioned ; it can
be recognized by the nuked eye, 385; pessible causes, 385 ; microscopically
solerosia of the cord can be recognized, 386.
A. Ju the posterior colwnn. Degoneration of the columns of Goll, 396: there

of Burdach also involved throughout their whole extent, but in varying
intensity according to the rogion of the cord, 286,

(©) reflexes connected with Gan geattal Scan, has Gapriaetacin tates Ale
oa » 287; bulbo-cavernoans reflex, 2885 atrophy and anmetheria of
ste erganein the fema‘o, 289; almost

Giving way of the logw in tabos, 161.
Glycosaria in insular sclerosis, ennmerated in two memoirs by Richarditre
Miss Biancho Edwards, 120; may occur in tabes, 249, and other changes in

with the masenlas pind of Kahne, or wih the Pcinan sorpusles in the
human tendons, 185.

Goll, column of, composed mostly of long fibres from the lower segments of the
cord, 45, 51; pathology of long Bbres which form it, 51; pass to the naolem
of the column of Goll, $1; degenoration of column of Goll not always the
same, 51; when affooting only the inferior roots of the cauda oquina it affects
‘the posterior and median part alone, 51; when divided at the upper part of
the cord the degeneration extends over a much larger area than in tho pre-
coding caso, 52.

The sclerosix of these columns ix directly due to the degeneration whieh
occurs in the long fibres, 363; degeneration of in combined bateral and
posterior sclorosis, 395 ; condition of these 463.

Gowers’, ascending antoro-latoral tract little affected in combined lateral und
posterior sclerovie, 398.

Gowers, tract of 53, 57, &c.; ite other name, 58; its origin, 58; pathology, 58;
its termination connected with the nncleas lateralis, 58; other fibres,
according to Tooth, passing into the cerebellum, 69; distinct from direct
cerebellar tract, 59; by its lator development, 60,

Grantlar bodies are seen both in islets of old date and of recent formation, M4;
in imwnlar sclerosis, cide, ‘infectious disease after which sclerosis has
appeared,” 150.

Grey matter of the cord, secondary descending degeneration within, 40,

Different thanges in, when Frivdreich's disease oxixts, 389; cella of
anterior horns may be affected in combined lateral and posterior nclerocia,
‘397; tho same may be said of the posterior horns, 397.

Gostatory organs, strange sensations of tarte, a persistent taste of sognr, oF
complete foxs of taste may exist in tabes probally on account of some change
im the nerves of taste, 200.

Hallopeau, opinion aa to hots ese pee ae ee eee oe
opinion *& to how the decossstion of the pyramids may be accompanied
degeneration, 26; little tendency to produce diffuse inflammation around it
whon eecondary degeneration oxists in pyramidal tract, 27; opinion of, ax to
canes of eaxgstsonhy seecmbeayiag 28.

Head and mpper prt f trunk nclned foemecd sil met 3 ssapsesin J

Hemiatrophy of the bl description of, by different authors, 258; |
connection with

and thinness of the skin, 419; callosities, 420; chil
it of the bonos, 420; intellectual state owing to the

lesions are very extensive and seated in the upper part of the spinal cord or
medalia oblongata, 424, 425; course and termination, 425; of the disease

according to different authors, 425; such n= tho progross of amyotrophy,
muscles of the limba or trunk, 425; second onset of amyo-

firet stated by Prévost and Vulpian in 1865, 453, 434; diminution of cells in
them and diminished size of the lateral columns on the aamo aide of the eord,

&

:
He
tine

Onset, 124; at times an attack of apoplexy, alone or eet
plogia signalizos the onset, or at times, hemiplegia without apoplexy, 124
ieee and giddiness, or sudden tvorders of sight ‘ara the frst

«symptoms, 124,
ate caromap nay rcerish soiree nape agp eee
ah spastic symptoms are then combined with certain symptome of tabes,

iT pall la cercbins 30)
The :

4, Hysteria. Vertigo, apoplexy, intentional tremor, disorder of
npecch, diplopia may exist in bysteria, 130 ; the porsibility of hysterin should
never be or the two diseases r, LBL;

male
sox suffers slightly more than female, 132; age moat affected is the first half
of adult life, viz., botween 20 and 30 years, 132; after the age of 40 insnlar
sclerosis need scarcely be considered, 132; it may, however, occur in child.
hood, as shown by the authors, Unger, Nolda, thongh rare at this time of Life,
182; causes mentioned, 192; the traly effective cause, in my opinion, i+
infection, or rather infections, 133.
Pathological anatomy, nataro, treatment, 187, &o,
Jatelligence, disordered at times with fits, inmbility to sit, &o., indicates condi-
tions termed by author tabid spasmodic conditions, 96 ; their causes, 96.

Internal capsule, localization of pyramidal tract in, 5; limbs of, 5; knee, 5;
tracts for face, tongue, limbs, 6.

Inwerval, Techirjew, in his observations upon spastic paralysie, showed that the
interval of the tendon reflex was in this disease shorter than in healthy

persons, 195.

Intoxication by ergot of rye may prodace lesions analogous to thone of tabes, 304;
also by fermented maize (pollagra), 304,

Intra-medullary afferent system proceeding from posterior roots, 41; anatomirts
who studied it, 41.

Tntra-muscular osseous ure eae papetrimigana taper
opinion they muxt be attributed to easi/ying myoritis, 213.

————

INDEX.

Latoral and posterior sclerosis (combined), possibility of, must be remembered in
eed 5g Nataponedeynaaed 129; spastic «ymptoma are then combined
with certain of tabes, 129,

‘Lateral columns, and losions of, in Friedreich's disease, 387.

Lateral colnmna, ctossed pyramidal tract pot wholly involved in combined latoral
and posterior sclorosix, 396,

Ponca nr Hh ll earned chin deminer ks ge aH Pt
really, , ousisting of short commissural fibres, 61; and only
within tho zone of traumatic degeneration, 61.

‘Lateral curvature in Friodreich's disease, 379.

Latoral movements easily produced in the knoo joint in. tabes, example of, £22.

Lega, extended and pressed against each other, and the feet adhurent to the soil,
so that the patient walks with difficulty in insular sclerosis, 103.

Lesions in the tendons in tabes, 243.

Lesions of insolar sclorosis, 102.

Lesions scen in a section of nerves stained by the hicmatoxylin of Woigert, 6 ;

1, 15.

Limbs of internal capsnlo, sont of pyramidal tract destined to the internal
capsule, 5.

‘Lips, muscles connected with, sometimes involved early in the disease, £12,

Lissaver, tone of, 325, 396; remarks by upon the lesions met with in tabor, 316,

Locomotor ataxy of Duchenne of Boulogne ix now replaced by the torm tabes, 154;
notwithstanding the barbarism and soleciem to which the use of this word
forcibly condemns us, 154.

Long fibres of postorior roots, 44; their pathology, 44.

‘Longitndinal comimiesural Shree, 25,

Loss of patellar tendon reflex in tabes, 193; except in norm cases when it porsiats
although the signs of tabes are quite characteristic, 198; tho lorions then most
often occupy almost exclusively the upper part of the cord, 193; its preeer-
‘vation in these casos is not constant, on account of the fact that the external
bandlets of the posterior column at the upper part of the lombar region of
the con! are mnaffectad, 194.

Lows of the sense of posture, 157; of differences in weight, 157 in tabos in which
disease a difference of 4, 4, or even more may not be distinguished, 157; plans
to decido whether differences in weight aro recognized, 157.

‘Loss of teeth, 248; has been observed since 1868, 448; description of 248; mechanism
of, 248; or its dependence on trophic disorder, 248; or on périostitis and
osteitis affecting the alvooli and teeth, 249.

EN erecta mete fo state of slight flexion at the hip and
kneo-joints in spastic paraplegia, 86.

Marginal tract, degenoration of, 34.

Marginal zone of Lissaacr, lesions of in Friedreich's disease, 389.

‘Mastication, unilateral puralysis of muscles of (Schultze) occurs in tabes, 105.

Medulla oblongata, atrophy of tho nuclei of the vagus, glosso-pharyngeal, and
*pinal accessory nerves mentioned by many authors, 282; changos in the grey
matter, 464; changes in the white substance in amyotrophic Intersl sclerosix,
45,

Mental condition, in spastic paraplegia, 93, 04; in insular sclerosis, 122.

Mercurial tremor, a similar tremor may be the predominating kymptom in insular

ecleronix, 126,
Mothod of Jendravatk in patellar tendon reflex, 190.

orres, changos in when dogenoration occurs, 6 hanges in norras, thoit roots,
Ni

nucle, and the medalla oblongata, n tabes, 28.

Nerves, periphoral, most pronounced peripheral nouritis, was first stated by

spinal ganel
Nourasthonfe pacndo:tabox, 813; how to recognize it (?), 313.
Neuroglia is more affected in insular sclorosia than in Friedreich's ditcano, 144,
Non-correspondence between the number of sensations and that of the impres-
sions, 170.
Non-dovelopment of pyramidal tract the Sarees in every cate of apastio
parapl &e., 96.

Normal and pathologioal anatomy show that it is throngh tho vascular

ment occur in « vertical dircction, 113; in two cases quoted by Ubthoff
the patients thought that they saw tho objects move, 113,
Nystagmus in Friedroich’s dineane, 375.

Ocular disorders may exist in insular sclorosis, 129; in Friodreich’s disease, 375,

Ooular mupeclos, paralyxis of, frequent, 166; paralysie of organs external to the
globe of the eye in tabes, 195; in Friodreich’s disenao, 375,

Ovular paralysis, in tho opinion of oculists may be ae to 8 porphin ov contend
cause of nuclear origin, in tabes, This however i* doubtfal, 198,

Ocular tone, diminution of, in tabes, 199.

Olfactory organs, Praag Por cmt rR ENRON Gere
ina Leslee: bt Ses Daosesy sar van pcs te seas 206.

Onset dates from time of birth in true epastic paraplegia, 94; in inralar sclerosis,
wudden in half, gradual in half the cases, 118; the onset may occur in am
attack of apoplexy alone or complicated by hemiplegia, 114; other forms of
onset ocour in different ways, 124,

Onset of tabes: this may occur by ocular paralysis, 204; or some alteration in
the micturation, or genital functions, or perhaps the larnygeal or gastric

crisis,
Ophthalmoplogia, external, may occur, but the 6th and 3rd norves are prin-
a L

portion papilla
usually of « somewhat grey colour, instead of being more red than the temporal
portion it bocomes of almost tho same lens, 200.

Pathological anatomy of tabia arthropathy, 290, and Joose bodies in the artieula-
tion, 230; of Friedreich's disease, 335, 386, 4c.

Pathology of the reflex may be compared with the practical jokes or games of
children, 191.

Patients enfforing from taben in whom arthropathy has existed for some timo,
221,

Perforating ulcer which may occur, 244; mild or perforating form, 245; neats of,

265.

Period, prodromie of tabes, 293; the ataxic do., the period of confinement and
that of paralysis, 903; the prodromic period that in which the lightning
pains occur, 293; often thought rheumatic, 293.

Periostenl roflex in tabes, 198.

Peripheral sensory nerve fibres and posterior root fibres, trophic centre of, 42.

Poriphoral nerves in Friedreich's dincase,

Persistent pain in tabos, as the “ girdle pain" (said in one case by Fournier to
‘bo #0 wevere as to produce dyspnaa) or the feeling of a bracelet, or “* gaiter,'’
or painful sensations in the trank, 170.

Pharyngo-laryngeal B.

Physical influences in tabea which’ may modify the sensibility, the anmsthosia
being increased by cold, whilat heat, the clectric current, &c., cause its
effect to cease for a time or diminish its intensity, 17.

Pierret, opinion a to the cause of amyotrophy accompanying degeneration of
pyramidal fibres, 28,

Pitros, bilateral degoneration of pyramidal tracts after a unilateral corebral lesion,
24; investigution of as» to degeneration of the two crowed pyramidal tracts
due to a unilateral cerebral lesion, 25.

Pitt, believes Friedreich's dieease to be duc to some defect in the development of
the cord, 392,

Miontar reflex, Vike many other cutancous reflexes, may belernibgieicce i.
period or lost, 194; in the Intter cae more or less anwethesia often exists in
the sole of the foot, 194; thix being the ease, the presence or absence of thin
roflex is of alight importanes in connection with tho diagnosia of tabes, 194,

Pnewmogaatric and spinal accewory nerves, no changes found similar to those in the
nucleus of the hypoglossal nerve in tabes, 262.

Polysthosia, oxplanation of, 176,

Polynouritis, in rare eases much excess ix observed (Striimpell and Mibins), 199.

Polyuria may exist from a similar cause, as diabetes in insular sclerosia, 120.

Pons varolii, path of pyramidal tract in, 6; in amyotrophic lateral sclerosis, 466.

Posterior column, in tabes, 385; tho external bandlets, 396; the colamn of Goll, 396.

Posterior colomes in Friedreich's disease, 358.

Posterior columns, secondary degeneration occurs in it, 37} comma-ehaped de-

posterior by degeneration
throngh tho whole breadth of the posterior columns, 33; and sta lower point
more at the posterior part, 23,

Posterior horn, anatomy of, 320 (ride fig, 165, p, 327); posterior horn properly «0
called, 329; and columns of Clarke, 331; one would expect the grey matter in
thix horn to be involved in the tabid lesions, as many authors have maintained,
Vulpian however doubting this beliof, 345.

Posterior root fibre, a fibre croming from the spinal ganglia, 42.

INDEX. 499

Relative anwathosia in tabes, 174

euie Tin cine Ine ae

Rigidity of leg preventing flexion of the knee in innular sclerosis, the progression
of each foot is accomplished by elevation of the corresponding half of the
trunk and pelvis, 109, 104,

Root cones of the porterior columns, &o., 323; anatomy of, 828, $24, 225,

Rubbing of the foot against the ground in insular sclerosia produces o noise
enabling the character af the gait to be known afar off, 104.

Rupprecht mentioned works of Little, &%,

Rupture of the tendons in tabes, 243 ; examples of the rupture, 243, 244.

Sobiefferdecker, “zone of traumatic degeneration of,’ 30.

Sclerosis, after ansputation of a limb, 75; if in an irregular form and disseminated,
the diffuse form exists, and the type of sclerosis in foei, 143.

Seats of pain in tabes, 168, 169, 170.

Seat of sensations in tabes, innbility to recognize, 175.

Seats of tabos, vis., the lower limbs, abdominal viscera or cervical enlargement
(cervical bpapecntrreet rage Ql eno | 66, ko.

Seated position in spastic paraplegia, 88.

Second period of tabon, 24; the ataxy of movement is almost always confined to
the lower Limbs, 204; and several ycars pass beforo the upper limb« #nffer,
204; ‘visoeral symptoms and the ooular affections may now bo observed, 24s

Secondary degeneration of pyramidal tract, 15, &. ; time of indication noticed by
Pitros, 17; secondary degeneration of the posterior roote in the «pinal cord,
46; not associated with ineular sclerosis, 107,

Senses, no observation need be made as to the power of tasting, hearing, or
smelling in Friedreich's disease, 375.

Sensibility, common, in insular «clorosis, 111; wscally absent, 111; and at times
pain of a somewhat diffused or lightning character, 111, or 4 sonee of eon-
striction round the trank may exist analogous to that occurring in tabes, 112;
objective disorders according to Freund, 112, do not form part of the
clinical features of insalar sclerosis, 112; they should be regarded o# a
clinical curiosity, 112.

Sensory disorders and lightning pains are absent, 369,

Sensory disorders in Friedreich's divas, 973; are rare, $14; thongh pain, anwe-
theria and analgesia may exist, 374.

Sensory metamorphosin, 175,

SENEORY SYMPTOMS AMENABLE TO OBJECTIVE CONTROL IN TARES. (0) Atma
thesia (analgesia), Tts occurrence, and consequences, 171; mccidentally
recognized to exist by the patient, 171; its sent, 171; its intemsity, 172.

Sensory symptoms of « purely subjective nature in tabes, 168.

Sensory tetanns, 176.

Sox, malo euffers more than female in insular sclerosis, 132; most Hable to tabes,
'223, 906; the latter sex ax supposed being lea liable to ayphilie, 307.

‘Sex most affected in Friedreich's diseaso, 865 ; in infantile spinal paralysis, 482.

Sheath of Schwann, rapture of, in degeneration, 69,

Shedding of the nails, 247; often observed in tabes, 247; mode of their being
shed, 247; a bateh of ecchymosis appears beneath the mail before it ix shod,
neually withont pain, 267.

Shooting, wrenching, or burning pains in tabes, 169.

Short fibres of roots, 42,

Special sensibility in insular Res 12,

Sight, disorders of, freqoent in insular acleroaix, and often much accontuated, 112;
difference of form and intensity in same direase, 117,

Sleep, diminishes the patellar tendon reflex, 159,

=

INDEX.

Pps pipes acres st
Supertioial

either {unotional or organi:
character ‘continnes during
weoks, tho motor nnclei ai Lene OnebaL eek Mids teckel rnlatoe teat

sclerosis, 415, to what is termed by the expressive name of cal-de-jatte,
410; when neither the lower part of

usod by the patients to maintain the upright position or for locomotion,
416; this deformity is rare, sclerosis being more frequent, 416; sensibility
is unaffected, 418; trophie disoniers which occur im the growth of tho
limba, subcutancous adipoxis, coldness of the paralysed limba, change ef
colour and thinness of the akia, 419) callonitios, 420; chilblains, 420;
changes in the skin, 420; fragility of the hones, £20; énvellectual state
to the life led and inherited tendency, 420.

and Vulpian, 103.
‘Symptoms divided into «pinal cerebral and bulbar, 103,
Symptoms indicating the existence of optic newritie in tabes, 002.
Symptoms of combined lateral and posterior sclerosis attract leas notice than

regions

Systemic sclerosis (combined) 400. With regard to the forms of this disease
Let ryeinipteliree cay prices campeon be rocalled, 40t; in thie the
blood was arrested and then re-established in the abdominal aorta, and the
degeneration which occurred in the grey matter asd antero-lateral columns
was obserred by Ehrlich and Briger, 401; Pee sn aleve eheerkia ae
| marginal zone corresponding to the direet cerebellar and Gowers’ tract was
in @ healthy coniition, #01,

Tanrs. Txewimonooy, Hisromr, Syaceroms, U4, ef sey. -
Locomotor ataxy of Duchenne of Boulogne, now replaced by the term tabes,
154; “ tabes"’ means “ consumption," Romberg baving described: ‘the disease

usually retained
during the first stages of tabes Many seemed Afiar ena 2 eae
Sere peat eR eee ee ee frequent in
those who have previously suffered from paralysis of the ocular murclos, 203;
Gowors states that he has never seen it ay the first evidence of taber, 202;
usually bilateral in tabes its onset may not be simultaneous in the tro eyes,
tho left eye boing most often first affected, 201,
Avprroxy Onoans.

with which auditory organs are involved, 204; often during tho
preataxio period, 204; diminution in the acvity of hearing, 206; deafness
absolute, and auditory neuritis may occur, 204. Aural vertigo (Menitre's
dlesnan) copes ta a erste: sa ee eee

the transmitting portion of the ear exixt, a lesion probably existing in what is
termed the nerre of spece, a lesion apparently directly dao to the existence
excessive

tabes in whom the eur is slightly affected, 206,

OLFACTORT OngAxs.

Disagroeable odours or anoxntia may exist, being probably due to a lesion
of the olfactory nerves, which ix special to tabos, 206,

GusTaTory ORGANS,

Strange sensations of taste, a persistent taste of mugar, or complete fons of
fuste may exist probably on account of some change in the nerves of taste,

foatures, 207; while some are stout and of healthy appearance. Spontaneous
Pana sat Sees oe entire aheenea of pain, S06 eee
swelling comiderable,

Albatt, Bustard and Volkmann also mentioned it, 229; a memoir of Weir

fThres from the nuclei of the fed and Oth nerves, 2695 tho same ix the caso ax
reyards paralysis of the soft palate and larynx which result from thie charge
in the hypoglossal nuclegs, 263,

Digestive System IN TAME.

(a) Gastric crisie, we teithe known o thi symptom to Charo 182),
265; cortain facts were also exposed by Fournier, 266; what characterises:
these are the pain, and the incontrolluble vomiting, 266 ; | the gastric crisis is
usually an early 4ymptor, 268 ; being Hable to occur and gradually to diminish
and cease, or to persist during the whole disease, 208; death at times occurs
during the co! lagsed condition of one of the crises, 268 ; abnormal forms of the
crisis, 269; its duration, 260; tabid anorvsia, 269. ‘The intestines 1, Inter
tinal tenosmus or «trong desire to evacuate the bowels; 2. tabid dinrrhora,
with little or no pain and no obvious cause, 270; may persist from two to four
years, 271; treatment is ineffectual.

TI. Vascu.ar sysrem in tabex heart, lesion of the aortic are more frequent
than those of the mitral valve, 271; cardine lesions occur somewhat!
in tabes, 271; vascular lesions, 272; arterio solerosia not infrequent, 272; the
bene of ciate Seca ee eee as
changes just mentioned, 272 Late aaa infrequently occurs,
presenting the usual aymptoms of ple yeindar al hares Be a
associated with gastric crisis, 273; disease of Graves ix not infrequently
associated with tabes, 273; opinions as to this coincidence, 274; tachyeardin
may occur, 275; absence of knee-jerk, 275.

TIS. THE PHARYNGO-LARNYGEAL STSTEM IN TABES

vines of Opec:
\" &B Larynx, tabid Inryugismas ; laryngeal erisie, 277; authors who men-
\ tioned Iaryngeal crises, 277; description of condition, 277; laryngeal

280; the thyro-arytenoid or lateral eryco-arytenoid may be parnlysed lees
often and to « stighter degree, 280; ataxy of the vocal cords seems to be

phorus, 283,

‘This tox is far from rare in tabes, 224; derangements of urinary excretions
may occur at the onset, 24; the patients usually consult a surgeon who:
specially treats affections of the urinary passages, and are termed by Guyon

eaten Cees patients,” 284; the patients may have to strain in order to

Tapes Don
‘suns DiAGNosts.
Diao 20. ciatineiats nea degen Bias HAs

!

i

Ait
Free
Hil

i
i

i
ip
Fe
et
i
i!
if

leas degree, 336, 397; rest of the column of Burdach, 237 ; scleronis exinta in
it, 357; zone of Lissaner, 337,

Lrsiox$ AT AN ADVANCED STAGE OY TAMES, 338,

Guey sunsTANcR, changes in the groy matter have been

tract of which vous sells ama igen, kage; AES: RODRD Sseneaean ees
the direct cerebellar tracts are affected, their degencration would perlaps bo
due to the fact that the cells in the eolumna of Clarke are by rare exception
involved, 345; one would expect the grey matter in the posterior horn to be
inpstye fe the bts tie as meesis ee eT Yalpian how-

Poaterice roots of the apinal nerves usually asifcr from atrophy, 347...

INDEX. 509

aking xp an Vesk ABRAM 266
how fractured at times when the patient drinks in insular scleromis, 106,

Each tibet nat pf aed yihncy ep arin sere
wnitable drugs may improve, 153.

Tondon reflex, which norre onding transmits the impulse in et ae
tendon-reflex is uncertain, 186; thongh such trnnsmisaion is
ible, 186 ; having reached diatcre Slaves até lnpdeh pauses wits afeed tats
the spinal cord, 186; in this care the fibres ure in the sensory portion of the
ernral nerve, 186.

Tendon reflexes, increase in, often indicates a tendency to contracture, 19; but not

ss more adopted, 192: owing to this being maintained by Tschirjew,

at

Tendons, nerves in, 183.

‘Tonuity of spinal cord, losion to be firat mentioned in Brledrelch’s dleense, which
can be noticed with the naked eye, 355.

Termination of infantile paralysis usnally consists in paralysis associated with
atrophy of those muscles which remained affected during the period of

regression, 424,
Saprcccanpnevme rt by fatigue and strong moral emotions (angor, fear), and
diminished OL.

Third nerve of the eye moat often affected in tabes, 196,

‘Third period of tabos, almost absolute loss of control over the movements of the
lower limbs, nggravations of the urinary disorders, oystitin with pyuria, &e.,
204, 295.

Tongue, part of pyramidal tract destined to in internal capstile, 5.

soveral fractures in tabes, 211; thus whon the pationt was crossing his lega,
or drawing off his boots, or lying in bed with the head resting on the elbow,
the fractare has been known to occur, 212.

Transformation of primary afferent impulse in patellar tendon reflex, 187,

‘Transient paral ysix in infantile paralysis, 424; improvement said to occur possibly
after some months or years, 424; paralysis possibly ends in death when
the lesions are extensive, and seated in the upper part of the spinal cord or
medalla oblongata, 424, 425.

‘Transvacoe nigalitie, tuiw dlethagetiliad fees pasts possptegtay nO
Treatment is

to the mouth, 108; mode of carrying glass to Hps, 108; belongs to tremors
with moderate rhythm (54 to 74 oscillations in a second), 109; why the rapidity:
seems greater, 109; the extent of the movements dao to their affecting nearly
all the muscles of the body, 110; the tremor may oceur in muscles of body,
and of upper and lower limbs, 110; the neck and hend may move forwards and

INDEX, oll

Vertebral fracturns in tabos first mentioned by Charcot and afterwards by
Pitres, and other authors, 214; there is deformity of the spine which forms
progressively and somewhat slowly, 214.

Vertigo in ingular sclerosis, 121, 122; in hysteria, 130.

Visceral disorders in insular xclerosis, 119; visceral pains in tabes, 170,

Vision, organ of, nearly all the structures in, may be affected in insular sclerosis,
Ls,

Visual disorder, development of, in inanlar sclorosiz, 118 penne
ocetr, 118; disorders bilateral, 118; hanger correspon scot
decoloration of the optic nerve, 118; rarictios of change ag vienal fleld
mentioned by Uhthof, 113.

Virual Sear symptoms connceted with, well known but ngain diecussed
recently,

Voluntary moverents, pre-eminently made by pyramidal tenct, 18; this tract
has an oxcitcmotor or inhibitory effect upon the cella of the cord, 13,

Vomiting of gastric crisis, 206; ite composition, 206, 267; gastric juice xocreted
in excess, 267 ; and more acid in reaction, 267 ; explanation of this change, 267.

Leper observations about degeneration of the pyramidal tract in
si 15,

Walking, mode of, in a little girl with spastic parsplegia, 87; and leaping almost
imporsible when spitstic paruplogia is severe, £3.

Weariness is produced by severe pain and repeated vomiting in the gastric
crisis of tabex, 267; un indifference to all which surrounds the patient, 268 5
and if the affection is severe n «tate of algidity may occur, and the colour of
the patient become livid as in cholera or sea-sicknoss, 268,

Wostphal, bilateral degeneration of pyramidal tracts after = unilateral cerebral
lesion firet pointed out by, 23; believes the nerve fibres of the patellar
tendon reflex to pass from the bend of the posterior horn to the posterior
surface of the cord, 126; showed the absence af knee-jerk after Erb had done
#0 in tabes, 102; termed the sign of Westphal, 192; gave the first knowledge
of combined lateral and posterior sclerosis, 394.

Works recently written upon insular sclerosis mentioned in theee lectures, 103 ;
upon combined lateral and posterior solerosis, 394,

Wrist eomi-flexed and fingers strongly flexed into the palm of tho band in amyor
trophic lateral sclerosis, 449,

Zone of Liesauer, $25; marginal rone, 326; external segment of xone of Lissauer,
926 ; inner segment of zone of Lissanor, 326; constituted alvicst entirely by
small fibres, $28; which insinuate themselves between the large fibros coming
in fascicali from the posterior root, &c,, 923; other small fibres accompany
the lange fibres of the posterior roots and pass with them into the gelatinous:
substance of the posterior corna or into the interior part of that cornu, 323 ;
this zone not increasing in «ize from elow upwards, 388; in the lumbar
region this group of fibres is most developed, its smallest size being in the
dorsal region, 320; small nerves which constitute the sone of Lissauer
almost entirely dimppear in tabes, 337; lateral colamn in no way affected
in these cases, 338; degeneration of the zone of Lissauer an early change in
tabes, 363,

LONDON

Printed by Jas. Tacncorr & Sox,
Suffolk Lane,

LONDO
Printed by Jas. Trrscort & Sox,
Suffulk Lane, EC,