BOUGHT WITH THE INCOME FROM THE SAGE ENDOWMENT FUND THE GIFT OF 1891 .../i...lik.A.k iVufJl n« .^^ .PS'"^" University Library RC 400.M33 1895 Lectures on diseases of the spinal cord. 3 1924 012 460 873 The original of tliis book is in tlie Cornell University Library. There are no known copyright restrictions in the United States on the use of the text. http://www.archive.org/details/cu31924012460873 THE NEW SYDENHAM SOCIETY. VOLUME CLIi Instituted MDCCCLVIIL LECTURES DISEASES OF THE SPINAL CORD Br De. PIERRE MARIE, De^mty-Professor at the Faculty of Medicine of Paris. Physician of the Hospitals, CONTAINING TWO HUNDRED AND FORTY-FOUR WOODCUTS. TEANSLATED BY MONTAGU LUBBOCK, M.D., P.R.G.P. Lond. THE NEW SYDENHAM SOCIETY. 1895. AUTHOR'S PREFACE. The lectures published in this book were taken from those delivered at the Faculty of Medicine in the summer of 1891. Although but little altered, some works which appeared during the course of their publication were of such great importance that it seemed unwise to neglect them, and they were consequently utilized by me. 1 Iiave advisedly given the name of " Lectures " to this publi- cation, more freedom of speech being thus allowed, while it may constantly be recognized during its perusal that the book is specially written for the purpose of " teaching.'' Since some diseases only of the spinal cord are described in this book, I have endeavoured to discuss these as completely as possible. Numerous works have been consulted upon this branch of nervous pathology, care being taken to name their authors, although it did not accord with the plan of this publi- cation to indicate the name of every book to which allusion is made. Those mentioned have been somewhat arbitrarily chosen, their importance, their novelty, and the difficulty in obtaining them being specially considered. Little will be found in this work about the pathology of any special symptom. Thus I have purposely omitted to discuss the mechanism of the inco-ordination which occurs in tabes. Our knowledge with respect to this and other similar conditions is so rudimentary, and the opinions expressed about them are so contradictory that, as I believe, it is better to await a more propitious epoch before doing this. The study of Etiology on the other hand owing to the immense progress made in general pathology is steadily advancing ; to this portion of the subject I have devoted special attention, since it is from a knowledge of it that Therapeutics worthy of the name must be derived. The symptoms have also been carefully studied. 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 SalpetriSre, 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. CONTENTS. LECTURE I. Anatomy of the Pyramidal Teact. Eeason for this denomination. Path of the pyramidal tract ; its origin in the motor convolutions ; its course in the centrum ovale its seat in the internal capsule, where it occupies the anterior two-thirds of the posterior limb ; its path in the eras cerebri, pons varoBi, and medulla oblongata ; its decussation, and path in the spinal cord. — Division into crossed (FPyC) and direct (PPyD) pyramidal tract.— Path of the crossed tract (PPyC) which is separated from the surface of the cord by the fibres of the direct cerebellar tract ; its form in the different regions of the cord. — Path of the direct tract (PPyD), which does not exist in the lower part of the cord ; variations in its size ; these variations correspond with those in the decussation of the pyramidal tract. — The pyramidal tract in certain animals.— Development of the pyramidal tract.— Collateral branches described by Ctolgi, Eamon y Cajal, KaUiker, v. Lenhossek. — Termina- tion of the pyramidal tract 1 LECTURE II. Secondary Deoeneeation op the Pyramidal Tract. History : Crnveilhier, Tilrok, Charcot, Vulpian, Leyden, Cornil, &c. ; Memoir of Bouchard (1866) ; Thesis of Brissaud (1880). Conditions in which secondary degeneration occurs in this tract. Clinical indications of the degeneration : spastic symptoms, excess of tendon reflexes, con- tracture, mode of production of these symptoms ; theories on this subject. Bi-lateral degeneration of the pyramidal tracts consecutive to a unilateral cerebral lesion. Works of Pitres and Dignat. Varying seat of the degenerated part. Theories proposed in explanation of these facts. Co- existence of secondary degeneration of the pyramidal tract with amyotrophy. Statement of such facts by numerous authors whose observations have given rise to different theories 15 LECTURE III. Descending Deoeneeation Consecutive to Teansveese Lesions of the Coed. A. — In the anteeo-lateeal column : — a. Pyramidal tract, its degeneration in a case of transverse lesion of the cord is far more complete than when it is due to a cerebral lesion. Theories proposed in explanation of this fact, p. Portion of the lateral column external to the pyramidal tract: fibres of the intermediate tract of the lateral column, y. Anterior column : fibres of the marginal tract. Nature, path, and origin of these fibres ; commissural fibres, commissural ceUs. Tract of descending degeneration of cerebellar origin (Marohi). B. — In the posterior column :— Comma-shaped degeneration of Schultze ; small number of confirming facts. Suppositions as to the nature of the fibres affected by this degeneration , 30 Vlll CONTENTS. PAGE LECTURE IV. ASCENDINO DEaENEBATION CONSECUTIVE TO LeBIONS Or THE Neete-koots. Anatomical study of the afferent system of the cord proceeding from the fibres of the posterior roots. Development of this system ; origin in the external layer of the blastoderm; division of the neural plate into a central segment which will form the motor portions of the oerebro-spinal axis, and two lateral segments from which the sympathetic nerve and ganglia of the spinal cord will be formed. Fibres of the posterior roots. Eesearches of Singer and Miinzer. These fibres may be classed in three groups, according to the portion of the spinal cord in which they end. Their terminal nuclei. Degeneration of the posterior columns after lesions of the posterior roots ; researches of Singer, Tooth and Hartley ; clinical observations of Kahler, Sohultze, &e 41 LECTURE V. Ascending Degehebation after Tjbansvbese Lesions or the Spinal Coed. A. In the posteeiob column : u. In the column of Burdach. P. In the column of Goll. Difference in seat and extent of degeneration according to the height of the transverse lesion. B. In the anteeo-lateeal column : a. Direct cerebellar tract, its description by Elechsig, its seat, form, origin, path, termination, and degeneration. /3. Tract of Gowers (antero-lateral ascending tract of English authors), its seat, form, origin, path, termina- tion, and degeneration. C. In the obey strBSTANOE of the coed. Degenerations in the grey matter are but little known, lesions found by Barbacci. Sound condition of lateral limiting layer {seitliche Grenzschicht of German authors^ 60 LECTURE VI. Degeneration of the Nerves and Spinal Cord AFTER amputation OF A LIMB. Such degeneration seems at first sight opposed to the doctrines of physiology. At the same time changes of this kind are frequent. They have been shown to occur by numerous observers. Works of B(5rard, Vulpian, Dickinson, Hayem, Dejerine and Mayor, Hayem and Gilbert, Fried- litnder and Kranse, &c. Changes in the central extremity of nerves after amputation of a limb ; frequent increase in size (does not always occur) increased diameter of the fasciculi constituting the nerve. Considerable diminution, in a transverse section, of the fibres containing myelin. Islets nf degeneration not to be confused with the primary islets, being in reality the traces of previously existing nerve fibres. The islets of degeneration are formed of a collection of nerve-fibres composed of a small axis cylinder, and fine primitive sheath, with or without myelin. Evolution of these islets, dispersion of the nerve fibrils, myxoid aspect of the partitions separating the different primary islets from each other, slight thickening of the fibrous bundles placed between the fasciculi of the nerve. Uncertainty with regard to the condition of the ganglia connected with the nerre roots, and the nerve roots themselves 62 CONTENTS. IX PAOE LECTURE VII. Degeneration oe the Nekves and Spinal Coed AFTER AMPUTATION OP A LIMB {continued). Alterations in the Spinal Coed :— According to Vulpian the atrophy especially ' iuvolTes the antero-lateral column and grey matter. Dickinson believes it to be more pronounced in the posterior column. The latter opinion seems preferable to the former. Opinion of Plechsig. Priedlander and Krause describe amongst their cases both atrophy of the posterior column and of the posterior horn, disappearance of a large number of cells in the postero-laleral group of the grey matter, diminution in size of the anterior horn with a reduction in the number of its cells, atrophy of the column of Clarice with disappearance of many of its cells. Kesults furnished by examination of the spinal cord in my own autopsies. Sand of sclerosis in the posterior column on the same side as the amputation. Thickening of the connective tissue in the posterior column of the opposite side. Inter- pretation of these different lesions : opinion expressed by Priedlander andKrause: personal conclusions 71 LECTURE VIII. Spastic Paeapleqia. History of the term spasmodic tabes dorsalis. Erb : Spasmodic spinal paralt/sis. Charcot : Spasmodic tabes dorsalis. Primary degeneration of the lateral columns. Different results obtained in the autopsies of adults. History of the disease itself. Heine : paraplegia spastica cerebralis. Little : con- genital spastic rigidity of limbs. The researches of the English surgeon were for a long time unknown to neurologists. Memoirs of Eupprecht and Peer. Symptoms of the disease. Spasmodic gait: attitude of the head and limbs. Attitude in the sitting position : position of the trunk and limbs due to difficulty in flexing the thigh upon the pelvis. Attitude of the upper extremities, which are often little affected. Muscles of the front and back of the neck. Disorders of speech. Strabismus. Appear- ance of the f ace . Awkwardness of movements . Pibro-musoular retractions . Influence of fatigue, of the emotions 83 LECTURE IX. Spastic Paraplegia (continued). Excess of tendon-reflex. Foot clonus. Normal cutaneous reflex action: Common sensibility retained. Muscular sense unaffected. Electrical irritability at times increased. Healthy condition of the bladder and rectum. Absence of intellectual change. Oddness of character. Special indications of degeneration. Attenuated forms of the disease. Tendency to progressive improvement. Congenital onset, often unperoeived by the parents. Such children very backward in walking. Their intellectual development usually enables them to live subsequently in the same way as others. Nature of the affection. True spastic paraplegia must be distinguished from the tabid-spasmodio conditions ; clinically the first is not accompanied by epileptic attacks, and very rarely by mental change ; anatomically it is due to a failure in the development of the pyramidal tract, and not to its destruction ; setiologically it is specially due to pre- mature birth. Diagnosis from infantile spinal paralysis, tetanus of the newly bom, tetany, Thomson's disease, the disease of Pott, transverse myelitis, insular sclerosis, spastic infantile hemiplegia. Hysteria. Treat- ment : methodical education of the limbs, gymnastics, massage, tenotomy S2 X CONTENTS. PAGE LECTUEE X. Instjlae Sclerosis. History : Crnveilhier, Eokitansky, Charcot and Vnlpian, Ordenstein, BoumeTille and Guerard, recent works. Symptoms : 1. Spinal. A. Motor. Disorders in the gait, true spastic gait, pure cerebellar gait, oerebello-spastic gait; their characters. Spastic paraplegia. Hemi- plegia : characters of the hemiplegia in insular sclerosis. Tremor : its characters ; occurs when voluntary movements are made, moderate rapidity of its rhythm, specially affects the muscles at the root of the limbs, but often also those of the trunk and neck, and may predominate upon one side of the body 102 LECTURE XI. Insular Sclerosis : Symptoms. B. Sensory symptoms. — Disorders of common sensibility (Freuud) are rare, and but slightly pronounced : they Vary in form from tingling to hemi- ansesthesia. Disorders of special sensibility : the hearing, taste and smell are little and rarely affected. Disorders of sight of great impor- tance : works of Gnauck, Parinaud and Uhthoff . Nystagmus and nystag- moid movements : precautions necessary in seeking them. Paralysis of the muscles of the orbit, associated paralyses (Parinaud). Disorders of the optic disc, myosis associated with excess of iris reflex. Changes in the optic disc, disorders of sight which result from them, changes 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 genital organs (Oppenheim) ; these are rare, and but little pronounced. D. Trophic disorders, specially amyotrophic. II. Bulbar symptoms : disorders affecting deglutition, or mastication, trembling of the tongue, glycosuria, polyuria. III. Cerebral symptoms : affections of speech, the articulation is slow, monotonous, scanning, spasmodic. Vertigo ; intellectual disorders, impulsive laughter, apoplectiform, epi- leptiform attacks Ill LECTUEE XII. Insular Sclerosis (continued). Course. Diagnosis. Etiology. Course: Onset acute, or gradual and progressive. The -course of insular sclerosis may follow different types : a chronic progressive type, a chronic type wiih sudden exacerbations, a. remittent chronic type, permanent improvement, and even recovery. Variable duration. Abortive forms. Diagnosis; A. Of fke abortive forms : with hysteria, paralysis agitans, mercurial tremor, chorea, general paralysis of the insane, cerebral tumour, transverse myelitis, compression of the cord, combined lateral and pos- terior sclerosis, tabes, focal cerebral lesions, amyotrophic lateral sclerosis. B. Of the typical form with Friedreich's disease, hysteria (coincidence of hysteria and insular sclerosis). .Etiology : Slightly more frequent in the male sex, most frequent between the age of 20 and 30 years, does not occur in old age, very rare, if occurring at all, in childhood. The special cause is infection. Narration of facts, different infectious diseases during or after which insular sclerosis has been found to occur. Explanations of this fact 124 CONTENTS. XI PAGE LECTUEE XIII. Insular Scleeosis (continuation and end). PATHOLoaiCAL Anatomt. Natube. Treatment. The meninges are usually but slightly, if at all, affected. Insular appearance of the lesions : abundance of the islets, extreme irregularity of their seat, dimensions, colour, and consistence, their seat upon the nerve roots con- nected with the medulla oblongata and spinal cord. MicroBoopioal examina- tion : the islet seems to have been cut out by means of a punch : the sclerosis essentially involves the neuroglia, containing many granular bodies in its substance, the sheaths of myelin being destroyed while the axis cylinders are preserved. Deductions which may be drawn from this fact with regard to the absence of secondary degeneration : remission or cure, the occurrence of tremor, lesions of the optic nerve, changes in the blood vessels. Pathology of insular sclerosis : inflammatory process of inter- stitial origin, commencing near the blood vessels. Pathological anatomy thus in accordance with ^etiological data. Hypotheses connected with this subject. Distinction between insular sclerosis and diffused multi- locular sclerosis. Treatment 137 LECTURE XIV. Tabes. Teeminolost. Histort. Symptoms. Terminology : locomotor ataxy, tabes, &C. History : Romberg, Duchenne of Boulogne, Charcot, &c. Symptoms: A. Motor disorders : 1. Alterations of the muscular sense, loss of the knowledge of posture, loss of the power to detect differences in weight, difSculty in maintaining the upright position. Sign of Romberg : disorders of the gait, indications given by Fournier in connection with the investigation of these disorders ; " giving way of the legs," inco-ordination of the upper limbs. 2. Athetoid move- ments an& muscular tremors. 3. Paralysis : its characters ; hemiplegia, paraplegia with sudden onset, local forms of paralysis 154 LECTUEE XV. Tabes. — Symptoms (continued). B. Sensory Symptoms. I. Sensory symptoms which are purely sub- jective. — a. Intermittent pain : a. Seated in the trunk, the limbs, or the face : lightning, shooting, wrenching, burning, often paroxysmal. P- Pain connected with the viscera (to be specially studied when the disturbances connected with each organ are considered), y. Sudden loss of muscular power, b. Persistent pain : girdle-pain, bracelet-pain, gaiter-pain, burn- ing-pain, c. Abnormal sensation of tingling or numbness. II. Sensory symptoms amenable to objective control. — a. Anmsthesia (anal- gesia) researches of M. Oolmont. S. Hypersesthesia ; hypersesthesia in islets, relative hypersesthesia, relative ansesthesia. c. Parassthesia : retarded transmission of impressions, altered character of sensations, localisation of sensations impossible, dissociated anaesthesia, return of sensations, non-correspondence between the number of sensations and that of the impressions, a. Sensory tetanus. /3. Polysesthesia : summation of impressions, loss of power to respond to impressions 168 xii CONTENTS. PAGE LECTURE XVI. Tabes.— Symptoms {continued). C Disorders of beflex action. PaUllar tendon reflex. PreoautionB to be taken in the attempt to obtain it : position to be given to the thigh ; percussion of the tendon, means of detecting the muscular contraoUon which is thus produced, principal causes of error. Pseudo-tendon reflex of Westphal. Nature of tendon-reflexes ; anatomical notions m connection with the nerves and nerve endings in the tendons. Eegion of the cord in which the afferent path of the patellar tendon reflex ends : external band- lets (Wurzeleintrittszone), the limits of this region in the transverse and longitudinal direction. Centrifugal paths. Conditions which may influence the production or intensity of the patellar tendon reflex : (a) local, (b) general. History of the discovery of the patellar tendon reflex ; sign of Westphal, theories in connection with this subject, reasons confirming its reflex origin. — Plantar reiex 179 LECTUEE XVII. Tabes.— Symptoms (continued). D. Obqans CONNECTED WITH THE SENSES.— 1. Visual organs. I. Those which are external to the globe of the eye. Paralysis of the external muscles of the eyeball, ptosis, shedding of tears, epiphora, exophthalmos, narrowness of the palpebral opening, diminution of ocular tone. II. Internal organs of the eye. Condition of the pupils, inequality, myosis, mydriasis, deformity. Reflex actions of the iris : on exposure to light, on accommodation, sign of Argyll-Robertson, reflex due to pain. Optic nerve : optic neuritis ; its onset, symptoms, and characters. 2. Auditory organs. Diminution in the power of hearing ; deafness, its characters ; M^nieres disease : excessive irritabilty of the auditory nerve by electric currents. 3. Olfactory organs. Anosmia. 4. Gustatory organs. Loss of taste 195 lecttjeb xvin. Tabes.- Symptoms {continued). E. Teophic disorders. — a. Disorders of the general nutrition, b. Spontaneous fractures ; nature of these fractures : absence of pain, tendency to con- solidation, tendency to form a large quantity of callus : special setiological conditions : the slightest injury may produce them, seat of these fractures, fractures of the apophyses, fractures of the vertebrae, their nature. Lesions of the bones : porosity, thinning of the compact substance, dilatation of the medullary cavity, dilatation of the Haversian canals, decalcification of the lamellse of bone, changes in the osteophytes, embryonic transformation of the marrow in the bones, diminution of the non-organic, increase of the organic material. Nature of these alter- ations 207 LECTUEE XIX. Tabes. — Symptoms {continued). F. Arthropathy IN TABES. — History: " Charcot's joint disease'' — opposite opinion of Volkmann. Onset more or less sudden : swelling, doughy condition, absence of true osdema ; crepitation ; absence of pain. Course of the affection ; mild and severe form. State of the articulation when the arthropathy has existed for a certain time ; flattening of the part, abnor- CONTENTS. Xlll , ^ PAGE mal relation oif the articular surfaces, abnormal mobility, absence of pain. Complications : fracture, pain, passage of the articular extremities through the skin, suppuration. ^Etiology: occurrence at different periods, greater frequency in the female sex ; seat in different joints. Pathological anatomy : articular capsule, intra-artioular ligaments, synovial membrane, effusion, floating bodies in the joints, extremities of the bones ; atrophic type, hypertrophic type, cases of combination of these two types. Path- ology of tabid arthropathy : different theories. A. The primary cause of the arthropathy is a nervous lesion (spinal cord, medulla oblongata, peripheral nerves). B. The primary cause is not a nervous lesion (traumatic, syphilitic, rheumatic arthritis, arthritis deformans). Conclu- sions 219 LECTURE XX. Tabes. — Symptoms (^continued). The foot in tabes : History. — Characters. Somewhat sudden onset, swelling of the foot, thickness of its inner margin, flattening of the arch of the foot, deviation of the metatarsus, shortening of the foot, enlargement of the malleoli, ankylosis of the joints of the foot, absence of pain. Pathological anatomy: spongy appearance of the tarsal and metatarsal bones, their destruction. Trophic disorders in the fibrous tissue : articular capsules ; intra-articular ligaments ; tendons, their rupture. Trophic disorders of the sHn : different eruptions — Perforating ulcer, its characters ; Gangrene. Spontaneous eoohymoses ; Loss of the nails and teeth, Hyperidrosis ; anidrosis. Nature of these trophic disorders 239 LECTURE XXL Tabes.— Symptoms (continued). Muscular atrophy: Distinction from the conditions of muscular emaciation, which are due to consumption. Is specially seated in the lower limbs, and most often bilateral; occurs also in the upper limbs and in the muscles supplied by nerves coming from the medulla oblongata. Onset. Extension. Fibrillar contractions. Electrical examination. Evolution. Tabid club-foot of Jeffrey. Pathological Anatomy of these forms of amyotrophy ; muscular lesions ; opinions as to the nature of the nervous lesion (in the spinal cord, in the peripheral nerves). Bemiatrophy oj the tongue: its symptoms. Appearance of the tongue, paralysis of the soft palate, and inferior vocal cord upon the same side. Lesions of the medulla oblongata in cases 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 arrange the forms of amyotrophy occurring in tabes in two distinct groups 251 LECTURE XXn. Tabes.— Symptoms {continued). Visceral disorders. — I. Digestive system : (a) Stomach. — a. Gastric crises, their character ; pain, uncontrollable vomiting, excess of secretion, and acidity of the gastric juice, researches of Sahli, Rosenthal, Hoffmann, state of nervous depression, its abrupt onset, and sudden disappearance ; tendency to relapses. Abnormal forms. — P. Tabid annorexia. — (4) Intes- tine. — »■ Intestinal tenesmus. —P- Tabid diarrhoea. II. Vascular system. — ((f) Heart : lesions of the mitral, and aortic valves. — (b) Blood vessels : Arterio-solerosis ; angiaa pectoris ; exophthalmic goitre ; works published upon this subject 265 xiv CONTENTS. PAGE LECTURE XXIII. Tabes. — Symptoms (conUnued). VlSCEKAL D180KDEKS. III.— PHABTNQO-LAETNaEAL SYSTEM. A— Pharynx. Researches of Fano. Pharyngeal crises of Oppenheim. B.— Larynx. Tabid Laryngismus : (1) Acute symptoms of tabid laryngismus ; laryn- geal crises, their description, laryngeal ictus, nature and mode of produc- tion of laryngeal crises. (2) Chronic symptoms of tabid laryngismus, whistling inspiration, unnatural Toice, having two tones. Laryngeal paralysis: the most frequent is that of the posterior orico-arytenoid muscles. Lesions of chronic laryngismus connected with the nerves and medulla oblongata. IV. — Urinary system. A. — Derangements of the urinary secretion : glycosuria ; changes in the amount of urea, phosphoric acid, &o. ; excessive secretion of urine. B. — Derangements of the urinary excretion: "false urinary patients" of M. Guyon ; relative retention, complete retention ; absolute incontinence, relative incontinence ; vesical colic ; nephritic crises. V. — GE^fEEATIVE system. A. — Derangements in the male : impotence ; generative excitement ; cremaster reflex, its research, its signification : bulbo-cavemosus reflex ; atrophy and anses- thesia of the testis. B. — Derangement in the female: genital depression; genital excitement ; pain in the genital organs ; vulvo-vaginal crises 276 LECTURE XXIV. Tabes. — Symptoms {continued). VI. Ceubbbal system. — Hemiplegia. Apoplectiform attacks. Epileptiform attacks. Acute symptoms connected with the medulla oblongata. Psychical derangements. Coincidence of general paralysis of the insane. Course of Tabes. — Prodromal period. Second period, period of inco- ordination. Third period, confinement to bed. Forms of tabes : upper or cervical, cerebral, mild, and severe forms 291 LECTURE XXV. Tabes.— iETioLOOY. .SJtioloqy. — Common causes: Wet and cold; Diatheses: arthritic, herpetic; sexual excess; injury. The true setiological element of tabes is syphilis. The discovery of this fact is due to Foumier (1876) . Erb (1879) adopts and defends this opinion. Statistics published by different authors as to the percentage of tabid patients known to be affected by syphilis. Recent statistics of Erb including 369 cases giving 89 per cent, of syphilis in the astiology of tabes. Nine-tenths at least of tabid patients suffer therefore from syphilis.— Increased number of adversaries to this opinion.— Refutation of their arguments. — Influence of hereditary pre-disposition to nervous diseases shown by Charcot. Doubtful effect of hereditary syphilis. Age: greatest frequency of onset between 30 and 45 years. Hace. Profession : most often occurs in the liberal professions ; its infrequency in the clerical profession 298 LECTURE XXVI. Tabes. — Diaqnosis. Diagnosis : Diflioulty in making a complete and methodical diagnosis. Differential character as regards : Cerebellar affections, Insular sclerosis Astasia-abasia, Syringo-myelia ; as regards Pseudo-tabes; A. Toxic Pseudo. CONTENTS. XV PAGE teJes, alcohol, arsenic; B. Diabetic pseudo-tabes ; C. Neurasthenic pseudo- tabes, THEEiPEUTics : A. Internal remedies ; nitrate of silver, strychnine, aconitine, atropine, ergot of rye ; anti-syphilitic agents : mercury, iodides ; B. External remedies : counter irritation applied to the skin, electricity, massage, nerve-stretching, suspension, hydrotherapy ; general summary of the treatment adopted 309 LECTURE XXVII. Tabes {continued). Pathological Anatomy. Anatomy of the pabts in a normal condition. A. Anatomy op the POSTEBIOE COLUMN. Description of the three kinds of fibres : long, of medium length, short, (a) Posterior column properly so called. Researches of Flechsig upon the order in which the zones composing it are developed. Origin and termination of the fibres which constitute these different zones. (4) Zone of Lissauer : its seat, limits, extension ; its division into two segments external and internal ; the fibres which constitute it ; their origin ; development of this zone. B. Anatomy of the posteeiok HOBN. (a) Posterior horn properly so called : apex ; gelatinous sub- stance of Bolando, its division into (») the spongy zone of the gelatinous substance, and (P) the typical gelatinous substance of Rolando. Spongy substance with its two zones, the anterior and the posterior. 0. Anatomy op the column of Clarke. Its seat, limits, and extension. Within this tract are found : (a) nerve cells ; (S) a network of nerve fibres 321 LECTURE XXVIII. Tabes (continued). Pathological Anatomy. History : Hutin, Monod, Cruveilhier, Rokitansky, Turck, Romberg, Charcot and Pierrot, &c. A. — Spinal Coed. Macroscopic appearance. Appear^ ance under the microscope. — (a) At the onset : Posterior column — a. External bandlets, their seat, their alterations ; /3. Column of Goll; y. Rest of the column of Burdach ; S. Zone of Lissauer. (b) At an advanced stage : 1. Posterior column — lesions of the external bandlets, the column of Goll, the rest of the column of Burdach. 2. Grey substance — ". Anterior horn ; (3. Coluimm of Clarke ; y. Posterior horn ; S, Central canal. B. — PosTEEiOE Roots, their lesions. C— Spinal Ganglia, their lesions. D. — PebiphebAl Neeveb, their lesions. E.— Bbain : dis- appearance of the nerve fibres of the convolutions 834 LECTURE XXIX. Tabes (continued). Nature op the Disease. Nature op Tabes : Theories about this subject. — The two lesions 'of tabes are seated in the brain (Jendr^ssik). — Rflle of the sympathetic nerve. — Pri- mary importance of the vascular lesions. — R61e of the posterior meningitis. — Primary sclerosis in the tracts of the posterior columns. — Objections to this which is the leading opinion. — The fibres of the posterior column, in the same way as is the case with those in other columns of the cord, do not degenerate until the cells from which they part are affected. — Study of the cells from which the fibres of the posterior column originate : — Cells of the spinal ganglia, peripheral ganglion cells, arguments in favour of the existence of the latter, changes in the different ceU elements. — Forms of XVI CONTENTS. PAG peripheral neuritis : — Lesions of the posterior roots. — The lesions of the cord in the course of tabes are due to ascending secondary degeneration of the nerre fibres which come from the posterior roots. — The seat of the islets of sclerosis in the cord of tabid patients differs in the different oases on aooount of the fact that in patients suffering from this disease the same posterior roots are not always involved, or at any rate are not affected to the same degree, and that the degeneration first occurs in certain groups of their fibres. — Hence the different clinical types rest upon a good basis from an anatomical point of view : inferior tabes, cervical tabes, &c.— Explanation of the symmetry which usually exists in the lesions of the cord in tabes. — Theprimum movens of the change in the nerve cells to which the lesions of tabes are duo is the toxio agent of syphilitic origin suspected by Strumpell 3J LECTURE XXX. Peiedeeich's Disease. History. — The first cases were described by Friedreich in 1861 : in England, the case of Carpenter (1871), of Growers (1880) ; in France, the thesis of Brousse (1882) ; Lecture of Charcot ; thesis of Sooa. Symptoms. — A. Motor disoedebs : — (a) Disorders of gait, (b) Difficulty in maintaining the upright position, (o) Atactic tremor, (d) Choreiform movements, (e) Paralytic symptoms. B. Sensoey disobdees : — (a) Pains, (b) Anass- thesia, analgesia, (c) Disorders of the muscular sense. C. Disoedees op THE EEPLEXES : — (a) Cutaneous reflexes, (b) Tendon reflexes. T>. Dis- OEDERS IN THE ORGANS CONNECTED WITH THE SENSES -.—Ocular disorders; nystagmus. E. CeeeeeAL disoedebs, vertigo; cephalalgia; state of the intellect; disorders in speech. F. Gbnito-urinaey disorders. G. Trophic and tasomotoe disobdees : — Club-foot ; muscular atrophy; curvature of the spine. Course of the disease : Progressive, remissions at times occur ; recovery impossible. Diagnosis from : — Tahes, insular sclerosis, chorea of Sydenham, a pseudo-disease of Friedreich recently described by Nonne 3( LECTTJEE XXXI. Feiedeeich's Disease (continued). aiTlOLOGY : It is a family affection.— \il hat is the r61e of the pathological antecedents in the parents or ancestry p What is that of syphilis ? The onset of Friedreich's disease nearly always occurs in childhood, and very rarely after the age of 16 years; Soca's law; is slightly more frequent in the male sex. Pathological anatomy: tenuity (gracilit<0 of the spinal cord. Sclerotic lesions : A. In the posteeioe COLUMNS as the columns of Goll and of Burdach. B. In the dieect CEREBBLLAE TRACT. C. In the LATERAL COLUMN (it WOuld not be the pyramidal fibres which are affected). D. In the zone of Lissauee. E. In the obey matter : columns of Clarke, their nerve reticulum is scanty, their cells are in smaller number ; posterior horns diminished in size ; anterior horns also somewhat altered. The central canal of the cord may be the seat of different lesions. The condition of the meninges the poslerwr roots, and peripheral nerves is the subject of very divergent opinions on the part of different observers. Nature of Friedreich's disease. Opinion of Dejerine and LetuUe, Pitt and Graseet. Therapeu- tics : its want of aucccBS CONTENTS. XVll PASB LECTURE XXXII. Combined Lateral and Posteriob Sclekosis. History : Westphal (1877), Kahler and Pick, Striimpell, Raymond and Arthaud, Babes, Ballet and Minor, Grasset, &o. ; Patholooical ANATOMY— Lesions : In the Posterior Columns : (o) column of Goll ; (i) column of Burdaoh. In the Lateral, Columns : (a) crossed pyramidal tracts ; (6) direct pyramidal tract. In the Direct cerebellar tract ; In Gowers' tract ; in the GREY matter : (o) cells of the anterior horns ; (b) cells of the posterior horns; (c) cells of Clarke's column. Symptoms : very diffuse and variable. Two large groups according as the tabid or spasmodic symptoms predominate. Diagnosis : from tabesj insular sclerosis and transverse myelitis. Nature of these affections : their classi- fication in different groups. Experience of Stenson ; results obtained by Ehrlich and Brieger, by Singer and Miinzer ; their apphoation to the study of the pathology of combined lateral and posterior sclerosis 394 LECTURE XXXIII. Combined Lateral and Posterior Sclerosis {continued). What is known ii.s to the normal anatomy of the arteries in the spinal cord. A. Extra-medullary branches. I. Anterior system: Anterior spinal arteries ; lateral spinal arteries. II. Posterior system : Posterior spinal arteries. B. Intra-medullary branches. I. System of the anterior spinal artery : anterior median and commissural artery ; branches to the anterior portion of the white substance; anterior radicular branches. II. System of the posterior spinal artery : posterior median artery : post- intermediate artery : posterior radicular artery ; posterior cornnal artery; posterior lateral branches ; middle lateral branches ; anterior lateral branches. The lesions in certain oases of the combined form of sclerosis, are seated round the vessels of the posterior cornua and the lateral arteries ; connection of the lesions with the above named vascular regions. .aSTioLOGY. Therapeutics 404 LECTURE XXXIV. Infantile Spinal Paralysis. History : From a clinical point of view, Heine, Rilliet andBarthez, Duohenne of Boulogne, Laborde. Prom an anatomical point of view, Prevost and Vulpian, Clarke, Charcot and Jeffrey. Symptoms: Fever, gastro- intestinal derangements, nervous symptoms consisting of somnolence, convulsions, &c. Paralysis, the nature of its onset and extension, its seat its regression. Period of deformities, points to be considered as regards their production ; the duration of the paralysis, early age of the patient at the time of its occurrence, atrophy of the bones. Nature of the paralysis, results of an electrical examination, loss of the tendon reflexes, flaocidity of the hmb. Integrity of the sensory functions, and of the superficial reflexes. Trophic disorders : subcutaneous adiposis, thinness of the skin, ulcerations, callosities, chilblains, hypertrophy of the hair, excessive secretion of sweat, fragility of the bones. InteUectnal condition. Xviii CONTENTS. PAGE Acute Spinal Paralysis of the adult. History : Dnohenne of Boulogne, Charcot, Moritz, Meyer, Bernhardt, Bourne- ville and Teinturier, E. C. Seguin. Symptoms : Onset, fever, nervous symptoms, &c. Parah/ds, its mode of onset and extension, its seat and regression. Briefly, the ago of the patient being excepted, the symptoms are quite analogous to those which occur in infantile paralysis : the few olinioal differences which exist are connected with the onset of the disease ^^^ LECTURE XXXT. Infantile Paralysis {continued). Abnormal forms. Insidious onset: onset during convalescence after an acute disease ; onset announced by pain. Forms of transient paralysis. Fatal determination. Late reappearance of paralytic symptoms, or occurrence of secondary progressive muscular atrophy. Theories upon thissubject. JHnf/nosis: irom birfh j/a/tty ; syphilitic pneudo-pnralysix ; infan- tile cerebral hemiplegia ; myopathic atrophy ; muscular atrophy of the Charcot-Marie type ; hysterical paralysis with amyotrophy in childhood. .Etiology: Cold, injury, ordinary causes ; dentition probably has .some influence but. is not a direct cause. Infantile paralysis really occurs from the influence of a general disease, usually of inj'ectiom character. Enumeration of the infectious diseases after which it has been known to occur. Epidemics of infantile paralysis : Cordier, Leegard, Medin, Bergenholtz, Colmer, Briegleb. Hereditar?/ injimnce. The age at which infantile paralysis usually occurs between one year and eighteen months. The sexes are equally affected 423 LECTURE XXXVI. Infantile Paralysis (continued). Pathological anatomy. — History of the discovery of changes in the anterior horns: Vulpian and Prevost (1865', Lookhart Clarke, Charcot and Jeffrey, Parrot and Jeffrey, Eoger and Damasohino. (a) Character of the lesions when the affected focus is of old date, lesions in theanterim- horns, nature of these lesions many foci usually exist which are rarely quite symmetrical ; atrophy of the half of the spinal cord which corresponds to the paralysed side, and in some eases of the cerebral hemisphere upon the same side (Eumpf, Colella, Fomario) ; lesions but slightly pronounced in the anterior roots and trunlis of the mixed nerves, explanation of Jeffrey and Aohard : alterations in the muscles ; alterations in the bones, diminu- tion of the diameter of the Haversian systems ; alterations in the blood vessels, (b) Character of the lesions when the affected focus is of recent date ; every appearance of a true inflammatory focus then exists, this focus of acute myelitis may extend to the adjoining white matter ; the reason of this fact probably connected with the distribution of blood vessels in that region. Normal Anatomy of the blood vessels supplied to the anterior horn. Anterior spinal avsTTSM.—Anterior spinal artery; anterior median artery ; anterior radicular branches ; each of these articles may he the seat of the intra-medullary vascular lesion which constitutes infantile paralysis. Identity of infantile spinal paralysis with infantile cerebral hemiplegia: my disagreement with Vizioli and Strilmpell. Observation of P. J. Mobius. This explanation of what occurs enables the late "renewal" of the clinical course of infantile spinal paralysis, which has been considered to be understood. Therapeutics.— Electricity massage, hydrotherapy, orthopaedic apparatuses ' 433 CONTENTS. XIX PAGE LECTUEB XXXVII. Amyotrophic Lateral Sclerosis. , History :—/)iseaife of Charcot. Symptoms: A. Spasitiodic fymploms: excess of tendon reflexes in the lower and upper limbs, foot-olonns, tendency of the limbs to pass into a condition of contracture, or at any rate of spasmodic rigidity. B, Paralysis. C. ^«5CM/ar «,iropAy, its seat, existence of fibrillary contractions; electrical reactions. D. Bulbar symptoms; paralysis and atrophy of the muscles of the lips, tonpie, and soft palate ; difficulty in mastication ; loss of the lateral movements of the lower jaw ; distorted action of the heart ; examination of the masseter tendon reflex (jaw jerk) ; examination of the pharyngeal reflex. No disorder of the sphincters or trophic derangement exist, frequent diminution of intel- lectual power, liability to emotion ; the existence of symptomatic neurasthenia. Co urse of the disease ; different modes of onset : A. by atrophy of the upper limbs ; B. by bulbar symptoms ; C. by spastic para- plegia. Duration of the disease. Termination inevitably fatal 447 LECTURE XXXVIII. Amyotrophic Lateral Sclerosis (continued). Patholosical anatomy. I. Spinal cord. A. Changes in the grey matter of the anterior horns : atrophy of the large ganglion cells, inflammation of the whole anterior horn. B. Changes in the white substance ; (a) lesions of the pyramidal tract (direct and crossed) ; (4) lesions of the trluile nntero- lateraJ. columns, while at times the lesions are apparently seated in the portion of these tracts which is between the anterior horn and surface of the cord, or in the part adjoining the anterior horns ; lesions in the part occupied by the columns of Goll. II. Medulla oblongata. A. Changes in the grey matter affecting the nuclei of the hypoglossal, 5th nerve, portio dura, &c. B. Changes in the mhlie matter : the pyramids, the posterior longitudinal tract (faisceau longitudinal posterieur), the band of Reil (Muratof). III. The pons varolii. IV. The CRUs cerebri. V. The BRAIN. The presence of granular bodies in the internal capsule in the motor convolutions. Atrophy of the large pyramidal cells of those convolutions. The.se lesions at the same time are not constant. The method of seeking the granular bodies. Theories which exist as to the nature of amyotrophic lateral sclerosis. Lesions of the anterior ROOTS, the motor nerve-trunks, the intra-musculah nerves, and the muscles. Diagnosis. A.. OtVaeamyotrophicfiyrmivoin: the muscular atrophy of Duchenne-Aran (?) ; muscular atrophy due to polyneuritis ; the different forms of myopathy; syi-ingo-myelia ; hypertrophic cervical pachy meningitis ; arthritic muscular atrophy. B. Of the form with spastic pa'-alynis from : transverse myelitis ; , spastic paraplegia ; insular sclerosis. G. Of the form with bulbar symptoms from : acute bulbar paralysis: chronic bulbar paralysis ; pseudo-bulbar paralysis 459 LECTURES ON DISEASES OF THE SPINAL COM). LECTURE I. ANATOMY OF THE PYRAMIDAL TRACT. Eeason for this denomination. Path of the pyramidal tract ; its origin in the motor oonvolationB ; its course in the centrum ovale ; its seat in the internal capsule, where it occupies the anterior two-thirds of the posterior limb ; its path in the crus cerebri, pons varolii, and medulla oblongata ; its decussation, and path in the spinal cord. —Division into crossed (FPyC) and direct (PPyD) pyramidal tract. — Path of the crossed tract (PPyC) which is separated from the surface of the cord by the fibres of the direct cerebellar tract ; its form in the different regions of the cord.— Path of the direct tract (FPyD), which does not exist in the lower part of the cord ; variations in its size ; these variations correspond with those in the decussation of the pyramidal tract. — The pyra- midal tract in certain animals. — Development of the pyramidal tract. — Collateral branches described by Golgi, Eamon y Cajal, Kolliker, v. Lenhossek. — Termina- tion of the pyramidal tract. Gentlemen, — Before commencing to study the diseases of the Spinal Cord the principal facts connected with its anatomy- will be considered, a knowledge of which is indispensable. Our object being to combine Pathology and Anatomy, the different parts of the cord will only be described anatomically in so far as this consideration relates to the diseases from which they suffer. Morbid and healthy conditions will be contrasted, normal and pathological anatomy being found to mutually enlighten each other. Thus the study of secondary degeneration will enable us to become rapidly acquainted with the principal tracts of the cord. In the first lectures such degeneration will be specially con- sidered, the anatomy and origin of the numerous tracts of fibres 1 2 LECTURES ON DISEASES OF THE SPINAL CORD. which compose the spinal cord showing it to be a cable of trans- mission rather than a central organ. The PYRAMIDAL TRACT (FPy) holds an , important place in many different alterations of the spinal cord. The knowledge which we possess about this tract, and the secondary degenera- tion to which it is liable will therefore be first considered. The name pyramidal tract was derived from the fact that this column, which is seated in the antferior portion of the bulb, is comprised within the elongated bundles of white matter in that region which are teTmed pyramids. The path of this tract is well known and has been long under- stood. In this, as in many other points, connected with neuro- logy, pathological anatomy preceded pure anatomy. The works of Tiirck, of Charcot and Bouchard, of which the importance is well known to you, have specially utilized the secondary de- generation which occurs in the spinal cord after hemiplegia in their description of the pyramidal tract. At a later date Flechsig by a new method of investigation reopened this apparently exhausted question, and the description which he has given is undoubtedly correct, and will be often utilized during the following lectures. Considered throughout its whole length the pyramidal tract extends from the cerebral convolutions through the hemispheres, crura cerebri, pons varolii, and medulla oblongata, to the lower pai-t of the spinal cord. The fibres of the pyramidal tract originate in the so-called motor convolutions, viz., the ascending frontal and parietal convolu- tions, and the paracentral lobule, or medial aspect of these two convolutions. Whether fibres from other parts of the brain also join this tract is uncertain, but its fibres undoubtedly come for the most part from these convolutions, in all probability from the cells of the cortex, and partly or entirely from the special cells which are termed the large pyramidal cells* of the motor convolutions. From the grey layer of the cortex the fibres of the pyramidal tract pass into the white substance of the motor convolutions, where they assume the form of a fan, as they converge towards the more internal parts of the brain. It is thus that they pass •The name of pyramidal cells is simply due to their form, anfl in no way to a real or supposed connection with the pyramidal tract. ANATOMY OF THE PYRAMIDAL TRACT. 3 through the centrum ovale subjacent to the motor cjon vo- lutions. The fibres follow this convergent direction, and before going TDrd blindttess temiopia agraphia ■word deafness Fig. 1. — Outer surface of the cerebral hemisphere showing the motor conTolu- tions (leg, arm, face). (This figure was extracted from the Atlas of Brissaud* upon the Anatomy of the Brain.) Fig. 2.— Median aspect of the cerebral hemisphere 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 can be localized in a more exact manner. This localization is * I have to thank my friend and colleague, Brissaud, for permission to use these figures before the publication of his Atlas upon the Anatomy of the Brain, from which they are taken. 4 LECTIKES 0^f DISEASES OV THE SPINAL CORD. speciallv interesting in tlie intenutl capsule on account of the difierentiation wliicli can be made of the numerous tracts which constitute this cross-road (carrefonr, Charcot). Fiff- 3. — S, carpus striatum ; NC, nucleus caudatus ; CO, thalamus opticus ; F, an- terior limb of the internal capsule ; G,knee of the internal capsule ; B, fibres of the internal capsule connectcil with the movements of the upper extremity. J, fibres of the internal capsule oonnectetl with the movements of the lower extremity ; CS, sensory cross-road. (This figure was extracted from the Atlas of Brissauil upon the Anatomy of the Brain) 'J'he position of the different tracts is most easily studied, as you know, by the horizontal division of the brain termed tlif ANATOMY OF THE PYRAMIDAL TRACT. section of Flechsig, and, according to the recommendation of Brissaud, the following is the best direction for the section to have : the knife should pass through the inner surface of the hemisphere in a direction which is slightly oblique downwards and backwards so as to divide the corpus striatum in the centre of its head, and the thalamus opticus at the junction of its upper third with its lower two-thirds. The anterior and posterior limbs of the internal capsule and the Tcnee which separates them can be recognized in this section, the pyramidal tract being found, as Charcot expresses it, in the posterior limb of which it occupies the anterior two- thirds. It may itselt be divided into secondary tracts, which from before backwards are connected respectively with the face, tongue, and limbs. Pig. 4. — Diagram Showing that the knee of the internal capsule varies in position according to the height at which the horizontal section of the hemisphere is made. (After the Atlas of Brissaud upon the Anatomy of the Brain.) NC, nucleus caudatus ; CO, thalamus opticus ; P, orns cerebri ; Tae, taenia semi- circularis ; XTZ, horizontal sections through the corpus striatum and thalamus opticus involving the taenia semioiroularis at the points 1, 2, and 3. The points 1, 2, and 3 correspond to three points in the knee of the internal capsule, which are seated at different levels. Upon reaching the crus cerebri the tract occupies the middle part of the lower portion, or crusta, having on its inner side a band of fibres which come from the anterior part of the brain and pass downwards into the pons, beyond which they cannot be followed. On the outer side of the tract (FPy) is another band of fibres which seemingly come from the temporal and occipital 6 LECTURES ON DISEASES OF THE SPINAL CORD. lobes, and which are, at times, though veiy rarely (Brissaud), found to degenerate (Bechterew), the degeneration ceasing when the pons is reached. The tract itself (FPy) may be divided into secondary tracts, which from within outwards are destined respectively for the face, tongue, and limbs. In the pons varolii the fibres of the tract (FPy) again occupy the crastal portion, being divided into flattened bundles, which are separated from each other by some of the transverse fibres of the pons. The size of the tract (FPy) is here much larger than in the iipper part of the pyramids in the medulla oblongata, and it seems probable that, as some authors believe, many of its fibres terminate in the pons. In the medulla oblongata, on the other hand, the tracts (FPy), as already stated, are completely isolated from the other fasciculi in that part, and form the prominent bundles known as pyramids. You will observe, gentlemen, that the summit of these " pyra- mids " is directed downwards, and that at the lower part of the medulla oblongata the tract (FPy) disappears from its fibres, remaining no longer at the surface, but passing into the interior 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 i)ijramids, 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 (I'PyC). Each of these tracts will now be separately studied. The CROSSED pyramidal 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, Lowenthal) 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. Very possibly it ANATOMY OF THE PYRAMIDAL TRACT. varies in length, 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 : throughout almost its whole course it is in relation internally and posteriorly with the posterior horn until almost the extremity of its 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 c,ervical pair of nerves) ; the consequence is that in those parts where the direct cerebellar tract does not yet exist, Fig. 5. Fig. 6. Fig. 7. Fig. 8. Fig. 9. Fig. 10. Sections of the spinal cord in which the two direct pyramidal tracts A, and the crossed pyramidal tracts B, present respectively the same symmetricaJ relations (after M. Flechsig). Fig. 5 at the level of the 2nd.cervical pair. Fig 6 at that of the 7th cervical. Fig. 7 at that of the 3rd dorsal. Fig. 8 at that of the 6th dorsal. Fig. 9 at that of the 1st lumbar. Fig. 10 at that of the 5th lumbar pair of nerves. that is to say, at some point below one of the last 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 extend beyond an imaginary line, passing transversely through 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 at any rate quite possible, the fact that individual differences may exist being always remembered. The form of the crossed pyramidal tract (FPyC) 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 oi'der to pass into the grey matter. The DIRECT PYRAMIDAL TRACT (FPyD) differs from the crossed pyramidal tract both by its different seat in the spinal cord, and "Br Fig. 11.— Section of tho spinal cord (upper part of cervical region) in a case of hemiplegia dne to a cerebral lesion. A, direct pyramidal tract, which has here a flattened form, and extends in an unusual way to the outer side of the anterior horn. B, crossed pyramidal tract. The white portion is affected by sclerosis (Da- maschino collection). Fig. 12.— Anterior horn and direct pyramidal tract of the same section as in the preceding figure more highly magnified. (By an error on the part of the designer the patch of sclerosis A is upon the right instead of the left side, as in fig. 11.) A, the direct pyramidal tract with its sin- gular flattened form (Damaschino collection) . its shorter course. According to Bouchard, this tract (FPyD) does not usually extend beyond the mid-dorsal region. Tooth, ANATOMY OF THE PYRAMIDAL TRACT. 9 however, has traced it as far as the second pair of lumhar nerves. It should be observed, however, that its length seems subject to numerous variations. Thus Bechterew states that he has known it disappear below the cervical region. Though exceptions may exist, it should be remembered, however, that as a rule the direct pyramidal tract does not extend beyond the middle portion of the dorsal region. When a section is made its situation and form are found liable to similar variations. In some cases this tract (FPyD) extends along the whole length of the anterior median fissure, beyond which it sometimes passes externally {vide figs. 11 and 12), while in others it seems to occupy a very small space, and to be, as it were, surrounded by the adjoining fibres. Fig. 13. — Anterior surface of the me- dulla oblongata, the shaded portion A representing the pyramids, which terminate far more abruptly than usual. In this case there was decus- sation of the whole pyramid , and not of part only, as is usually the case. There was, therefore, no direct pyra- midal tract. (After Plechsig.) Fig. 14.— Anterior surface of the me- dulla oblongata, the shaded portion A representing the pyramids. These extend far lower than is normally the case. The decussation of the pyramids in this patient was very incomplete, the greater number of fibres remaining in the direct pyra- midal tract. (After Plechsig.) It must not be supposed that the relation between the sizes of the direct (FPyD) and crossed pyramidal tract (FPyC) is by any means invariable; the individual differences which exist, as Flechsig has shown, are by no means small. In some cases, in fact, symmetrical semi-decussation occurs. 10 LECTURES ON DISEASES OF THE SPINAL CORD. both pyramids furnishing a direct and crossed pyramidal tract, which more or less correspond with each other. This, according to Flechsig, is what usually happens (in 75 per cent, of the cases). At other times the whole pyramid decussates, and the direct Fig. 15. — Section of the spinal cord in wliich the direct (A) is far larger than the crossed pyramidal tract (B). From the same patient as fig. 14. (After Flechsig.) Fig. 16. — Section of the same cord (upper part of dorsal region) as figs. 14 and 15. Same comparative size of the direct (A) and crossed pyramidal tracts (B). (After Flechsig.) Fig. 17.— Section of the same cord Fig. 18.— Section of the same cord (lower part of the dorsal region) as (level of 4th lumbar pair of nerves) in figs. 14, 15, and 16. Same com- as in figs. 14, 15, 16, and 17. In this parative size of the direct (A) and cord, in which the pyramidal fibres crossed pyramidal tracts (B). (After are mostly contained in the direct Flechsig.) pyramidal tract, traces of this tract (A) are seen in the lowest part of the lumbar cord, instead of its disap- pearing, as usually happens, in the lower part of the dorsal region. (After Flechsig.) pyramidal tracts are completely absent. Inversely the direct pyramidal tracts (FPyD) are in some cases of larger size than those which cross to the other side (FPyC). (Vide figs 14 l5 16, 17, 18.) ' ANATOMY or THE PYRAMIDAL TRACT. 11 In some cases the right and left pyramidal tracts act differently, and the decussation 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 arid 20). Fig. 19. — Section of a cord (cervical region) in which the direct and crossed pyramidal tracts are sym- metrically unequal- in size. The crossed pyramidal tract B, which is smaller than B', corresponds to a direct pyramidal tract A, which is larger than A'. Pig. 20. — Section of a cord (cervical regionl in which the direct pyramidal tract (A) only exists upon one side. The crossed pyramidal tract (B') upon the same side far exceeds that of the opposite side in size, as if it contained, in addition to its own 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 Bechterev*r, 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 run easily from the first. Thus there is a curious 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. 12 LECTUUES ON DISEASES OF THE SPINAL CORD. The size of the pyramidal tract also depends upon the move- ments which the animal is able to perform, In those animals of which the movements are but slightly differentiated, such as the whale and the elephant, the pyramidal tract is almost entirely absent, while in those living beings by which varied and delicate movements are performed, such as men and monkeys, the pyramidal tract attains its full development. DiflFerences again exist as regards the division into a direct and crossed pyramidal tract ; some animals, for instance, such as the dog and cat, have no direct tract, while in others, such as the white rat, the pyramidal tract passes into the anterior segment of the posterior column. These facts are of interest, and show that the knowledge obtained from the study of degeneration in the cord of some animals cannot be applied in an absolute manner to the degeneration which occurs in man. The position and path of the pyramidal tract and its divisions being understood, the general facts which relate to its study 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 (4| in.) in length the pyramidal tract is completely absent although the other tracts of the cord can be distinctly perceived. Those who have investigated this question believe that the pyramidal tracts are in all probability formed towards the middle or end of the 5th 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. Since 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, Kolliker, &c.). These collaterals, * Vide Medical Microscopy, by P. J. Wethered, p. SO (Translator). ANATOMY OF THE PYRAMIDAL TRACT. 13 according to Lenhossek, do not pass as one would expect, and as most authors believe, into the anterior, but into the posterior comua. There they form arborescent divisions in such a way as only to be connected with the cells of the anterior comua by the medium of other intervening cells. I must remind you that the pyramidal tracts do not form an absolutely compact mass, but between the fibres composing them others of different origin exist which are not affected by secondary descending degeneration, a lesion which indicates that the pyramidal tract is involved. As regards the connections of these fibres I have already had occasion, gentlemen, to mention their origin in the cortex of the brain ; I still have to speak of their termination in the spinal cord. This part of their path is certainly one of the least known. According to the generally accepted opinions the connections of these tracts are as follows : — As regards the crossed pyramidal tract it is connected with the cells of the anterior comua, the lateral parts of the grey matter, and the crossed pyramidal tract (FPyC) of the opposite side. The direct pyramidal tract is connected with the cells of the anterior comua, the internal portion of the grey substance of those horns, and the crossed pyramidal tract (FPyC) of the opposite side. It must be acknowledged, however, that these connections, specially as you have already seen, the direct connection of the fibres of the pyramidal tract with the cells of the anterior comua, have by no means been objectively shown to exist. The intrinsic mode of action of the pyramidal tract is little better understood. "We certainly know that it is pre-eminently the tract by means of which voluntary movements are made, but in what way we are completely ignorant. It may be supposed that the nervous influence which passes through the pyramidal fibres has either an excitomotor or inhibitory effect upon the cells of the cord, a point which will be again considered when, the contracture associated with secondary degeneration is mentioned. Before ending this chapter I would ask you to remember an assertion of Homen, according to which an isolated lesion in the direct pyramidal 14 LECTURES ON DISEASES OF THE SPINAL CORD. tract (PPyD) 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 II. SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. HiSTOET : Cruveilhier, Tiirok, Charcot, Vulpian, Leyden, Comil, &o. ; Memoir of Bouchard (1866) ; Thesis of Brisaaud (1880). Conditions in which secondary degeneration occurs in this tract. Clinical indications of the degeneration : spastic symptoms, excess of tendon reflexes, contracture, mode of production of these symptoms ; theories on this subject. Bilateral degeneration of the pyramidal tracts consecutiTe to a unilateral cerebral lesion. Works of Pitres and Dignat. Varying seat of the degenerated part. Theories proposed in explanation of these facts. Co-existence of secondary degeneration of the pyramidal tract with amyotrophy. Statement of such facts by numerous authors whose observations have given rise to different theories. Gentlemen, — 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 -Yfill now be studied, the portion of the pyramidal tract contained in the spinal cord being alone considered. The 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 given to Tiirck, who described such degeneration in 1851 — 55. A few yesirs later these observations were confirmed and extended bj' Charcot, Vulpian, Leyden, Cornil, &c., and in 1866 the important memoir of Ch. Bouchard appeared, which marks, as you know, an important epoch in the history of secondary degeneration. The fact is that after this work nothing was observed upon the subject during several years. In 1880 the inaugural thesis of Brissaud was written, this being the most complete and suggestive account of the subject in which we are now interested which has hitherto been given. Although different varieties of cerebral lesion may produce -secondary degeneration in the pyramidal tract, the lesion must 16 LECTURES ON DISEASES OF THE SPINAL COKD. te connected with some point in the path of the pyramidal fibres, and be also destructive in nature. You know in what part of the brain the lesion must be seated in order to fulfil the first of these conditions, namely, at the very origin of the pyramidal fibres in the so-called central convolutions near the fissure of Rolando, or in the path of these fibres in the centrum ovale, or lastly in the anterior two-thirds of the posterior limb of the internal capsule where these fibres are seated. The second condition that the lesion must be destructive in nature should be explained. Mere compression of the pyramidal fibres, as by cerebral tumours, or meningeal lesions which are superficial would not probabljr produce secondary degeneration in the spinal cord. Opinions differ as to whether removal of the grey substance of the convolutions, without any lesion of the white matter, could be followed by secondary degeneration. It seems quite possible that this may be the case if the lesion is not too superficial, since it is in the deeper portion of the grey matter of the cortex that the large cells are seated, from which the fibres of the pyramidal tract seem to take origin. When the two conditions which have just been mentioned exist, the trophic centre of the pyramidal fibres being seated in the cortex, the peripheral termination of these fibres in the spinal cord degenerates accoi'ding to the law of Waller. The axis cylinder perishes, the medullary sheath of myelin undergoes the changes which usually occur in degeneration, and during the somewhat long period of its absorption granular bodies are observed in greater or less number at the part where degenera- tion is taking place. At the same time or shortly afterwards there is proliferation of the neuroglia. Is this due, as some authors have stated, to inflammation of the neuroglia by extension of the inflammatory condition which exists in the fibres of the pyramidal tract, or as Bouchard maintains, to simple proliferation of the neuroglia which fills the empty spaces resulting from disappearance of these fibres? For my part I am much inclined to agree with the latter opinion. How soon after production of the cerebral lesion do the first indications of secondary degeneration appear ? It is to Bouchard that we must again refer for an answer to this question. According to that author the first traces of de- scending degeneration can be recognized anatomically upon SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 17 about the sixth day. Clinical examination enables it to be traced some time before this. Thus Pitres has noticed the existence of the foot phenomenon as soon as 20, or even 10 hours after the occurrence of hemijilegia. 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. I need not again describe in detail the parts in which degeneration occurs. I should merely have to repeat word for word what I liave alread}- 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, 21, 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 solelj' the spasmodic condition which occurs. Fig. 21.— Section of the medulla ob- longata at its lower part. A, de- R-eneration of the pyramid on the left side from a case of hemiplegia due to a cerebral lesion. The white zone was affected by sclerosis. Fig. 22. — Secondary degeneration of the direct pyramidal tract A, and the crossed pyramidal tract B, in a case of hemiplegia from a cerebral lesion (cervical cord). 18 LECTURES ON DISEASES OF THE SPINAL COKD. Tliis spasm does not always present the same characters ; in some cases it is specially marked, a contracture existing which attains a most pronounced degree, while in others there is bo Fig. 23. — Secondary degeneration of the crossed pyramidal tract in a case of hemiplegia dne to a cerebral lesion. (Section of the cord in the dorsal region where the direct pyra- midal tract no longer exists.) Fig. 2t.— Secondary degeneration of the crossed pyramidal tract A from a case of hemiplegia dne to a cere- bral lesion. (Section of the cord in the lumbar region.) external indication of this state. The patients in whom the latter is the case have been truly said by Brissaud to be " ow the point of suffering from it," being really, it may be said, in a condition of latent spasm. The appearance of hemiplegic patients suffering from spasm is well known. In the upper extreinity the position is usually one oi flexion with pronation, in the lower extremity of extension with adduction. The attitude of these patients is so characteristic that they could be recognized by any observer even at a distance when walking in the street, while in the case of those who suffer from latent spasm the foot phenomenon or other forms of reflex action alone reveal the condition which exists. In these, not only is there an exaggeration of the movements, but as Brissaud has shown, both stronger muscular contraction and a diminution in the length of time which passes hetween i/ie hlmv being given and the movement produced (38 instead of 45 thousandths of a second), and , at times the increased irritability causes recurring contractions -to oeeur, although but one blow has been given to the tendon. This increase in the tendon reflexes very frequently indicates SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 19 Fig. 25. — Longitudinal section of the right side of the cord from a case of hemiplegia due to a cerebral lesion. The section passes through the cord at ahout the union of the anterior two-thirds with the posterior third of the posterior cornu. A, healthy zone, representing the fibres of the direct cerebellar tract; B, zone of degeneration, formed by the crossed pyramidal tract; C, healthy zone, formed by the longitudinal fibres which ascend along the external margin of the posterior cornu and are connected with the system of the lateral limiting layer ; D, healthy posterior cornu ; E, healthy posterior column. a more or less pronounced tendency to contracture. This is certain, but it must not be thought to be always the signification of this symptom. Those in whom apoplexy has occurred may present the same increase of reflex moveroents for some hours after an attack upon one or both sides without the occurrence of any paralysis or, for a better reason, of contracture. Thus too much importance must not be attached to such increase when it occurs during the first few days after an attack of hemiplegia, and only indicates secondary degeneration, when a certain length of time has passed since the attack occurred. Why the spasm occurs in cases of secondary degeneration of the pyramidal tract will now be considered, though but little can be said upon this subject. Many different explanations have been given of its occurrence. FoUin believed that the deformities which exist in hemiplegia of long standing are due to retraction of the muscles and soft parts. It is certainly true that such retraction does exist, but this is merely an associated symptom, and in no way the cause of the deformities. Hitzig maintained that the contracture was due to the fact that the motor impulses extended from the healthy to the paralysed side, and produced, as it were, an uninterrupted series of movements associated with muscular spasm in the limts upon that side. This explanation, which is ingenious, and to 20 LECTURES ON DISEASES OF THE SPINAL CORD. some extent probable, seems to be too exclusive. The more generally believed opinion is that already adopted by Straus in his Thesis,* written in 1875, according to which the tonic spasm is merely an increase of the muscular tone. D E Pig. 26. — Tracing of the patellar tendon reflex taken from the thigh of the non- paralysed side in a case of hemiplegia with secondary degeneration. A, myo- graphio line ; B, line upon which the time of the blow being given is indicated; C, line upon which the duration of the reflex is indicated ; DE, interval of time between the time of the blow being given and the commencement of muscular contraction ^ time of reflex. In this case the time occupied by the reflex was 40 thousandths of a second (with the correction 32 thousandths), that is to say, somewhat less than the normal time ; this shows that in hemiplegia the limb upon the " sound" side has also a tendency to spasm. (After Brissaud.) Pig. 27. — Tracing of the patellar tendon reflex taken from the thigh of the side upon which paralysis and contracture existed, in the same case of hemiplegia with secondary degeneration. Same letters as in fig. 26. It will be observed that upon this side the time of reflex DE is much shorter ; it was only 36 thousandths of a second (with the correction. 28 thousandths). (After Brissaud.) It is certain in fact that in hemiplegia the spasm is due to permanent contraction of the muscles alone, as Brissaud showed in his Thesis, by a most elegant demonstration. An Esmarch's * These d'agre'gation, 1875. SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 21 bandage being applied to a limb affected by contracture, so as 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 secondary degeneration 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- appearance 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. It is certain, gentlemen, that we cannot completely under- stand the role played by degeneration of the pyramidal tract in the production of contracture, until we are better acquainted with the precise functions of that tract. As stated in the last lecture there is much difference of opinion as to the action of the fibres in the pyramidal tract. Are they directly motor fibres, the irritation of which has for its consequence 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 cells 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 suggested 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 made to work, and the instrument immediately plays the air indicated. In the human organism the spinal cord may be looked upon as a musical box such as the one just mentioned. On account of education, on account of the local and special memory with which it is endowed, each part of the cord holds itself in readiness to execute such or such a movement, it might 22 LECTURES ON DISEASES OF THE SPINAL CORD. be said, to play such or such an air. A wish exists to walk : the pyramidal tract produces whatever change in the coi-d is necessary, in order that the centres of walking may act ; the lower limbs then execute the required movements, under the control, be it understood, of the higher centres which watch over the direction — the maintenance of equilibrium, &c. In a word, it is the motor impulse excited in the centres of the grey matter by the fibres in the pyramidal tract which determined the movement, but these are not the fibres by which it was executed. If this very seductive theory is admitted, the con- tracture which occurs after hemiplegia must be looked upon as the result of a permanent impulse which sclerosis of the pyramidal tract, after degeneration of that part, excites in the grey matter of the cord. This opinion has been most favourably received, and, as already observed, Vulpian and others support it. I myself find it very difficult to agree with them, since I can scarcely admit that a morbid process, such as that which produces sclerosis, of which the active period is limited, and the inflamma- tory effect can therefore be but temporary, would produce per- manent spasm lasting until the death of the patient. Nor do I understand how such a contracture could exist in spastic para- plegia, when there is not degeneration but non-development of the pyramidal tract, and no morbid process therefore exists which resembles the sclerosis due to secondary degeneration. Might it not be supposed that the function of the pyramidal tract is one of inhibition, analogous in a proportional degree to that of the vagus nerve upon the heart ? If its action is con- sidered to be of this nature the grey substance of the cord would still be the true motor power, but a power which is always under pressure, though at the same time ever ready to perform its functions (and in support of this suggestion, gentlemen, might not the rapidity, the suddenness with which the different reflex movements are made, be invoked?). The function of the pyramidal tract would be to exercise a restraining influence upon the cord and to prevent its ill-timed and unceasing action. If the will interferes to suspend temporarily this inhibiting effect upon the fibres of the tract which correspond to the centres of the grey substance, to the influence of \^'hich the contraction of a definite muscle is due, this muscle at once contracts in order SECONDAKY DEGENERATION OF THE PYRAMIDAL TRACT. 23 to produce the desired movement. If, on the other hand, this inhibiting action is permanently suspended owing to destruction of the fibres of the pyramidal tract, the cord being no longer undei! control, acts indefinitely. The muscular contraction due to -its influence is similarly uninterrupted, and persistent spasm occurs. I would remind you that this, gentlemen, is but a hj'pothesis, but one which, as I think, accords with clinical observation, and it is on this account that I lay it before you. Thus far, gentlemen, we have considered facts in their greatest simplicity; not rarely, however, they are slightly more complicated. Fig. 28. — Hemiplegia of the right side of a female, aooompanied by contracture, not only in the flexor muscles of the right leg, but also in those upon the ' ' sound ' ' side. (After Brissaud. ) I allude especially to the cases in which (A) secondary de- generation after a cerebral lesion occurs in the pyramidal tract on both sides of the cord, and (B) secondary degeneration is accompanied by more or less pronounced mu-scular atrophy. A. Bilateral degeneration of the pyramidal tracts consecutive to a unilateral cerebral lesion. — Westphal was, it appears, the first whose attention was drawn to the existence of such de- generation, since, in 1875, he observed that in some cases of 24 LECTURES ON DISEASES OF THE SPINAL CORD. hemiplegia there was foot clonus in the leg of the healthy side. In 1878 Dejerine made the same observation, and in the following year Brissaud called especial attention to contracture in the 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 learn from clinical observation, and let us suppose a hemiplegic patient suffering from contracture, due to secondary degeneration 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 unafiected, as one might d 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 hemiplegia. In addition to this, the side presents evidence of functional loss ef 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 execution is amply sufficient. Decided increase of the knee-jerk upon the "sound" side (already noted by Brissaud), and at times foot clonus also occur. Lastly, when the tendency to spasm is still more pronounced, as Brissaud has remarked, hemiplegia may be accompanied by contracture in the two lower extremities. The upper extremity on the " sound " side is somewhat similarly affected. Though it is not in a condition of rigidity, and hand clonus never occurs, the tendon reflexes are more or less excessive. The muscular force is diminished by about 38 per cent. (Dignat), this diminution being greater in proportion as the time of the observation is nearer to the occurrence of the SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 25 hemiplegia. As regards this point Friedlander made an in- teresting remark in comparing the upper extremities with each other. According to his obseTYations the diminution in the muscular power of the left upper extremity in right hemi- plegia is greater than that of the right upper extremity in left hemiplegia. Such, from a clinical point of view, is the participation of the limbs upon the " sound" side. The anatomical cause of the symptom was shown to us by the investigations of Pitres. This author collected 10 cases of degeneration of the two crossed pyramidal tracts, due to a unilateral cerebral lesion. Minute examination of these cases led him to conclusions which I shall ask your permission to mention. In six cases the lesion of the two crossed pyramidal tracts (PTyO) was equally developed, that is to say, the degeneration of the crossed pyramidal tract (FPyC) in the cord upon the same side as the cerebral lesion, was as pronounced as that of the corresponding tract (FPyC) upon the opposite side. In these cases the sclerotic area was slightly more extensive than when one side is alone affected, while on the other hand the sclerosis was more moderate in degree, as if a smaller number of fibres was affected than when the sclerosis is unilateral. In the four other cords the sclerosis was decidedly more pro- Fig. 29. — Section of the cord (cervical enlargement) in a case of hemiplegia from a lesion in the left hemisphere. The secondary degeneration inTolves the direct pyramidal tract (C), and the crossed pyramidal tract (B), besides the crossed pyramidal tract (A) upon the healthy side. nounced in the crossed pyramidal tract (FPyC) upon the side 26 LECTUBES ON DISEASES OF THE SPINAL CORD. opposite to the cerebral lesion, than in the TPyC upon the same side. .The direct pyramidal tract (FPyU) in the first six of the cases just mentioned wias affected as follows :— In ivw cases the tract (FPyD) V, as quite unaffected upon both sides ; in the fifth case it was unequally affected upon the two sides, while in the sixth case the tract of degeneration only occurred upon one side. As regards the four cords in which the crossed pyramidal tract (FPyC) on the same side as the cerebral lesion was less affected than that (FPyC) upon the opposite side, in two the direct pyramidal tract (FPyD) was in no way involved. In the third bilateral but unequal degeneration existed in the two direct pyramidal tracts (FPyD). Lastly, in the fourth, degenera- tion only occurred in the direct pyramidal tract (FPyD) upon one side alone, namely, upon that in which the crossed pyramidal tract (FPyC) was most affected. I have quoted the results of these ten autopsies at length, since they are the most interesting illustrations of the anomalies which occur in tbe distribution of the fibres of the pyramidal tract.* More than one explanation has been given of bilateral degene- ration of the crossed pyramidal tract (FPyC) after a lesion in one cerebral hemisphere alone. Charcot has expressed the opinion that the fibres of one crossed pyramidal tract (FPyC) passed to the same tract (FPyC) upon the opposite side by the anterior commissure, and the fibres might thus degenerate equally well upon either side of the cord. According, to Hallopeau the mechanism of the lesions is different. In his opinion, where the decussation of the pyramids occurs and the fibres of these tracts are closely interwoven, the inflammation of the degenerating crossed pyramidal ti-act (FPyC) would extend by contiguity to the corresponding tract on the opposite side. Thus the fibres of this tract would be equally involved in the degeneration. This theory, otherwise ingenious, is diflScult to accept, since as the knowledge of the pathological anatomy of the nervous system becomes greater, the more con- * Sherrington has observed in the dog and reproduced in the figures of his memoir: "On Secondary and Tertiary Degenerations in the spinal cord of the Dog" (Jcurn. of Physiology, Vol. VI., No. 4), a degeneration of the two crossed pyramidal tracts, after a lesion in one hemisphere alone. SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 27 vinced one becomes that secondary degeneration, properly so called, has little tendency to produce diffused inflammation around it such as that which Hallopeau believes to exist. The opinion of Pitres seems to me the true one. These facts are considered by him as fresh proof of the extreme irregularity in -the distribution of the pyramidal tracts and of the close con- nections which exist between the tracts on the two sides. These connections might d priori be believed to exist on account of the synchronism and association of the movements which occur in health. The existence of bilateral degeneration of the pyramidal tract completely explains the troubles which occur on the " sound" side in hemiplegia ; namely, decrease of muscular force, loss of power to co-ordinate certain movements, excess of the tendon reflexes, foot clonus, &c. I would make one observation before closing these remarks upon degeneration of the pyramidal tract. It has been stated by me that the upper extremity on the " sound" side never suffers from contracture, which on the other hand is frequently the case with the lower extremity. There seems to be an anatomical reason for this fact. The upper extremities are accustomed to act in an isolated way and separately from each other, whereas in standing or walking, which are the real functions of the lower extremities, the latter always move and work simul- taneously. It is thus explained why the bilateral distribution of the pyramidal tracts would not produce the same effects upon the upper and lower limbs, and why the symptoms due to degeneration of these tracts would be different in the two cases. B. Association of secondary degeneration of the pyramidal tracts with amyoi/rophy. — This subject, gentlemen, will be considered, although the connection of amyotrophy with secondary degene- ration of the pyramidal tract is not equally well recognized in the different hypotheses suggested to explain its existence. One thing is certain, that in some cases hemiplegia is followed by pronounced muscular atrophy in the paralysed limbs. This amyotrophy is specially marked in the small muscles of the hand, the thenar and hypothenar eminences being flattened, the interosseous spaces hollowed, and although the muscular atrophy is less pronounced than in the hand called that of Duchenne-Aran the appearance of the extremity is quite characteristic. The 28 LECTURES ON DISEASES OF THE SPINAL CORD. muscles of the fore arm, and even of the arm and lower ex- tremity, may be similarly affected. It should be noted that this is true amyotrophy, affecting some muscles in preference to others, and not the simple condition of general muscular wasting, which is so frequently observed in the paralysed limbs when the hemiplegia is of long duration. Different opinions have been held, gentlemen, as regards the anatomical cause of this muscular atrophy. , Charcot, who was one of the first to consider this condition, recognized in one case, which was soon followed by several others, that a characteristic lesion existed in the cells of the anterior comua, and expressed the opinion that this lesion was consecutive to degeneration of the pyramidal fibres and due to Fig. 30.— Section of the cord (cervical region) in a case of hemiplegia on the left side associated with amyotrophy. (After Pitres.) In the left anterior comu (the side of the degeneration in the crossed pyramidal tract (P) ) some cells alone of the anterior group (A) and the lateral group (L) are unaffected, the others being all destroyed by atrophy. the fact that inflammation of those fibres extended to the grey matter. This explanation was generally accepted, and confirm- atory facts had been published by Carri6re, Hallopeau, Pierret, Pitres, Brissaud, &c.,* when within the last few years Babinski, in publishing the account of an autopsy in a case of this kind, stated that no lesion was found, either in the comua of the spinal cord or the peripheral nerves. Shortly afterwards Quincke observed a case of the same kind, as did other authors, viz., Eoth, Muratow, Darkschewitsch, &c. On the other hand Dejerine found in four cases of atrophy, after hemiplegia, * During the publication of these lectures Joffroi and Achard published a work containing new facts and an interesting theory as regards the occurrence of this condition. (Arch, de mi!d. experim., Nov. 1, 1891.) SECONDARY DEGENERATION OF THE PYRAMIDAL TRACT. 29 peripheral neuritis without any lesion of the cornua or anterior roots. On account of the frequency of peripheral neuritis these cases may, in all probability, be classed with the preceding. Owing to the above facts one thing may be considered cer- tain, viz., that degeneration of the pyramids of the cord almost constantly produces, at any rate after a sufficient length of time, marked diminution in the size of the anterior cornua upon the same side. Fiirstner and Knoblauch called attention to this fact, and showed that it is in the lateral horn that this dimi- nution in size is specially pronounced. It can be easily con- ceived that in some conditions this alteration in the grey matter may extend to the cells of the anterior cornua and produce the lesions described by Chai'cot. The so-called "tertiary" degeneration mentioned by Sherrington and Langley may be justly regarded as confirming this idea. On the other hand there seems no reason to doubt the results obtained by the authors previously named, by whom no lesion was found in the grey substance of the anterior horns. Perhaps these facts, as Borgherini believes, are due to the trophic effects of the brain upon the muscular system. This explanation is also confirmed ly the cases in which muscular atrophy occurs rapidly after the production of a cerebral lesion. This, it is true, is a mere sup- position, but it is one which is by no means inadmissible when it is remembered, gentlemen, that the most pronounced muscular atrophy is at times observed in certain hysterical patients, of which the cause is in all probability some trophic disturbance of cerebral origin. 30 LECTURE III. DESCENDING DEGENERATION CONSECUTIVE TO TRANSVERSE LESIONS OF THE CORD. A. — In the anteko-latebal column :— «. Pyramidal trad, its degeneration in a case of transverse lesion of the cord is far more complete than when it is due to a cerebral lesion. Theories proposed in explanation of this fact. p. Portion of the lateral column external to the pyramidal tract : fibres of the intermediate tract of the lateral column, y. Anterior column: fibres of the marginal tract. Nature, path, and origin of these fibres : commissural fibres, commissural cells. Tract of descending degeneration of cerebellar origin (Marohi) . B. — In the POSTERlOE COLUMN : — Comma-shaped degeneration of Schultze : small nnmber of confirming facts. Suppositions as to the nature of the fibres affected by this degbneration. Gentlemen, — In the preceding lecture the degeneration of the pyramidal tract which follows cerebral lesions was studied. The secondary degeneration which follows a lesion seated in the spinal cord will now be considered. In order that the subject may be more clear it will be supposed that the cord has been almost completely divided by a trans- verse lesion. As you know, gentlemen, in such a lesion a general, and more or less total destruction of the anatomical elements of the cord exists to the extent of four, five, six millimetres (^, i, ^ inch) or more in height, according to the mode in which the lesion has been produced. In this zone of total destruction, " the zone of traumatic degeneration " of Schiefferdecker, the way in which each tract is affected cannot naturally be studied. It is only in a lower portion of the cord, when the lesions are sufficiently localized, that their distribution can be advantageously con- sidered. A. — Antero-lateral Column. This column being composed of afferent and efferent fibres, the degeneration of many distinct tracts within it, both in the ascending and descending direction, must be described. DESCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 31 a. Pyramidal trad. — The fibres of this tract have (in all probability) their trophic centre in the cerebral cortex. It is therefore most probable that if, owing to some cause, they are divided in any part of their path in the cord, the portion seated below the point of division will be undoubtedly affected by secondary degeneration. This, in fact, has been already shown to occur when the continuance of these fibres is interrupted by a lesion in their path through the brain. At the same time, degeneration of the pyramidal tract has a special character according as a lesion in the brain or cord is the cause of its existence. Bouchard first remarked that when the initial lesion Fig. 31. — Degeneration of the crossed Fig. 32. — Degeneration of the lateral pyramidal tract following a lesion colnmn following a transverse lesion of the cerebral hemispheres (dia- of the cord a few centimetres above grammatic). the region which is here represented (diagrammatic). It wiU be observed that the extent of the degeneration is notably greater than in the pre- ceding fignre. is in the cord the secondary degeneration of the pyramidal tract is notably more extensive than when it is seated in the brain. This statement is absolutely true, having been confirmed by all observers, although the explanations given of this difference vary. These explanations may be connected with one of the two following opinions : — a. A more considerable portion of the cord is affected by degeneration, because not only the pyramidal fibres but also those belonging to other tracts are involved (the commissural fibres of Bouchard connecting points of the grey substance which are at a more or less different height in the cord) ; these latter fibres being intimately mixed with those of the pyramidal tract, and degenerating at the same time necessarily render the diseased part more extensive. 32 LECTURES ON DISEASES OF THE SPINAL CORD. b. 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 less 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, gentlemei;!, that these two ojjinions 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 case of transverse lesion seated on the dorsal region. Degeneration of the two crossed pyramidal tracts (semi-diagrammatic) . So far, the whole pyramidal tract has been considered ; it need scarcely be added- that degeneration maj^ 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. ;3. Portion of the antero-laleral 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 are 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 DEGENERATION 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 a dog after removal of one of the cerebral hemispheres.- (After Singer and Miinzer.) It will be remarked how much the extent of the degeneration due to the cerebral lesion differs from that which occurs (fig. 33) after a transverse lesion of the cord. those of the crossed pyramidal tract. At this point, in fact, nerve fibres are found which difier much in size. Pig. 35. — Descending degeneration in the cord of a dog after division of the antero-lateral column upon the same side, in the upper cervical region. (After Singer and Miinzer.) Two regions are seen to be affected by degeneration (indicated by dots) in the right antero-lateral column ; the smallest at the anterior angle of the cord is the descending suIco-Tnarginal tract* while the larger is in the intermediate tract of the lateral column.f The pyramidal fibres are included in this degenerated part. 7. Anterior column. — In this column also certain fibres are affected by descending degeneration after transverse lesions of * Vide p. 34 (Translator). t f'«'''« P- 32 (Translator)'. 3 :34 LECTURES ON DISEASES, OF THE SPINAL CORD. the cord in opposition to the opinion of Schiefferdecker, who thought that .these were stray fibres of the pyramidal tract, whereas it seems certain (Flechsig and Singer) that they are quite unconnected with this tract, since they do not degenerate after lesions which are confined to the brain. This is the degen- eration of the marginal tract (Lowenthal). The degenerated fibres of this marginal tract are found in man and in the monkey in the part of the anterior column which adjoins the anterior fissure, which I shall call the sulco-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 zo7ie. Fig. 36. — Diagram of the principal seats of descending degeneration in cases of transverse lesion of the cord in the dorsal region. A, Sulco-marginal region, in which two kinds of fibres are found : firstly, those of the direct pyramidal tract (+ + + +); secondly, those of the descending system of the sulco-marginal zone (. . . .). B, C, Intermediate tract of the lateral column ; some fibres of this tract are found at the peripheral part of the cord intimately mixed with those of the tract of Gowers. D, Zone o^ the crossed pyramidal tract. This zone contains two sorts of fibres : firstly, those of the crossed pyramidal tract {+ + +); secondly, those of the inier^nediaie tract of the lateral column (. . . .), which are intimately mixed with the preceding fibres but in smaller 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 course, since'traces 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 commissiMral fil/res, 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 such 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 commissural cells seated, as Golgi had already shown, in many points of the grey matter. From these, which are smaller than the motor cells, an 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. During 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 degeneration, 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. It is doubtful, however, whether such a statement can be made with regard to all the fibres, and an opinion recently expressed by Marchit should at any rate be mentioned. This author, having removed the whole or part of the cerebellum, studied the subsequent degeneration in the cerebellar peduncles. He was able to trace such degeneration in the inferior cerebellar peduncle, and found that at about the height of the olivary » p. 31. t Marohi, Origine e decorso dei pedtmcoU cerebellari {Rivitta sperimeniale di Freniatria e Med. leg., Vol. XVII., p. 367). 36 LECTURES ON DISEASES OF THE SPINAL CORD. body, the posterior longitudinal tract joined the band of Reil,* the conjoint mass of fibres passing into the antero-lateral columns. In these a somewhat large number of degenerated fibres existed, forming a mass of some size in front of the crossed Fig. 37. — Diagram representing the longitudinal aspect of tli-e cord as seen through a transparent medium. A, Commissural cell ; B, Prolongation of that cell, passing through the anterior commissure, and dividing into an ascending (BG) and descending branch (BD) ; GG', divisions of the descending branch, forming branohlets round the nerve-cells of the grey matter. E, Prolongation of the same kind as B, but not passing to the other side through the anterior com- missure; from this prolongation branohlets (PF') are given off, analogous to those (GG') which are connected with the descending branch (BD), and which are also divided into branohlets surrounding the nerve cells of the grey matter. These cells are not represented, that the figure may not bo un- necessarily complicated. (Diagram after a figure of Eamon y Cajal.) pyramidal tract, and extending also into other parts of the antero-lateral and anterior columns, with a pronounced tendency to occupy the peripheral parts of the cord. According to Marchi, therefore, fibres exist in the antero-lateral columns of * Vide p. 45 (Translator). DESCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 37 which the trophic centre is seated in the cerebellum, so that when a transverse lesion of the cord occurs these fibres would be affected by descending degeneration. Their seat in the cord exactly corresponds to the regions in which degeneration has just been studied by us, and wbich are termed the intermediate tract of the lateral column and the descending s'lilco-ma/rginal tract. Such, gentlemen, may be said with regard to the anterior and antero-lateral columns, but it must not be supposed that descending degeneration after transverse lesions of the cord is Fig. 38. Fig. 39. Fig.40» Section of the cord of a monkey (?), from which the right half of the cerebellum was removed (after Marchi). Fig. 88, Lumbar region; Fig. 39, Dorsal region ; Fig. 40, Ceryical region. It will be remarked that the zone of degeneration (indicated by points), which occupies in the thfee figures the periphery of the antero-lateral column, just extendi) within the region of the pyramidal tract, and encroaches slightly upon the anterior part of the direct cerebellar tract. In the original figure some points of degeneration also exist in the anterior roots, which are here omitted. limited to these columns, as it may occur also in the ^posterior columns. B. — The Posterior Column. It is to Schultze that we specially owe the knowledge that secondary degeneration occurs in this part, although in his work (Archiv fur Psychiatrie, 1883) that author points out that similar facts had been previously observed by Westphal, Kahler and Pick, and Strumpell. It is none the less true, however, that attention was specially drawn by his observations to this form of degeneration. The case to which allusion was made in these initiatory remarks was one of compression with complete division of the cord in the middle part of the cervical enlarge- ment. At a distance of 2 or 3 centimetres (f inch to 1 inch) 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 some distance. Owing to its form this degeneration may very justly be termed the comma- 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 a focus of compression, seated in the middle part of the cervical enlargement. (After Schultze.) A, Degenerated pyramidal tract ; B, Comma-shaped degeneration in the posterior columns. The original figure indicated a small islet of degene- ration in the antero-lateral 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 occupied 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 require, 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. This is as yet unknown, so that the uncertainty * 0. Barbacci, Contribulo anatomico e sperimentale alio studio delle der/enerazioni >econdarie, ^c. (to Sjierimentale, 1891, parts III. and IV., pp. 335 and 406). DESCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 39 still exists. According to Schultze the fibres whose alteration produces comma-shaped degeneration are the descending branches pi 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 after 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. 42. Fig. 43. Fig. 44. Fig. 45. Fig. 46. Fig. 47. Sections of the cord from a case of fracture of the spine with destruction of the cord between the 8th cervical and 1st dorsal pair. (After Tooth.) Fig. 42. — Transverse lesion ; the destrnction is almost complete. Fig. 43.— let dorsal pair; the greater part of the white columns is afEected. The zone of traumatic degeneration exists here also. Fig. 44. — 2nd dorsal pair ; in the antero-lateral column the region of the direct cerebellar tract and the tract of Gowers is already almost free from de- generation. In the posterior column the " comma-shaped degeneration " of Schultze is most clearly seen. Fig. 45. — 3rd dorsal pair ; the same observations may be made. Fig. 46. — 6th dorsal pair ; the degeneration is confined to the crossed pyramidal tract, the intermediate tract of the lateral column, the descending sulco- marginal tract, and the direct pyramidal tract. Fig. 47. — 7th dorsal pair ; the degeneration of the pyramidal tract is still most clear, while that of the intermediate and even more that of the sulco-marginal tract are far less apparent. of the commissural fibres. Possiblj-, and this is a simple suggestion on my part, the condition of the grey matter in the cord should be considered, and in future it will be observed whether this comma-shaped degeneration only occurs when the transverse lesion involves to a greater or less extent the grey substance of the cord. If so, this would be a fresh argument in 40 LECTURES ON DISEASES OF THE SPINAL COED. favour of the theory that the commissural fibres are affected in this form of degeneration. With regard to the lesions which exist in tabes 1 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. As 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 are 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. 41 LECTURE IV. ASCENDING DEGENERATION CONSECUTIVE TO LESIONS OF THE NERVE-KOOTS. Anatomical study of the afferent system of the cord proceeding from the fibres of the posterior roots. Development of this system; origin in the external layer of the blastoderm ; division of the nenral plate into a central segment which will form the motor portions of the oerebro-spinal axis, and two lateral segments from which the sympathetic nerve and ganglia of the spinal cord will be formed. Fibres of the posterior roots. Researches of Singer and Munzer. These fibres may be classed in three groups, according to the portion of the spinal cord in which they end. Their terminal nuclei. Degeneration of the posterior columns after lesions of the posterior roots; researches of Singer, Tooth and Hartley; clinical observations of Kahler, Schultze, &c. Gentlemen, — Before commencing to study ascending de- generation in the cord it will be useful to review the anatomical disposition of the intramedullary afferent system proceeding from the posterim- roots. That this description may be more easily understood, the posterior roots will be themselves con- sidered, which constitute, so to speak, the affluent branches of that system. In this exposition the names of numerous distinguished neurologists or anatomists must be mentioned, who first acquired the knowledge which we possess upon this subject. Of these I would specially mention Singer, His, Eamon y Cajal, Edinger, Lenhossek, Kahler, Lissauer, &c., as those whose works are of special interest in connection with this subject. Without entering into very complex details in reference to the development of the cerebro-spinal axis, I would merely remind you of the principal facts connected with its develop- . ment which are specially applicable to the subject which now occupies our attention. As His has shown, the nervous system derives its origin from the external layer of the blastoderm of which it forms at first a longitudinal thickening. This neural plate (Neuralplatte) 42 LECTURES ON DISEASES OF THE SPINAL COED. becomes divided longitudinally into three segments, from one of which, the median, the central nervous system is developed, and notably the motor portions (the motor cells of the cord with their prolongations vi'hich 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 cells 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 root 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, gentleraen, 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 entering the grey matter. These varieties have been recognized to exist • by different authors, and were very accurately described by Singer and Mtinzer. The fibres of the first group (short fibres') 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 (fibres of medium length) ascend to a certain height in the posterior column, and as they pass • onwards are seated more and more internally, so tliat their ASCENDING DEGENERATION AFTER NERVE-ROOT LESIONS. 43 general direction is oblique' upwards and inwards. As, how- ever, this group ascends, each of the fibres composing it curves outwards and passes into the grey substance of the posterior cornu, which it joins, not at its point, but more anteriorly, and to a special extent where the columns of Clarke are seated. This group thus becomes smaller and smaller as '^f^ Fig. 48. — Diagram of the path and termination of the posterior root fibres ; the black fusiform patch G, placed below and upon the right side, represents a spinal ganglion, the line which crosses it indicating the point of section of the posterior root which issues from it. This posterior root divides into three branches ; each of these gives descending branches : + + + + short fibres pass- ing to the apex of the posterior cbrnu ; fibres of medium length entering the base of the posterior horn after having ascended within the column of Burdaoh ; long fibres passing into the nucleus of the column of Goll (nucleus gracilis) D, after having ascended within the interior of that column. The nucleus of Burdaeh (nucleus cuneatus) is at C receiving the long fibres and those of medium length issuing from the roots in the cervical region. higher portions of the cord are reached, and finally, when all the fibres composing it have entered the grey substance of the posterior cornu, totally disappears. 44 LECTURES ON DISEASES OF THE SPINAL CORD. The fibres of the third group (long fibres) though least numerous are by far the longest, since instead of being lost in the grey matter of the cord they are prolonged as far as the medulla oblongata. When this part is reached they join the grey substance in a similar manner, and the points at which they do so are known by the name of the nucleus of the columm, of Goll (nucleus gracilis) and nucleus of the column of Burdach (nucleus cuneatus). It should be observed that on account of the tendency which all the root fibres of the posterior columns have to pass inwards being thrust back as it were on the outer side by fibres from the Fig. 49.— Diagram showing the constitution of the column of Goll. The fibres which constitute this column come from the posterior roots A, A', A", A'", A'^-, A^-. In quite the lowest part of the cord the column of Goll therefore contains a smaller number of fibres than in the parts which are at a higher level. This increased number of fibres in the columns of Goll is not found to exist throughout the whole length of the cord, since, according to most authors, the roots in the lumbar region alone enter into the formation of this column. new roots penetrating into the cord, those of the root fibres which have joined the cord at a lower level occupy the portion of the posterior columns which is closest to the posterior median fissure, constituting what are termed the columns of Goll. The long fibres of the third group coming from the roots which pass into the upper portion of the cord are external to these, and join the nucleus of the column of Burdach (nucleus cuneatus). At what height in the cord the root fibres cease to join the nucleus ASCENDING DEGENERATION AFTER NERVE-ROOT LESIONS. 45 of GoU, and are directed towards the nucleus of Burdach, even in the opinion of Singer and Miinzer, is quite uncertain. It should be observed that the columns of Goll are not universally believed to be entirely composed of long fibres coming from the lower segments of the cord alone, especially from the roots of the cauda equina. Thus, in the opinion of Barbacci, the division of the roots seated in the upper segments of the cord, especially of those in the cervical region, is followed by degeneration in some fibres of the column of Goll. However this is, it may be considered certain that by far the greatest number, if not all of the fibres which compose this column come from roots connected with the lower part of the cord. Such, then, considered briefly, is the path of the fibres in the posterior roots. It may be said in addition that some of these fibres pass through the anterior commissure, join the antero- lateral column of the opposite side, and eventually reach the corresponding band of Reil* (Auerbach), Some fibres also pass through the posterior commissure into the posterior horn of the opposite side. In a general way it may be said that all the fibres of the posterior roots pass eventually into the grey substance of the cord or medulla oblongata. According to recent observations (Golgi, Ramon y Gajal, Lenhossek, Kolliker) these fibres having reached the grey substance divide into numerous ramifications, which surround the nerve cells, but do not penetrate within them. The cellular groups in the grey substance are not the nuclei from which they originate (which, as we have seen, are constituted by the cells of the spinal ganglia) but those in ivhich they terminate (Endkerne, His). Both in the cord and medulla oblongata fresh fibres are given 00" from these terminal nuclei, which cross each other in both structures. Joining each other in the medulla oblongata, they constitute the plexus of fibres which is termed the hand of Reil* {Schleife, Germ.). As to the final termination of these fibres in the brain our knowledge is more limited. Some fibres of the band of Reil, according to Meynert, join the corpora quadrigemina, while some, according to other authors, pass into the expansion of the lenticular nucleus. More recently Flechsig * Vide p. 36 (Translator). 46 LECTURES ON DISEASES OF THE SPINAL CORD. and Hosel* have shown that a large number of fibres, specially of the middle portion of the band of Reil, pass into the motor convolutions adjoining the fissure of Rolando. This summary description having been given, you will be able, gentlemen, to understand the path of that portion of the afferent system which proceeds from the ganglia of the spinal cord. The cells of these ganglia are centres of origin, while those in the grey substance of the cord or medulla oblongata are centres of termination, with the modification that no fibres pene- trate into their interior, expansions being formed by the fibres 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 continuous, 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 posteriw roots in the spinal cord, 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, &c. If, for instance, as was done by Tooth, one of the posterior roots is divided in the cord of an animal such as the monhey, and a suf- 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 * Fleohsig and Hosel, Die CerOralwindmigen, em Centralorgan der Hlnteratrange (Neurologis:hev Centralblai, 1890, p. 417). 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 posterolateral 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 Fig. SO. Sections of the cord from a monkey in which Horsley had divided all the posterior roots of the oauda equina at the distance of about 1 centimetre (§ in.) above the inferior extremity of the conus meduUaris. ' (After Tooth.) Fig. 50 : 5th lumbar ; A, Section of roots which have undergone ascending degeneration, and which after being placed round the cord ascend along its surface until one after the other penetrates into its substance ; B, Degeneration of the posterior column where it is in contact with the posterior cornu (entrance of the pos- terior roots into the. cord, true external bandlets). Pig. 51 : 3rd lumbal:; the posterior column has undergone more degeneration (B) than at the level of the 5th lumbar, because at the latter point it had not yet received all the fibres of the posterior roots which had undergone ascending degeneration. The degenerated zone (B) commences at the level of the 3rd lumbar nerve to approach the middle line. Fig. 52 : 11th dorsal ; the degeneration (B) has completely left the posterior cornu in order to approach the posterior median fissure. Fig. 63 : 8th dorsal. Fig. 54 : 4th cervical ; the degeneration (B) is seated in the posterior part of the column of Goll. or sacral region) the degeneration occupying a more and more internal position, finally affects the postero-median column or that of Goll, and may then be followed as far as the medulla oblongata, where it is seated near the nucleus of the column of 48 LECTURES ON DISEASES OF THE SPINAL CORD. Goll (nucleus gracilis). This statement is certainly more or less theoretical ; it would be difficult to follow for such a distance the degeneration of a single root, since as a higher and higher level is I'eached the number of fibres lost in the grey substance is so great that at last the sclerosis almost entirely disappears. When, however, many of the posterior roots have been divided or injured the long fibres which degenerate are so numerous that they can be followed throughout their entire length. It is unnecessary to describe at greater length the 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 as they- enter the cord a Y-shaped division of the posterior roots occurs, an ascending (which has alone been considered) and a descendiTUj 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 bifur- cation of the divided posterior roots. These branches, however, being shorter, their degeneration 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 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 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 changes in the grey matter must also 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 ASCENDING DEGENERATION AFTER NERVE-ROOT LESIONS. 49 found to be much diminished in size. The effect produced by division of the posterior roots in the cauda equina upon Clarke's column should also be specially noticed. It is most clearly seen in fact that after such division the fine reticulum of nerve fibres disappears throughout the whole section of Clarke's columns ; whilst, however pronounced the alteration of these fibres may be, the cells of Clarke's columns remain in a perfectly healthy condition. This also shows, what has been already stated, that the fibres of the posterior roots terminate close to the cells of the grey matter, but without penetrating within them ; thus the former may disappear without degeneration of the latter. These facts are well known, and have been mentioned by different authors, especially by Schultze, Lissauer, Mott, and others. If I have thus dwelt upon the degeneration which follows lesions of the roots it is not, gentlemen, on account of its being frequently observed in cases of injury or compression of the cord, or cauda equina, which but rarely occur, but because in my opinion a very important part of the pathology of the spinal cord is intimately connected with lesions of the posterior roots, and I have therefore felt it my duty to consider them at some length. 50 LECTURE V. ASCENDING DEGENERATION AFTER TRANSVERSE LESIONS OF THE SPINAL CORD. A. In the posterior coitjmn : a. In the column nf Burdach. p. In the coltimn of Goll. Difference in seat and extent of degeneration according to the height of the transverse lesion. B. In the ANTebo-latkeal column : a. Direct cerebellar trad, its description by Plechaig, its seat, form, origin, path, termination, and degeneration. ?■ Tract nf Gowers (antero-lateral ascending tract of English anthors), its seat, form, origin, path, termination, and degeneration. C In the GEET SUBSTANCE or THE COED. Degenerations in the grey matter are but little known, lesions found by Barbacci. Sound condition of lateral limiting layer {seitliche Grenzschicht of German authors). Gentlemen, — In the preceding lecture ascending degeneration of the cord after lesions of the posterior roots was considered, and the path of those roots in the cord and composition of the posterior column was carefully explained, although in a somewhat summary manner. The characters of ascending de- generation when it is secondary, not to a lesion of the roots, but to one of the cord itself, will now be discussed. A. In the posterior column. — The fibres of this column having been just considered, it is unnecessary to enter into minute details. Whichever part of the cord is the seat of the transverse lesion, all the ascending fibres of the posterior columns in this part would evidently be totally destroyed to a distance of some millimetres (1 mm.= 0'039 in.) above the lesion, this con- stituting the area of traumatic degeneration of Schiefferdecker. But as this area is left, the effect of the lesion upon the long fibres, and those of medium length, which constitute the tracts of Goll and of Burdach, may be studied with advantage. a. Column of Burdach. — This column, of which almost the whole at first undergoes degeneration, receives from each fresh posterior root as it enters the cord a contingent of sound fibres, which are placed iit first along the inner side of the point of the posterior ASCENDING DEGENERATION AFTEE TRANSVERSE LESIONS. 51 horn, pressing the degenerated fibres inwards; thus the pos- terior and external portion of the column of Burdach is that from which the indications of ascending degeneration most quickly disappear. The anterior part of the same column, which adjoins the posterior commissure, remains altered over a some- what longer portion of its path (usually over a distance corre- sponding to the entry of from two to four pairs of nerves into the cord) ; this is because the fibres which constitute the anterior part do not belong to the system of the posterior roots, but to that of the commissural fibres which bind together the grey substance at different heights ; and the addition of such fibres appears to be less rapid and less extensive than that of the posterior root fibres. In short, the degeneration of the column of Burdach diminishes little by little, and disappears from without inwards, and from behind forwards, until completely ceasing in this tract, though still existing near the posterior median fissure in the region which has been termed the column of Goll (vide figs. 64, 65, 66). 0. Column of Goll. — The long fibres of the posterior roots ■ which join together to form this tract pass to a certain point in the grey matter of the medulla oblongata, which is termed, as you know, gentlemen, the nucleus of the column of Goll (nucleus gracilis). The greater part of, or all the fibres of this tract are derived, as you also know, from the posterior roots of the cauda equina and of the pairs of nerves connected with the lower part of the cord. Thus every transverse lesion of the cord which aflects these fibres will be followed by their degeneration, which can be traced almost as far as the floor of the 4th ventricle. At the same time the degeneration of the column of Goll must not be supposed to be always identically the same, gentlemen, whichever portion of the cord is divided. Thus, for instance, if this occurs quite at the level of the conus terminalis so as only to affect the inferior roots of the cauda equina the degeneration of the column of Goll which follows only affects its posterior and ■median part. This is the same as stating that the nerve fibres of the posterior columns are placed so much the nearer to the middle line and surface of the cord as they have the longer distance to go. If, on the other hand, the cord is divided in its upper part, as. 52 LECTURES ON DISEASES OF THE SPINAL COED. 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 united fibres each of which has its own individuality. Thus the column of Goll, as Schultze also believes, cannot be absolutely dis- tinguished from that of Burdach, since during part of its path Pig. 55.— Division of the cord (cervical region) in a case of ascending degeneration in the columns of Goll (A) dne 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 ao-ree- ment as to the facts of the case, it is unnecessary to dwell upon this point. Such, gentlemen, is what I 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 occupies also, and over a considerable area, the antero-lateral columns. 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 Turck, who traced it as far as the restiform body, but 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 A Pig. 56.— Division of the mednlla oblongata at its lower part, in a case of ascending degeneration of the colnmns of GoU (A) due to compression of the dorsal region. 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 difier as to the point in the cord at which this tract 54 LECTURES ON DISEASES OF THE SPINAL CORD. can be first recognized. Some (Kahler and Pick) state that it is at the level of the 9th dorsal pair ; others (Schultze) believe it to be at the height of the 10th pair ; Tooth concluding from his observations that it is at the level of the 8th pair. Quite recently Barbacci states that he has observed degeneration of this tract when the lesion of the cord was seated at the level of the 11th and even of the 12th pair. I shall soon have occasion to consider this latter point, and when the origin of the fibres of this tract is better known to you the interpretation Fig- 57. — Longitudinal aspect of the cord and lower part of the medulla oblongata seen, as it were, through a transparent medium.' This schematic figure shows the origin of the fibres of the direct cerebellar tract (P) and tract of Gowers (E). (The tract should have been prolonged beyond the ascending root of the fifth nerve (B), in which by an error of the designer it seems to end.) The fibres of the direct cerebellar tract issue from the cells of the columns of Clarke (G). The fibres of the tract of Gowers would issue on the one hand from the cells of the lateral horn (H), on the other from those of the columns of Clarke (G). which may be given to this statement on the part of the Italian author will be considered. The study of the direct cerebellar tract will now be continued by examining its path from beginning to end. The size of this tract varies according to the height at which it is considered. It is thus smaller in the lower dorsal than in the cervical region. The fibres which constitute it ascend the whole length of the dorsal and cervical cord to the medulla ASCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 65 oblongata, being always seated behind an imaginary line dividing the cord and medulla oblongata transversely in its middle part. Gradually the posterior horn of grey matter leaves the surface of the cord, while the direct cerebellar tract remains there, but pressed backwards to a slight extent by the ascending root of the 5th nerve, which is placed in front of it ; the tract is then contained in the restiform body, within which it passes into the cerebellum, having reached which it terminates, according to Flechsig, in the superior worm. It must not be thought, gentlemen, that the fibres of the cord seated in the direct cerebellar tract all belong, on this account, to that column. It is, in fact, recognized that even when pro- nounced degeneration of the direct cerebellar tract occurs a small number of fibres remain unaSected in 'the midst of the degenerated area. It is certainly true that some of the unaSected fibres do belong to the direct cerebellar tract, and having joined it above the point at which the cord was divided, are, owing to that fact, in a sound condition ; others, however, apparently belong to a different system, in which degeneration occiirs in a. descending direction. These are fibres which seem to form in part that tract of Marchi.of which I have already had occasion to speak. You remember, gentlemen, that in the opinion of Marchi the origin of this tract is in the cerebellum, that it passes by the inferior cerebellar peduncle, and descending into the cord is dispersed through the antero-lateral column, and to a large extent near its surface. What is the origin of the fibres of the direct cerebellar tract ? According to what has been alreadjr remarked about the anatomy of the cord, it is probably to be found in a group of nerve cells,. none other in fact than that termed the cells of the columns of Clarice. The columns of Clarke will again be considered with regard to the pathological anatomy of tabes. - I will merely observe that by this name are designated two important groups of cells, each of which is seated at the base of the corresponding posterior horn, and which are much developed in the dorsal region. The pyramidal prolongation of each cell passes obliquely across the lateral columns to the surface of the cord, where it seems to curve upwards and constitute one of the fibres of the direct cerebellar tract. According to some authors the cells of the 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 a 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 J :._B Tig. 58. — Division of the cord (cervical region) in a case of transverse lesion seated in the dorsal region. Ascending degeneration : — A, Columns of GoU ; 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 Barbacci,* 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. Fig 59. — Section of the spinal cord (upper part of the cervical region immediately below the decussation of the pyramids) in a case of transverse lesion of the cord in the dorsal region. A, Columns of Goll; B, Direct cerebellar tracts and tract of Gowers; C, Marginal tract of the anterior column. (Damasohino collection.) Fig 60. Fig. 62. Fig. 63. Sections of the medulla oblongata from a monkey in which a division had been made through half of the cord between the 7th and 8th cervical vertebrse (after Tooth). At the lower part of the medulla oblongata (fig. 60) the direct cere- bellar tract (B) and the tract of Gowers (A) are in contact, and apparently confused together. As a higher and higher point in the medulla oblongata is reached, the two tracts tend to separate, and in fig. 63 are seen to be quite distinct from each other. jS. Trad of Gowers (antero-lateral ascending tract of English , authors) . — Although the altered part which resulted from degene- 58 LECTURES ON DISEASES OF THE SPINAL COED. ration of this tract had been previously seen and mentioned in the account of some autopsies, it may fairly be said that Gowers was the first who described it clearly and distinguished it from the change due to degeneration of the direct cerebellar tract with which it had been previously confused. It is for this reason that I shall call this tract, as some other authors do, by the name of the tract of Gowers, a term which seems to me preferable to antero-lateral ascending tract, a name which some English authors employ, especially Tooth, in the interesting chapter which he devotes to the study of these fibres. The tract of Gowers is seated at the surface of the cord, where it forms a narrow band ; its posterior extremity is immediately in front of the direct cerebellar tract, while its anterior extremity terminates at the point where the anterior roots enter the cord. The origin of this tract is at a much lower level than that of the direct cerebellar tract, since its fibres are found even in the lower part of the lumbar enlargement (Bechterew), and it is found to degenerate after transverse lesions seated in the lumbar region, whereas the direct cerebellar tract is unaffected. It is doubtful from which part of the cord or from what cells these fibres originate. According to certain authors, some, but not all the fibres proceed from the cells of the columns of Clarke in the same way as those of the direct cerebellar tract, while the others, which are of larger size and more numerous, take origin from the cells of the anterior horns. However this may be, the tract formed by these fibres passes upwards along the whole length of the cord, increasing gradually in size and becoming more and more distinct from the direct cerebellar tract until it reaches the medulla oblongata. It there becomes comma shaped, the head of the comma being opposite the gelatinous substance while its tail is upon a plane posterior to that of the ascending root of the 5th nerve. It is always seated in front of the direct cerebellar tract, but instead of being in contact therewith is distinctly separated from it. The termination of the fibres of the tract of Gowers is little better known than their origin. According to some authors they are in intimate connection with the nucleus lateralis* in * This nucleus is seated in the lower part of the medulla oblongata on a level with the commencement of the olive. Anteriorly to it is found a group of large multipolar cells of the same type as those which are seen in the anterior horns of the spinal cord. The nucleus itself is composed of small cells of the bi-polar type. ASCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 59 which a certain number of fibres terminate (Bechterew) ; according to Tooth such is the destination of the narrow fibres contained in Fig. 64. Fig. 65. Fig. 66. Sections of the cord from a case of fracture of the spine with destruction of the cord between the 8th cervical and 1st dorsal pair of nerves (after Tooth). Fig. 64 : 7th cervical ; almost the whole posterior column is degenerated, except in the vicinity of the posterior horn, where the 8th and 7th cervical roots enter the cord introducing a contingent of sound fibres. The whole surface of the autero-lateral columns is degenerated, the degeneration at some points invading the cord to such an extent that the zone of traumatic degeneration seems to have been again reached. Fig. 65 : 4th cervical ; in the posterior columns the distance of the degeneration from the grey substance is gradually increasing. In the antero-lateral columns degeneration has only occurred at the surface of the cord. Fig. 66 ; 2nd cervical ; in the posterior column the degeneration is almost entirely limited to the tract of Goll. That of the antero-lateral column (degeneration of the direct cerebellar tract, and of the tract of Gowers) no longer extends anteriorly as far as the margin of t&e anterior fissure. this tract. The thicker fibres pass into the cerebeUum through the superior cerebellar peduncle. It will be noticed, gentlemen, that all the observations which Fig. 67- — Lower part of the medulla oblongata from the man in whom there was a transverse lesion with destruction of the cord between the 8th cervical and 1st dorsal pair of nerves, the subject of figs. 64 to 66. A, Degeneration of the tract of Gowers ; B, Degeneration of the direct cerebellar tract. Fig. 68 — Middle portion of the same medulla oblongata as in fig. 67 (after Tooth). A, Degeneration of the tract of Gowers ; B, Degeneration of the direct cerebellar tract. we have just made tend to show that the direct cerebellar tract is distinct from the tract of Gowers. This fact is confirmed by their mode of development. Bech-i 60 LECTURES ON DISEASES OF THE SriNAL COED. terew has in fact shown that the tract of Gowers appears at a relatively late date, since it does so at the commencement of the 8th month, immediately before that of the pyramidal tract, while at that time the development of the direct cerebellar tract has already occurred. Such anatomical considerations I wished to put before you with regard to the tract of Gowers, which contain all that refers to the degeneration of this ti'act after transverse lesions of the cord, and it appears to me useless to say more upon this subject. The changes in the GREY substance of the coed which are Fig. 69. — Diagram representing the principal seats of ascending degeneration in transverse lesions of the cord (dorsal region) . A, Column of Goll. BB, Column of Burdach ; the degeneration in the part of this column adjoining the column of Goll is more pronounced than in that which is close to the posterior horn. The degeneration in tiie column of Burdach can only be traced to a short distance ahove the point where the transverse lesion occurs. C, Direct cere- bellar tract. D, Tract of Gowers. This tract is largest at its posterior part, becoming gradually narrower anteriorly. The point of its termination is somewhat uncertain. It is doubtful whether the fibres in which ascending degeneration occurs by the side of the anterior fissure (they should have been prolonged along the whole length of the fissure) belong to the tract of Gowers or not. In my opinion they are distinct from that tract, the fibres seeming to be shorter than those of the tract of Gowers ; they might be denominated the Bulco-marginal ascanding tract. due to the ascending degeneration which follows transverse lesions of the cord, have as yet been little studied, and their description (Tooth, Hofrichter, Barbacci) is still incomplete. According to the latter author it is found that the grey matter above the transverse lesion has a granular appearance ; that the nervous reticulum at this level is scanty, and may even completely be lost, while the cells disappear, or exhibit changes which are clearly retrograde in character, some of them becoming full of ASCENDING DEGENERATION AFTER TRANSVERSE LESIONS. 61 pigment, while in others more or less atrophy exists. The changes are always more marked in the region of the posterior than in that of the anterior horn. As the distance from the point of division increases, these changes diminish. The granii- lar appearance first ceases, then the lesions in the cells of the anterior horns, then those in the cells of the posterior horns, and lastly those in the nervous reticulum. As regards the true seat of the degeneration with respect to tracts or systems which are clearly determined, no knowledge at present exists which permits any opinion to be formed upon this point. The study of ascending and descending degeneration after transverse lesions of the cord, gentlemen, is now ended, and, as might have been foreseen, all the tracts are affected in one or other direction according as their trophic centre is seated above or below the transverse lesion. There is, however, one tract which does not seem to degenerate in either direction, and which is only affected within the " zone of traumatic degenera- tion." This tract is that which has been termed the lateral limiting layer (seitliche Grenzschiclit). The escape of this tract, however, is but apparent ; it really degenerates in the same way as the adjoining tracts, but since it is composed of what appear to be very short commissural fibres their degeneration only occurs for a limited distance, and is entirely contained within the " zone of traumatic degeneration." 62 LECTURE VI. DEGENERATION OF THE NERVES AND SPINAL CORD AFTER AMPUTATION OF A LIMB. Such degeneration seems at first sight opposed to the doctrines of physiology. At the same time changes of this kind are frequent. They have been shown to occur hy numerous observers. Works of Bdrard, Vulpian, Dickinson, Hayem. Dejerine and Mayor, Hayem and Gilbert, Priedlander and Krause, &c. Changes in the central extremity nf nerves after amputation of a limb ; frequent increase in size (does not always occur), increased diameter of the fasciculi constituting the nerve. Considerable diminution, in a transverse section, of the fibres containing myelin. Islets of defeneration not to be confused with the primary islets, being in reality the traces of previously existing nerve fibres. The islets of degeneration are formed of a collection of nerve fibres composed of a small axis cylinder, and fine primitive sheath, with or without myelin. — Evolution of these islets, dispersion of the nerve fibrils, myxoid aspect of the partitions separating the different primary islets from each other, slight thickening of the fibrous bundles placed between the fasciculi of the nerve. Uncertainty with regard to the condition of the ganglia connected with the nerve roots, and the nerve roots themselves. Gentlemen, — The consideration of secondary degeneration in the cord would be incomplete unless a few words were said with regard to the occurrence of such degeneration after division of the peripheral nerves, especially when this is due to amputation OF A LIMB. At first sight, owing to ideas derived from the study of physiology, such degeneration seems impossible. "We are told that since the cells in the anterior horns of the cord are the trophic centre of the motor nerve fibres, the cells of the ganglia connected with the nerve roots being that of the sensory fibres, no degeneration of the cord can occur after the lesion of a peripheral nerve fibre. Whilst, in fact, descending degeneration could alone affect the motor fibres, ascending degeneration of the sensory fibres would be prevented by the ganglia connected with the nerve roots. The so-called fibres of recurrent sensibility are not, as it is genei'ally acknowledged, sufficiently numerous to produce ascending degeneration which is at all extensive. Whatever the value of these conclusions formed in the DEGENERATION 'OF NERVES AND COED AFTER AMPUTATION. 63 laboratory may be, it is an undeniable fact that in most cases after amputation of a limb changes which are truly singular are found to occur in the central extremity of the divided nerve, and upon the corresponding side of the cord after a certain time. The study of these changes will form the subject of this lecture. Without referring to the observations of B6rard (1829), which are not quite accurate, it is to Vulpian that the honour of first studying and methodically 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 Hayeni (1875) had. devoted his attention to similar facts, and as you know that author has more recently (1884) been again occupied in a work written in conjunction with my distinguished colleague and friend Gilbert. It is but fair to name Dickinson, who, in the same year as Vulpian (1868), gave a most remarkable description of the lesions which he had observed in the spinal cord after amputation of a limb. Dickinson very clearly indicated the posterior column as the principal seat of the atrophy, whilst, as I shall soon ' observe, Vulpian specially localized that atrophy in the antero-lateral column. I do not wish, however, to insist upon this historical review, since in the course of this lecture more than one opportunity will occur of stating the name and opinion of the different authors who have made observations or written works upon the subject which now interests us. Amongst these works there is one to which I must draw attention, namely, that of Friedlander and Krause, in which a thorough investigation may be found of the forms of degenera- tion which follow amputation. The articles which that work contains are numerous, and of the greatest value, while the interpretation given by the authors is new ; in fact I must allude more than once to this work, though I am far from agreeing with the conclusions which are formed in it. That you may be able to form a clear idea of the changes which occur in the cord after amputation, the study of the cord alone must be, to some extent, abandoned in these lectures, of which the special subject is "Diseases of the Spinal Cord," and the condition of the nerve trunlcs above the region in which the amputation occurred be investigated. We will not, however, go so far as to study the alterations which are shown to exist in 64 LECTURES ON DISEASES OF THE SPINAL CORD. connection with terminal neuromata. Those who are interested in these changes will find them described in the work of Hayem and Gilbert. Let - lis take as example and basis for the description of ascending degeneration after amputation a case which I have had occasion to observe during the course of this year, and from which I shall be able to put before you many interesting microscopical specimens. The patient was a man aged forty years, in the middle part of whose left thigh amputation had been performed twenty years previously, and whose death was due to phthisis while under my care. The sciatic nerve upon the left side was considerably increased in size along its whole length, its dimensions being of about double the size of those of the same nerve upon the right side. This fact, gentlemen, which has been already mentioned by several authors, is by no means constant; I did not, in fact, find it to exist in two cases of amputation of the arm, or in one of amputation of the foot. In a case of amputation of the arm Hayem and Gilbert remark that " the radial nerve was slightly diminished in size, while the ulnar gradually increased from the brachial plexus to the terminal neuroma, near which it was twice as large as the sound nerve on the opposite side ; the median nerve was of the same size on the two sides." It would be difiicult to find a, better example of the uncertainty which exists with regard to such increase in size, since in this case the three nerves of the stump were afiected in a difierent way. It should be noticed that the increase of volume when it exists is more pronounced in the peripheral parts of the nerve, and tends to diminish or disappear gradually as the spinal cord is approached. Leaving thfe macroscopic aspect of the nerve, the microscopical characters of the lesions which it presents will now be studied. If a section of' the sciatic nerve be examined, which I have asked you to take as the basis of this description, it is at once evident, even to the naked eye, that the primary fasciculi which compose it have for the most part a much larger diameter (2, 3, or 4 times as large) than those in a healthy nerve, an increased degree of transparency corresponding with the increased size of the fasciculus. It will presently be explained that these fasciculi have degenerated, those which have most increased in DEGENERATION 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 hasmatoxylin 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, a very small number of such sheaths is Fig. 70. — Section of the left sciatic nerve in a case Of amputation of the thigh on the same side 20 years previously. A, Primary fasciculus in which degenera- tion has occurred. B, Primary fasciculus in a normal condition.- C, Primary fasciculus in half of which degeneration has occurred, while the other half is in a, normal condition. D, Portion of the nerve between the fascioulse having a myxoid appearance. E, Blood vessel. perceived ; .these sheaths are also scattered throughout every fasciculus, 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 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 Pig 71.— Part of the section of a healthy sciatic nerve (stained by the hsema- toxylin of Weigert) (from nature, and semi-diagrammatic). The segments isolated by the white lines are the primary islets. very singular appearance, which have been observed by difierent authors, but to which special attention seems only to have been drawn by Friedlander 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 of Weigert is employed, but are distinctly coloured by carmine ; they vary in 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 or 1 of Verick) 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 islets 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. — Section of the left sciatic nerve after amputation of the thigh upon the same side-20 years previously. AAA, Nerve fibres in a normal condition which have retained their axis cylinder and sheath of myelin. BB, Islet of degene- ration of rounded or polyhedral 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 myelin in the islets. CC, Islets of degeneration in the margin of which a rupture has occurred so that the nerve fibres escape and become separated from each other. D, Blood vessel with thickened wall , . (much exaggerated in diagram). The segments separated by large white tracts are the primary islets. (From nature, semi-diagrammatic, coloured by the haematoxylin of Weigert.) distinguish them from those which are primary, I shall give the name "islets of degeneration." According to Friedlander and Krause, the islets of degeneration are merely primitive islets, of which the fibres have wasted, and %yith considerable reduction of their size in consequence. At first sight this explanation 68 LECTCRES ON DISEASES OF THE SPINAL CORD. seems extremely probable, but upon examining it more closely I was convinced that this is not the case. The primary islets, far from being diminished in size, are found to be of larger size in a degenerated than in a sound nerve. As regards the islets of degeneration, each of them merely represents, in my opinion, a degenerated nerve fibre, of which the size has increased 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 o of Verick) i^; is found to be com- posed of a mass of very na,rrow 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 a 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 varfes, but * The existence of these narrow narva fibres has been mentioned by Hayem, and they were very clearly d9sor;bed in a memoir by Hayem and Gilbert. (Arch de Phyaivl:, 1884, p. 430 et seq.) DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 69 is sometimes considerable (5, 10, 15, 20, or more). Owing to the development of these nerve fibrils, the sheath of Schwann, which I shall call " primary," becomes rapidly distended, and the islets of degeneration thus become of much larger size than the sound nerve fibres by which they were preceded. Owing also to the influence of the development of the nerve fibrils and the external pressure 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 other. Such, in my opinion, is the way in which evolution of these islets of degeneration occurs, which will be observed, gentlemen,, to be in direct opposition to that professed by Friedlander and Krause ; when they believe atrophy of the nerve fibres to occur,, my 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 used. In considering th& different nerve tracts it is found that in the degenerated tracts the partitions separating the primary islets from each other are twice or three times as large as those existing in healthy tracts ; they seem also to be more relaxed, and have a truly myxoid appearance. An analogous band of myxoid tissue is also found at the surface of the most altered tracts, separating them from the fibrous sheath furnished by the neurilemma, so that, when contraction has taken place in the sec- tion, these tracts have a pronounced tendency to escape from the sheath formed by the neurilemma. The connective tissue which separates thedifierent fasciculi from each other is moderately thickened. The spaces surrounded by many degenerated tracts are alone much increased in size, this seeming especially due to increase in the diameter of the surrounding tracts. The blood vessels contained in the degenerated tract are not increased in number, and their walls are at most but moderately thickened. Thus the most important factor, gentlemen, in increasing the 70 LECTUKES ON DISEASES OF THE SPINAL COED. size of the nerve trunks seated above the point of amputation is certainly the multiplication of the small nerve fibres, which has just been described. I cannot say to vrhat lesions atrophy of the nerve trunks is due, having never had the opportunity of making a histological examination in cases of this kind. The changes presented by the nerve trunks are now known, healthy fibres being found in greater number as the distance from the point of amputation increases. The degeneration will now be followed in its ascending path. As regards the ganglia connected with the nerve roots, which some obsei-vers considered unaffected, I should prefer to ^ive no opinion. I have examined many sections of these organs, but must confess to having formed no opinion with respect to them, and that I am unable to say whether they are or are not altered. During a long time observations have been made with regard to lesions in the spinal nerve roots, but with very different results. Vulpian believed them to be unaffected, while Dickinson claimed to have observed a certain degree of atrophy in the pos- terior roots. Subsequent observers showed the same difference of opinion, a small number of whom found no lesion in the nerve roots, while some observed degeneration in the posterior roots alone, others (Hayem and Gilbert) in both the anterior and posterior roots. In the case of amputation of the thigh which was taken as basis and example in this lecture, lesions were not very clearly seen to exist in the nerve roots. Almost the only change noted was the somewhat large increase in the number of small nerve fibres as compared with those of larger size, a disproportion which existed both in the posterior and anterior roots. As in the normal condition the narrow fibres are numerous in the roots connected with certain parts of the cord, this estimate is of such an extremely delicate nature, that the reserve with which I men- tion these facts will be understood. 71 LECTURE VII. DEGENERATION OF THE NERVES AND SPINAL CORD AFTER AMPUTATION OF A LIMB (continued). Alterations in the Spinal Cord : — Aooording to Vulpian the atrophy especially involves the antero-lateral column and grey matter. Dickinson believes it to be more pronounced in the posterior column. The latter opinion seems preferable to the former. Opinion of Fleohsig. Priedlauder and Krause describe amongst their cases both atrophy of the posterior column and of the posterior horn, disappearance of a large number of cells in the poiiero-lateral group of the grey matter, diminution in size of the anterior horn with a reduc- tion in the number of its cells, atrophy of the column of Clarke with disappear- ance of many of its cells. Results furnished by examination of the spinal cord in my own autopsies. Band of sclerosis in the posterior column on the same side as the amputation. Thickening of the connective tissue in the posterior column of the opposite side. Interpretation of these different lesions : opinion expressed by Friedlslnder and Krause : personal conclusions. Gentlemen, — In the preceding lecture the lesions of the peripheral nerves and nerve roots after amputation of a limb were considered ; the alterations which occur in the spinal cord itself will now be discussed. It may be henceforth said that these alterations have been very differently a,ppreciated by the authors who have studied this subject. Whilst Vulpian supposed the antero-lateral column and grey substance to be especially affected by atrophy, Dickinson believed the latter to be far more pronounced in the posterior column. Lastly, Vulpian, Dejerine and Mayor observed cases in which atrophy was found to an almost equal extent in all parts of the cord upon the side which corre- sponded to the amputated limb. Yet another opinion is held by Flechsig, who, on account of the fact that malformation, or at least want of symmetry, frequently exists in the spinal cord of those who are healthy, concludes that the atrophy in one half of the cord or one of the tracts composing it observed after 72 LECTURES ON DISEASES OF THE SPINAL CORD. amputation, is really congenital absence of symmetry and totally unconnected with the amputation.* In 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, 1 would almost say constancy, in the results, in my opinion it is 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, bori'owed from the memoir of Friedlander and Krause (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 hern, 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.f With regard to the anterior cornu 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 Clarice 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 I borrow a diagram (fig. 76), which shows most clearly the atrophy ♦ In a recent woik {Des affections nerveiises centripetes consecutives a la section des nc^fi ft aux amputations des membres, Acad. Royale de Med. de Belffique, 1891) Vanlair considers the alteration in the nerve roots and cord inconstant. He thinks that when existing in man they are due rather to the surgical affection which re- quires amputation than to removal of the limb. As regards the operations which he has performed in animals Vanlair summarises the results obtained by saying that "on account of their age and the absence of any pre-existing peripheral lesion, adult animals usually escape, to a great extent, the consequences of amputation ; in them myelitis due to section of the nerves or even removal of the limbs is usually absent. It is rarely met with except in bilateral operations." t In the observations published in Arch, de Physiol., 1873, Hayem had already stated the fact that when the sciatic nerve is torn away in young rabbits rapid atrophy of the nerve cells belonging to the group in the intermedio-lateral tract is found to occur. DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 73 and rarefaction of the cells of the ganglia after the loss of an arm. As regards ray case of amputation of the thigh to which Fig. (3.— Section of the cord (middle part of the lumbar enlargement) from a case of amputation of the lower extremity on the left side (after FriedlSnder and Krause). It will be observed that the left half of the cord in almost every part, but especially in the posterior column and anterior horn, is smaller in size than the right half. (The notch opposite the lateral column on the right side is due to a slit made by the authors before the preparation was hardened with the object of placing the sections in a better position.) Fig. 74. — Section of the cord (lower part of dorsal region) in a case of amputa- tion of the left lower extremity (after Priedlander and Krause). The left half of the cord is smaller than the right, and the cells of the columns of Clarke are in smaller namber. Fig. 75.— Section of the cord (middle part of dorsal region) in a case of amputation of the left lower extremity (after Friedlander and Krause). Same remarks as under fig. 74. 74 LECTDRES ON DISEASES OF THE SPINAL COED. allusion has been frequentlj^ made, the same condition of atrophy affecting the most different parts could not be observed, while on the other hand the posterior column was found to be clearly- diminished in size. This diminution extended through the whole or almost the whole length of the cord. (The medulla oblongata was not examined.) The antero-lateral column on the opposite side seemed to me also of smaller size than that on the same side (a fact already mentioned by Bignami and Guarnieri). As regards the posterior column on the side of the amputation it is my opinion, which is based both upon that of other authors, as well as the results of my Fig. 76. — Section of the cervical cord in a case of amputation of the right arm at its lower part (after Hayem and Gilbert). This sketch only represents the two anterior horns j the small volume of the right anterior horn (the side of the amputation) and the small number of nerve cells in that part will be noticed. own experience, that this column is the one which presents most constantly, and in the most pronounced way, the diminution in size which is now being considered. > Whether this diminution affects the whole or some parts only of the posterior column will now be considered. In opposition to the general opinion, especially that ex- pressed by Friedlander and Krause, I believe the latter to be the true state of the case. After amputation of the arm or thigh I have always clearly observed at the centre of the wasted posterior column a band, which if not very dense, is at any rate well defined, in which the fasciculi of connective tissue were more thick and numerous. This seemed to indicate that it is specially at that point that atrophy of the nerve fibres coming DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 75 from the posterior roots on the same side an that of the amputa- tion occurs. 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 sketches of this band of sclerosis at different heights in the cord. Fig. 77.— Cord in the lumbar region from a case of amputation of the left thigh performed twenty years previously. In the left posterior column, viz., on the side of the ampu- tation, degeneration has ooourred ; there was also in the posterior column of the opposite side an islet of degeneration, which however was less pronounced in degree, and of smaller size. It will be observed that the zone of degeneration in the left posterior column is at the postero-external part adjoining the posterior comu, which seems to indicate that it is due to degeneration of the fibres of the posterior roots seated below the point of section. The reduction in size of the left half of the cord is very pronounced in degree. Fig. 78. — Cord in the dorsal region from the same case of amputation of the thigh as fig. 77. In the left posterior column (the same side as that of the amputation) degeneration has occurred. In the posterior column on the opposite side there was also an islet of degeneration, which how- ever was less pronounced in degree and of smaller size. It will be observed that the area of degenera- tion is much nearer the posterior median fissure than in the lumbar cord, and that it is clearly seated in the column of G(j(ll. The diminution ' of size on the left side of the cord is very 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 COED. of the atrophy. This may possibly indicate that some fibres of the posterior roots upon the side of the amputation have Fig. 19. — Spinal cord in the cervical region from the same ease of amputation of the thigh as figs. 77 and' 78. In the left posterior column degeneration has occurred upon the same side as that of the amputation, there is also an islet of degeneration, 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 less extensive and slighter in degree than in the sections of the cord in the dorsal or lumbar region. The diminution in the size of the cord upon the left side is less pronounced than in the preceding figures. Fig. 80. — SectiOii ua wuw \^xjl\^ ^^/cj.YAua 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 * I shall have oocaaion to explain this statement more fully in discussing the {)athological anatomy of tabesi DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 79 alteration in the nerve fibres might be of septic origin (whatever the mechanism of its occurrence may be), and that some morbific agent may have penetrat?cl into the extremity of the nerve whilst it was seated in the deep part of the wound. This is certainly but a supposition, which may not be the true one, but the occurrence of sympathetic ophthalmia being remembered, it may certainly be said that this hypothesis has at least a appearance of probability. This, then, is my idea as to the origin of the considerable lesions which are observed at the central extremity of the divided nerve. Two causes tend to produce them : one is the occurrence of ascending Wallericm degeneration, which exclusively involves fibres, of which the originating nerve cell (trophic centre) is seated near the surface (in muscle, tendon, aponeurosis skin, &c.) ; the other, the existence of ascending neuritis, which occurs under the influence of the decomposing material within which the extremity of the divided nerve is placed. The nerve is then penetrated by some morbific agent,* which produces a special form of ascending neuritis at its central extremity, the sheath of myelin is soon absorbed, while the cylinder axis disappears, though I cj,nnot say that it is entirely destroyed. A second stage then occurs, namely, that of regeneration. The principal characters of this are known to you, namely, that in the sheath of Schwann, which is now without myelin, a somewhat large number of nerve fibrils (10 to 20) now appear, being formed of a thin sheath of Schwann, and a narrow cylinder axis with or without a small covering of myelin attached to it. By what means is this regeneration effected ? Is it to be supposed that the nerve cells from which the fibres originate (or trophic centres) contained in the spinal ganglia throw out prolongations into the empty sheaths in greater number, and constitute the fibrils which are being considered ? This is possible ; but the regeneration might also be due to another cause : the fibrils which compose the cylinder axis of the nerve fibre not being all destroyed by the ascending neuritis (the communication of some of them' with their trophic centre still existing) the morbid process merely separates them. Bach of * It should be stated that the microscopical examinations of nervous trunks made by Lesage, at my request, in two cases of amputation, merely gave negative results. 80 LECTURES ON DISEASES OF THE SPINAL CORD. Fig. 80 bis. — Diagram of the tracts of the cord (at the junction of the cervical with the dorsal region) according to their mode of development (after the diagrams and descriptions of Fleohsig). A, Direct pyramidal tract. B, Fundamental tract of the anterior column. C, Bemaining part of the lateral column. D, Direct cerebellar tract. E, Crossed pyramidal tract. F, Lateral limiting zone. G, Anterior root zone. H, Middle root zone composed of two systems of fibres ; some + + + + + = the fibres of the first system of the middle root zone ; = fibres of the second system. I, Median zone. J, Column of GoU. K, Postero-intemal root zone. L, Postero- external root zone, or zone of Lissauer. DEGENERATION OF NERVES AND CORD AFTER AMPUTATION. 81 Pig. 80 ter. — Diagram of the tracts of the cord (at the junotion of th3 eervioal with the dorsal region) in connection with the study of secondary deoener- ATION. A, Fibres liable to ascendingj degeneration : 1. Fibres of the ascending sulco-marginal tract. 5. Fibres of the tract of Gowers. 7. Fibres of the dirett cerebellar tract. 8. Fibres of the cornU'Commissural tract. 9. Fibres of the column of Burctach. 10. Fibres of the external bandlet properly so called. 11, Fibres of the zone of Lissauer. 12. Fibres of the tract of Gull. B, Fibres liable to dbscendino DBasNERATiON ; 2. Fibres of the descending sulco-marginal tract, 3. Fibues of the direct pyramidal tract. Fibres of the intermediate tract of the antero-lateral column; some of these are stray fibres, and scattered through the tract of Gowers, the direct cerebellar tract, and, above all, the crossed pyramidal tract. 6. Fibres of the crossed pyramidal tract. 13. Fibres of the comma shaped 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 tho term spasmodic tabes dorsalis. Erb : Spasmodic fpinal paralysis. Charcot : Spasmodic tabes dortalis. Primary degeneration of the lateral columns. Different results obtained in the autopsies of adults. History of the disease itself. Heine : paraplegia spastica cerebralis. Little : congenital spastic rigidity of limbs. The researches of the English surgeon were for a long time unknown to neurologists. Memoirs of Euppreoht and Peer. Symptoms of the disease. Spasmodic gait : attitude of the head and limbs. Attitude in the sitting position : position of the trunk and limbs due to difficulty in flexing the thigh upon the pelvis. Attitude of the upper extremities, which are often little afPected. Muscles of the front and back of the neck. Disorders of speech. Sti^abismus. Appearance of the face. Awkwardness of movements. Fibro-musoular re- tractions. Influence of fatigue, of the emotions. Gentlemen, — 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 order to avoid the confusion 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 lectures, so long as care is taken to make no error and to consent to no sacrifice to current ideas, which are in any way inexact. It is necessary to review shortly the history of this disease in order that the position of spastic paraplegia, from a nosological * Synonyms: spasmodic tabes dorsalis (Charcot}. Spasmodic spinal paralysis (Erb). Congenital spastic rigidity of limbs (Little). Primary spastic paraplegia (Gowers). Spasmodic spinal paralysis (Eustace Smith). ^Primary lateral scle- rosis (Gowers), &o. — (Translator.) 84 LECTURES ON DISEASES OF THE SPINAL C^RD. / point of view, may be understood. In 1875 Brb, 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 that in all probability this was due to primary degeneration in the lateral columns of the cord. Charcot A\as of the same opinion with some modifi- cations.- This idea as to the nature of spasmodic sinnal jjaralysis (name given by Erb) or spasmodic tabes dorsalis (name given by Charcot), thus at once gave it the right of being included in nervous pathology, but owing to a singular misfortune the autopsies of adult patients in whom the diagnosis of spasmodic tabes dorsalis had been made, there was found either disseminated sclerosis, or transverse, or focal myelitis, or perhaps amyotrophic lateral sclerosis ; not a single case in fact occurred in which primary degeneration 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 scarcely ever made. Should this affection be definitely excluded from a nosological catalogue ? If 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 aiBrmative, 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 desideratum exists. If, however, instead of looking for this disease in the adult, one seeks it in childhood it is quite otherwise. You will meet some day with a 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 absoluteljr normal, and the special senses quite unaffected. SPASTIC PARAPLEGIA. 85 "It is with regard to these young patients and them alone that in the present state of our knowledge upon this subject the name of spasmodic tabes dorsalis should be applied ; this term will be thus perceived to have quite a different meaning from that applied to it when the first works of Erb and Charcot were written, since at that time a disease which occurs in adults was alone considered. Such is the history of the term spasmodic tabes dorsalis ; as regards the disease itself, when understood, as I have just stated, its history is very different. It was Heine who in 1840 first described some cases under the name paraplegia i^pastica cere- hralis, a name which, I may say, is most suitable, and deserved to be permanent. This description however was merely sugges- tive, and the author, to whom we are indisputably indebted for making this disorder a true clinical entity, is the English surgeon Little, who in different works written between 1846 and 1870 clearly traced the picture of this morbid condition, and studied the causes with much talent ; he termed the affection "congenital spastic rigidity of the limbs." For a long time the researches of Little were almost unknown to neurologists, 'and it is only in recent works, notably in that of Rupprecht, that they are mentioned with' the consideration which they deserve. I shall have more than once to mention the name of Little ih the study of this disease, and to quote extracts from the interesting memoirs of Eupprecht and Feer, which are monographs entirely devoted to thfe disease which we are actually considering. I have already indicated, gentlemen, the principal characters of this aflfection, but that you may more fully understand it a detailed study of its' symptoms will now be made. Since this affection almost exclusively consists in motor dis- turbances of a spasmodic nature, the different movements and attitudes of the patient must be necessarily considered. It is specially when the patient is walking that rigidity is most clearly observed in almost all the muscles of the body. Even when the patient is in the upright position, as is the case with this girl whom I now place before you, the following symptoms are observed : — The head and upper part of the trunk are inclined forward and move as if they were inseparable. The upper extremities 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. 01.— Girl sufferiofr from spastic paraplegia (Damasohino 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 equiao-varus 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 trochanter, and inner surface of the iliac bones are somewhat rigid. The feet usually present, when the motor SPASTIC PARAPLEGIA. 87 disorders are very accentuated, a manifest tendency to assume the position of equinus on account of the contraction of the gastrocnemii muscles. The little girl, upon attempting to walk, presents a simsmodic form of gait which is absolutely typical. She is seen to draw the point of each foot firmly and noisily along the ground, carrying it forward, as it were, by a semicircular movement. Fig. 82.— Same girl as in fig. 81, seen in profile. The attitude of the lower exfcretnities and the position of the feet in equino- varus 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 waj^ that, owing oe LECTURES ON DISEASES OF THE SPINAL CORD. to reflex contraction of the calf muscles, tlie 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 infg,nts are so accustomed 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. There is one other attitude in which I would ask you to observe this little girl, because the diflJculties connected with the position present singular characters. I mean the seated position. It ■\\'ill 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 tenable 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 sufiiciently upon the trunk, they necessarily fall in the reverse direction. It 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. In 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 SPASTIC PARAPLEGIA. 89 Fig. 83. — Attitude of a patient suffering from spastic paraplegia, in the seated position (collection of Charcot). The legs, which cannot be entirely flexed, remain half extended, 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 movernents of the upper extremities it is easily understood that, owing to the spasms which occur in the muscles producing them, they are diiBcult 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, &c.), are amongst those movements which the patients find it the most diflBcult to execute. There are fortunately a large number of cases 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 spasmodic paraplegia can alone be said to exist ; this is really the " paraplegia spastica cerebralis " of Heine. It 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 90 LECTUKES ON DISEASES OF THE SPINAL CORD. to some authors, be equally involved. This statement, taken in a general way, is true, but an exception must be made 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 most 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 baoJc of the nech are often involved and slight extension of the head backwards, or spasmodic lateral deviation are then observed. Bificidties in deglutition are mentioned by some authors, which are due to the existence of a tendency to spasm in the pharyngeal and oesophageal 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 ^^-ben 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 is that of the muscles of the orbit. In more than 30 per cent, of the cases (Feer) strabismus, usually convergent strabismus, occurs. Many explanations of its existence have 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 occurs 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 of these two causes. SPASTIC PARAPLEGIA. 91 The muscles of the face are also liable to the same functional disorders, and on account of the spasmodic rigidity to which they are liable the appearance of the face is changed, and what has been mentioned by most authors is "the expression of stupidity" which the patients have, although in reality their mental power is no way inferior to that possessed by a person of ordinary intelligence. Such is the condition of the patient's face in the ordinary con- ditions of life, but the immobility and placid appearance of the features may, as I have myself seen, be replaced by exaggerated contraction of the muscles under the influence of passion. Thus for example in the case of a 5"oung boy who was very fond of teasing his companions, at this time, or when he became angry, the face was observed by me to assume quite a " diabolical" expression, this being accompanied by a grin which was truly frightful. Little 'on his part observed a case of " risus sardonicus." Thus, gentlemen, almost the whole muscular system may be involved, the mviscles, however, being affected to a different degree ; without becoming notably weaker it is the inability and spasmodic awkwardyiess in their movement which specially prevail in this disease. There is no true paralysis, but at most pseudo-paresis (Striimpell). Add to this that there is no trace of muscular atrophi/, while at times the more prominent muscles become unnaturally enlarged, and a true idea of the illness will be obtained. In some cases fihro-muscular contractions occur which may produce permanent deformity (foot), or an abnormal position of some of the joints (knee), contractions which may also increase the difficulty which exists in using the muscles ; contractions of a similar kind may be also observed in the muscles of the orbit when strabismus exists. Such are the principal disorders connected with motion. It is also an interesting fact that the tetanic rigidity of the limbs diminishes during sleep or even after simple rest ; while on the other hand it is increased by fatigue or strong moral emotions (anger, fear). 92 LECTURE IX. SPASTIC PARAPLEGIA (continued). Excess of tendon-reflex. Foot clonus. Normal cutaneous reflex action. Common sensibility retained. Muscular sense unaffected. Electrical irrita- bility at times increased. Healthy condition of the bladder and rectum. Absence of intellectual change. Oddness of character. Special indications of degeneration. Attenuated forms of the disease. Tendency to progressive im- provement. Congenital onset, often unperceived by the parents. Such children very backward in walking. Their intellectual development usually enables them to live subsequently in the same way as others. Nature of the affection. True spastic paraplegia must be distinguished from the tabid-spasmodio conditions ; clinically the first is not accompanied by epileptic attacks, and very rarely by mental change ; anatomically it is due to a failure in the deve- lopment of the pyramidal tract, and not to its destruction ; setiologically 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, spastic infantile hemiplegia. Hysteria. Treatment: methodical education of the limbs, gymnastics, massage, teno- tomy. Gentlemen, — 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 struck. 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 forejarm, 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 cases be looked upon as a distinctly spasmodic manifestation. SPASTIC PARAPLEGIA. 93 As regards the cases (notably those of idiots) in which tendon- reflexes are said not to have occurred, my opinion is, either that they were not properly sought, and I realize (vide the article on " RSmiplegie infantile," in the Dictionnaire Encyclo- pedique) that this examination is at times difficult, or that a mistake was made in the diagnosis, and that the patients in question were not really suffering from spastic paraplegia. In my opinion increase of the tendon reflexes should be looked upon as a constant symptom in this affection. As regards the suferfidal reflexes little need be said, since they may be found to be in the normal condition, increased, or diminished in different patients without these variations being apparently of the slightest importance. The common sensibility is absolutely unaffected, as you easily observe to be the case in this patient who feels not only when he is pricked but when lightly touched, or the temperature is changed to a very small extent. The muscular sense again does not appear to be changed to any appreciable extent. The electrical irritability of the muscles and nerves is normal as regards its quality ; the only change which occurs is in relation to its quantity (tetanoid contraction being produced by the faradic current), and this effect seems only to be produced in isolated cases. Vaso-motor disorders have been mentioned by some authors, the feet being said to be cold, livid, or marked with a marbling of red colour ; these troubles, however, are slight and of no great importance. The rectum and bladder are unaffected, or, at most, a tendency to spasm has at times been noted in the sphincters. The different positive or negative characters of this affection have now been mentioned, but the mental condition of the patients must still be considered. Owing to the expression of stupidity of which I have already spoken when studying the aspect of the face, and which you yourselves observed in the little girl whom I placed before you, you would suppose that the mind is in the most unsound condition. This, however, would be a mistake, since the intellectual faculties of most of these patients are in quite a normal condition, so that at a later period they can pursue the same studies as healthy children, and adopt the same 94 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 pr(5duce 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 diflScult as was found to be the case in the patients already considered ; a certain degree of more or less 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 infants 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. At the same time, as you would expect, it is not durino- the first days of life that the diagnosis is made; this usually SPASTIC PARAPLEGIA. 95 occurs at a later date, as at the end of a few months when the parents remark that the movements of the child are not entirely free. 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 or extending them, or at a still later date, when he is eighteen 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 been 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 diiferent from that of others. They may certainly be occasionally in worse circumstances 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 increase. 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 in 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. 96 LECTURES ON DISEASES OF THE SPINAL CORD. spastic paraplegia as you hnoiv not being a heredita/nj disease. The duration of their life is of good length, and quite inde- pendent of the illness from which they suffer. I have 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 ^,3 I explained at first totally different opinions have 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 are 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 equallj' certain that other very analogous conditions may be observed of more or less general muscular 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 tahid-spasmodic conditions, that the mal- formations of the skull are found, which are mentioned by many authors. We will first consider the cetiology 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 aetiology of these spasmodic conditions : premature birth, or the existence of a severe laboiir. 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 influence of certain inflammatory diseases of the fcetus or neidy- horn child, which give rise to lesions in the meninges or brain. The ordinary lesion in every case 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 SPASTIC PARAPLEGIA. 97 its being congenital. 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 cour siderable length of time, instruments being often used, it is easy to understand that the brain may be injured. Whether, as Little supposed, hasmorrhage 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 Eoss 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 cause mentioned, viz., the occurrence of an inflammatory affection in foetal life, or during the first few 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, intellectual 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. In cases due to premature birth, which I have exclusively taken 7 98 LECTURES ON DISEASES OF THE SPINAL COED. 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 should 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 cases might be termed '' talid-spasmodic conditions " whether due to injury at birth, or to destruction 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-spasmodic 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 SPASTIC PARAPLEGIA. 99 spasmodic tabes dorsalis was expressly given by Charcot to this primary and isolated degeneration of the pyramidal tract. It will now be understood, gentlemen, how totally distinct these two 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 cases, and that these must therefore be necessarily separated from each other. The varieties of muscular rigidity having been distinguished, the DIAGNOSIS of the affection must now be made by comparing with it other affections which resemble either spastic paraplegia or the tabid-spasmodic conditions. Infantile 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. Thomsen's disease might possibly give rise to a mistake since in this affection also there is muscular rigidity which may incon- venience 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 paraplegia 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 increase of the tendon -reflexes and foot- clonus are notably absent. The only affections which in my opinion it may be difficult to distinguish 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 are : — Potts^ disease and the different varieties of compression of the cord. These are distinguished by their course, by not being 100 LECTURES ON DISEASES OF THE SPINAL CORD. congenital, by the deformity of the spine, and by the frequent occurrence of pseudo-neuralgic pains (Charcot), &c. Transverse myelitis, whatever its cause may be, very possibly, if chronic, bears a similar resemblance, specially in the adult. Its aetiology suffices to distinguish it ; the fact that the affection appears at a later, even at the adult age, proves that the disease cannot be spastic paraplegia. The same may be said with regard to insular sclerosis, in which, as will be seen, the spasmodic symptoms often prevail to a special degree. Other symptoms, however, and the most frequently many of them, such as the tremor, the peculiar change in articulation, nystagmus, &c., enable this disease to be at once recognized. Lastly, thei'e is one affection of a clearly spasmodic character with regard to which the diagnosis may be most difficult, namely, spastic infantile hemiplegia. I do not refer, it must be understood, to infantile hemiplegia associated with tonic contraction and pronounced atrophy of the limbs, which could not fail to be recognised, but to those forms of hemiplegia which are unaccompanied by true paralysis, but in which the movements are performed with some difficulty and attended by the pronounced occurrence of spasm. The clinical aspect then much resembles that of spastic paraplegia, and to such an extent that some of the cases described by me recently under the name of tabid-spasmodic conditions should be referred in my opinion to spastic infantile hemiplegia. These would be cases of incomplete bilateral hemiplegia due to lesions seated in both the cerebral hemispheres but involving the pyramidal tracts to a moderate degree. Having sufficiently dwelt upon the clinical aspect of that affection I need not return to the subject. The facts recently observed by Bernhardt * should be men- tioned in connection with the diagnosis of the disease. This author observed the existence of all the symptoms of spastic paralysis in many members of the same family at the conclusion of the 30th year of life, without its being exactly known to what affection these symptoms were due. This chapter must be ended by my once more putting you on your guard with respect to hysteria ; both in the adult and child paraplegia may occur associated with tonic contraction, which * Bernhardt, Seitrag zur Lehre von den familiaren Erkranghungen des central, nerventyittnu {Virchow; Arch. 1891, 126,7). 59). SPASTIC PARAPLEGIA. 101 closely resembles that existing in spastic paraplegia, but whicli 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 rectified. 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 metlwdical education of the limbs, gymnastic exercises, massage, and passive 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. Boumeville at the Bicetre, 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 less pronounced idiocy. 102 LECTUKE X. INSULAR SCLEROSIS. History : CruTeilliier, Eokitansky, Charcot and Vulpian, Ordenstein, Boumeville and Guerard, recent works. Symptoms : 1. Spinal. A. Motor. Disorders in the gait, trne spastic gait, pnre cerebellar gait, cerebello-spastio gait; their characters. Spastic paraplegia. Hemiplegia : characters of the hemiplegia in insular sclerosis. Tremor : its characters ; occurs when voluntary movements are made, moderate rapidity of its rhythm, specially affects the mnscles at the root of the limbs, but often also those of the trunk and neck, and may pre- dominate upon one side of the body. Gentlemen, — It will certainly have been observed that at the beginning of each 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 mj'self 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 sclerosis and other affections which will be considered by us. The lesions of insular sclerosis are undoubtedly seen in Oruveilhier's admirable atlas of pathological anatomy, in that of Carswell and in the treatise of Eokitansky. Many obser- vations upon this affection were published by Turck, Frerichs, Rindfleisch, &c., but it was only regarded by them as a patho- logical curiosity and the account of the autopsies was alone given. INSULAR SCLEROSIS. 103 It is Charcot and Vulpian who deserre the honour of having given to insular sclerosis the dignity of an anatomical and clinical existence in 1866, and of describing in a masterly way the symptoms and pathological anatomy of the disease. The thesis of Ordenstein, inspired by Charcot, was written in 1867 and lastly in 1869 the memoir of Bourneville and Guerard appeared, a 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 Symptoms 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 nervous system they originate. The spinal symptovis will be first considered and studied in the following order : — I. SPINAL SYMPTOMS. A. Motor Symptoms. Amongst 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 clea/rly spastic in charader. )8. The form which is purely cerebellar. y. 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 LECrURES ON DISEASES OF THE SPINAL COED. each foot is accomplished by elevation of the corresponding half of the trunk and pelvis, 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. These 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 pv.rely cerebellar form (/3), which certainly but seldom occurs, 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-spasiic form (7) 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 in different ways. As regards rhythm, length, since the steps may be longer or shorter, and dvreotion 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 to 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. INSULAR SCLEROSIS. 105 i ^ d s p 4 ^ [/ p ^ ^ D P .n ') ^ 'AL Organs of the Eye. The iris is liable to many disorders which are of great 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. 116.— Diagram representing the size of the pupils in tabea. The black figure in the "middle represents the pupil of a healthy man moderately illuminated. The black figure on the left side represents the pupil when mydriasis exists (it may be of even larger size). The black figure on the right side represents the pupil when myosis exists. between the two pupils, without its being possible to say that one is too large or the other too small ; sometimes the pupil is much reduced in size, the condition to whicli the term myosis 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 case. It consists of dilatation of the pupil, which may be carried to an 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 ohliquely oval shape, that is to say, instead of being round, it becomes more or less oval (Berger) with the 200 LECTURES ON DISEASES OF THE SPINAL CORD. large diameter from above downwards, or from without inwards, the two sides remaining symmetrical. We have not, however, yet ended the disorders affecting the iris in tabes, and its different reflex actions must still be con- sidered. You know, in fact, that reflex contraction of the iris may be produced by three forms of irritation. (a) Light reflex. When a light is brought near the eye, or when, after having covered the eye by the hand, one quickly removes it opposite a window in full daylight, the pupil is seen to contract at once in a decided way, and to a considerable extent. In tabes this reflex contraction produced by light is absent in a large number of cases, viz., from 80 to 90 per cent, whether myosis or mydriasis exist. This disorder usually occurs almost simultaneously in the two eyes. Sometimes the loss of the light reflex is not absolute ; the first effect of the light is to produce contraction of the pupil, but this is almost immediately followed by dilatation which persists. (b) Accommodation reflex. This reflex occurs normally when a near object is looked at, specially if that object is placed very close to the eye, that is to say, when the eye must necessarily accommodate in order to see the object. This reflex also consists in contraction of the pupil. It often remains until an advanced period of the disease. This persis- tence of the accommodation reflex associated with loss of the light reflex constitutes an excellent sign of tabes at the onset ; although this fact had been already mentioned by Vincent and Coingt, many authors continue to call it by the name of the sign of Arrj-yll^Bohertson. In connection with this point I must give you one piece of advice which may be of practical use ; when the condition of the light reflex is investigated, be careful to ask the patient to look at some distant object and not at the light which is close to his eyes; otherwise, on account of the eye being fixed on a near object the pupil is seen to contract, and you are liable to mistake for light reflex the change in the pupil which is really due to accommodation. The accommodation reflex is, as I have just observed, usually retained during the first stages of tabes, but after a certain time it usually also ceases to exist. (c) Pain reflex. This reflex consists in the fact that when irritation is produced by pain in a healthy person (by means of TABES DORSUALIS. 201 a pinch, &c.), 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 iierue is often afiected 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 : in all the cases seen by him it had been preceded by loss of the tendon reflexes or lightning pains. Optic neuritis is almost always, if not always, bilateral in tabes ; 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 afiected. The length of time which passes between the onset of this affection of the optic nerve and the occurrence of loss of sight is variable ; the mean time was found by M. Berger 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 neuritis 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 amaurosis. 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 202 LECTURES ON DISEASES OF TPIE SPINAL CORD. which the optic nerve is involved, constitute, to a certain extent, a special form of the disease, the upper form of tabes, or cerebral tabes ; in this variety the evolution of the affection occurs favourably as far as any organs are concerned, except those con- nected with vision. But as regards any direct action of optic neuritis upon the course ' of the disease this seems to me very improbable. The symptoms which indicate the existence of optic neuritis are the following : — rt. Diminution in the acuity of vis bn.. In this case the patients often complain that there is, as it were, a veil or mist in front of the eyes diminishing their acuity, and an examination by means of different typographical scales enables the extent of this diminu- tion, which may be considerable, to be estimated. h. Bijsclirortiatopsi/d. This condition specially exists with regard to certain colours. Thus yellow and blue are usually seen by the patient during a certain time after he has become unable to see the other colours. c. Contraction of the visual field. Authors are by no means agreed as to the form which this takes. Leber speaks of a concentric contraction ; Galezowski of peripheral contraction ; von Graefe of nasal contraction; Schweigger of temporal con- traction. It would not be my place, gentlemen, to give an opinion on this point, but in the presence of such different statements I must confess that I feel strongly inclined to agree with Berger that no form of contraction of the visual field is characteristic of tabes. According to the latter author temporal contraction is most liable to occur. Charcot found that in the majority of cases an irregular form of concentric contraction existed. d. A ssotoma may be present over half or a section of the field of vision in certain cases, and may even be seated in such a position that hemianopia, due to a central cause, seems at first sight to exist. Ophthalmologists are of opinion- that this form of scotoma, in the same way as amblyopia, without apparent lesion of the pupil depends upon neuritis, due to some cause connected with the part of the brain seated above the medulla oblongata. As rej;ards the ophthalmoscopic appearances of these lesions I will briefly quote their principal characters as indicated by TiBES DORSUALIS. 203 Berger, who has specially studied the ocular disorders which occur 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 Pig. 117.— Extreme concentric con- Fig. 118. — Contraction of the field of traction of the field of vision in a Tiaion in the left eye, in a case of ease of tabes. (Damaschino col- tabes with atrophy of the optic lection.) nerve. (Damaschino collection.) Fig. 119. —Scotoma simulating contraction of the visual field with the hemiopic form in a case of tabes. (Damaschino collection.) 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. This 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 204 LECTURES ON DISEASES OF THE SPINAL CORD. still well defined. The vessels are considerably altered ; quite at the onset the arteries and veins are normal ; the arteries then become contracted and the veins swollen; lastly, considerable atrophy of both arteries and veins occurs towards the end of the disease. Auditory Organs. The AUDITORY ORGANS are almost as frequently involved as the visual, and the troubles which they present are numerous; * they often occur early in the preataxic period of the disease. The diminution in the acuity of hearing is in most cases slowly progressive, being at first but slight, increasing slowly, and possibly becoming most pronounced ; but the deafness may occur rapidly, as in a few days, or even suddenly. The diminii- tion in the acuity of hearing is almost always bilateral, although it may be more pronounced upon one side than upon the other. One important character of the deafness is that it is absolute, and presents neither the irregularitj^ nor the varieties which are so apt to occur when it is due to disease of the middle ear or its appendages. In tabes in fact the disorder is not one con- nected with the transmission of sound, the receptive organ, viz., the auditory nerve being involved ; the auditory neuritis of tabes, analogous to the optic neuritis of the same disease, may exist. This explains the fact that when the extei-nal portion of the ear is examined it is found to be free from disease. At the same time, since the middle ear may be affected in tabes, it is better to ascertain how far the deafness depends upon the transmitting, and how far upon the receptive portion of the ear. Recourse should therefore be had to the method of Rinne,t and to that of * M. Morpurgo {Arch./. Ohrenheilkimdt:; 1890), who examined the patients of Marina, and some others who were sufEering from tabes, with regard to associated disorders of the auditory organs admits that 81.13 per cent, of these patients suffer from some aural disorder ; according to him the receptive portion of the ear is most often affected, changes in the transmitting portion being less frequent, and of but secondary importance ; affections of both parts may how- ever coexist, and both kinds may depend upon trophic disturbance. t The method of Binne consists in placing a vibrating tuning fork in contact with the mastoid process, and holding it there until it ceases to be heard ; it is then placed opposite the external auditory meatus, and if it is again heard, the " positive Rinne " is said to exist, as when the ear is in a normal condition ; if it is no longer heard, the "negative Einne" exists, this indicating the probable existence of an obstacle in the middle ear ; if therefore more or less pronounced diminution in the acuity of hearing is associated with the " positive Einne," and no lesion can be detected by means of the otoscope, it is probable that some lesion exists in the internal ear. TABES DORSUALIS. 205 Weber,* which may give interesting if not absolutely reliable information. Suhjedive sounds are frequently hear-d in tabes, 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. Awal vertigo (Meniere'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 true paroxysms as in Meniere's disease properly so called. This fact was first mentioned by Pierret, f and by Charcot ; I myself studied this subject from^ a special point of view in conjunction with Walton in 1883. J 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. In 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 ihe 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 avditory nerve to electric currents * The method of Weber ooiiaists in placing a vibrating tuning fork in contact witli the vertex, which is usually more distinctly heard upon the side which is the seat of the diseased ear, when the middle or external ear are afEeoted. t Pierret, Contribution a IMtnde des ph^nomfenea o^phaliqnes du tabes dorsualix. Symptomes sous la dependance du nerf auditif . Sev. mensuelle de mid. et de chir. , 1877, p. 101. X p. Marie et Walton, Des troubles vertigineux dans le tabes (Vertige de Meniere tab^tique). Etvue de Medecine, 1883, p. 42. § Al. Marina, Zur Symptomatohgie der Tabes dorsualis mit, etc., Archiv f. Psych. t. XXI, p. 156. 206 LECTURES ON DISEASES OF THE SPINAL COED. in tabes lias 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 1 1 electrical reactions were produced in the auditory nerve by means of currents whose intensity was less than 15 milliampferes, 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 Organs. 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. Gustatory Organs. Gustatory 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 taste, 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. 20; LECTUEE XVIII. TABES. SYMPTOMS (continued). E. Trophic disobdeks.— o. Uisorckrs of the general iiuiii:itn, t, Spontaneous fractures: nature of these fraetnres : absence of pain, tendency to consolidation, tendency to form a large quantity of callus : special setiologioal conditions : the slightest injury may produce them, seat of these fractures, fractures of the apophyses, fractures of the vertebrae, their nature. Lesions of the bones : porosity, thinning of the compact substance, dilatation of the medullary cavity, dilatation of the Haversian canals, decalcification of the lamellae of bone, changes in the osteophytes, embryonic transformation of the marrow in the bones, diminution of the non-organic, increase of the organic material. Nature of these alterations. Gentlemen, — The study of trophic disorders in the course of tabes will occupy us during several lectures. They are so varied as to constitute a certain number of entirely distinct groups, as distinct, in fact, as a fracture of the thigh is from a cutaneous affection. We will therefore consider them apart from each other and describe each one separately. a. Disorders of general nutrition. These disorders have as yet been very little studied. With the exception of some works upon the quantity of excrementitious matter contained in the urine of patients suffering from tabes, works, again, which have contained no very conclusive evidence, scarcely anything has been written upon this subject. And yet, gentlemen, one fact connected with it is of great importance. This the good sense of the medical profession has quite understood : tahes = con- sumption, ataxy is a consumptive disease ; this is what first struck the observers. They were right, since those attacked by tabes are in the large majority of cases persons suffering from consumption, from " medullary phthisis," as it was termed in old times. It is this condition which causes them to have so characteristic an appearance : they are thin, their muscles are flaccid, their skin has an earthy tint or one of bistre, the eyes are somewhat sunken and surrounded by a dark circle, the features are pinched, in short the appearance is entirely that of 208 LECTURES ON DISEASES OF THE SPINAL COKD. persons sufFering from a high degree of malnutrition. It will be observed, however, gentlemen, that this appearance, though very usual is not absolutely constant. Some patients will be found to have the appearance of being in excellent health ; they are stout, and their aspect is most satisfactory. This, however, is the exception, and the latter patients are those in whom the medullary process is apparently exhausted and remains in a stationary condition. However this may be, I determined, gentlemen, to direct your attention to these disorders of general nutrition in the course of tabes, since they form part of the clinical aspect of this disease. The question may be asked whether at some future time, on account of the incessant pro- gress which it makes, pathological anatomy will not explain this phenomenon. It will, perhaps, demonstrate that this con- sumptive tendency of tabes is due to the degeneration of certain extra or intra-medullary nerve fibres analogous to the visceral filrres described by Gaskell in the posterior columns of the spinal cord. h. Spontaneous fractures. These fractures occur somewhat frequently in patients suffering from tabes, much more so, in fact, tlian one would be at first inclined to think. Mentioned for the first time by Weir Mitchell, their existence was not generally recognised until 1873, after they had been studied by Charcot. The works of Forestier, Eichet and Eaymond con- tributed to increase our knowledge upon this subject, whilst an excellent critical survey by Talamon generalise'd it. We shall in these lectures review the works of Regnard and Blanchard ; speaking also of the opinion expressed by Volkmann and of the effect which it had upon the development of the question of tabid fractures in Germany ; I shall also have more than once to quote the conclusions formed in some of the investigations recently made, to which I shall call your attention as the lectures progress. These fractures present in most cases, without considering their eetiological conditions, certain objective characters which are sufficiently pronounced to distinguish them, and to justify their being separately described. One of the principal amongst these characters is the entire absence uf jjain due to the fracture ; this absence of pain is so complete that many of the patients do not even perceive that TABES DOESUALIS. 209 the fracture has occurred, and still 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. In 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 I 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 tendency 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 jDlace 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 qther 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 been also stated that the spontaneous fractures which occur in tabes are specially liable to be associated with shortening 14 210 LECTURES ON DISEASES OF THE SPINAL CORD of the limb. This 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 the 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 large quantity of callus. (Collection quantity of callus. (Coreotion of of Charcot.) 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 cetiological conditions ; you will again see that in this case one important fact confirms what is said by those who maintain, as Charcot was the first to do, that the spontaneous fractures which occur in tabes constitute a separate group. This fact is the disproportmi which exists between the slightness of the injvry and the fracture of the bone, which is so pronounced that these were consequently termed " spontaneous fractures." TABES DORSDALIS. 211 The history of tabes contains numerous observations in which one or several fractures have been produced by mere traction or Fig- 122. -A woman suffering from tabes with fracture of the left femur (the figure having been turned round it is represented as the right femur) ; after 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, femur in a case of arthropathy in tabes. C femur in a case of fracture in tabes. Such absorption of fragments occurred that the bone scarcely attained half the length of that of a normal femur. 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 perhaps lying in 212 LECTURES ON DISEASES OF THE SPINAL COKD. bed with the head resting on the elbow when the fracture occurred. A fracture of the inferior maxillary bone has even , been known to occur while the patient was breaking a piece of sugar between the teeth. Lastly, in some cases fractures have occurred without the patient being able to ascribe them to any definite act, being probably due to some irregular movement. It is in fact incontestable that the movements of patients suffering from tabes are often so abrupt and violent that fractures may easily result from them. To look upon this occurrence, however, as some authors do, as the principal and even exclusive cause of fracture in the course of tabes is, in my opinion, to make a mistake. The true cause of these accidents is the fragility of the bones, produced by morbid conditions of the bone, which will be considered by us presently. The period of the disease in which these fractures occur varies. Most often they take place' during the stage of inco-ordination, but frequently also before that time; this being another proof that inco-ordination is not the only cause of these accidents. As regards sex one fact deserves to be mentioned, namel}^ that these fractures are more frequent in women. To such a point is this the case that of 32 cases of spontaneous fracture occurring in tabes, collected from medical literature by Max Flatow, 15 were found to occur in the female sex. When the infrequency of tabes in women is considered this is a large number, and the great predominance of fractures in that sex must be recognized, unless one admits that the value of these statistics is to a certain extent impaired by the first observations made at the SalpitriSre Hospital, into which for a long time only women \^'ere admitted. Even, however, when this correction is duly considered, one must, gentlemen, in my opinion, allow the fact that these fractures are far more common in the female sex. So far fractures have only been considered in a general way, let us now study them in connection with the different bones, con- sidering in the first place in what part they are most often seated. In connection with this question I will quote the statistics given by Max Flatow : — Cl^ cases : taking place specially in Fractures of the femur \ the diaphysis, fracture of the (. neck occurring in 4 cases. Fractures of the leg below the knee 17 cases. Frantures of the forearm 7 oases. TABES DORSOALIS. 213 It 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. I must also observe, and this is the objection which occurs most readily 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 diflerent 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 multiple fractures exist in the same patient, either from many bones being simultaneously fractured, which in some cases has happened in as many as 5, or even 6, or from more than one fracture occurring in the same bone (as many as 3 in one case). 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 bony prominences are produced which penetrate into the muscles and tendons. I am ready to admit that this may occasionally happen, but in some cases I have seen the intra-muscular osseous prolongations attain such a length that I cannot help attributing them to ossifying myositis. However this may be, gentlemen, I call your attention to these 214 LECTURES ON DISEASES OF THE SPINAL CORD. facts from feeling sure that they would be more frequently observed was their existence better known. Another class of fractures which is also very interesting is that which includes fractures of the vertebra. These were first mentioned by Charcot, and the diagram which I now put before you, and which comes from his collection, is the representation of one of the first, if not the first case in which a fracture of the vertebrae was observed. Pitres* published a case in 1885 ; since then some other cases have been mentioned, notably by Kr6nig,+ who on his part studied three such cases. These fractures usually present a collection of characteristic symptoms ; there is more or less considerable deformity of the spine, .a deformity which could only form progressively, and in a some- what slow manner. At the seat of the fracture angular curvature is found to exist posteriorly, owing to projection of one or more vertebral spines ; anteriorly a hard body is occasionally felt in the abdomen by palpation, owing to displacement of one of the vertebrae. On account of these changes in the form of the spine, the attitude of the patients is considerably altered, the upper part of the body being curved or rather ient anteriorly or laterally, while on the other hand, owing to diminution in the length of the spine the upper part of the trunk is as it were forced into the abdomen and pelvis, giving rise to the formation of strange horizontal folds in the skin. I should be afraid, gentlemen, of giving you a wrong idea with regard to these fractures of the spine, if I omitted to mention that they rarely exist alone ; they are most often complicated by more or less pronounced arthropathy affecting the inter-vertebral articulations ; to such an extent is this the case that the lesions may often be classed indifferently as joint affections, or fractures. Besides these fractures involving tbe bodies of the vertebra, they may also occur in the arches or processes. After the description which has been given of these different varieties, you will easily understand that if the diagnosis of spontaneous fractures in tabes is often easy to make, it is at times very much the reverse. Many difficulties may present * Pitres, Sodke de Sioloffie, 21st November, 1885. t KrSnig, Wirbelfr/cranlungen bet Ttibikern. Zeitschrifl fur kliniache Medicin 1888, XIV, p. .51. TABES DOESUALIS. 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 oedema, which in the fractures of those suffering from tabes is usually more pronounced than in Fig. 124.— Fractnre of the spine. On account of the sinking of the vertebrse which has occurred there is considerable lateral curvature of the spine. (Col- lection of Charcot ) ordinary fractures, may cause so 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 swelling of the adjoining parts and mobilitj^ 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 connection with the flat bones which are situated somewhat deeply (sternum, pelvis) that these surprises are wont to occur. 21G lECTUr.ES ON DISEASES OF THE SPINAL CORD. Such observations may be made about the clinical varieties of these fractures ; the lesions v^rhich accompany them will now be considered, and these will explain some of the characters which cause the fractures occurring in tabes to have a special character. The changes in the hones which are visible to the naked eye are the following : — The open cancellous siructure at the surface causes the bone to have a " worm-eaten " appearance in certain parts. At times this condition is so pronounced that marked ulceration exists (Fere) : when this condition is very evident the bone can occa- sionally be indented by the pressure of the finger. If a longi- tudinal section of a bone which is thus altered be made by means of a saw it will be found that — The compact svhstance is more thin, being possibly so much reduced as not to have more than half or a third part of its normal thickness ; this also causes the bone to give way when it is pressed upon, the compact tissue being the part which is the most capable of resisting pressure. The dilatation of the medullary canal which exists, so that the hollow part at the centre of the bone is enlarged, also diminishes the solidity of the bone. The microscopical examination shows other causes of the friability which exists in the bones ; and it is to R. Blanchard that we owe the precise and detailed knowledge which we have of these lesions from a histological point of view. These consist in — Dilatation of the Haversian canals, specially of those which are seated most near to the medullary canal. When this dilatation is at the surface of the bone it is to this which produces the " worm-eaten "or " lacunar " aspect already mentioned ; when very pronounced near the medullary canal it contributes to produce its enlargement. Absorption of the osseous material near the Haversian canals is shown to occur by the fact that the diseased bone is coloured more deeply by picro-carmine than that which is healthy. This absorption seems one of the first symptoms which occur in the bones in tabes, since it is detected near the Haversian canals, which are not as yet dilated ; this would therefore be one of the primary lesions. TABES DOESU^LIS. 217 Changes in the osteoplasts. These sufier 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 canals. 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. — Longitudinal section of the upper extremity of a long bone (humerus) affected by the disease which exists in the arthropathy of tabes, showing the reduced size of the compact tissue and cartilage, where the arthropathy (A) exists. (Damasohino collection.) portion of the bone only forms 24 per cent., a large deficiency in phosphates being specially found to exist (10 per cent, instead of 50 per cent.). Increase 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 218 ' LECTURES ON DISEASES OF THE SPINAL CORD. case. In consequence of these facts it is easy to understand why it is that the changes in the bone are ascribed by some of the medical profession to peripheral neuritis. It cannot be denied that the coincidence is most suggestive, but it scarcely suffices to show that, in opposition to the opinion of Charcot, the changes in the nutrition of the bone are not dependent upon medullary alterations. However this may be, the fact upon which T would lay special stress, gentlemen, is that the changes which I have just, described are found not only in the fractured bone, but in all or nearly all the bones of the skeleton, so that in certain patients suffering from tabes all, or nearly all, the bones are " liable to fracture," and multiple fractures may therefore occur in the same patient. It should be added that lesions of the bone do not exist in every case of tabes, nor can it be indicated, even to an approxi- mative degree, in what proportion of cases these lesions oecur. It will be explained, gentlemen, in the next lecture, that the lesions in the bone are precisely those which are found at the commencement of the arthropathy which occurs in tabes, so that fractures and arthropathy are, in reality, but different ex- pressions of the same pathological condition, and, cannot be separated from each other, at any rate from an anatomical point of view. 219 LECTURE XIX. TABES. SYMPTOMS (continued). F. Arthropathy in tabes. — History: "Charcot's joint disease'' — opposite opinion of Volkinann. Onset more or less sudden : swelling, doughy condition, absence of true oedema ; crepitation ; absence of pain. Course of the affection mild and severe form. State of the articulation when the arthropathy has existed for a certain time ; flattening of the part, abnormal relation of the . articular surfaces, abnormal mobility, absence of pain. Complications: fracture, pain, passage of the articular extremities through the skin, suppuration. JEtiology : occurrence at different periods, greater frequency in the female sex j seat in different joints. Pathological anatomy ; articular capsule, intra-articular ligaments, synovial membrane, effusion, floating bodies in the joints, extrem- ities of the bones ; atrophic type, hypertrophic type, cases of combination of these two types. Pathology of tabid arthropathy: different theories. A. The primary cause of the arthropathy is a, nervous lesion (spinal cord, medulla oblongata, peripheral nerves). B. The primary cause is not a nervous lesion (traumatic, syphilitic, rheumatic arthritis, arthritis deformans). Conclusions. Gentlemen, — 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 oases. 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 v. 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 once have to quote from important works written by German authors. The following numbers, taken from a memoir by Weizsacker, 220 LECTUEES ON DISEASES OF THE SPINAL CORD. published in 1887,* represent more or less what has been written about this disease: — of 109 cases to which this writing alludes, 53 are French, 36 English, and 18 German. I shall put before you several patients suffering from this affection, and we shall be able to observe in them some of the characters which it presents. They are all cases in which arthropathy has already existed for a certain time. Since I had not the opportunity of showing you a patient in whom the symptoms were just commencing to occur, I must describe to you in full the first stage of the affection. The onset of tabid arthropathy varies but little. In some cases it is absolutely sudden. A patient while walking perceives crepitation in the hip ; his legs give way under him, and he falls. Almost immediately, or after some hours, considerable swelling occurs in the region of the joint, which soon extends over the whole thigh. In other cases the onset is not quite so sudden, though it is always abrupt. Thus, while the patient is undressing, he by chance perceives that one of his joints is swollen without, however, having suffered any pain ; or upon rising in the morning he makes the same observation without having had any abnormal sensation during the night. It may, again, happen that during the few days which precede the onset of the affection premonitory crepitation occurs in the joint which will be involved. However this may be, one of the principal initial symptoms is the svwlling of the joint, and soon afterwards of the whole limb. This swelling presents certain characteristic features. As I have just said, it occurs more or less suddenly, and acquires its maximum degree in a few hours or days. It almost always affects the whole segment of a limb or even the entire extremity which becomes of truly enormous size, having the same appearance as exists in elephantiasis. It is certain that this is not due to ordinary oedema, since the swelling is firm, un- yielding to the finger, does not pit on pressure, or leave an indentation after being pressed with the finger. The skin of the part is pale, often shining, the veins are dilated, while redness, heat, pain, and in fact all the signs of inflammation are absent. In order to explain this singular appearance Debove * Weizsacker, Die Arlhropathie bei Tabes. Beitrdge zur Minischen Chirurgie von Brum, 1887. 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 structures. 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. Crepitation often, but not always, occurs 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 he is soon fatigued. But of all these symptoms, that which combined with the swelling is most characteristic of tabid arthropathy, is the absence 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 : In the mild., form the effusion disappears in a short time, and the joint retui-ns 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. In 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. 222 LECTURES ON DISEASES OF THE SPINAL CORD. It is from this form that the patients who are assembled here suffer, though it has not the same form in the different cases. In this woman, for example, the arthropathy has only existed for a few months in .the left hip joint; the oedema, 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 ^ M vi^^^l ^KbJ'^^ ^m u ^^^H^^S V ^B W m ^1 ^^^^k|i' Mb wiM ^1 N 11 1 1 I 1 Fig. 126.— Eeoent tabid arthropathy in the left hip joint. (Damasohino collection.) The enormous increase in the size of the left thigh will be observed. The lesion having existed but a short time, the upright position can be maintained without pronounced deviation of the lower limb. case ther« is but little swelling, but on account of the lesions in the bone, some deviation is already beginning to occur in the direction of the limb, and upon my attempting to produce lateral movements in the joint, I can do so, as you see, without difficulty, whereas when the joint is in a healthy condition the lateral movements are barely perceptible; hence there is apparently much difficulty in walking. Lastly, in the third patient, the woman who is lying upon TABES DOESUALIS. 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, flattening 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. (Damaaohino collection.) The knee and thigh are swollen, the knee presents prominences which canse it to appear of a square form. The lateral movements were very pronounced, and could be produced by slight pressure of the hand upon the 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 a poor figure; at the same time, however extensive these movements 224 LECTURES ON DISEASES OF THE SPINAL CORD. may be, no resistance is felt in the joint. The reason of this 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 yourselves can again easily understand what occurs, even at a distance, since the subcutaneous prominence which you perceive in the hip is reallj' the upper extremity of the femur, which changes its place whenever the thigh is moved, and to an extent which corresponds with the amplitude of the movement. Owing to its Fig. 128. — Tabid arthropathy in the knee. (Damaschino collection.) The most extraordinary dislocations can be produced in the lower limb of this patient without the slightest pain being produced. being possible in this way to bring the upper extremity of the femur, so to speak, underneath the skin, it is easy to feel its different parts, and to recognize that both the head itself and a large part of the neck have almost completely disappeared, and that this extremity is almost solely represented by the great trochanter. It is unnecessary to add, gentlemen, that with such lesions the patient is quite unable to walk ; but the fact which I am anxious to repeat and impress upon you is, that however much I move or drag the limb no pain whatever is felt by the patient ; if she does not walk it is solely because the mechanical conditions of the joint do not enable it to support the weight of the body. TABES DORSUALIS. 225. Fig. 129.— Eeoent tabid arthropathy in the shoulder (from a patient named Berthel). (Collection of M. Charcot.) Much enlargement of the shoulder. The condition of the shoulder joint a few years later is seen in the following figure. Fig. 130. — Ancient 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 acromion, forms a prominence beneath the skin in front of the angle of the inferior maxilla. 15 226 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 kneea. (CoUeotion of M. Charcot.) In this case the deformity which existed was lateral ; though pronounced in degree it did not prevent the patient from walking a few steps with the help of two sticks. Fig 132.— Tibid arthropathy in the two knees. (After M. Dreschfeld.) This patient presents in the most pronounced way the deformity which is known by the name of " genu reourvatum." considerable change in the articular surfaces, neither of which conditions produces the slightest pain. TABES DORSUALIS. 227 Such are the ordinary features of this affection ; in some cases other symptoms occur which should perhaps be regarded as complications. Thus — A spontcmeous 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 usually almost entirely absent, may occur in some cases and persist during several days or weeks, this being specially the case when arthropathy affects the small joints (of the hand or foot). The articular extremities may pass 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 so rare that its occurrence need not be feared. At the same time, I might mention cases in which it has happened either after an im- prudent puncture, or during the course of a general affection (pneumonia), or without obvious cause, but I would again saj"", gentlemen, that its occurrence is quite exceptional. In order to finish what is connected with the clinical aspect of tabid arthropathy, gentlemen, its jETIOLOGY 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. The time at wliich it takes place is very variable. Kredel* found that in 132 cases it appeared — 21 times iu the premonitory period of tabes. 38 times between the 1st and 3rd year of tabes. 32 times between the 5th and 10th year of tabes. 41 times after the 10th year of tabes. It is therefore very difficult to specify the time of its occur- rence, the truth being that it occurs at any period of the disease. * Kredel, Die Arthropathien und Bpontanfracturen bei Tales. VoVcmann's Samm- uvg klinisc' er Vorlrdge, 18f8. 228 LECTURES ON DISEASES OF THE SPINAL' CORD. With respect to tlie age of the patients, no definite information can be given. At the same time, a case under the care of Charcot and Fere sliould be mentioned in which arthropathy occurred at the age of 20 j-ears, tabes having commenced to exist in the 18th year. This is probably the first case published in whicli the onset of tabes occurred at so early an age. As regards sex, in 100 cases of Weizsacker 72 of the patients were males, and 39 females. On account of the infrequency of tabes in females one must conclude from this that arthropathy is more frequent in the female than in the male sex, even when the correction is made with respect to the female cases which were published at the Salpetri^re Hospital. What is the freqitency of arthropathic cases as compared with all the cases of tabes ? With respect to this question I can only Pig 133 — Ancient tabid arthropathy in the knee (in a patient named Berthel). (Under the care of Charcot.) The lower extremity of the femur projects con- siderably above the upper extremity of the tibia and fibula. quote the numbers given by Erb, who found 2 cases of arthro- pathy in 56 cases of tabes. These numbers represent, in my opinion, the true state of the case, and 4 or 5 cases of arthro- pathy may be supposed to exist in 100 cases of tabes. In connection with the seat of the arthropathy several interesting facts are brought to light in the following statistics of Max Flatow, which include 149 cases, in 41 of which there was bilateral arthropathy. It was most frequent in the knee, which was affected in 60 patients, in 13 of whom it was bilateral. After the knee it occurred most often in the foot, which was affected in 39 patients, and in 8 of them bilaterally. The hip follows, having suffered in 38, and on both sides in 9 patients, and the shoulder in 27, and on both sides in 6 patients. The elhovj joint, the joints of the hand and fingers, and the temporo- TABES DOESUALIS. 229 maxillary joint were far more rarely affected, giving numbers which vary between 6 and 4. Yoii will have observed that all Fig. 131.— Tabid arthropathy in the thumb. The last phalanx of the thumb is turned abruptly outwards, so as to be almost at right angles with the first phalanx. The metacarpal bones connected with the fingers are somewhat swollen at their lower extremity. fig. 135. — Tabid arthropathy in the right elbow. the joints may be affected, but that they are so with a varying degree of frequency. 230 LECTURES ON DISEASES OF THE SPINAL COED. The jMthological anatomy of tabid arthropathy will now be considered. The articular capsule, the lax condition of which was indicated by the clinical symptoms, is flaccid, softened, dilated, often ruptured, and at times more or less completely destroyed. The intni-a/rticular ligaments, such as the long portion of the biceps, the crucial ligaments of the knee, and specially the round ligament of the hip-joint, are the seat of somewhat accentuated lesions, and may completely disappear. The synovial membrane is usually pale, its surface is often covered by vascular fringes, it is thickened, adherent to the adjoining parts ; like the capsule it may entirely disappear. In Fig. 136. — Tabid arthropathy in the shoulder. (Damasohino oolleotion.) The capsnle, which is extremely dilated, has been opened, and the head of the humerus is seen to be dialooated into the subscapular fossa. some cases plates or nodules of bone are found in the thickened membrane. The effusion into the joint cavity is usually serous, transparent, fluent, and of a clear yellow hue ; it sometimes contains fibrinous flakes, in a few cases blood, and very rarely pus. Abundant at the onset, it disappears after a certain time. So much was said about these facts when the symptoms were discussed that it is quite unnecessary to dwell upon them now. The existence of loose bodies in the articulation has been often mentioned, commonly of osseous structure, and either free, or possessing a pedicle. Usually newly formed within the joint they are sometimes due to a fracture of the epiphysis. In the former case these newly formed osseous bodies are frequently TABES DORSUALIS. 231 many hundred in number, so that tlie 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 less complete atrophy of the cartilage and articular Fig. 137. — Tabid arthropathy in the shoulder. A. Normal head of the humerus. B. Head of the humerus upon the side in which arthropathy exists ; the articular surface of the head of the humerus has much diminished in size upon this side. (Damaschino collection.) rig. 138.— Tabid arthropathy in the Pig. 189. — Arthropathy in the shoulder. hip-joint. (Collection of Charcot.) The lower part A of the articulating portion of the head of the femur is completely absorbed and has dis- appeared, a plane surface alone remaining ia its place. (Collection of Charcot.) The glenoid cavity of the scapula can scarcely now be seen, and the head of the humerus has almost completely dis- appeared. surfaces of the bone, the wasting being possibly so considerable that a somewhat long portion of the epiphysis may entirely disappear. Thus, in the femur, for instance, not only the whole head, but even the neck of the bone may be completely absorbed. In this way, as in the patient whom I showed you just now, the great trochanter, which is itself much eroded, may be the only 23-2 LECTURES ON DISEASES OF THE SPINAL CORD. part which remains ; the femur has then, as has been said, an appearance resembling that of a " drum-stick." Atrophy of a similar kind occurs in the articulur cavities, their margins are effaced, their surface flat, their walls become thin and are at times depressed (ths cotyloid cavity) by the epiphysis which rests upon them. B. The Jii/iwrtrophic type, also termed the type with the form of arthritis deformans. In this type there arise, specially at the edges of the cartilages and bone, either more or less numerous and irregular outgrowths of bone, or merely isolated osteo-cartila- ginous projections, closely resembling those which are seen Fig. 140. — Tabid arthropathy in the elbow, of the hypertrophic form. A con- siderable mimber of bony nodules are seen in the cavity of the joint, some in a free condition, others fixed in the periarticular tissues. The articular ex- tremities are thickened. in the joints affected by chronic arthritis deformans. At times? the vclvet-lihe dissociation of the cartilage is also observed, from this being worn away at the points of friction, the bone present- ing in the same parts an open cancellous appearance or that of ehurnation. The synovial fringes become affected by hypertrophy, and a frequent corollary to this event is the formation of intra- a/rticular osseous masses, either free or pediculated. The capsule also presents changes ; it is thickened, sometimes ossified in certain points, and may even become adherent to the bones and articular extremities. Though these two types are quite different from each other, TABES DORSUALIS. 233 however, they are undoubtedly both examples of true 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 tubercular forthritis, 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 hypertrophic type B is more frequentlj' 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 combination 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 intra-articular 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 234 LECTURES ON DISEASES OF THE SPINAL CORD. absolutely free from disease. Jiirgens has in fact shown that in many patients suffering from tabes all or almost all the joints, even when no arthropathy exists, present dilatation of the cap- sule and elongation of the ligaments ; the vessels of the synovial membrane are also dilated, and the intra-articular ligaments of a somewhat red hue ; in short most of the joints in tabes are frequently in a condition of "virtual arthropathy." The existence in the bones of these patients of arthropathy, and all the osseous lesions enumerated in the chapter devoted to the consideration of spontaneous fractures, should be compared with these facts (dilatation of the medullary canal and of the Haver- sian canals, decalcification, changes in the osteoplasts, trans- formation of the marrow in the bones, &c.). Now that the principal characters of tabid arthropathy are known, its nature may be discussed, and I shall make use of this opportunity to say a few words about the different theories which exist, or have existed about this interesting question. These theories may be ranged in two large classes : A. The primary cause of tabid arthropathy is a nervous lesion : Charcot, when he described arthropathy, regarded it as a trophic disorder occarring in the bones of the joints, and depending upon a lesion of the spinal cord, probably its anterior cornua, a lesion which in all probability had extended to the cornaa from the posterior columns of the cord ; Charcot and Jeffrey, Pierret, Liouville, Seeligmuller have observed cases which testify in favour of such a lesion in the anterior cornua. Other authors have assigned a different seat to the nervous lesion ; Buzzard, stating that laryngeal and gastric crises occur frequently in patients with tabes who suffer from arthropathy, concludes from this fact that the trophic centre of the bones and joints is seated in the medulla oblongata, these lesions in the bones being associated with disorder in that part. This supposi- tion however, as far as I know, has not been verified anatomically, and the coincidence which he mentions does not appear to exist, since Weizsacker remarks that in the 109 cases of tabid arthro- pathy from which his statistics are formed, gastric crises only occurred in 30. Lastly, certain authors, basing their ideas upon the researches of Pitres and Vaillard, "Westphal, Siemerling, who in some cases of spontaneous fracture or arthropathy have found lesions in TABES 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 perypheral nerves. B. The pi'imary cause is not a nervous lesion. Amongst those who have held this opinion the first who must be mentioned is Volkmann ; according to this author the arthro- pathy is purely and simply a lesion of traumatic origin, and the reason of its occurring in those suffering from tabes is solely because injuries to the joints are far more frequent in these patients on account of the inco-ordination which exists in the movements. Either on account of falls, or because the joints are being constantly irritated by the irregular movements, they are more liable to suffer than those in healthy persons ; to these unfavourable 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 sypldlitic artJiropathy , 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 Koyal 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 I do 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 learnt to see." To return to the supposition that the arthropathy in tabes is of a purely rheumatic character I must also quote the opinion 236 LECTURES ON DISEASES OF THE SPINAL COKD. of Vircliovv, according to whicli this affection is only a form of arthritis deformans. In fact, according to that learned path- ologist, in both these affections the morbid process commences in the cartilage, which it causes to swell, and then to split and disappear. The only difference is that in tabes, from the effect of bad nutrition, the lesions occur more rapidly. There are many who hold this opinion, and the points of resemblance between tabid arthropathj?- and arthritis defm-mans are certainly at first sight numerous. But the differences are still more pronounced. Already in 1875 it was the object of Michel to explain this in his thesis, (a) In arthritis deformans the onset is slow, gradual, and usually accompanied by pains, which are at times most severe ; (6) there is no effusion, or if one exists is in very small quantity ; (o) the process is from the first one of hypertrophy, whereas in the arthropathy of tabes atrophy first exists ; {d) the movements of the joints are difiicult and limited, on the contrary, we have seen that in the arthropathy of tabes the mobility is much increased; (e) pronounced deformity may occur in both affections, but its character is quite diflferent. Other points of distinction might be mentioned, specially in connection with their pathological anatomy, but in my opinion those to which I have alluded are sufficient to show that tabid arthropathy and arthritis deformans are .totally distinct affections. Such, gentlemen, are the principal theories which exist with respect to the nature of tabid arthropathy, they are, as you see, numerous, and differ much from each other. It is, in my opinion, useless to increase the difficulty in which this enumeration places us, and though I cannot oblige you to have the same opinion I must be allowed to add that it is still the first theory, that of Charcot, which seems to me the best. The arthropathy •cannot be simply and purely due to the traumatic injuries occasioned by the inco-ordinate movements which occur, since, as we have already seen, it often occurs in the prodromic period when no inco-ordination of movement exists. Nor can it be simply the result of syphilis since unfortunately the number of persons affected by that complaint is legion, and yet unless tabes also exists they never present this singular variety of arthro- pathy. With regard to .the connection of tabid arthropathy with TAKES DOKSCALLS. Zo7 peripheral neuritis as its initial lesion, I must confess, gentlemen, that I cannot believe this to exist, ancT for a reason analogous to the one "which I have just given with respect to its pureh' syphilitic origin. We see, in fact, gentlemen, a large number of cases of so-called peripheral neuritis, and 3-et never, even in those who present the most characteristic sjunptoms of that affection, does an_y symptom occur which resembles tabid arthro- pathy. On the conti'ary an affection is known, due to a special Fig". Ul- — The innominate bone and femur in a case of tabid arthro- patliy of the hip joint. (Collection of Charcot. ) The acetabulum has almost entirely disappeared, and its margin barely remains, the bottom of the cavity being continuous "^ith the adjoining surface of the bone. The head and neck of the femur have also completely disappeared, and the great trochanter alone remains. Fig. 142. — The innominate bone and femur in a case of chronic rheumatism (arthritis deformans). The margin of the acetaliuluin is much increased in size, and the cotyloid 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 tliat \A-hich occurs 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 diflSculty in supposing that in some cases traumatic injury, either directly inflicted or due to the irregular movements, is an occasional cause of tabid arthropathy, and determines its seat. 239 LECTURE XX. TABES. SYMPTOMS (continued). The fool in, taies: History. — Characters. Somewhat sudden onset, swelling of the foot, thickness of its inner margin, flattening of the arch of the foot, deviation of the metatarsus, shortening of the foot, enlargement of the malleoli, ankylosis of the joints of the foot, absence of pain. Pathological anatomy : spongy appearance of the tarsal and metatarsal hones, their destruction. Trophic disorders in the fibrous tissue : articular capsules ; intra-artioular ligaments ; tendons, their rupture. Trophic disorders of the shin; different eruptions — Perforating ulcer, its characters ; Gangrene. Spontaneous ecohymoses ; Loss of the nails and teeth, Hyperidrosis ; anidrosis. Nature of these trophic disorders. Gentlemen, — 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 Fere. 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 parts was almost identically the same, This condition usually exists at a comparatively e(wly period, occurring most often at the end of the first or commencement of the second period of tabes, in other words in the praeataxic stace. 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 ba:ck of the foot, specially pronounced near the tarso-metatarsal 240 LECTURES ON DISEASES OF THE SPINAL CORD. , articulation, and causing this region to present a uniform curve, and not allowing any of the bones in the foot to form a promi- nence above its surface. It must be also observed, gentlemen, that pressure does not cause this swelling to disappear or pro- duce any subsequent depression in it ; this is precisely what has been alreadj- stated in connection with other forms of arthro- pathy. The inner border of the foot becomes thickened, and at the same time as it were of rounded form, presenting occasionally a some- what pronounced prominence, answering to the tuberosity of the scaphoid and internal cuneiform bone. The arch of the foot is flattened and loses more or less com- Fig. 143. — Impressions and lateral appearance of the feet of a patient suffering from a tabid foot on the right side. (On the left side the foot was sound j the right diseased.) (After Fe'r^.) pletely its curve, so that in some cases the sole of the foot is absolutely flat, and at times even slightly convex in form. This deformity is clearly seen in the impressions made by the foot. The arch of the foot is not always flattened (Troisier, Pavlidds), but on the contrary may be raised (Chinese foot ; Damaschino, PavlidSs). Displacement of the metatarsus outwards is sometimes observed, being less constant than the changes already mentioned. TABES DOESUALIS. 241 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 malleoli 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. Crepitation, whether spontaneous or during passive movement of the joint, occurs but little if at all. The absence of pain on pressure is as complete as in other forms of spontaneous fracture and arthropathy. Sometimes anaesthesia 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 I 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 Salpetridre 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 margins 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 cuneiform bone can be with diflficulty recognized. All the bones of the metatarsus and tarsus present a 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 fragm-ents of bone ; one can there- fore understand and agree with the following statement of Fig. 144.— Bones of the foot in tabes showing the os calcia to be formed of extremely loose oanoeUons tissue, while the bones of the tarsus are almost completely destroyed. Fragments of bone which have passed beneath the foot are of loose cancellous tissue and jagged, coming from the bones of the tarsus, and found at the autopsy to be separated from each other. (Collection of Charcot.) Fig. 145. — Tabid arthropathy in the foot. The os calcis forms, as it were, a thin osseous cup at its lower and anterior part, into which the astragalus has descended. (After Tuffier and Chipault.) A. ChaufTard, made in his excellent laconic expression, " In the tabid foot there is not more osteopathy than arthropathy." TABES DORSUALIS. 243 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 occur more in the tabid foot than in other forms of arthropathy, or in spontaneous fractures. It is un- necessary to say more about these points, 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 a peculiar appearance. Teophic Dtsoedeks in the Fibrous Tissde. Whilst considering these changes in the bones and articula- tions I should say a few words about the trophic disorders in the fibrous tissue. In speaking of the different forms of arthropathy the lesions which occur in the articular ea/psules 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. In a similar way we know that the intra-articular ligaments may be much altered, and even completely disappear. What occurs 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 aao doubt that it will be more frequently seen to occur when attention is drawn to this point. I must quote the case of Hoffmann, in which rupture of the tendo Achillis occurring when the patient made the simple 244 LECTURES ON DISEASES OF THE SPINAL CORD. movement required to enable him to turn round in the street. In the case of Lepine there was rupture of the quadriceps extensor cruris. The same muscle was ruptured in the case of Lowenfeld whilst the patient was walking quietly. Although the number of cases in which rupture of the tendons has been known to occur is small it is still possible . to see that there is obviously some analogy between the rupture of the tendons and that of the bones (spontaneous fractures). The fact that they specially occur in the lower limbs, and are produced by causes which are quite insignificant when compared with the effect which they produce is to be noticed in both cases. Trophic Disorders of the Skin. These disorders have been noted by many observers, amongst whom the names of Charcot, Fournier, and L'eloir should be specially mentioned. My intention is not to describe these in detail, but simply to enumerate them, since, owing to their infrequency, and but slight severity, they belong rather to the province of pure dermatology. Many forms of eruption have been observed on the skin of those suffering from tabes, of which the name alone will indicate the character. Thus, lichenoid, herpetic, ecthymatous, erythe- matous, urticated, and pemphigoid, &c., eruptions fnay occur. Zona has been occasionally met with, being specially seated upon the trunk. Vitiligo (Leloir) may also be observed. Ballet and Dutil * noted hypertrophy of the epidermis, which was thickened and desquamating, the hypertrophy affecting not only the papillae, but even the whole thickness of the skin. This is dry and can be raised in large folds, specially that of the upper limbs (the back of the hands). It is this condition to which authors have applied the term ichthyosiform state of the shin. One of the most interesting trophic disorders affecting the skin, at any rate on account of its frequency, is the perforating ■ulcer. \ It commences usually in a corn, which, after a variable * Ballet and Dutil, Note sur uu trouble trophique de la peau, &o. {Progres Medical, 1883, p. 379.) t Ball and Thibierge were the firat to call attention to these facta in 1881. On perforativg ulcer of the foot as connected with progressive locomotor ataxy. {Inter- national med. Congress, 7th sesaion, London, 1881, vol. ii., p. 52—55.) TABES DORSUALIS. 245 time ulcerates in its central 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 Review upon the perforating ulcer, he stated that it was of rare occurrence. As regards the first form, on account of which the patients never requine 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 Tuffier 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), but 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 must 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, usuallj' below the head of the 1st, and often below that of the 5th metatarsal bone, or sometimes below the heel. In some cases again it is found at 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 * Chipanlt, Le mal perforant. General Eeview in the Gazette des EopUaux, 1891, No. 83, p. 765. 246 LECTURES ON DISEASES OF THE SPINAL COED. cutaneous nerves, and the anaesthesia, which so often exists in and around the corn, is a strong argument in favour of this supposition. It is, however, evident that the mechanical effect rig. 146. — Sgle of the foot, in which is a perforating ulcer, A, and traces of two other similar ulcers of more ancient date are seen. produced upon the sole of the foot by walking or standing upright, takes a large part in its production, since the ulcer always occurs where the greatest pressure is made, either against the ground or the boot. Both feet may be affected, although frequently only one foot suffers from the complication, or two or more perforating ulcers may again occur in the same foot. GangreTie has been observed by some authors in the course of tabes, not only towards the end of the disease, which would not be surprising since this is liable to occur in all wasting diseases, but at the beginning of the complaint. These would be exam- ples of early gangrene with a rapid course : spots of blackish, violet, or reddish blue spots are first observed upon the buttocks, or over the trochanters or heels ; vesicles then form in the same parts, the liquid of which soon becomes turbid and of a red or brown colour ; the vesicles rupture and leave a bare, excoriated gangrenous surface of violet or black colour ; this part is then eliminated with suppuration,, and all the septic troubles with which you are acquainted. It is certain, gentlemen, that in all these cases some disorder of the nervous system exists, since it is to such an affection that the diminished nutrition of the skin which enables these complications to occur would in all pro- bability be due. On the other hand, the progress of bacteriology has enabled us to know that gangrene, or mortification occurring TABES DORSUALIS. 247 with such rapiditj' 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 aflfections 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, occurring 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 classed with them ; these are shedding of the nails, and loss of the teeth, as also the existence of hyperid/rosis and anidrosis. The shedding of the nails has been often observed in tabes, and I would specially quote the observations and memoirs of Joffroy, Pournier, Domecq-Turon, 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 dystrophy which sometimes occurs. They then * I. Straus, Des eoohymoses tab^tiques a la suite des crises douloureuses, Arch, de Newroloqie, 1880—81, No. 4. 248 LECTURES ON DISEASES OF THE SPINAL CORD. present the same appearance as that which occurs in some cases of eczema or psoriasis. Fournier, who has carefully studied them in these cases, describes varied forms. The nails, specially in the toes, are found to be much thickened, and the free extremity seems to be stratified, the surface is irregular and presents longitudinal or transverse streaks ; the nails are usually hard and brittle. In short, they are affected by true dystrophy and pronounced alteration of nutrition due in all probability to the lesions of the nervous system. The loss of the teeth has been observed for many years, since 1868 and 1869. Labb6 and Dolbeau have mentioned cases of its occurrence. Amongst the most important memoirs on this subject those of Vallin, Demange, Galippe and David should also be mentioned, and the different opinions which are held will be stated to you. This is what usually happens in such a case : without the patient having perceived that his teeth were in any abnormal condition, they gradually become loose and fall out without pain, and "as if they had been plucked," while after their separation the saliva may be but slightly tinged with the colour of blood, and the patient scarcely perceives what has happened. It sometimes occurs that when the tooth falls the corresponding fragment of the alveolar edge does so also; thus it has been possible (Dolbeau) to introduce through the opening which is thus produced, the finger or some instrument into the rtiaxillary sinus, besides which considerable atrophy of the alveolar margin is found to occur after a certain time in the case of all the patients whose teeth have been lost. Very different opinions have been formed as to the mechanism of this loss of the teeth ; on the one hand Demange looks upon it as purely and simply due to trophic disorder depending upon change in the fifth nerve. David, whose opinion is somewhat analogous, found that a change occurred in the pulp of the teeth, and that there was atrophy of the crown. Comparing these lesions with those found in other organs connected with the senses he believes them directly due to the nervous disorders which exist in tabes, and the more so on acccount of the pulp being undoubtedly a sensory organ. On the other hand, Galippe does not consider that the nervous lesions sufficiently explain the existence of this symptom ; in his opinion it is TABES DOESUALIS. 249 principally due to periostitis and osteitis affecting the alveoli and teeth, as well as to the loss of the latter, and destruction 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 sjinptoms connected 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 sudoriparous 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 vary 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 secreted. It should be stated that in some patients who suffer daring 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 diarrhcBa, 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. Lastly, an enquiry should be made as to the pathology of these trophic disorders in the skin. Are the nervous changes, upon which these disorders more or less directly depend, seated in the spinal cord or peripheral nerves ? This is a question 250 LECTUKES ON DISEASES OF THE SPINAL CORD. ■which it is very difficult to answer ; during the last few years the tendency of many authors has been to refer these symptoms to the existence of peripheral neuritis, which is observed in the course of tabes. Lesions in the spinal cord had been previously held responsible for their occurrence. It is certain that with the means of investigation which we possess it is impossible to decide what part is taken by each of these. Allow me, however, to remark that on the one hand, notwithstanding the continually increasing number of cases of polyneuritis from some cause or other, trophic troubles, analogous to those which I have just described as occurring in tabes, have scarcely even been mentioned ; on the other hand, in an affection which seems clearly connected with the spinal cord, syringomyelia, very similar symptoms do at times exist. This argument, as I admit, is of an indirect character, but, as it appears to me, it enables us to state that some, at any rate, of these trophic disorders has for its origin a lesion in the spinal cord. 251 LECTURE XXI. TABES. SYMPTOMS (continued). Muscular atrophy ; Distinction from the conditions of muscnlar emaciation, wMoli are due to consumption. Is specially seated in the lower limbs, and most often bilateral ; oecnrs also in the upper limbs and in the muscles supplied by nerves coming from the medulla oblongata. Onset. Extension. Fibrillar contractions. Electrical examination. Evolution. Tabid club-foot of Joftroy. Pathological Anatomy of these forms of amyotrophy ; muscular lesions ; opinions as to the nature of the nervous lesion (in the spinal cord, in the peripheral nerves). Hemiatrophy of the tongue : its symptoms. Appearance of the tongue, paralysis of the soft palate, and inferior vocal cord upon the same side. Lesions of the medulla oblongata in cases of hemiatrophy of the tongue. Conclusions which may be drawn owing to the oo-exiatence of paralysis of the soft palate and vocal cord. Attempt to range the forms of amyotrophy occurring in tabes in two distinct groups. 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 atrophy which occurs deserves special con- sideration. This symptom was some years ago studied in the most complete way by Condoleon,* the pupil of Jofiroy. I shall have occasion to quote more than once from his remarkable inaugural dissertation. Condoleon was preceded in the study of this subject by several 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, Joffroy, &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 * CoUdole'on, Contribution k I'^tude pathog^nique de I'amyotrophie tab^tique These de Paris, 1887. 252 LECTURES ON DISEASES OF THE SPINAL CORD. name we term the condition of muscular emaciation, flaccidity, and weakness, which so often occurs in patients suffering from this disease, specially in its later stages. As I have said more than once, tabes = consumption ; this is a condition of the muscles which is found to exist in every consumptive disease, and has nothing in it which is special to tabes. Thus, true musculwr atrophy will now be alone considered. This muscular atrophy, according to Condoleon, is usually seated in the loiver limbs, specially in the muscles of the leg heloiu the knee, and the foot ; it usually occurs on both sides but is sometimes unilaieral. In some cases, however, it occurs in the upper limbs, usually then occupying the STnall muscles of the hands. More rarely it exists in the forearms, or arms, at the shoulder, and in certain muscles of the back. The muscles supplied by nerves coming from the medulla oblongata may be similarly affected ; thus Schultze found it to occur in the muscles supplied by the motor root of the 5th nerve. We shall soon see that it also occurs somewhat frequently in those supplied by the hypoglossal nerve, facts to which I shall return, as they are of considerable interest, on account of the definite seat of the lesions to which they are due. Very different opinions have been held as to the /re^wence of this symptom. Eulenburg, for example, considers it rare, and says that it is only found to occur once in 250 patients suffering from tabes. This, gentlerrien, is a mistaken estimate. The symptom is far less uncommon, I would almost say that it occurs with some frequency, specially in the lower limbs, and cases of the tabid club-foot of Joffroy are notably observed in large numbers. The onset usually occurs in a late period of the disease. In some cases, however, it has been found to occur in a much earlier stage, and even during the prodromic period (Charcot, Fournier). The degree to which the muscles waste is sometimes con- siderable, and you will easily recognize this for yourselves in the photographs which I put before you. The hands may present a somewhat " claw-like " attitude, while the width of the muscles of the shoulder and trunk is found to considerably exceed the thickness. The fibrillar contractions are usually, but not always, absent ; TABES DORSUALIS. 253 when they exist it is usually when the amyotrophy occurs early in the disease. Fig. 147.— Atrophy o£ the mnscles of the right shoulder and of the hands in a, case of tabes. (Collection of Charcot.) The electrical examination, according to Condoleon, 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 course of these forms of amyotrophy is but relatively pro- gressive ; that is to say, the atrophy may gradually increase in 254 LECTURES ON DISEASES OF THE SPINAL COED. one group of muscles, but in other ways it rarely if ever shows any tendency to progressive change, and will not be found to extend slowly from one group of muscles to another, as occurs in some forms of progressive amyotrophy. Such are the general facts which the present state of our knowledge enables us to state with regard to the muscular atrophy which exists in tabes. I would now consider in a somewhat detailed way a special form of amyotrophy — the tabid dub-foot. The tabid club-foot has only been studied and described within the last few years by Joffroy. The observers who met with such cases before that time merely nqted their occurrence in a summary way, attributing them to the existence of tonic con- traction, the effect of which was combined with that produced by the weight of the bedclothes upon the feet. I cannot better describe the deformity which constitutes the tabid club-foot than by quoting the words which Joffroy himself uses. He alludes to the evolution of this deformity and its characters in the following terms : — " The affection begins in the foot being permanently placed in the position of pronounced extension ; in addition to this the extremity of the foot is inclined inwards, the inner side of the sole being hollowed out and raised, so that in reality talipes equino-varus exists, and since the lesion is usually symmetrical, the result is, that when the patient is in the dorsal decubitus, the heels are separated by 10 centimetres (4 inches), the two great toes as they approach each other leaving an angular space between the feet. •' When the condition becomes more accentuated the toes are bent to a very pronounced degree; the toes cannot be then straightened, or the foot be bent upon the leg ; the impediment ■is clearly seated in the position of the tendo Achillis, but the calf-muscles are not necessarily contracted, as Leyden supposes, since palpation of the wasted muscles shows them to be in the same relaxed flaccid condition as the antero-lateral muscles of the leg, in addition to which, if the leg is raised and moved about, lateral displacement of the foot can be easily obtained, which can scarcely be efiected when the calf muscles are con- tracted, and extend the foot. Electrical irritability is retained in all the muscles of the leg. (It has already been observed, TABES DORSUALIS. 255 however, that in one case Joffroy found partial reaction of degeneration to exist.) Pig. 148.— Tabid clnb-foot of Joffroy. (CoUeotion of Charcot.) "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 occurs 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 bedclothes from weighing down the foot, or if necessary, to apply a simple orthopEedic apparatus." Such is the tabid club-foot, gentlemen, which, notwithstanding tlie 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 256 LECTURES ON DISEASES OF THE SPINAL CORD. this muscular atrophy, another element takes part in its pro- duction ; this element is constituted by the trophic disorders of the articular ligaments which Charcot has taught us to know, notably in cervical hypertrophic pachymeningitis and alcoholic paralysis, and in which he has shown us the good effect of tenotomy. What, as regards its pcdhological anatomy, are the characters of the muscular atrophy which occurs in tabes ? The muscles are found to be wasted, of yellowish hue, and have sometimes the colour of dead leaves ; but it should be observed that in the midst of the degenerated muscular fasciculi others are nearly always found in greater or less number in a healthy condition. Thus by dissociation fibres differing in appearance may be obtained :— (a) Fibres which are much degenerated, varicose, and are totally deprived of their striee enclosing a large number of the nuclei of the sarcolemma and fine granulations of a yellowish brown colour and strongly refractive. (b) Fibres which are much reduced in diameter but retain their normal striation. (c) Fibres which are absolutely healthy. You will observe, gentlemen, that the different fibres of a muscle are not usually in the same condition ; but on the other hand, as in the micro- scopical preparation which I put before you, the different varieties of fibres form distinct fasciculi which degenerate independently of each other. This association of healthy and diseased fibres will explain (Rumpf) the fact that the reaction of degeneration is usually absent since the muscle nearly always contains enough healthy fasciculi to produce the normal response to electrical irritation. What, however, is the cause of this muscular atrophy ? Upon what lesion of the nervous system does it depend ? According to some authors it is due to pmplieral neuritis, the spinal cord, anterior roots, and large nerve trunks remaining unaffected. These authors specially rely on the researches of Westphal, Pierret, Dejerine, &c., who have shown that lesions frequently exist in the peripheral nerves in tabes. The opinion first promulgated by Charcot and Pierret, pro- fessed by Leyden, and accepted by numerous authors, is that muscular atrophy in tabes is due to a lesion in the grey TABES DOESUALIS. 257 matter of the anterior horn ; this lesion according to Charcot and Pierret is consecutive to extension of the change which occurs in the posterior columns. c A Pig. 149 —Section of a portion of the thenar muscles from a patient with tabes snffering from very pronounced amyotrophy. (From a preparation taken from the patien:t ■wlio forms the subject of fig. 151.) AA. Muscular fibres in a healthy condition ; BB. degenerating muscular fibres ; C. band of conneotiye tissue between the fasciculi. It will be obserred that the fasciculi of wasted fibres alternate with those which are sound, and that the degenerated fibres are dis- seminated in the midst of those which are sound. On account of this difference of opinion the researches of Condoleon 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 Condoleon 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 Condoleon 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 recognized. 17 258 LECTURES ON DISEASES OF THE SPINAL COED. In support of tlie opinion that the lesion which causes the muscular atrophy of tabes is seated in the nerve centres, and only secondarily in the peripheral nerves, I shall mention to you, gentlemen, in my turn, as an argument which cannot be refuted, facts which have been shown by many authors and by myself in connection with the liemiatrojilnj of the toncjue which occurs in tabes. In this case the anatoraical investigation is made in conditions which are specially favourable since it is known in which definite group of cells in the medulla oblongata the lesion should be sought, and the hemiatrophy of the tongue being a unilateral symptom the nuclei of the medulla oblongata can be compared with each other, and the least alteration which occuis in one of them be definitely recognised. The result of these researches was to show clearly that characteristic lesions existed in the nerve centres. Before mentioning these it would not, I think, be useless to describe somewhat in detail this hemiatrophy of the tongue which occurs at times in tabes and is accompanied by an interesting series of symptoms. As was the case with regard to most of the trophic disorders in tabes which we have already considered it is again Charcot who first called attention to the hemiatrophi/ of the tongue which occurs in that affection; this subject was again taken up at a later date and developed by Gilbert Ballet ; amongst the principal observations upon this symptom I must quote that of Raymond and Artaud, with the autopsy and histological examination very carefully made by Mathias Duval ; the thesis of Arnaud * contains interesting documents from a clinical point of view. I had the good fortune to obtain possession of the diseased parts taken from the patient under the care of Charcot and observed by Ballet and Arnaud ; P. D. Koch (of Copenhagen) and I made the microscopical exam- ination, and this enables me to put before you certain original sketches which represent the appearance of the patient and of the lesions found at the autopsy. Hemiatrophy of the tongue is not extremely rare in the course of tabes, and, as we shall see, occurs also without being connected with this affection. Its onset occurs slowly, without the occurrence of any inflammatory symptom, so that the patients * J. Arnaud, De rhcmiatrophie de la langue dans le tabss dorsal ataxique. These de Pari/, 1885. TABES DOESUALIS. 259i 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 protruded 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 (th6 small crescent) is shrivelled, reduced in size, placed Fig. 150. — Hemiatrophy of the tongue in a, ease of tabes (in this diagram the hemiatrophy is seated on the right side, the figure having been turned round • in reality it was on the left aide). (Colleotion 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 ot the atrophy. When the tongue is taken between the fingers (J. Hutchinson) it is found that when that organ moves the half in which atrophy has occurred does not harden, whilst, on the other hand the ordinaiy 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. Hencd 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 hemiatropihy in this organ. 260 LECTURES ON DISEASES OF THE SPINAL CORD. In some cases slight fibrillaiy movements are found to occur upon the affected side. Lastly, and this is a fact which is specially remarkable, and upon which Koch and I dwelt in our memoir upon hemiatrophy Fig- 1-51. — A patient suffering from tabes with hemiatrophy of the tongne on the right aide : the tip of the tongue is turned to the right side, the whole organ being "in the form of a crescent." In this patient there was also ptosis in the right eye (collection of Charcot). This is the patient whose autopsy P. D. Koch and I made, and whose medulla oblongata we examined. of the tongue, there exist in the same patients paralysis of one side of the soft palate, upon the same side as the atrophy of the tongue, and of the corresponding inferior vocal cord. I shall endeavour in a few moments to explain this singular coincidence. The course of the affection is otherwise benign ; although TABES DOESUALIS. 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. Fig, 152. —Section of the tongue from a case of hemiatrophy on the right tide (from the patient who is the subject of fig. 151). (After P. D. Koch and P. Marie.) I have already said, gentlemen, that hemiatrophy of the 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 P. D. Koch. Gouguenheim and Leudet had also mentioned it some weeks previously. This eetiology 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 tabes are connected affections, and the former depends directly upon the latter, or the two are merely associated dis-r 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 hemiatrophy is much smaller in size than that on the opposite side, a certain number of nerve cells having disappeared, as seen in the diagram which I now place before you. In addition to this the accessory nucleus upon the same side shows analogous changes 262 LECTURES ON DISEASES OF THE SPIRAL CORD. (Mathias Duval and Eaymond, Westphal, P. D. Koch and P. Marie) ; the afferent fibres in the nucleus of the hypoglossal rig. 1£3.— Section of the medulla oblongata at its lower part from a case of hemiatrophy of the tongue upon the left side. (After P. D. Koch and P. Marie.) The middle part of the floor of the 4th verticle is alone represented (the sinuous line below the figures) ; above this are the nuclei of the hypoglossal nerve. That on the left side is much reduced in size, as are the cells which it contains and the fibres which emanate from it. (In its reproduction the figure has been turned round, it is therefore the nucleus of the hypoglossal nerve upon the right side which is wasted.) nerve, described by P. D. Koch, are not apparently affected. On the contrary, the radicular fibres of the hypoglossal nerve present clearly atrophic changes upon the same side as that of the lingual hemiatrophy. It is a singular fact that no changes similar to those in the nucleus of the hypoglossal nerve are found either in that of the pneumogastric or spinal accessory nerve, although, as we have already seen, there is paralysis of the corresponding vocal cord, and half of the soft palate. In order to explain this anomaly P- D. Koch and I made a different suggestion from that first proposed by Hughlings Jackson and Henschen. In our opinion the muscles of the larynx and soft palate, besides the fibres which connect them with the nuclei of the pneumogastric and spinal accessory nerves, receive fibres also from the hypoglossal nucleus. Something of an analogous nature occurs in the eye ; thus, for example, the internal rectus receives at the same time fibres from the nuclei of the 8rd and 6th pair of nerves TABES D0ESUALI3. 263 (6raux-F6reol, Mathias Duval). When a change Has occurred in the nucleus of the latter nerve this muscle is found to be Fig. 15i. — Ssotion of the left hypo- Fig. 155.— Section of the right hypo- glossal nerve (normal) from a case of glossal nerve (diseased) from a case hemiatrophy of the tongue in tabes. of hemiatrophy of the right side of (After P. D. Koch and P. Marie.) the tongue in tabes. (After P. D. Kooh and P. Marie.) The nerve fibres which are in a normal con- dition and have retained their sheath of myelin (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 nucleus. Considerable disorder is seen to exist in these two organs, although there is, so 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 I can do at present is to call your attention to their existence. This is what I wished to say about lingual 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. I said " one form at least of the amyotrophy which occurs in tabes," since I must confess, gentlemen, that I am doubtful 264 LECTURES ON DISEASES OF THE SPINAL CORD. whether all the cases of amyotrophy in tabes are of the same kind, and whether it is not possible, at any rate from a clinical point of view, to establish a decided difference between them. As regards my own views, if at the end of this lecture, after having mentioned the actual condition of the question according to the ideas formed about it, I may be permitted to state my opinion I should be inclined to range the varieties of amyotrophy in tabes in two large distinct groups : — In one (A) would be placed the forms of amyotrophy which usually occur at a late period of the disease, are disposed symmetrically, and in which fibrillar contractions can scarcely be said to occur. In the other (B) I would place those varieties of amyotrophy which occur at an earlier period of the disease, of which it may be specially said that they are not disposed symmetrically, and in which fibrillar contractions are more frequent, and the reaction of degeneration exists more frequently than in those of the group A. The tabid club-foot of Jofiroy is the type of the forms of amyotrophy which belong to the first group, and which are in consequence very analogous to those which occur in different forms of intoxication, which have been ranged in quite a transi- tory way, as I think, among the forms of multiple neuritis ; it is to this type alone that the opinion of authors who contend with Dejerine that the amyotrophy of tabes is due to peripheral neuritis can refer. The forms of amyotrophy belonging to the second group would include lingual hemiatrophy, some varieties of atrophy affecting the muscles of the shoulder, the back, and perhaps the hands ; they would be secondary to lesions which clearly exist in the grey matter of the medulla oblongata or spinal cord. The classification which I have just sketched, gentlemen, is simply a suggestion of which the value must not be exaggerated ; it seems to me that from a clinical point of view the differences which existed were sufiiciently pronounced to justify the formation of these groups, and that, even when the pathological anatomy of this condition is considered, such a distinction makes it possible to explain the different opinions which have existed. This suggestion, gentlepien, I leave in your hands ; the future will judge of its value. 265 LECTURE XXII. TABES. SYMPTOMS (continued). ViscEKAL DISOEDEBB. — 1. Digestive system: (a) Stomach. — a. Gastric crises, their character ; pain, uncontrollable vomiting, excess of secretion, and acidity of the gastric jnice, researches of Sahli, Eosenthal, Hoffmann, state of nervous de- pression, its abrupt onset, and sudden disappearance ; tendency to relapses. Abnormal forms. — p. Tabid annorexia.— (A) Inteetine. — ». Intestinal tenesmus. — P. Tabid diarrhoea, II. Vascular system. — (a) Heart : lesions of the mitral, and aortic valves.— (6) Blood vessels : Arteriosclerosis ; angina pectoris ; exophthalmic goitre, works published upon this subject. Gentlemen, — 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. — Digestive System. A. Stomach. — Amongst the symptoms which occur in tabes in connection with this organ, the most interesting is undoubt- edly the gastric crisis. a. Gcistrio 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, " Lepons da Mardi"* the eminent professor of the Salpetrifere * J. M. Charcot, Legons da Mardi, 1888—89, p. 333. 266 LECTUEES CN DISEASES OF THE SPINAL COKD. Hospital again discussed this symptom, adding other facts and extending his classification ; it is this description which should guide us in the study which we are about to commence ; I must mention also to you certain facts which were brought to light by Fournier. Many of you have without doubt been already present at one of the dramatic episodes of tabes which are termed cjastrio crises since these are far from rare ; in whatever medical ward you may prosecute your studies, one case at least is almost certain to come before your notice. The two symptoms which specially characterise the gastric crises in tabes are : (a) the pain, (6) the uncontrollable vomiting. (a) The pain corresponds to that of gastralgia ; it is felt in the epigastrium, being at times limited to that part, but more often extending in different directions, towards the abdomen, the side or the back ; very severe in the great majority of cases, it is at times absolutely agonising, seeming to exceed what a human being can bear in the degree of suffering. -• (li) The uncontrollable vomiting is at first of the food taken, but soon afterwards exclusively of slimy liquid, or may perhaps consist of a clear liquid, mucous in character and more or less abundant ; at times the vomited matter is coloured by the bile or more rarely by blood ; it may quite exceptionally present the appearance of "coffee grounds" (a case under the care of Vulpian, one under that of Charcot). Usually the vomiting occurs very fr?quently, but varies in abundance ; at times but a few grammes (1 grm. = r5'432 grs. Troy) of liquid are vomited on each occasion, but sometimes a great deal more ; these different characters vary according to the patient and in the same patient from day to day. During the last few years different works have appeared about the composition of the liquid vomited in the gastric crises ; the conclusions formed are certainly not as yet quite established, but that is no reason whatever, gentlemen, why you should fail to be interested in the researches which have taken place. In 1885 M. Sahli* stated that an exscess of the secretion and of the acidity of the gastric juice occurred but without giving a quantitative analysis of the secretion. In the following year (1886), upon examining the liquid vomited during the existence * H Sahli, Corretpondbl./. Schweieer Aerale, 1885. TABES DORSUALIS. 267 of the gastric crisis, Rosenthal found during the first daj's 0"30 per cent, of hydrochloric acid, and during the last days only 0" 10 per cent. : now according to Gh. Richet the mean quantity of hydrochloric acid in normal gastric juice is 0-174 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 tlie acid was somewhat largely diluted. On the other hand Hoffmann was not able to prove the fact stated 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 Hoff- 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 less acid in reaction. Such great technical difficulties are associated with the study of these facts, and so few attempts have been made to elucidate them, that it is quite intelligible that no definite results have yet been obtained, though it seems most probable that such will soon be the case. In order to terminate this question of the acidity of the gastric juice in gastric crises, I must add that in some cases besides the hydn-ochloric acid, luetic acid has been met with in small but varying quantities. You will easily understand, gentlemen, the sensation of iveari- ness which is produced in the unfortunate patient by the severe pain and repeated vomiting. Thus you would have but an incomplete idea of the gastric crises if I omitted to describe this third symptom. (c) A state of nervous depression more or less pronounced. 268 LECTURES ON DISEASES OF THE SPINAL COKD. varying from simple indolence, from an indifference on the part of the patient to all which surrounds him, absorbed as he is in his own suffering, to true stupor. In the cases of exhaustion from these terrible shocks the unfortunate patients become almost unconscious, not answering when they are spoken to, or having even the strength to replace their bedclothes when they are displaced. At the same time this precaution is by no mean? useless, since when the affection is of this severity the patient is not rarely found cold, in a state of algidity, while the surface becomes of a livid colour, and to such an extent, gentlemen, that medical advisers who have not been forewarned, being struck by the severity of the vomiting and the algid condition, havQ made the diagnosis of cholera. In most cases the strongest ancj most obvious resemblance is to those who suffer from sea sickness, and it occurs in some people who are specially susceptible to it. , Another character of the gastric crises is the rapidity of theii; evolution. (d) Sudden onset. Abrupt cessation. — The onset is usually quite unexpected, and the crisis may continue without remission by night and day during 2, 3, 4, 8, 15 days, rarely more, be- coming more severe when the least attempt is made to take food, or to swallow liquid, although the thirst is often truly dis^ tressing. Then as if by enchantment the vomiting and pain- cease, a sensation of relief is felt by the patient ; the appetite, sometimes returns almost immediately, and in this case the; patient, who during several days had not been able to retain the smallest part of his food, is able to take a somewhat abundant meal without being at all inconvenienced by it. The gastric crises are usually early symptoms in tabes, often occurring even in the prodromic period. They are rarely isolated, that is to say, the patient seldom suffers from but one crisis of this kind ; usually, when they have once occurred, they tend to recur more or less frequently, at times indeed taking place somewhat periodically ; as for example every year, every 6 months, or every 3 months. As regards what ultimately happens, either of the two following sequelae are possible : not infrequently the gastric crises after a few years gradually diminish in number and intensity so as to completely cease ; or they may persist during the whole duration of the disease, death at times occurring during one of the crises. TABES DORSUALIS. 269 whilst the patient is in the collapsed condition, or after the onset of coma. Such is usually the aspect which the gastric crisis presents, but you should know, gentlemen, that it does not always occur in. this manner. In his lectures Charcot did not fail to study its abnormal forms. / A. The gastric crisis retains all the fundamental characters of the type, but the pain 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. 0. 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 no pain, but vomiting exists, pre- senting the characters which have been already mentioned (Vulpian, Pitres, Fournier); B, Fournier has described a flatulent variety of gastric crisis,, characterised by the large quantity of gas discharged by eructa- tion. F. The crisis is sometimes of very and exceptionally short duration, lasting at the most but a 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 duration of the gastric crisis may again be prolonged, and continue during from 15 or 20 days to a month, or even more. A patient obsei-ved 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. y8. 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, ceases to 270 LECTURES ON DISEASES OF THE SPINAL CORD. be nourished ; he has an Insuperable aversion to food, and only eats or drinks from a sense of duty, since, as I have already said, the feeling of hunger is completely absent. Notwith- standing this loss of appetite, and the vomiting which occasionally occurs, the tongue remains moist, clean, and free from deposit. This, briefly speaking, is a more or less exact reproduction of the singular condition which Charcot made known to us by the name of hysterical anorexia. Owing to its complete resemblance, owing to the frequent co-existence of tabes and hysteria in the same patient, it seems to me, gentlemen, that the occurrence of tabid anorexia may at times be due to the association of these two affections. Subsequent observations will enlighten us upon this point. B. Intestine. — Fournier described not only the derangements of the stomach which I have just mentioned, but also intestinal symptoms which occur in tabes, which he carefully studied, and which he has graphically delineated. With this author we may divide these symptoms into two principal groups : — (1) intestinal tenesmus ; (2) tabid diarrhoea. a. Intestinal tenesmus. This consists in a desire to evacuate the bowels, which, as Pournier expresses, is remarkable in three ways : (1) on account of its strong character (it must be gratified at once ; while fearing to soil his clothing the patient finds it absolutely impossible to wait) ; (2) on account of its unusual frequency (4, 6, or 10 times in the same day) ; (3) on account of the absence of any cause for it (when the patients have the opportunity of relieving their pressing need very little or perhaps nothing at all is passed). The defecation is again quite inactive, not being associated either with spasmodic con- traction of the sphincter, or the sensation of a foreign body in the rectum. /8. Tabid diarrhoea. This symptom is somewhat frequently observed in the prodromic period (Fournier) ; it thus usually occurs as an early symptom. It consists of frequent pasty or liquid stools, usually in small quantity ; the diarrhoea being in most cases attended by little or no pain, and unaccompanied, so to speak, by colic. The characters specially characteristic of this form of diarrhoea are, on the one hand, the absence of oaiise, the diarrhoea occurring in fact quite spontaneously, and without the occurrence of any Tabes dorsualis. 271 irregularity in the diet, exposure to cold or other cause, being, as Foumier expresses it, a form of " essential diarrhoea." 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 suffer from it. II. — Vascular System. Lesions and derangements frequently exist in this system, much more often, in fact, than is usually believed to be the case, since, as 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 derangements are numerous, and affect the heart and blood vessels in different manners. In connection with the vascular derangements I shall have 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 mitred 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 cases 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 exisb, 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 large number of cases ; I woiild 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 272 LECTURES ON DISEASES OF THE SPINAL COED. definite indications of a cardiac lesion would be found in at least one of them. (6) Blood vessels. The vascular lesions in the course of tabes are comparatively less known than the cardiac ; one fact is how- ever certain, namely, that they are • frequent, and may be observed in the small arteries as much as in the large ones. Arteriosclerosis, with its different aspects and various con-v sequences, occurs with some frequency in tabes. The existence of this vascular lesion has indeed been the base of certain theories formed about the nature of tabes, and, a point which now specially interests us, about the nature of the cardiac changes of which I have just spoken. The following are the opinions which have been formed in order to explain the production of cardiac lesions : — According to some authors (Berger, Rosenbach, Grasset, &c.), these lesions are due to real trophic disorder of tabid origin, which affects the valves or the heart itself. J. Teissier goes so far in this respect as to pronounce the expression "perforating ulcer of the aortic valve." According to other authors (Adamkiewicz, Rumpf, Hippolyte Martin, Letulle, &c.), tabes and the vascular lesions would pro- gress in a parallel manner, the lesion in the cord being the direct consequence of vascular lesions localised therein, and occurring at the same time as in other regions of the body. I must confess, gentlemen, that I am not quite satisfied with either of these opinions, and for my part am much inclined to doubt whether tabes and the cardio-vascalar affections are thus intimately connected as cause and effect ; the. explanation of the frequency with which cardio-vascular lesions occur in tabes is more probably, in my opinion, the fact that in tabes the great majority of patients have suffered from syphilis ; now syphilis is one of the most important causes of cardio-vascular lesions, specially of those connected with the aortic valve; it is not therefore surprising that in tabes lesions of the spinal cord and of the heart and blood vessels occur simultaneously in the same way as symptoms connected with the skin and mucous mem- branes. As regards cardio-vascular disorders there is one of which a few words should be said, namely, angina pectoris (Vnlpian, Leyden) which not unfrequently occurs in tabes, and presents the usual TABES 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, &c. 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 during 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 " Societe des Hopitaux,"* by Barie and Jeffrey. 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 undoubtedly suffering from tabes, since the lightning pains, the symptom of Westphal, the symptom of Romberg, the inco-ordination of movement, the plantar anaesthesia, 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, &c., 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, Soc, mid. des hdp. de Paris, s&nce du 14th December, 1888. 18 274 LECTURES ON DISEASES OF THE SPINAL CORD. one. Besides those presented to the Soci6t6 des Hopitaux hj Bari6 and Joffroy I can name many others : one of Marcus Gunn,* which occurred in 1883, another of A. Marina t ; Charcot- also presented a case to his audience; lastly, a recent thesis, J written under the inspiration of Mendel, has for its subject a case of this affection. As to how this singular connection between tabes and the disease of Graves should be understood opinions differ consider- ably. It must be remarked, however, that nothing is settled, from a chronological point of view, between these two affections. Thus, in the case of Bari6, tabes had preceded the occurrence of the symptoms of the exophthalmic goitre, whilst the contrary took place in several cases of Joffroy. Naturally the opinion of these two authors was influenced by this difference in their observations. According to Barie these are cases of tabes during the course of which lesions occurred in the medulla oblongata which produced secondarily the disease of Graves. Joffroy believes, and Ballet is of the same opinion, that there is simply a combination of two distinct nervous diseases, neither of which is in any way the cause of the other. For my part, gentlemen, I had the opportunity of making the autopsy of the case which I had been able to observe. From a clinical point of view I can affirm positively that a typical case of the disease of Graves was combined with one of confirmed tabes ; there is no doubt whatever of this fact. The microscopi- cal examination of the cord revealed changes which were characteristic of tabes at the onset of the disease. But I have not yet been able to examine the medulla oblongata, and regret that I cannot therefore definitely settle this interesting question. At the same time, I am strongly inclined to think that it is by the influence of tabes that the disease of Graves was caused to * Marcus Gtmn.— I have not been able to find this work, which was only- indicated to me secondhand. (The acconnt of this case is found in the Trans. Ophthalm. Soc, Vol. III., p. 236— Tbanblatoe.) t Al. Marina, Arch. f. Psch., XXI. { J. Weiner, Ueber einen Fall von morbw Basedowii mit tabes incipiens. Inaugural Distert., Berlin, 1891. During the publication of these lectures another case has been published by Mobius, Ueber die Basedow'ecke Krankheit. Deutsche Zeitschriftfiir Nervenheilhunde, 1891, 1. 1, p- 423. I have also been able to consult since writing this lecture the following work : Art. Seochieri, communicazione fatta al congresso di medicina tevutosi in Padova nel Settembre, 1888, in Sivista veneta di scienze mediche. November 1889. TABES 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 Barie believes. It must also be observed that, if the coincidence of tabes and the disease of Graves is not very frequent, symptoms 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. Kahler 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 reflex to he absent. In 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. 276 LECTUEE XXIII. TABES. SYMPTOMS (continued). Visceral disorders. III.— PHABTNao-LARYNGEAL system. A, — Pharj,nx. Eesearches of Fano. Pharyngeal crises of Oppenheim. ^.—Larynx. Tabid Laryngismus : (1) Acute symptoms of tabid laryngismus ; laryngeal crises, their description, laryngeal ictus, nature and mode of production of laryngeal crises. (2) Chronic symptoms of tabid laryngismus, whistling inspiration, unnatural voice, having two tones. Laryngeal paralysis : the most frequent is that of the posterior crico-arytenoid muscles. Lesions of chronic laryngismus connected with the nerves and medulla oblongata. rv. — Urinary system. A. — Derangemenia of the urinary secretion : glycosuria ; changes in the amount of urea, phosphoric acid, &o. ; excessive secretion of urine. B.— Derangements of the winary excretion: " false urinary patients " of M.Guyon ; relative retention, complete retention; absolute incontinence, relative inconti- nence ; versioal colic ; nephritic crises. v.— Generative system. A. — Derangements in the mole : impotence ; generative excitement ; cremaster reflex, its research, its signification ; bulbo-cavernosus reflex ; atrophy and anaesthesia of the testis. 3. — Derangement in the female : genital depression ; genital excitement j pain in the genital organs ; vulvo- vaginal crises. Gentlemen, — The visceral disorders, or rather those of the internal organs, which we shall now study are those which affect the pharyngo-laryngeal system. III. — ^The Phahyngo-laeyngeal System. A. Pharynx. — The derangements of the pharynx have been recently described by several authors. Their existence at the present time, however, is far from being generally recognized, and works controlling their existence not having been written, I look upon it as my duty to at least enumerate the derange- ments which may exist. In 36 patients suffering from tabes, v^ho formed material for the researches of A. Marina to which I have already alluded, TABES DORSUALIS. 277 Fano found hypercesthesia or anaesthesia of the soft palate to exist in 30 ; in 14 cases the sensibility of the pharynx was diminished, and in 9 cases that of the larynx. In 4 patients this author found the pharyngo-laryngeal reflexes to he excessive and associated ivith hypercesthesia of the pharynx. In 5 cases the epiglottis was much lower 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 suffering 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. Larynx. — The derangements of the larjmx 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 practice. 1. Acute 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 full 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 which * Oppenheim, iVtue Beilrcye zur Path, der Tabes dorsalis (Arch. f. Pti/ch. XX., 1888). 278 LECTURES ON DISEASES OF THE SPINAL CORD. recalls the "kink" of whooping cough. In the latter case, a coiiigh is associated with the dyspnoea which occurs in paroxysms presenting at times, on account of the noisy inspiration to which I have just alluded, all the characters of the cough which exists in pertussis ; the cough is dry and unaccompanied by expectora- tion, except at times when it occurs in small quantity at the end ofthe paroxysms. The dyspnoea which occurs in the two cases and which really constitutes the laryngeal crisis differs much in character, being sometimes slight and of short duration, whilst at other times it is pronounced, and continues for a longer time, whereas in some cases it is extremely severe, constituting the laryngeal idvs of Charcot. This symptom is then trulj"- alarming, and for my part I shall never forget the conditions in which I witnessed it for the first time. It was in 1879, during the first year of ray being house physician at the Salpetri^re Hospital, and I was in the room of the suparintendent of "incurable patients," when a female patient entered to ask a question of my esteemed friend and colleague Brissaud. The patient was slightly out of breath whilst she was speaking, each inspiration being accompanied by a pronounced whistling sound ; in my mind I made the diagnosis of "compression of the recurrent nerve" by some mediastinal tumour ; when suddenlj^ whilst Brissaud was explaining matters, the patiently fell violentlj' to the ground in an unconscious condition, making convulsive movements and with the face of a purple colour. I feared that the patient was about to die suddenly, and undoubtedly betrayed my emotion, since Brissaud hastened to reassure me and explained that this patient waa liable to attacks of laryngeal ictus. This was an attack of such a nature, and the patient was one of those of whom Charcot made use when describing this com- plication. The following, according to this author, are the characters of the laryngeal ictus : usually a burning sensation is first felt in the region of the larynx, representing a form of aura, which is followed by a dry cough occurring in short hacks, the patient then falls to the ground in a state of unconsciousness, and most often general flaccidity, whilst epileptiform convulsions sometimes occur, causing the attack at times to resemble those which occur in epilepsy ; the face is usually livid. The laryn- geal ictus generally lasts but a few seconds, at the end pf which TABES DORSUALIS. 279 the patient rises of his own accord and immediately regains his senses without the occurrence of any condition resembling the *' confusion," which is liable to occur after an attack of true epilepsy. You have seen, gentlemen, how severe the attacks of dyspnoea 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 dyspnoea 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 necessary. As regards the frequency and course of laryngeal crises it is difficult to make any precise statement, on account of tbe variations which occur ; frequent in some patients, they occur seldom in others ; constantly increasing in frequence and severity in some patients they diminish in others, and are even found at times to cease altogether. Nor are authors agreed as to the pathology of this symptom. 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 hypersesthesia 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, &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 280 LECTURES ON DISEASES OF THE SPINAL COED. to Fano, who attempted to verify it in 36 patients suffering from tabes, and whom he examined in regard to the laryngeal symptoms, this sensitive point was only found to exist in one case, and this was in a patient who was not subject to laryngeal crises. The supposition of muscular spasm, due to hypersesthesia of the mucous membrane, accords also with the favourable effect which is produced by anti-spasmodic remedies (ether, chloroform, cocaine, and bromides), and upon the duration and intensity of the laryngeal crises. 2. Chronic symptoms of tabid laryngismus. In some patients true laryngeal crises do not occur, but only a sensation of pricking and uneasiness in the region of the larynx. The breathing of these patients is often more or less permanently wliistling in character, and the voice often becomes unnatural and of two different tones. At times there is also slight dyspnoea, the inspirations at least being difficult and painful, while the cough, should one exist, may be muffled, unnatural, and accompanied by a tendency to eructation, the symptoms, in fact, of laryngeal panralysis. Laryngeal paralysis is comparatively frequent in the course of tabes, and deserves brief consideration. The posterior crico- arytenoid muscles are most often affected, which, as you know, dilate the glottis, and are therefore essentially respiratory muscles. They are not infrequently paralysed on both sides, and according to some authors this condition is essential for the production of the whistling breath sounds, due to the fact that abduction of the vocal cords is impossible ; this symptom would not result from unilateral paralysis of the muscle. The posterior crico-arytenoid muscle being, as has just been observed, a muscle connected with respiration and not with speech, you will easily understand, gentlemen, that its paralysis does not produce loss of voice, though it probably contributes to make it unnatural, and to produce the double tone to which allusion has been made. Other laryngeal muscles may be also paralysed during the course of tabes, as for example, the thyro-urytenoid or lateral crieo-arytenoid, but these muscles are paralysed less often, and to a slighter degree than the posterior crico-arytenoid muscles. TABES DORSUALIS. 281 l^he coincidence of the laryngeal 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. — Bight 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 on the left side is the septum. A, nucleus of the pneumogastric nerve ; B, ascending root of the glosso-pharyngeal nerve, in which are fibres of the pneumogastric and spinal accessory nerves — the " slender column " ; C, ascending root of the 5th pair of nerves ; D, root fibres 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. In the nerves, changes have been found to exist in certain nerve trunks, either in an isolated condition, or conjointly with 282 LECTUEES ON DISEASES OF THE SPINAL CORD. lesions of their roots or their nuclei themselves in the medulla oblongata. These nerve trunks are the vagus, glosso-pharyngeal, and spinal accessory; Oppenheim has also been able to show that the electrical irritability of the recurrent nerve is lost. In the medulla oblongata, atrophy of the muslei of these same nerves (the vagus, glosso-pharyngeal, and spinal accessory) has been mentioned by a somewhat large number of authors (Jean, Pig. 157. — Right half of a section of the medulla oblongata, from a case of tabes with laryngeal crises, (To be compared with the preceding figure.) A, nncleus of the pneumogastrio nerve, which is altered and no longer contains nerve fibres ; B, ascending root of the glosso-pharyngeal nerve, in which are fibres of the pneumogastric and spinal accessory nerves — the "slender column"; this tract is very much altered, its nerve fibres having completely disappeared ; C, ascending root of the 5th pair of nerves which is affected and of a white colour; Djthe root fibres of the pneumogastrio nerve have almost disappeared. (After Oppenheim.) Demange, Landouzy and Dejerine, J. Eoss, Kahler and Oppen- heim) . To these facts I must add those which I have already men- tioned, in which hemiatrophy of the tongue coincides with laryngeal paralysis, or rather with atrophy of the vocal cord on the same side. In these cases the laryngeal symptom would, in TABES DOBSUALIS. 283 the opinion of P. D. Koch and myself, be solely due to the lesion in the nucleus of the hjrpoglossal 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. — Urinary System. A. Derangements -of the urinary secretion. These may be qualitative or quantitative. Amongst the former I must mention glycosuria* which is perhaps due to the existence of tabid lesions in the floor of the 4th 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 a proportionate increase in the earthy phosphates. Lastly there is a great variety in the quantity of chlorine eliminated, with a tendency to the prod action of hypochlorites, after the researches of Livon and Alezais. Albert Robin (verbal communication) has remarked that in certain patients suffering from tabes the quantity oi phosphorus eliminated by the urine in an incompletely oxidised state is more considerable in the early morning than at bedtime. This difference would be due to the nervous Trritation 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 Souques, in the Archioes de Neurologie, 1891, maintain the interesting opinion that in some patients suffering from tabes glycosuria might be the expression of a hereditary diathesis, since as Charcot has shown, tabes is frequently met with in families which suffer from diabetes. ' 284 LECTURES ON DISEASES OF THE SPINAL COED. giving rise to the term " urinary crisis," which was proposed by M. Fere for cases of this nature. This is one of those fluxes (gastrorrhcBa, sialorrhoea, persistent diarrhoea, &c.) which are far from rare in tabes. B. Derangements of urinary excretion. These are extremely frequent, and may occur in the earliest period of the disease, a fact which, from their being conjoined with other special symptoms, enables them to be classed amongst the best signs of tabes at the onset. They constitute at times the first indication of tabes, and the patients naturally almost always consult a surgeon who specially treats affections of the urinary passages. A local examination is made, and, as would be expected, no lesion of the urethra, prostate, or bladder is detected, to which the disorder from which the patient suffers might be due. This is the reason why Guyon very rightly terms these persons " false urinary patients." When tabes is suspected the patient should always be asked whether he has remarked anything unusual to happen when he passes urine. Sometimes, specially in the case of those who observe but little, the patients will say that they have not remarked anything abnormal. You must not, however, be satisfied with this reply, but put the definite question, " Are you obliged to wait, or to strain in order to pass urine ? " This time the patient, who now understands the question, will give an afifirmative answer, a fact which I can guarantee before hand, and often he will add characteristic details. The fact is, that though the bladder may not be paralysed at the onset of tabes, the control over that organ is partially lost ; it is, as it were, an instrument upon which the patients cannot play with the necessary precision ; instead of passing water freely they are obliged, so to speak, to " pump," to strain with all their might, and before the first drop of urine is seen to appear they have to icait, not a few seconds only, but one or two minutes, or even more. As Fournier says, " these patients only pass water in several acts," and I would add that the intervals between the acts are often so long that the spectators, should there be any, are unable to -wait. Thus Fournier gives the history of many of liis patients, who dared not pass water in the public urinoirs, innumerable quarrels arising with those who were about to use them, and who, desiring to follow in their TABES DORSUALIS. 285 turn, were provoked on account of the long time which passed before the place was unoccupied. Gomjplete retention of urine sometimes occurs, though it is rare, and most often of temporary duration ; I 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 rare ; when it occurs it is often like retention of temporary duration. 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 anaesthesia 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. In some cases micturition is abnormally frequent, and " pol- lakiuria" exists, or, on the contrary, it may be infrequent, sd 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." I have just spoken, gentlemen, of the ancesthesia 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, true 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, but on account of the special rdle of the male or female sex in the sexual act causing them to have peculiar characters. 286 LECTURES ON DISEASES OF THE SPINAL CORD. a. In the male. — It is in the male sex, as you will easily understand (the part taken being more active, the investigation more easy), that the derangements of the generative system have been specially observed. a. Impotence. This is the most frequent symptom of tabes connected with the generative organs. At first this impotence is but relative, erection occurs but slowly, and the patient con- fesses, while shaking the head with an air of resignation, that " he has lost much power in that respect," and when erection pccurs it has an unsatisfactory character, and should it by chance be sufficiently pronounced it is the ejaculation which is but slowly produced should it fortunately occur at all. This disorder gradually, or at times rapidly, increases until absolute impotence exists. Fortunately for these unhappy persons sexual inappetency increases in a corresponding manner. A patient suffering from tabes who was quite impotent observed to me once at the hospital, "When, sir, hunger no longer exists it is of little consequence that the teeth are lost." Those suffering from tabes are "no longer hungry." It is on this account, as I said just now, that these sufferers from impotence are resigned, in which they differ from many affected by disease of the nervous system who, though impotent, are starving. This, however, is not always the case. In some cases the onset of tabes is marked by the opposite condition, and the period of impotence is preceded by one of excitement. b. Genital excitement. This, which is a rare condition, may affect the different stages of the sexual act at the same time (the desire, the erection, the ejaculation), and the patients in whom it occurs feel sometimes flattered on account of their power, but more often somewhat alarmed, and not unreasonably so. Usually, however, this excitement barely exists except in appearance, and is a false indication ; the frequent persistent erection amounting in some cases to real priapism is often unassociated with any desire and followed by no ejaculation, or perhaps the ejaculation is premature, occurring at the first contact and producing no pleasure, but sometimes, in fact, actual pain. In some patients pollution simply occurs, and Poumier, who has known the ejaculations to occur in groups at intervals of time more or less distant from each other, suggests that they are really "crises" TABES DOHSUALIS. 287 more or less analogous to the different visceral crises with which we are alrea'dy acquainted. So far all the information which it is necessary to have with respect to the different genital derangements must be obtained from the patients, who should be questioned, their answers being then believed to be true. We have, however,- the means of controlling their statements up to a certain point, and at least of knowing whether the patients are, or are not, impotent. These means are furnished to us by the examination of the different reflexes connected with the genital organs. 1 . Gremasteric reflex. No practical examination being usually made with regard to the existence of this reflex, at any rate in France, it would be useful, I think, at any rate to some of you, gentlemen, if I indicated the modus faciendi. One of the two persons whom I now place before you is in good health, while the other is a tabid patient suffering from impotence ; the con- dition of the reflex will be examined in each of the two patients. 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 ; as often as I repeat this action the same elevation of the testis occurs ; this is what is termed the cremasteric reflex. We will now 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 having as yet been so 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 nervous system, as in tabes ; on the other hand, when impotence exists in other nervous diseases and is not due to an organic lesion of the nerve centres, the 288 LECTUKES ON DISEASES OF THE SPINAL, CORD. cremasteric reflex persists, and it may even be hoped that the impotence will subsequently cease to exist. There is another mode of testing the cremasteric reflex, with which I should make you acquainted ; instead of the inner sur- face of the thigh being rubbed, somewhat strong pressure need alone be made with the thumb upon the inner surface of the thigh opposite the opening which exists in the adductor magnns muscle for the passage of the femoral vessels ; the same result occurs. Both these means of producing cremasteric reflex should be tried before it is pronounced to be absent. Lastly, I would put you on your guard against the error which consists in mistaking contraction of the dartos for that of the cremaster muscle. This contraction of the dartos, being due to the con- tact of the air when the part is uncovered, has no connection with the cremasteric reflex; the fact that it is not seated in the testis in the same way as the latter reflex, but generally occupying the whole scrotum enables it to be distinguished; again, the movement which is produced is slow and vermicular, whereas the elevation of the testis in the cremasteric reflex is on the contrary abrupt and rapid. 2. Bulho-cavernosus reflex. This reflex has been recently described by Onanofi",* who also mentioned the changes to which it is liable in tabes ; these and the mode of investigating them are in the words of Onanofi" as follows : — " The index of the left hand being placed over the region of the bulb of the urethra, the right hand rapidly rubs the dorsal surface of the glans with the edge of a small piece of paper, or again, the mucous membrane is slightly pinched by it ; in these conditions the index which is applied to the bulb feels the movement to occur, which is due to contraction of the ischio- and bulbo- cavernosus muscles." This reflex which constantly exists in healthy persons, and even in those sufiering from nervous diseases, in which the genital functions are unaffected, ceases to occur in some cases of tabes ; in these complete erection no longer occurs according to OnanofF. This author is the first to admit that this statement is liable to some modifications, his researches only having been made as yet in cases of tabes. 3. Atrophy and anaesthesia of the testis. -These two symptoms * Onanoff, Du r^flexe "biilbo-cavenieux," Societe du Kologie, stance of May 3rd, 1689. TABES DORSUALIS. 289 Have bepn mentioned and studied by Pitres and EiviSre.* The atrophy of the testis was found by them to exist in 3 out of 20 cases; with regard to the anaesthesia of this organ when exposed to pressure they found it to exist in 16 out of 20 cases ; this form of anaesthesia may occur in a veiy pronounced degree. These, gentlemen, are symptoins of tabes connected with the genital organs, of which there is direct evidence, and which give valuable information as to the condition of their functions in the male sex. j8. In the female. In the female sex, as I have already observed, the derangements connected with the genital organs are 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 a healthy condition having no sexual desire whatever. In females sufferi- 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. (b) Genital excitement. This sometimes occurs to a very pro- nounced degree, as for example, when the condition occurs which is termed by Pitrest 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 voluptuous 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 precisely the same way as during ordinary sexual connection. This spontaneous crisis was almost always repeated three or four times during the same day, after which one or two weeks * Eirifere, De I'anesthesie et de I'atrophie testiculaires dans I'ataiie loooruo- trice, These de Bordeavx, 1886. f Pitres, Pes crises clitoridiennea au d^but ou dana le coiirB do I'atazie locomo* trice. Progres medical, 1884, No. 37, p. 729. 19 290 LECTURES ON DISEASES OF THE SPINAL CORD. passed without ' its returning. It was followed by a painful ■ sensation of languor affecting the stomach. It should be noted that at this time the patient was living with her husband, 's\'ith whom sexual connection occurred to a full though not im- moderate extent." The clitoridean crises therefore, as this example shows, consist merely of pollution, accompanied by the ordinary voluptuous sensations. The singular point about them is that they occur spontaneously, and usually in paroxysms. They are quite unaccompanied by pain, which is not the case with regard to ths other variety of crisis which I have still to mention. , (c) Pains in the genital organs. 1 have already had occasion to mention the occurrence of pain in the male sex, and have alluded to the painful ejaculation, the sensation of a foreign ■body in the urethra or bladder, &c. In the female tabes may also be accompanied by pain in the genital organs, which at .times are more or less analogous to those just mentioned. In other cases they are of a special character, having been described by Morselli* by the name of vulvo-vaginal crises. In these there ,is no erotic element, as in the clitoridean crises, the patients experiencing severe pain, which seems due to a very painful .spasm of the constrictor vaginae muscle. Thus Morselli com- pares the vulvo-vaginal crises with those connected with the larynx, in which, as we have seen, the spasmodic element is found to preponderate. Such, gentlemen, is an enumeration of the principal derange- ments of the genital function, which are observed during the course of tabes in the male and female sex. Before this chapter is ended it should be mentioned that these symptoms vary .to a considerable degree in different subjects, although some resem- blance always exists between them. It should not be forgotten again that these symptoms often occur at quite an early stage of the disease, belonging to the pi'^ataxic period, during which we have seen so many tabid symptoms occur, and which in the opinion of every observant medical practitioner may be regarded as really a stage of confirmed tabes. * Morselli, Sulle crlsi vuho-vaijinali ntW attasia hcomo'.riije. Giornak di Neuropa- .(ptoyto, 1890, March— Juno, p. 117. 291 LECTURE XXIV. TABES. SYMPTOMS (continued). VI. Ceebbbal system. — Hemiplegia. Apoplectiform attacks. Epileptiform attacks. Acute symptoms connected with the medulla oblongata. Psychical derangements. Coincidence of general paralysis of the insane. CoTiBSE or TABES. — Prodromal period. Second period, period of inco-ordination. Third period, confinement to bed. Forms of tabes : upper or cervical, cerebral, mild, and severe forms. VI. — Cerebral System. The hemiplegia which somewhat frequently occurs 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 attacks which sometimes occur, and the psychical derangements. I would also remind you that many authors (Berger, &c.) have observed migraine to indicate, so to speak, the 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 consciousness, and may be accompanied by aphasia, 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 epilepsy at times occurs. By what mechanism are these complications produced ? It is probably multiple ; in some cases foci of real hgemorrhage or sofcening exist, or some morbid process of long duration in the meninges or ependyma, 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 lesions alone, and it seems far more 292 LECTURES ON DISEASES OF THE SPINAL CORD. probable that they are connected with syphilis, which, as you will soon see, is the origin of tabes. It seems that the acute symjptoms connected with the medulla oblongata, mentioned by Hanoi and JofTroy* as occurring at the onset of tabes, may, at least as far as their clinical aspect is concerned, be compared with those which precede. The jpsychical derangements which occur in the course of tabes are not infrequent but vary considerably, and Dieulafoy was able to apply the term " tabid insanity " to the condition which existed in some patients ; while others present similar symptoms which are but slightly pronounced, and may be either transitory or permanent, or possibly occur " in paroxysms " (Fournier). I should not like to exaggerate what happens, and on that account it is, I think, necessary to distinguish the moral from the intellectual derangements; the former are, I must allow, frequent, I would almost say constant, but whether they are due to the existence of tabes is doubtful. Is it not rather the consumptive tendency of this affection, the weak condition in which the patients are placed, which are the cause of this change in their character ? However this may be some apathy, some indifference, are usually found to exist, which in certain ex- ceptional cases may reach a condition of melancholic stupor, or more or less pronounced irritability. Notwithstanding this tendency, and the agonizing pains which the patients frequently feel, they are rarely found to commit suicide, whilst on the contrary, in certain affections, notably those of the bladder, suicide is relatively more common ; this is an interesting point in connection with the psychology of the tabid patient. As regards the intellectual disorders, these, gentlemen, are far . less frequent, and if truly pronounced depend upon the association of cerebral lesions with those of the spinal cord ; tabes is in that case most often found to be complicated by general parah/sis of the insane. This, gentlemen, is a real combination, and in most cases intellectual disorders, if at all pronounced in a tabid patient, should be referred to this complication. Such cases, however, are somewhat rare, I would almost say exceptional ; on the contrary the mental condition of the tabid patient is usually found to be good, as good in fact as before the onset of the * Hanot and Joffroy. Des aooidontsbulbairesaigusaud^butdu tabes. Congrh d' Alger, 1881. TABES DORSUALIS. 293 disease, whatever . some authors 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 not 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 but those who are dead : the artist Manet sufiered 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, I think, that intellectual disorders are not usually associated with locomotor ataxy. Course and forms of the Disease. I 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 retrospective point of view, how the principal symptoms occur, that is to say, what is the COURSE of the disease. In ordinary cases 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. Prodromic period. — This is pre-eminently the time at which the lightning pains occur, which are most often referred by the patient to some totally difierent cause, and he voluntarily terras them " rheumatic." Thus reassured as to their nature he troubles 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 occurrence of these lightning pains enables the existence of tabes to be recog- nized. In most cases something more is required, some things 294 LECTURES ON DISEASES OF THE SPINAL CORD. which has more effect upon the'patient's mind. This is usually either the onset of some form of ocular paralysis, or one of the numerous derangements affecting the internal organs, of which I spoke in my previous lectures, such as an altered character of the micturition or genital functions, or perhaps the larnygeal or gastric crisis, &c., or lastly some difficulty in moving the lower extremities. This, it must be understood, is not true, confirmed ataxia, being an indication which an observant patient alone remarks. Certain movements are not performed with the necessary pre- cision ; thus, for example, they find it difficult to descend a staircase, and can no longer walk at night owing to the dark- ness, being also unable to indulge in certain exercises, such as riding. With these exceptions the gait in ordinary circum- stances presents nothing abnormal, and an experienced eye can alone recognize that a certain amount of hesitation exists at times when the patient is obliged to turn rapidly round. No considearable change occurs in the symptoms during a time which may be of some length. In some cases in fact the disease makes no further progress, it is to these latter cases that the name of mild tabes is given. Then at the end of 2, 3, 5 years or more the symptoms become progressively aggra- vated, or ataxy of the movements suddenly occurs ; this is the second period. Sometimes in the cases of so-called acute tabes, this second period may occur almost immediately after the onset of the affection, it is thus impossible to make any definite statement as regards the duration of the first period. In the second period the ataxy of movement is almost always confined at first to the lower limbs, and it is only at a later date as at the end of several years that it extends progressively to the upper extremities, which in fact does not invariably occur. It must be understood that numerous other tabid symptoms coincide with these during the period of motor ataxy, and the most different visceral symptoms as well as the ocular affections which we have already' considered may be specially observed at this time. In some cases this period brings" the patient by slow or rapid aggravation of the symptoms to the third stage, while in others it perpetuates itself, so to speak, and the patient remains in almost a stationary condition. The third period is constituted by the almost absolute loss of TABES DORSUALIS. 295 control over the movements of the 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 once understand to what' great dangers the patients suffering from tabes are exposed. There are 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 dif- ferent forms of tabes. Of these forms some are connected with the seat of the morbid process, others with its mode of develop- 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 so, is 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 account 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-myelia as examples of cervical tabes.. 296 LECTURES ON DISEASES OF THE SPINAL CORD. The tahes with cerebral form of some authors is that in which the symptoms connected with the cranial nerves predominate, without the existence of psychical derangement being much more frequent than in the other forms. All authors, however, do not admit that the special characters of this form are suf- ficiently decided to justify its separation. It must at the same time be acknowledged that the cases of tabes with atrophy of the optic nerve and persistence of the patellar tendon reflex have a very special character. The mild form of tabes (Charcot) is that in which the symptoms are not very pronounced, and either remain stationary or improve. In these patients the lightning pains are but slight, the loss of the knee-jerk, the inactivity of the iris-reflex, and the symptoms of Romberg are but little pronounced. These would be almost the only indications of tabes, and the symptoms far from increasing as time progresses, remain of almost pre- cisely the same severity during 8, 10 years, or more. It is evident that in these cases, which, however, are not very frequent, the morbid process has ceased to increase, and the patient may perhaps be said to recover ; as regards, however, recovery consisting in "restitutio in integrum," I must confess, gentle- men, that this seems to me impossible, on account of the destructive degeneration which has occurred in the spinal cord. Some authors have thought it right to connect certain symp- toms with the prognosis of the disease. M. Eemak contended that when the pain is very intense in tabes, " tabes dolorosa," the subsequent course of the disease is seldom very severe. This fact may be true in some cases, but cannot certainly be applied to every patient. Other authors, and of this I have already spoken, while discussing the ocular disorders which occur in tabes, assert that the patients suffering from tabes, in whom the symptoms of optic atrophy occur early in the disease, may be assured that the affection of the spinal cord will not be severe. This observation has some truth in it, but the theory which these authors deduce from it must be accepted with some reserve, as I have already explained to you. The severe forms of the disease are far from being rare, this being specially the case as they may be produced by morbid processes of different kinds. In some cases they are severe on account of the intensity of TABES DOKSUALIS. 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 oi acute tabes. Within some months pronounced 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 an 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 occur, 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 disease. Lastly, the severity of the disease may be due to the coinci- dence of general paralysis of the insane with tabes, of which you already know; the head and lower limbs then suffer simul- taneously, the unfortunate patient is entirely helpless, and death inevitably occurs. 298 LECTURE XXV. TABES. jETIOLOGY. Etiology. — Common causes : Wet and cold; Dlalheses; arthritic^ herpetic; sexual excess; ivjury. The true setiologioal element of tabes is .'j/j/mKs. The disooTery of this fact is due to Poumier (1876). Erb (1879) adopts and defends this opinion. Statistics published by different authors as to the percentage of tabid patients known to be affected by syphilis. Eecent statistics of Erb including 369 cases giving 89 per cent, of syphilis in the aetiology of tabes. Nine-tenths at least of tabid patients suffer therefore from syphilis. — Increased number of adversaries to this opinion. — Reputation of their arguments. — Influence of here- ditari/ pre-disposifion to nervous diseases shown by Charcot. Doubtful effect of hereditary syphilis. Age : greatest frequency of onset between 30 and 45 years. liace. Profession : most often occurs in the liberal professions ; its infrequency in the clerical profession. Gentlemen, — The knowledge of tabes from a clinical point of view, and of its pathological anatomy will not enable an exact idea to be formed as to its nature ; the study of the Etiology will alone enable this to be done. As you will see, I do not allude to the commonly given aetiology; far from it. There are certainly authors who have invoked, in connection with the getiology of tabes, a long list of possible causes; allow me, gentlemen, to pass rapidly over these and only to dwell upon the opinions which appear to me to be of some interest. I shall therefore say nothing more about the effect of wet and cold, the arthritic or even the herpetic diathesis. Sexual excess is generally believed to play an important role in the aetiology of the disease ; the reason of this is that tabes is ordinarily considered to be a " consumption of the spinal cord"; while again genital derangements are frequent in this affection, so that it is easily understood that popular malice has taken advantage of this fact to apply the aphorism " punish- ment affects the parts by which an error has been committed." In my opinion, gentlemen, the mistake and effects of sexual TABES DORSUALIS. ' 299 excess have been mucli exaggerated, as also of onanism, sexual connection in an upright position, or too frequent venereal congress. That such excess may have the effect of producing such pronounced organic lesions of the nervous system is a fact which I absolutely deny. The only concession vi^hich 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 a larger place is made for an impure than for an immodest Venus. The effect of injury has 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 the use of the sewing machine. This, 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 as 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 is 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 has 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 tabes 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. 300 LECTURES ON DISEASES OF THE SPINAL CORD. I should be more disposed to agree with the theory pro- pounded by Straus, according to which certain facts, without proving the absolute connection of cause and effect between injury and tabes, at the same time show that traumatic injury may exercise some influence on the seat of the first tabid symptoms (the lightning pains first occurring in the limb which has been injured). It is also possible that injury to the sacral region may in certain cases hasten the occurrence of impotence in this disease. For my part I have had the opportunity of seeing patients sufiering from tabes M'ho date the commencement of their disease from the time of a fall. One, for example, whilst hunting fell into a ditch, from that moment he had difficulty in walking, and ataxy of movement rapidly occurred. In another patifent the first symptoms of tabes occurred after a fracture of the leg. By careful inquiry I was soon enabled to ascertain that both these patients were previouslj- sufiering from tabes, and presented symptoms of the disease at the time when the accident occurred ; in the second, in fact, a tabid fracture had actually occurred. Be careful, therefore, gentlemen, not to consider as causes of the disease in the spinal cord fractures which are really its consequence. All these sstiological elements are therefore invalid, or may be neglected. The true, I would almost say the sole, cause of tabes is syphilis. This opinion as to the syphilitic nature of the disease is due to one of the most eminent masters of our faculty, Fournier, who, in 1876, commenced to teach this in his lectures, and since that time has written works, which you all know, in order to demonstrate its truth. In 1879 and 1881 Erb expressed himself in favour of this opinion, and the number of adherents who followed those authors is very large, so large in fact that I will not undertake to name them, contenting myself with mentioning to you, as the lectures continue, the authors of the statistics which contain the most information, since it is by means of statistics that we must advance in a question of this nature. The number of tabid patients in whom syphilis can be found to have previously occurred is, according to Fournier, from 91 to 98 per cent. According to Erb (first statistic) it is 88 per cent. ; according to Kumpf from 80 to 85 per cent. ; according TABES DORSUALIS. 301 to Althaus 90 per cent. By the side of these numbers I ought to mention, in fairness, those of Gowers and Seguia, 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 = 11% B. Cases in which syphilitic infection had occurred ... ^ 89% The latter included : — o. Cases in which symptons of secondary syphilis had existed ... = 63-3% 0. Cases in which the chancre had alone existed, no secondary syphilide having occurred =257% The 50 patients of the poorer class were composed as follows : — A. Cases in which it was impossible to find any indication of syphilitic infection = 24% B. Oases in which syphilitic infection had occurred ... = 76% The latter included : — a. Cases in which symptoms of secondary syphilis had existed = ^^°Jo /3. Cases in which the chancre had alone occurred, no syphilide having existed =^ 24% Thus, in the first 300 patients suffering from tabes the total 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. Erb, Zur ^tiologie der Tabes. Berliner hliniache Wochenschrift, 1891, No,. 29, p. 713. This work was published after the time at which the lecture was given. I thought it my duty to rearrange it in order to add these paragraphs, of which the importance will be evident to all. 302 LECTURES ON DISEASES OF THE SPINAL COED. SO large, being only 76 per cent. This difference, however, is another reason for believing in the truth of the numbers iirst given, since it shows that syphilis is more often found to exist in tabes in proportion as the patients are more observant, and their intelligence greater. It is doubtful whether the total of 89 per cent, indicates the exact proportion of those affected, since as you know, gentlemen, or soon will know, that persons who are very intelligent may certainly in their first youth have a chancre which is quite unperceived, or merely regarded as an insignificant abrasion, the existence of which is quite forgotten. Thus, everything being considered, the percentage of those suffering from syphilis in tabes would be considerably higher, and it is impossible to say at what number the limit would be reached. Is the fact then definitely established that niThe-tentJis of those affected by tabes have previously suffered from syphilis ? Some authors have looked upon this as a simple . coincidence, while others by many reasons, or rather much reasoning, have sought to prove that tabes could not be due to syphilis. Their mode of arguing is usually as follows : — 1. At the autopsy of tabid patients the lesions in the spinal cord are not fouiid to resemble gummata in the slightest degree,' nor are they in any way analogous to the ordinary specific lesions, no such lesion being again discovered, in the other viscera. To this it may be answered that many syphilitic affections of the skin or viscera exist which are also quite unaccompanied by lesions having a specific character. 2. The failure of treatment by mercury and mercurial preparations is an argument against the syphilitic nature of tabes. The- fact, which is perfectly true, does not however disprove our statement. -In the first, place writers on syphilis are agreed as to the point that certain syphilitic symptoms exist against which treatment by mercury and potassic iodine is almost inactive. Again it seems scarcely possible that it could have much effect when the lesion consists in complete destruction of certain fibres of the spinal cord. Would one, for instance, expect cerebral softening which is due by syphilitic arteritis to be cured by the influence of specific treatment ? Why then should one suppose the spinal cord to act differently from the TABES DOESUALIS. 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, so to speak, is a common element, and cannot be invoked as a cause of the disease more than any other chronic disease 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 unaffected hy 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. Noegeli- in 1,450 patients arrives at an even higher per- centage. ■ 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 his hands with respect to the existence of syphilis, taking care' to eliminate those suffering from tabes, and all the patients who came 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 : 22'5 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 these two numbers : 22-5 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 ai'guments is not really very difficult. 304 LECTURES ON .DISEASES OF THE SPINAL CORD. It would be an exaggeration to maintain, in the present state of our knowledge, that syphilis is the only cause of tabes, since Fig. 160. Fig. 161. Fig. 162. Spinal cord of a patient in whom degeneration of the posterior columns had occurred after intoxication by ergot of rye (according to Tuczek). The most severe lesions are designated by a black mark, those which were less pro- nounced being indicated by a grey tint. Fig. 158. — Section of the lower part of the medulla oblongata ; the lesion is fonnd upon both sides at A. Fig. 159. — Section of the spinal cord at the level of the decussation of the pyramid. Fig. 160. — Section of the spinal cord at the level of the second cervical nerve-root. Fig. 161. — Section of the spinal cord at the level of the 8th cervical nerve- root. Fig. 162. — Section of the spinal cord at the level of the 11th dorsal nerve-root. The lumbar and sacral regions present alterations of the same kind. Tuczek has shown that intoxication by ergot of ryg- may produce lesions which are quite analogous to those of tabes. Symptoms of the same kind may occur in those affected by pellagra. It must therefore be admitted that from a theoretical point of view tabes may be due to certain special forms of infection or intoxication. Practically, however, you may feel sure of one thing, namely, that in ordinary practice tahes invariably has a syphilitic origin. In addition to syphilis a very important place should be given to hereditary predis2'>osition to nervous affections, to that singular condition of the organism which is transmitted from generation to generation, and owing to which the nervous system is specially vulnerable. As regards this tendency Charcot has truly observed that in a large number of tabid patients this hereditary tendency is very pronounced. It may also be observed, gentlemen, that the heredity in these cases need not be direct, that is to say, TABES DORSUALIS. 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 aetiology 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 future 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 (???). Leaving, gentlemen, the question of the causes of tabes, which are now I hope understood by you as far as the great majority of cases is concerned, the other aetiological 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 (??) ; it is rare also after the age of 55 years. In 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. Brb has given a statistical table of 300 patients in connection with this point, and arrived at the following results — The onset of tabes occurred — 1 to 5 years after the syphilitic infection in 12'3 per cent, of the cases. 9 to 10 „ „ „ 37 11 to 15 16 to 20 21 to 25 26 to 30 31 to 35 247 14-2 4-8 1-9 07 20 306 LECTURES ON DISEASES OF THE SPINAL CORD. The onset of tabes occurs therefore most often from 6 to 15 years after the syphilitic infection and it may be said in a general way, as Erb observes, that tabes may occur during the 20 years which follow the existence of syphilis (88 per cent, of the cases). This knowledge, or the length of time which passes between the infection and the onset of the afiection in the spinal cord, will enable you, gentlemen, to understand why it is that tabes specially occurs between the ages of 30 and 45 years. It is, in fact, between the ages of 20 and 30 years that syphilis is usually contracted; by adding to this period 6 to 15 years, which may be called the time of the iTicubation of tabes, the age of from 30 to 45 years is reached, during which clinical experience shows that tabes most often occurs. When again the infection has taken place at some other age, tabes is also found to appear at a correspondingly altered period; thus one of Brb's patients who had contracted syphilis at the age of 19^ years suifered from tabes at the age of 22, whilst in two others who only suffered from syphilis when mature age was approached, tabes occurred at a later age ; in the one syphilis took place at 57, tabes at 66 years ; in the other syphilis at 54, tabes at 59 years. It will be interesting to consider the relation which exists between the form of syphilis and the onset of tabes. According to the information furnished by most of the patients it is the mild forms of syphilis which are most often followed by tabes. The chancre had not been severe and had healed in the normal length of time, secondary symptoms were entirely absent or but little pronounced, while tertiary symptoms have scarcely ever occurred ; it is very rare that patients suffering from tabes present any evidence of a severe attack of syphilis. The mild character oi the syphilitic symptoms explains, gentlemen, the hesitation with which some of the medical profession have recognized the connections which unite this disease to tabes, and the opposition which some authors still manifest with regard to this idea. With respect to sex an important fact must be mentioned, namely that tabes is far more frequent in males than in females ; so great is the difference, in fact, that in a number of cases collected since 1883 by Erb 350 males and only 19 females are found to be affected. Must this infrequency of tabes in the TABES DOESUALIS. 307 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, plays the same part in the astiology 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 ? It 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 Eussia where the Jews are somewhat numerous, 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 tetiology of tabes. 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 unequg,lly from loco- 308 LECTURES ON DISEASES OF THE SPINAL CORD. motor ataxy. If the relative number of persons in society belonging to the diiferent professions is considered it is found that tabes is most frequent in officers and those belonging to the liberal professions : artists (those connected with the theatres, musicians, painters), clerks, journalists, &c. What then explains the greater frequency of tabes in these professions ? Is it excessive use of the brain ? Perhaps, — but is it not more probably inconsiderate and indiscreet activity of the feelings ? I can bear witness to the great frequency of syphilis in these professions ! An exception to the rule which exists with regard to the liberal professions should however be made, which is of some importance, namely that connected with the priests of the different religions. Thus in the statistics of Erb but one person belonging to the church is found, while there are at least 60 officers, and 26 in the medical profession. You will certainly divine, gentlemen, the reason of this immunitj' ; it is that syphilis, it must be allowed, is less frequent in priests than in other classes of society. I should add that the only priest included in the statistics of Brb suifered also from syphilis ; in this case again, as in all the facts which I have put before you in regard to the connection between tabes and syphilis, the exception confirms the rule. It is really, gentlemen, this concord in all the writings about the disease which has induced me, after mature deliberation, as you may be sure, to believe in the syphilitic origin of tabes. 309 LECTURE XXVI. TABES. DIAGNOSIS. Diagnosis : Difficulty in making a complete and methodical diagnosis. Differ- ential character as regards : Cerebellar affections, Insular sclerosis, Astasia- abasia, Syringo-wyelia; as regards Pseudo-tabes: A. Toxic Pseudo-tabes, alcohol, arsenic; "B. Diabetic pseudo-tabes ; C Neurasthenic psevdo-tabes. Therapeutics: A. Internal remedies ; nitrate of silver, strychnine, aconitine, atropine, ergot of rye ; anti-sjrphilitio agents : mercury, iodides ; B. External remedies : counter irritation applied to the skin, electricity, massage, nerre-atretohing, suspension, hydrotherapy ; general summary of the treatment adopted. The DIAGNOSIS 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 diiferent disorders, either connected with the limbs and skin or with the diiferent organs, may open the scene, and owing to their intensity occupy 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 genito-urinary derangements of these patients often cause mistakes to be made, and lead to an organic afiection 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, so often termed rheumatoid, as also of the ocular or auricular troubles. I 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 310 LECTURES ON DISEASES OF THE SPINAL CORD. voj lectures ; in these cases it will be better to discuss the diagnosis when the diseases themselves are specially considered. We will therefore only regard tabes at the present time under the aspect which it most usually presents, and compare it with certain diseases which present more or less exactly the same appearance. The cerebellar affections have at times a very similar appear- ance ; that is to say the patients who suffer from them present the same disorders in connection with the gait and upright position as well as various ocular troubles. A more careful examination, however, quickly reveals the differences which exist ; there is no true inco-ordination but chiefly staggering ; the lightning pains are nearly always absent, being often re- placed by headache ; there are no sensory disorders, the knee- jerk has not ceased to exist, and as regards the lesions in the fundus oculi these consist specially in the optic neuritis and not in optic atrophy. Insular sclerosis may, as we have already seen, assume some of the characters of tabes. But in this disease also the changes in the gait resemble those which occur in cerebellar, or cerebellar spastic disease, and are not purely ataxic ; the knee-jerk is usually retained or perhaps excessive, the sensory disorders or pains are but slightly pronounced; in addition to this numerous other symptoms exist (the tremor, nystagmus, scanning utterance, &c.) which are not observed in Duchenne's disease. The disorders of the gait in tabes might, if the physician is not on his guard, be confused with those which were described by Charcot and Richer, and by P. Blocq under the name of Astasia-abasia, which consists in a loss of the memory as regards the movements necessary in walking. These cases, however, are distinguished by the fact that the knee-jerk is retained, and that the pains and other symptoms of tabes are absent. Again, whenever the patient is found to be seated or lying down the movements of the lower limbs are unaffected ; and not only does he know exactly in what position these are placed, but can also direct them accurately towards a point indicated to him — -in a word there is no inco-ordination, no true ataxy. The other modes of progression again are completely unaffected ; thus as Charcot showed, the patient is quite able TABES DORSUALIS. 311 to advance either bj'' 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 seat 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 syringo-myelia 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 between syringo-myelia and tabes being usually un- attended by diflSculty. The same is not the case with regard to a group of affections to which, owing to their resemblance to tabes, the name of Pseudo-tabes has been given. These pseudotabid affections, depending upon morbid conditions which are very 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 pseudo-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 alcoholic pseudo-tabes. In these cases, gentlemen, there is often most severe pain, very obvious disorder of the gait, and considerable diminution or loss 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 distinguished from the real disease ? A careful analysis of the symptoms will enable this to be done. 312 LECTURES ON DISEASES OF THE SPINAL COKD. The fact is that the uncertain gait in these cases of pseudo- tabes is much less due to inco-ordination of movement than to a certain degree of paralysis or paresis which exists in the muscles of the leg and foot ; these patients do not " mow " with the feet but " step," that is to say, on account of the paresis in the extensor muscles of the foot, they are obliged, in order to lift it from the ground and carry it forward, to raise the knee much higher than a healthy person would do, and hence the special mode of their progress which exists. As regards the pains, they have not precisely the same characters as the lightning pains of tabes : they are rather lancinating and cramp-like, beirig specially seated in the muscles, and increased by pressure ; lastly, they are more persistent, and less fugitive than the lightning pains, and occur rarely as these do in paroxysmal attacks. The visual disorders also differ from those which occur in tabes, usually consisting at any rate in cases of alcoholism in more or less pronounced central dyschromatopsia, whilst with the ophthalmoscope atrophic discoloration is found limited to the temporal side of the optic disc. B. Diabetic pseudo-tabes.— In this affection the gait is heavy, painful, undecided, much more often than ataxic ; sometimes sensory disorders and powerlessness exist, often pain, which may be very severe, and lastly, loss of the knee-jerk, as Bouchard has observed, and of which he has shown the importance as regards the prognosis of the disease. I have just said, gentlemen, that the disorders of the gait when they exist rarely present an atoxic character ; as regards the pains they are usually described by the diabetic patients as neuralgia (Worms, Dreyfous, &c.), and especially bilateral neuralgia. I will not dwell upon these points since the examination of the urine will remove every doubt. It must not be forgotten, however, gentlemen, that, as I have had occasion to observe before in the proper place, glycosuria sometimes, but rarely, occurs in the course of tabes. Until the present time, with the exception of alcoholic pseudo- tabes, the forms mentioned are but seldom met with in practise: the last group of pseudo-tabes, of which 1 have still to speak, is far more important both on account of the complete way in which it resembles tabes and of the frequency with which it TABES DORSUALIS. 313 occurs. I am referring, gentlemen, to an affection with wliich j'^ou 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, Neuras- thenia. In order to give you an idea of neurasthenic pseudo-tabes I 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 feel, 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 pseudo-tabes for the 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 purely 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. (b) The weakness of the genital functions, even when im- potence exists, is not associated with loss of the cremasteric 314 LECTURES ON DISEASES OF THE SPINAL COKD. reflex ; I would ask you, gentlemen, to bear this remark specially in mind, as it may be of real service to you. (c) The loss of the knee-jerk is not so absolute as it appears to be, that is to say if the precautions which I mentioned to you, when the reflexes were considered, are adopted. Thus, for instance, slight contraction of the quadriceps may be felt to occur by the left hand placed over that muscle at the moment when the blow is given to the tendon ; or better still, if the patient is directed to exert the muscles in some way at the same time as the tendon is struck, according to the plan suggested by Jendrassik, the knee-jerk will be felt to be clearly produced. In a word, the knee-jerk is not absent, but only so much diminished that it cannot be produced in the ordinary way, though more delicate means of observation at once reveal its existence. (d) Other neurasthenic symptoms exist, which you well know, and upon which I need not now dwell (headache of a special character, gastric derangements, psychical changes, &c.) . (e) Nothing is found in the pupil which recalls what is termed the symptom of Argyll-Robertson. In a few words, gentlemen, it is not very difiBcult, as you perceive, to distinguish tabes from the affections which resemble it ; the essential condition, that any error may be avoided, is to know that a mistake may be made. An account of the methods of treatment which have been employed against this disease must now be rapidly given in order to complete the study of tabes. Therapeutics have unfortunately in most cases but a very theoretical value; the greater reason, gentlemen, for your knowing all the means which may be employed against this disease. A. Internal remedies. — From a historical point of view two phases may be distinguished in connection with these remedies : In the first those medicaments were almost indifferently employed which for some reason were believed to act specially upon the nervous centres. Among these the place of honour may be given to nitrate of silver ; this salt was usually given to the amount of from 1 to 5 centigrams (gr. | to gr. f) or more daily. Some patients took this medicine for months or years ; I insist upon this point as you may meet with cases of the kind, TABES DORSUALIS. 315 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. 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. 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 hj ergot of rye, and it is when they exist that this drug should be specially used, though at times it has also a favourable effect upon some of the other symptoms which occur in tabes. In the employment of this remedy, however, certain pre- 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 during a short period of time ; thus, for example, 2 or 3 doses of 30 centigrams (4J grs.) of the powder of ergot of rye should be given upon each of the first 3 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 Eetiology 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 having declared myself as I have done, an absolute believer in the syphilitic nature of tabes ; but at the same time it m ust be 316 LECTURES ON DISEASES OF THE SPINAL CORD. candidly acknowledged that this treatment has apparently no beneficial effect upon the symptoms of tabes. On the other hand, if it does no good, it is only right to say that it may do harm, specially to the patients who are cachectic. At the same time when a patient'who is under my care seems able to support the anti-syphilitic treatment, I do not hesitate to employ it ; nor should this practice be considered inconsistent or illogical. This form of treatment is not adopted as a cure for the actual symptoms of tabes, as I know but too well that it would be powerless in this respect. I only prescribe these remedies in the hope of making my patients secure from other lesions of a syphilitic nature, which at times are severe complica- tions in tabes, as for example chronic arteritis, which is often followed by cerebral hemorrhage or general paralysis of the insane, the result of syphilitic affection of the meninges or brain. I also adopt the anti-syphilitic treatment in hopes that the progress of tabes will be thus arrested. I dare not maintain that this is not an illusion, but it seems to me that during the last few years the patients in whom the symptoms are severe and pronounced, those suffering from "tabes major," as it is termed, have become less numerous ; if this is true, would it not be due to the fact that in many cases the administration of specific treatment has been able to arrest the course of the disease ; and it has in these cases remained stationary instead of being pitilessly progressive.* After this profession of faith in these remedies, I will dwell no longer upon them, leaving you free, gentlemen, to adopt whichever mode of treatment seems the best. If you decide to use the specific remedies, I should recommend a mixed form of treatment, mercury being employed by means of friction ; as regards iodides, you should prescribe them at the dose of at least 30 or 45 grains (2 or 3 grammes). It must be remembered, however, that in certain cases this form of treatment cannot be borne, and bad results occur, in which case it must be at once abandoned. This must be done, gentlemen, immediately and without hesitation, since, as I at first observed, there are some * I am aware that in certain oases a severe attack of tabes has been seen (Pournier, Charcot) to occur in patients in whom anti-syphilitic treatment had been adopted and continued in a satisfactory way, from the time when the chancre existed ; are not these, however, exceptions p TABES DORSUALIS. 317 patients suffering from tabes in whom the specific treatment, far from being useful or inactive, is absolutely injurious. B. External remedies comprise a large number of agents, among which I should mention the following : — ■ (a) Counter irritation through the sltin. — Setons, the cautery 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 laminfe of the vertebra ; they should be numerous, very superficially placed, and re-applied about once a week. (b) Electrlciti/ will consist either in the form of the faradic current applied to the sldn, 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 be, as Erb recommends, galvanism of the peripheral nerves. (c) Massage and electro-massage may produce some eifect, 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 trunk of the sciatic nerve was reached ; more or less violent efferent traction (stretching) was then exercised upon this nerve. This plan, if I am not mis- taken, had been previously employed by Niissbaum in a patient suffering from obstinate neuralgia (and who in short was hysteri- cal, and hence the brilliant success). Langenbuch applied the same 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, hffimato-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 Progres M§dical in 1889 and 1890. I need merely remind you that suspension 318 LECTURES ON DISEASES OF THE SPINAL COED. has a special effect upon certain symptoms, such as the lightning pains, the inco-ordinatlon, and the genito-urinary disorders. Nor must it be forgotton that different contra-indications may exist to its employment in tabes, as in patients in whom either cardio-vascular affections, or atheroma, or enphysema, or advanced pulmonary phthisis, or, lastly, obesity, or even simply a very bad state of the teeth exists. (/) Hydrotherapy. — Every process has been recommended in the treatment of tabes, and it must be acknowledged that some have proved effectual as regards one or other symptom, hot or cold douche, hot or cold baths, and even baths of steam or electrical baths ; residence at different thermal stations is also recommended by many authors. Amongst these I should mention Balaruc, Neris, Plombifires, Uriage, and specially Lamalou which seems to be specially efficient in the treatment of tabes. To sum up, gentlemen, and to guide you in the right direction through the therapeutic labyrinth, how should one usually treat a case of tabes ? On the one hand, as you have seen, the most different symp- toms exist ; on the other, a nervous affection essentially due to syphilis. Since one is as powerless as we now are with regard to tabes itself, it would be ridiculous and culpable to treat the cause alone. Eemedies must be prescribed carefully in connection with the symptoms of the disease, the indications which I have just mentioned being borne in mind. For the inco-ordination and genital disorders you will specially recommend suspension. When urinary derangements exist you will give ergot of rye, the precautions being taken upon which I have, I think, sufficiently dwelt. The lightning pains, which in too many cases cause the patients to be true martyrs, will be opposed by all the anodynes which therapeutics have or do put into our hands : antipyrin, antifebrin, exalgin, phenacetin, salicylate of soda, aconitine, &c. Recourse will also be had to the different narcotics : hydrate of chloral, cannabine, hyosciamine, sulphonal, &c., and the prepara- tions of opium, codeine, narceine, laudanum, thebaic extract. In connection with these remedies I must mention suhcutaneous injections of morphine. Ought they to be practised or not in TABES DOESUALIS. 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, &c. As regards the different ■wisceraZ crises, they should be treated, as I have already said, by injections of morphine or other nar- cotics, 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 iis to the necessity of anti-syphilitic 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 I have 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 be used, and a go-cart, used for several years at the Hospital of Bicetre in order to enable 320 LECTURES ON DISEASES OF THE SPINAL CORD. patients suffering from ataxy to walk, even in the advanced disease, may be of service in this respect. It must be well understood that the exercise of the muscles which I recommend should never be carried so far as to produce fatigue. Fig. 163. — Go-cart, used at the Hospital of Bic6tre for many years to enable patients suffering from tabes to walk. It forms, as it were, a movable bar, which surrounds the patient on all sides and, moving with him, constitutes a basis of support which is always close at band. A bench placed at one of the extremities enables the patient to rest whenever he wishes to do so. (From a stereotype of Damaschino.) Lastly, gentlemen, I would remind you of the suggestion* which, whatever may have been said to the contrary, has never cured any organic affection of the spinal cord, but should on no account be forgotten, and on the other hand, stated in the clearest and most agreeable manner, the expression, I mean, of such an opinion as will calm the fears of the patient, and even in the most severe case will still cause the patient not to lose hope. • It must be well understood that no reference is made here to hypnotic practices but only to the moral action of the physician. 321 LECTURE XXVII. TABES (continued). PATHOLOGICAL ANATOMY. Anatomy of the parts in a normal condition. A. Anatomy of the POSTEEIOK COLUMN. Description of the three kinds of fibres : long, of medium length, short, (a) Posterior column properly so called. Researches of Plechaig upon the order in which the zones composing it are developed. Origin and termination of the fibres which constitute these different zones, (b) Zone of Lissauer : its seat, limits, extension ; its division into two segments external and internal ; the fibres which constitute it ; their origin ; development of this zone. B. Anatomy of the postebiok hoen. (o) Posterior horn properly so called : apex ; gelatinous substance of Eolando, its division into (a) the spongy zone of the gelatinous substance, and (fi) the typical gelatinous sub- stance of Eolando. Spongy substance with its two zones, the anterior sind the posterior. C. Anatomy of the column of Clabee. Its seat, limits, and extension. Within thia tract are found: (o) nerve cells; (6) a network of nerve fibres. Gentlemen, — We have now to commence a study which is extremely difficult, full of obscurity and uncertain — that of the Pathological Anatomy of Tabes. Fortunate indeed are those 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 included 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 columns 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 21 322 LECTURES ON DISEASES OF THE SPINAL CORD. secondary degenerations. I sliowed you that for the most part it was constituted by the conjunction of the fibres of the posterior roots. You will remember, gentlemen, that amongst; these fibres we agreed with Singer and Miinzer in distinguishing three groups which vary in the length of their course. One of the groups passes almost immediately into the extremity of the posterior cornit. the intermediate group into the column of Burdcwh, while the third group composed of the longest fibres joins the column of Goll. The knowledge of these facts is sufficient when it is a question of degeneration produced by a coarse lesion of the root-fibres (division of the roots, or transverse mj'elitis, compression of the cord). When, however, the disease is distinctly localised, as is the case in tabes, the principal tracts which constitute the posterior column must no longer be considered together, but the difierent groups of fibres which compose it must be taken into account. Until the last few years the only parts of the posterior column which were clearly distinguished were the columns of Goll, the columns of Burdach, and the external hand- lets described by Charcot and Pierret. Recent works of which the principal are due to Flechsig and Lissauer have much extended our knowledge in this respect. These works are specially' interesting to us inasmuch as the knowledge which they contribute as regards the normal anatomy of the spinal cord has often a special connection with the seat of the initial lesions in tabes ; I should therefore be glad to convey this knowledge of the normal anatomy of the part to you before discussing these lesions. (a.) Posterior column properly so called. — It is to Flechsig, as I have just said, that we owe the increased knowledge which now exists in connection with this part. The researches of this author were made according to the same |)lan as that which gave such remarkable results as regards the other white tracts of the cord being based upon the distinct development of the different groups of fibres in the foetus. In the embryos of from 28 to 35 centimetres (11 to 14 inches) in length, Flechsig found that in the cords of which the myelin was coloured by any means whatever the posterior columns contained zones of different hues. The parts which were least coloured, and in which the myelin was consequently least TABES DORSUALIS. 323 developed, are those marked by the letters A, D, and G, in figure 164 ; on the contrary, in the other parts the colour was much more marked, and consequently the myelin in far larger quantity . In G, the fibres of the column of Goll, as you already know, will be developed, and which as you are aware, after a very long course along the pDsterior fissure, join the grey substance of the nuclei called " those of the column of Goll." The lettters A, D, represent the postero-internal root zone ; you will observe, gentlemen, that in the lumbar 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 GoU's column j ' according to Flechsig, this is by no means the case, the segment being in no way connected with the fibres of that column. 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 zone of the posterior columns. We have elsewhere had occasion 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 cornu-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 fibres of the first system of jthe middle root-zone develop cotemporaneously with those of the middle zone ; while the fibres of the second system of the middle ropi- zone develop cotemporaneously with those of the column of Goll. At the inner part of the posterior column, immediately ndjoining the posterior fissure, is the middle zone F, 0, which 324 LECTURES ON DISEASES OF THE SPINAL COED. Flechsig believes to be quite distinct from the columns of GoU. In the cervical region and in the upper two thirds of the dorsal region of the spinal cord, it is seated between the columns of Pig. 164. — Scheme of the different zones in which the posterior columns of the spinal cord are developed. (After the description of Flechsig.) Upon the left of the line XT a section of the cord in the cervical region is represented in the figure; upon the right a section of the cord in the lumbar region; AD, postero-intemal root-zone; it will be observed that in the spinal cord of the lumbar region, this zone A hag the form of a gourd of which the smallest segment might (wrongly according to Flechsig) be considered as connected with the column of GoU. G, Column of Goll. B,E, Middle root-zont containing two kinds of fibres : + + + +=fibres of t\i6 first system of the middle root zone ••••^fibres of the second system of the middle root-zone. The part of the ■ posterior column seated in front of and externally to BE and adjoining the posterior cornu (not represented by a number in the figure) is the anterior root-zone. C,F, middle zone. The column of Lissauer is not marked in the figure. Goll and anteriorly to them, whereas telcw this point it is distinguished with greater difficulty from the middle root-zone. • 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 columps of Goll come tiext in order with the second system of the middle root-zone, as also "yvith the postero-internal root-zone. Such is the anatomy of these different tracts. Howeve;i' interesting the study may be, it would not be complete without inquiry being made as to the origin and termination of . the fibres hj which the tracts are constituted. In regard to these points the opinion of Flechsig will be given. The cmterior root-xone receives a large number of its fibres directly 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 first system of the middle root-none 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 rpot-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 as- a description such as the one which I have just given you would indicate. (6) Zone of Lissauer. — Marginal zone (Lissauer), postero-ex- i32G LECTUEES ON DISEASES OF THE SPINAL CORD. ternal rooi-zom (Flechsig).^ — A group of fibres is contained. in this zone which essentially belongs, in reality, to the posterior column, but as they encroach upon the lateral column, specially as they have been unrecognized until the last few years, and their description does not seem to have made its way into the inanuals which are placed in your hands, it seems to me a good plan to separate to some extent the studj' of these fibres from that of the rest of the posterior column. As the name which I have just used for this tract indicates, it is to Lissauer, a pupil of Weigert,' that the honour must be given of having first mentioned the existence of this zone, and of having described- its structure. This zone is seated in the interval which separates the exterior angle . of . the posterior Column from the internal and posterior angle of the lateral column. Thus it occupies the part through which the posterior roots pass in joining the cord and passing into the posterior cornu. Such are its limits in the transverse sense (as con- sidered in a transverse section of the cord). In the antero-pos- terior direction this zone is limited in front by the spongy zone of the gelatinous substance which covers the extremity of the posterior cornu ; posteriorly, it extends as far as the surface of the cord, which it just reaches (hence the name of marginal f.one giveti by Lissauer). The fibres of the posterior roots in entering the cord pass through the zone of Lissauer, which they divide into two un- equal parts. One of these is on the outer side of the fibres of the posterior roots, the other being internal to them ; the latter segment is the smallest in size, being, in fact, at times scarcely recognizable. The external segment of the ;.one of Lissauer, which, as I -have just said, is the largest in size, and which has a trapezoid form, extends along the outer edge' of the posterior cornu and en- croaches more or less upon the postero-interal angle of the lateral column, which it presses back to the same extent. ■Although essentially belonging to the posterior column, this segment appears at first sight to be an integral part of the lateral columii. I shall have, while discussing the pathological anatomy, to return to the inistaken interpretation to which this singular appearance has been able to give rise. The inner segmmit of the zone of Lissauer; ot which the size TABES 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. — Sclieme of the different tracts and fibres of which the posterior cornu is composed (lumbar region), after Lisaaner. A. — ^Anterior zone of the spongy substance. B. — Posterior zone of the spongy substance. C. — Fasciculi of large fibres ascending in columns. D. — Gelatinous substance. E.— Fibres insinuating themselves between the spongy zone of the gelatinous substance and the fibres of the lateral column. F. — Spongy zone of the gelatinous substance. G. — Fibres of the lateral column adjoining the external margin of the posterior cornu. H. — External marginal zone (txtesmud segment of the zone of Lissauer). I. — Small root-fibres passing into the zone of Lissauer. K. — Pos- terior root-fibres. L. — Internal marginal zone {internal segment rf the zone nf Lissauer. M. — Fibres of the posterior column. N. — Fasciculi of transverse fibres becoming mixed with the posterior root-fibres at the moment of their penetration into the anterior cornu. O. — Large fibres joined in fasciculi, with a direct passage into the gelatinous substance. P. — Radiating fibres of . the posterior column. 328 LECTURES ON DISEASES OF THE SPINAL COED. posterior roots and the vertical fibres of the postero-external angle of the posterior column. The zone of Lissauer is found to be composed almost exclus- ively of small nerve-fiires, of much less size than those of the posterior roots or of the lateral or posterior columns ; these small fibres are massed together in the zone in a larger number than is found to be the case in any other part of the white columns of the cord. You must now however suppose, gentlemen, that the part which is named zone of Lissauer contains only small fibres ; some large fibres coming either from the posterior or lateral column may be scattered therein. Not only do the small fibres which are special to this part insinuate themselves between the fasciculi of large fibres coming from the posterior roots, but they are also found in the intervals which separate the large vertical fibres of the posterior column immediately adjoining the apices of the posterior cornua from each other. What is the origin of the small fibres which constitute the zone of Lissauer ? These fibres come from the posterior roots and are merely, according to Lissauer, the continuation of the small fibres seated in the posterior roots in which they are known to be far more abundant than in the anterior roots. (According to some authors these 'small fibres are specially formed by the collaterals of the posterior roots, the knowledge of which we owe to the works of Golgi, Kamon y Cajal, KoUiker, von Lenhossek, &c.) However this may be, small fibres separate from the posterior roots at the point of their entrance into the cord, and pass almost horizontally towards the zone of Lissauer, being principally directed towards its external segment; they then curve upwards and pass to a certain height in the zone of Lissauer, from which they enter the gelatinous substance. Besides this group another portion of the small fibres of the posterior roots forms a second group which accompanies the large fibres of these roots, and passes with them either into the gelatinous substance of the posterior cornu, or into the interior of that cornu. In short, these small fibres, as you see, have but a short course in the zone of Lissauer which they soon leave • this explains how it is that notwithstanding the fact that fibres are incessantly brought to it by each posterior root, this zone does not increase in size from below upwards : on the contrary TABES DOESUALIS. 329 it is in tlie 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 description, gentlemen, jf on will be able to under- stand how it is that the small fibres which constitute the zone of Lissaiier, 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 qtiite at the end of foetal life. B. Anatomy of the Posterior Horn. — Under this name we will study the posterior horn, properly so-called, and the columns of Clarice, these two parts of the grey matter in the cord being apt to present changes in the course of tabes. (a) Posterior horn properhj 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 subpiamaterian layer, which in fact occupies the whole surface of the cord (vide the 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 stratum zonale, of which the tissue is analogous to that of the subpiamaterian layer 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. 330 LECTUKES ON .DISEASES OF THE SPINAL CORD. As regards the gelatinous substance of Rolando, Lissauer divides it into two zones, which, however, are not separated from each other by anj- precise limit. One of these zones is seated posteriorly, viz., the spongy zone of the gelatinous sub- stance ; the other which is in front of this is the typical gelatinous substance of Rolando. a. In the spongt/ zone of the gelatinous substance the root- fibres are more closely pressed together, and irregularly placed than in the rest of the gelatinous substance-; it often extends to a slight distance along the inner margin of the posterior cornu. This zone is constituted by an admixture of fibres which are isolated from each other, and disposed in every direction : thVee principal origins may, according .to Lissauer, be discovered for these fibres : 1. some fibres coming from the marginal zone of Lissauer ; 2. some directly from the posterior Joots ; 3. some from the posterior column. /3. The typical -(Jelatinous substance of Rolando, vs'hich, as I have just said, is placed immediately in front of the preceding zone, contains large fibres coming directly from the posterior roots, and narrow fibres coming from the spongy zone of the gelatinous substance. In this substance a number of cells are found which have been recently described and studied by Gierke and H. Virchow. Under a magnifying glass of moderate power the cells have the appearance of large spherical nuclei within round and clear spaces, since their protoplasm, which is almost colourless, can Scarcely be recognized at first, though with a more highly magnifying power the protoplasm and its fine pro- longations can be distinguished. The nucleolus of these cells is small, but the authors who have described it believe that it is constantly present. Owing, to all these characters Gierke, H. Virchow, Waldeyer, believe these cells to differ from all other cells which exist with the- exception of nerve-cells, and regard them as true ganglion nerve-cells. I would also add that contrary to the opinion expressed by some authors, the gelatinous sub- stance of Ilolando is, according to Weigert, the part of the spinal cord in which the neuroglia is least abundant. If we still consider the parts from behind forwards we then meet with a new layer which, while surrounded to a great extent by the gelatinous substance of Rolando, is found to constitute the special substance of the posterior cornu. TABES DORSUALIS. 331 This layer is the spongy substance properly so-called in which Lissauer also distinguishes two zones : a. Posterior zone ; 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 fprm a small number of fasciculi, seeming to be the direct continuation of the posterior roots. /3. Anterior zone ; 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 large 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. C. Anatomy of the column of Claejce. — These columns, notwithstanding some claims as to their having been first described in Gernlanj^ (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 themselves, 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 a 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 upjJer 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 332 LECTURES ON DISEASES OF THE SPINAL COED. by scattered cells ; these cells also completely disappear in tlie cervical enlargement,^ reappearing in the upper cervical region. Masses of cells are again found, according to Tooth, in the medulla oblongata, within the nuclei connected with the columns of Goll and of Burdach, resembling those of the column of Clarke. Waldeyer also thinks that the columns of Clarke do not exist in the dorsal region of the cord alone, but throughout the whole length of the cord without exception ; but th&t in other ' parts than the dorsal region it is not a large mass or r.eal column that is found, but simply a group of two, three, or four isolated cells which so strongly resemble those of the columns of Clarke in their form and seat that they must be considered of the same nature. In these columns we must, while considering how the patho- logical anatomy can be applied, specially consider two elements : (a) the cells, (6) 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 the description 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 a trophic centre, having the same office perhaps in regard to some fibres of the column of Gowers. (6) Reticulum of the columns of Clarke. — The knowledge of this reticulum is as you will se^, gentlemen, very important in the study of the lesions which occur in tabes. In the normal cord coloured by the hsematoxylin of Weigert the general tint of the section through the columns of Clarke is almost analogous (an exception being made of the brownish-yellow points Repre- senting the nerve-cells) to that of the remaining grey substance TABES DOESUALIS. 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. 334 LECTURE XXVIII. TABES (continued). TATHOLOGICAL ANATOMY. History : Hutin, Monod, Crnveilhier, Rokitansky, Tii.-ok, Eomberg', Charcot and Pierret, &c. A — Spinal Coed. Macroscopic appearance. Appearance under the microscope. — (n) At the onset : Posterior column — a. Exlervnl bandlets, their seat, their alterations ; (3. Column of Goll; y. Jlett of the cnlumu of Burdach; S. Zone if Lisanver. (b) At an ADVANCED STAGE: 1. Posterior column — lesions of the external bindttts, the column of Goll, the Test, if tkn column of Burdach. 2. Grey substance— "i. Anterior horn; p. Column of Clarice ; y. Posterior horn; S. Central canal. B. — Posterior Roots, their lesions. C— Spinal Ganglia, their lesions. D. — Peripheral Nerves, their lesions. E.— Brain : disappearance of the nerve fibres of the convolutions. Gentlemen, — We are now, I tbink, in a position to discuss the seat of the lesions which occur in tabes, or at any rate to form an approximative idea of where they exist. I will com- mence by enumerating the different changes which may be observed in tabes, and we will then endeavour to bring these lesions together as regards each other, and thus to discover the nature of the morbid process to which they are due. You will pardon me, gentlemen, for not spending much time in speaking to you of the history of this disease, which is some- what dull, and which, beginning at Hutin (1817)/ is continued in Monod, Cruveilhier, Rokitansky, Tiirck, Romberg, &c. The fact is that the lesions of the spinal cord which occur in tabes were known by those who studied pathological anatomy long before this affection was raised by clinical observers to the dignity of a morbid entity. A passage of Froriep quoted by Jaccoud is in this respect of great interest. It does not appear to me therefore that the idea of sclerosis of the posterior columns existing in tabes can be connected with one or other of these authors. On the other band, however, I would specially men- tion the progress which has been made in the pathological anatomy of tabes when proof was affotded by Charcot and TABES DORSUALIS. 335 Pierret that in this form of sclerosis the external bandlets tyere first affected, 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 also 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. Spinal cord. — rA 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, ^^'hen, 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) At 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 suflRcient to show that these, changes are really due to the formation of true zones of sclerosis. _ Posterior column. — Its different segments should be con- sidere'd separately : .1 i. 336 LECXUKES ON DISEASES OF THE SPINAL CORD. a. The external bandlets, that is to say the part of the posterior column which adjoins the posterior horn upon each side, not throughout its whole extent but specialljr in its middle Jig. 166 —Section of the spinal cord in the lumbar region from a case of tabes at the onset. (The white parts are affected by sclerosis.) The change in the zones of Lissauer at the extremity of the posterior horns, and the seat of the Fclerosis in the posterior column will be observed ; this extends from the part immediately adjoining the posterior horns (external bandlets properly so- called), and spreading transversely as far as the posterior fissure, occupies the whole thickness 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. Ilie 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 in 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 usuallj^ in a healthy condition. /3. The column of Gall. — 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. Whj'- they are seated in -^JP^ Fig-. 167. — Section of the spinal cord (dorsal region) from a case of tabes at the onset. The intensity of the lesion is proportionate to the light colour of the diagram. The colnmn of Goll is affected; the column of Burdaoh on the other hand is free from disease except at its posterior part ; the lesions are most pronounced in the external bandlets, properly so-called. this part is not yet understood, and future researches \'iill bo required to explain the fact. 7. The rest of the column of Burdaoh. — The extent to wliiclr 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 ssgment 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 ; thus I prefer merely putting before you sketches which faithfully represent the appearance of the posterior columns in cases of tabes which are not yet in an advanced stage. S. Zone of Lissmier. — As you know, gentlemen, this zone is ssated between the apex of the posterior horn and the surface of the cord, and is composed of a mass of narrow nerve-fibres, lir tabes this zone, as Lissauer has shown, is much altered ; all or almost all the small nerve-fibres which constitute ib entirely disappear. Thus, in preparations coloured by the hematoxylin of Weigert, this region is found to have a special appearance, 2.^ 338 LECTURES ON DISEASES OF THE SPINAL CORD. seeming to be quite denuded. It is this appearance wliicli has induced some authors to describe sclerosis of the postero- internal part of the lateral column as frequently co-existing in Pig. 168.— Section of the spinal cord (cervical enlargement) from a case of tabes at the onset. The colnmn of GoU is affected throughout its whole length. The middle zone of the column of Burdach is also involved, as 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 said, belongs entirely to the posterior root-zone. I 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. (h) At 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. Posterior 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 none 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 bandlets are, as you would expect, TABES DORSUALIS. 339 completely degenerated, at any rate at the level of the lumbar and cervical enlargements, and in a large part of the dorsal region. Pig. 169. — Section of the spinal cord in a case of tabes. The posterior eolnmn is affected throughout its whole extent, with the exception of a few healthy fibres which are completely isolated, and of which the number is slightly greater in the anterior portion adjoining the posterior commissure. The exten- sion of the islet of sclerosis in the form of a fan will be specially obserred. This appearance is due to the fact that th^ marginal zone of Lissaner 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 lateral 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 tabes (the parts light in colour are affected by sclerosis). The fibres B (commissural?) should be observed seated in the anterior part of the posterior column ; extension of the lesions in the posterior column in the form of a fan on account of the zone of Lissaner being involved. whole of the column of Goll is affected ; in others, a part only of this column is involved. 340 LECTURES ON DISEASES OF THE SPINAL COKD. Lastly, Strumpell lias shown that a 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 a caao of tabes (the parts of a light colour are affected by sclerosis). The (commissural ?) fibres (C) seated in the anterior part of the posterior colnmn are sound. A small part (A) of the posterior column upon each side of the posterior fissure is also sound. The unaffected condition of this small part mentioned by Striimpell is not infre- jjuent in the lumbar region of the spinal cord in tabes. Fig. 172. — Section of the spinal cord in the lumbar region from a case of tabes (the parts of a light colour are affected by sclerosis). The (commissural?) fibres (B) seated in the anterior part of the posterior column are sound. It will be remarked that the sclerosis extends into the white columns at the extremity of the posterior horn, the small quadrilateral which is thus formed, and which one sees to be marked by small points, is the marginal zone of Lissaner affected by the degeneration ; its colour should be less dark. the posterior with the middle third of the posterior fissure. This small band of fibres corresponds to the middle zone of Flechsig. TABES DORSUALIS. 341 As regards the rest of the column of Burdach it is in the middle part of this tract that the lesions are usually most pro- nounced, while certain 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 column which adjoins the posterior .1 Fig. 1T3.— Section of the cord in the cervical region from a case of tabes. Almost the whole of the posterior oolnmn is affected with the exception of a few fibres at its posterior and external angle, which are sound, as are also the fibres seated behind the posterior commissure and a:long the base of the posterior comu, the cornu-oommissural zone. It will be also observed that lines of isolated fibres extend from the posterior commissure along the posterior fissure which they then quit in order to pass to the surface of the cord. These isolated fibres are the last traces of the healthy tracts which are found at this point in a less advanced stage of the disease. Compare fig. 191 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 comu. 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 iij that part as in the anterior portion. * We have already seen, in connection with the secondary degeperation asso- ciated with transverse lesions of the cord, that a certain number of commissural fibres exist in this portion of the posterior column. It is probably these fibres which remain for a time unaffected in tabes, the so-called coruu-commissural zone. 342 LECTDEES ON DISEASES OF THE SPINAL CORD-. WJien almost the wliole 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-fibres surrounded Fig. 174.— Section of the cervical region in the ceryical cord from a case of tabes. This figure shows that some asymmetry may exist in the lesions of the pos- terior columns. Upon the right aide, in fact in the angle formed by the junction of the posterior and external margins of the posterior column, a patch of sound tissue is found which does not exist upon the left side. by this tissue is much diminished, and this diminution seems to me to occur specially and primarily in the fibres of small size #hich 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 is usual to describe an abundance of granular bodies in the pos- terior columns. I must confess that for my part, although they have been most carefully sought, I have been rarely able to find any such bodies in the posterior columns of those who have suffered from tabes. On the other hand, I have recognized the absence of these elements both in cases in which the lesion was TABES DORSUALIS. 343 commencing to exist and in those in which it was in an advanced condition. In this point I therefore completely disagree from most of those who study pathological anatomy. As regards the blood-vessels, their walls are much thickened at the spots in which they are surrounded by a fibrous zone, but only at these points. I have already said a few words about the meningeal changes and the opacity of the pia-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 tha membrane or, as it may be said, of this chronic meningitis is unknown. 2. Ch-ey 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 liorn. — I spoke of the lesions which may be found in this part in a previous lecture, 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. /3. Columns of Glarhe. — 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 reticulum is alone affected in tabes, the cells remaining free from disease, in such a way th^t the latter, in sections coloured by the hsematoxylin 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 disc 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 columns of Clarke ; he explains this difference in the intensity of the lesion by the fact that the 344 LECTURES ON DISEASES OF THE SPINAL CORD. posterior root fibres which come from the lower part of. the spinal cord (and which are usually the most affected) pass specially into the inner part of these columns, whereas those Fig-. 175 — Column of Clarke in a healthy spinal cord (semidiagrammatic). A. Column of Clarke ; B. posterior column. At the upper part and upon the left side traces of the posterior commissure are seen. Upon the right side and helow the column of Clarke are seen tracts in the form of a fan which repre- sent the posterior horn. The part marked by points which occupies the whole height of the figure upon the right side is the lateral column. The large black points in the column of Clarke represent the ganglion-cells (they should be in larger number) ; while the very small black points represent the reticulum of nerve-fibres which exists normally in Clarke's column. Pig. 1T6.— Column of Clarke in the spinal cord from a case of tabes (semidia- grammatic). A', column of Clarke; B', change in posterior column (more evident) except, however, near the posterior horn (band of darker colour) ; C, posterior horn, &c. ; D', posterior column which is affected (the lighter part), except in the part adjoining the posterior born (the darker band) ; C, posterior horn ; D', lateral column. The number of ganglion-cells is almost the same as in a healthy cord, but the reticulum of small neive-fibres has almost entirely disappeared, and hence the clear appearance presented by the cnlnmn of Clarke. TABES DORSUALIS. 34,5 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 yon know, the ti'ophic centres. In the cases, which, however, are very rare, Fig. 177. — Section of the spinal cord (lower dorsal region) from a case of tabes. The whole of the posterior column is affected by sclerosis. The appearance of the columns of Clarke will be observed ; their aspect is quite clear on account rf the disappearance of the reticulum of nerve-fibres which exists in the healthy cord ; the number of ganglion-cells has undergone no diminution. in which the direct cerebellar tracts are affected (Jenclrassik), their degeneration would perhaps be due to the fact that the cells in the column of Clarke are, by rare exception, involved. 7. Posterior horn. — One would, d priori, expect that the grey matter of the postferior 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. 346 LECTURES ON DISEASES OF THE SPINAL CORD. In his work upon the condition of the posterior horn in tabes Lissauer analyses in a more exliaustive manner than had liitherto been done the lesions which may be met with in tabes. Amongst other things he mentions the disappearance of a large number of the small fibres seated in the posterior none of the spongy substance, as also of the radiating fibres 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. S. Central 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. Fig. 178.— Section through the lower part of the medulla oblongata in a case of tabea (the white colour in the posterior columns indicates the parts affected by sclerosis). A. Lesions in the column of GoU. B. Lesions in the columns of Burdach. 0. Decussation of the pyramids. D. Traces of the anterior horn. E. Traces of the posterior horn. ^ Before terminating my remarks upon the pathological anatomy of tabes in connection with the spinal cord, I would observe that TABES DORSUALIS. 347 the latter may be affected throughout its whole length, from and incluBive of the filnm terminale to the medulla oblongata and even higher. The study of the lesions which occur 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 column of GoU can be very clearly traced almost as far as the nucleus of that tract, while those of the column of Burdach can be similarly traced almost as far ^s the nucleus of the same name. B. Posterior roots. — The atrophy of the posterior roots which occurs in tabes is so 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 cord which occur in tabes. The roots of the cauda 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 I am unable to say whether this increase really exists or is simply relative. It should be observed, gentlemen, that the cranial nerves, which for the most part (with the exception, certainly, of the optic and 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 liable to analogous changes. 0. Ganglia of the spinal 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 dcr scription whatever of the lesions which occur has been written. 318 LECTURES ON DISEASES OF THE SPINAL CORD. Some authors mention atrophy of the cells, others degeneration of the fibres which join them. In the ganglia which I have been able to examine the latter lesion has seemed to me by far the most clear, and notwithstanding my most careful investigation I was unable, to my great yegret, to discover the existence of such or such an alteration of the cells. At the same time, as I shall soon say, it appears to me that some lesion very probably exists in these cells. D. Peripheral nerves. — Extremely pronounced alterations undoubtedly exist in the peripheral nerves in tabes, and peri- pheral neuritis occurs with such frequency and intensity in tabes that it can scarcely be left unconsidered when the pathological anatomy of that affection is discussed. Westphal (1878) was apparently the first to mention its existence, but in quite a secondary manner, while referring to the lesions existing in the sensory branch of a nerve in an ataxic patient. Pierret followed, to whom the honour should undoubtedly be given of calling the special attention of the medical profession to the existence and cause of peripheral neuritis in tabes (1879). In 1883 Dejerine published other examples of this lesion, and ascribed to its exist- ence a preponderating influence in the production of some of the symptoms which occur in tabes. Since that time Pitres and Vaillard, Oppenheim and Simmerling, Nonne, and many others, have shown how frequently peripheral neuritis occurs in tabes. As in all other affections in which it occurs (from the effect of a toxic agent, diabetes, pulmonary tuberculosis, different forms of cachexia, &c., &c.), the neuritis is more pronounced in the small branches of the nerves than in the large trunks, although it must not be supposed, gentlemen, as has been said, that the sensory fibres are alone affected ; every branch of the nerve, whatever its functions may be, is liable to changes of this nature. E. Brain. — It is not my intention to speak now of the focal lesions, which, as I have already said with regard to the hemi- plegia which occurs in tabes, are sometimes found in the cere- bral hemispheres or pons varolii. There is in fact nothing peculiar in their nature. The cerebral changes which are special to tabes consist in a more or less marked disappearance of the nerve-fibres contained in the convolutions. This change, which is very analogous to that described by Tuczek as occurring in TABES DORSUALIS. 3'49 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. 350 LECTURE XXIX. TABES (continued). NATURE OF THE DISEASE. NATtTEE or TABES : Theories about this subject.— The two lesions of tabes are seated in the irain (Jendr^ssik). — E61e of the sympatJietic nerve. — Primary im- portance of the vascular lesions. — E61e of the posterior meningitis. — Primary sclerosis in the tracts of the posterior coIuthtis. — Objections to this which is the leading opinion. — The fibres of the posterior column, in the same way as is the case with those in other columns of the cord, do not degenerate until the cells from which they part are affected. — Study of the cells from which the fibres of the posterior column originate : — Cells of the spinal gavglia, peripheral gatiglioh cells, arguments in favour of the existence of the latter, changes in the different cell elements. — Forms of peripheral neuritis : — Lesions of the posterior roots. — The lesions of the cord in the course of tabes are due to ascending secondary degeneration of the nerve fibres which come from the posterior roots. — The seat of the islets of sclerosis in the cord of tabid patients difPers in the different cases on account of the fact that in patients suffering from this disease the same posterior roots are not always involved, or at any rate are not affected to the same degree, and that the degeneration first occurs in certain groups of their fibres. — Hence the different clinical types rest upon a good basis from an anatomical point of view : inferior tabes, cervical tabes, &c. — Explanation of the symmetry which usually exists in the lesions of the oord in tabes. — The primum movens of the change in the nerve cells to which the lesions of tabes are due is the toxic agent of syphilitic origin suspected by Striimpell. The NATURE of tabes is one of the points in neuro-patliology which has given rise to the most numerous and contradictory- theories. Unfortunately, gentlemen, we have not yet escaped from the period of suppositions, and to my great regret I shall have little to offer you in this chapter, which treats of so important a branch of nervous pathology, which is not hypo- thetical. On the other hand it is quite useless to mention all the theories which have been advanced, and you will permit me only to place before you those which are apparently of special interest. The BRAIN, according to M. Jendrdssik, is the seat of the most important lesions in tabes, and the greater number of the TABES DORSUALIS. 351 symptoms of that affection are directly due to these cerebral lesions ; this author in fact is much inclined to consider the Fig. 179.— Section of the second frontal convolution (normal) made at right angles to its surface. (After Jendrassik.) The free surface of this convo- lution is at the upper part, while the lower part of the section (E) is continuous with the corona radiata. The vertical lines B represent the fasciculi of fibres which, in the healthy convolutions, contribute to form the rays of the corona 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 iirst time with a 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 suffering from tabes, even when there is no indi- cation of general paralysis. I am unable to say that they 352 LECTURES ON DISEASES OF THE SPINAL CORD. are present in every patient, whicli I do not believe to be the case. A *» -<;-^ ^' c? a Si £i ^ . / ^^ -a "" , ; Fig. 180. — Section of the oecipito-temporal convolution from a case of tabes made in a similar way to that in the preceding figure. (After Jendrassik.) The upper horizontal line represents the surf ace of the convolution. — A. Granular bodies in somewhat large number. The fasciculi of nerve-fibres (B') in this affected convolution are far less numerous, and thinner than in the preceding figure which represents a convolution in its normal condition. The fact, however, is in itself true ; as to its interpretation, I cannot accept that v*'hich is proposed by Jendrassik, which is specially based upon physiology, a frail science. The cerebral lesions which he regards as the cause of sclerosis in the cord, have in my opinion no direct connection with it ; the con- ^'olutions suffer of their own accord in the same way as in certain cases a portion of the crus cerebri, pons vai'olii, or medulla oblongata. The cerebral lesions of tabes in fact are purely and simply due to the same cause as that which produces the lesions in the spinal cord, namely syphilis. They are very analogous to, if not identical with those which occur in general paralysis of the insane ; like them they are due to the direct and injurious effect of syphilis upon the fibres of the cerebral convolutions. TABES DORSUALIS. 353 independently of any destructive action it may have upon the posterior coliimns 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, tlie reverse is said to have occurred. It is unknown at the same time why in certain cases general paralj^sis or tabes develop, either separately or conjointly, and whj^ 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 suiFering 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 func- tional. MM. Raymond and Arthaud have been able to show, however, that organic lesions undoubtedly exist in that nerve. In the opinion of Ordonez, Adamkiewicz,*, Buzzard, &c., 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 pronounced 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, however, admits that sclerosis may be due to other causes. 23 354- LECTURES ON DISEASES OF THE SPINAL CORD. ft p-imary systematic sclerosis of the' posterior columns ; this, I may say, is the opinion which the greater number of neuro- pathologists have professed during the course of the last thirty years. Quite recently Flechsig has also discussed this subject, and owing to data which were furnished to him in his Study of the development of the nervous tracts, has attempted to show- that the points which are first affected in tabes correspond to fasciculi, which are quite definite from the developmental point of view. Fig. 182. Fig. 183. Diagrams of the spinal cord showing the lesions which exist in tabes at its onset. The pointed parts indicate the lesions ; those of the zone of Lissauer have not been indicated. (After M. Flechsig.) Fig. 181.— Middle part of the cervical enlargement ; the lesions would be specially seated in the zone m (middle root zone) and in the median zone. Fig. 182. — Upper half of the dorsal region of the cord in which lesions exist both in the middle root zone and in the median zone. Fig. 183. — Lnmbar enlargement ; the lesions are seated in the middle root zone. The signification of the letters is as follows ; V, anterior root zone ; m, middle root zone ; G, column of Goll ; A, external part of the posterior root zone ; h', internal part ; I, zone of Lissauer. These diagrams are made from written accounts and correspond to the lesions which are most often found ; they do not, however, represent every case of tabes at the onset. According to him, in fact, the first lesions which exist in tabes are seated in the parts of the posterior column which you have TABES DOKSUALIS. 355 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 posterior and internal root zone, and only at the last period of the disease in the anterior root zone. I believe, gentlemen, that as regards our knowledge at the present time it is impossible to admit that primary sclerosis of orie or other column of the cord can possibly exist. In order that a nerve fibre may degenerate, it must either be separated from the cell from which it takes origin, and which is its trophic 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 conductors (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 disease 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 reasons exist for supj)osing 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 plates 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 thickness of the spinal cord ; their fibres during this course are disposed in a direction parallel with each other, and the posterior column is thus formed. This, gentlemen, is a fact of the greatest importance which should 356 LECTURES ON DISEASES OF THE SPINAL CORD. never be forgotten, namely, that the fibres of the fosterior column only belong to the spinal cord by accession. Which, then, are the cells from which the fibres of the posterior roots take origin ? The opinion which generally exists is that all these fibres come from the nerve cells of which the spinal ganglia are composed. This, gentlemen, is said to be the fact as regards many, the greater number, in fact, of the posterior root fibres ; is it, however, the case with respect to them all without exception ? I should be very inclined to think that some of the posterior root fibres have a difierent origin, and come from nerve cells which, during embryonic life, are separated from the group of cells which formed the Neural Plates, and then made their way to the surface, and into the different regions of the body, being carried along by the centri- fugal development of the different organs with which they had connected themselves (skin, muscles, tendons, aponeuroses, &c.). Eor this reason I propose to call these elements peripheral ganglion cells. It is very probable that all, or at any rate some of the nerve corpuscles which are called terminal, and which are found in difierent organs (the skin, aponeuroses, tendons), are really the peripheral ganglion cells to which I have just alluded. One difference alone, but an important one, exists between my opinion, as to the nature of these corpuscles, and that of other authors, namely, that I for my part, far from regarding them as terminal organs, believe them to be cells from which the fibres take origin, and from which they pass into the nerve trunks, posterior roots, and spinal cord, in which they contribute to form the posterior columns. You will ask me, gentlemen, upon what I found my belief as to the existence of these fibres of peripheral origin. Unfortunately, the arguments which I shall not put before you are not, I fear, such as will at once convince you when they are heard. In the first place, I must admit, that according to the opinion ■of the anatomists who most understand these questions the existence of a fibre of this nature has never been directly shown to exist. At the same time we know that in certain organs connected with the senses (olfactory mucous membrane, taste- buds, &c.) cells exist which are almost certainly seated at the origin and not at the termination of the nerves. In animals a^ain the existence of such peripheral ganglion cells is very TABES DORSUALIS. 357 mucli more clear ; these animals are certainly far removed from us : the amphioxus, lumbricus,* &c. On the other hand the experiments of Joseph showed him 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 great opposition. In the third place, and I feel that this reason has a more solid foundation, since it concerns 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 Friedlander and Krause had made the same observation, we must necessarily, as I 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 constituted 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 Lenhossek. Ursprang, Verlauf und Endigung der sensibeln Ner- venfaBem bei Lumbricus. ArchiT. fiir Mikroskopische Anatomie, t. XXXIX. 1892. 358 LECTURES ON DISEASES OF THE SPINAL CORD. iii tabes. The condition of the spinal ganglia in that disease must now be considered. As I have already observed to you, gentlemen, the recognition Fig. 184. Pig. 185. Pig. 186. Fig. 184 — Section of the cord in the lumbal- region. Pig. 185. — Section of the cord in the dorsal region. Fig. 186. — Section of the cord in the cervical region. From a case of ampntation of the left thigh It will be observed ; 1. That in the lumbar region into which the nerve fibres coming from the divided nervous parts the external bandlet the column of GoU and that of Burdach are affected by degeneration, on the contrary in the dorsal and cervical regions, which no longer receive the affected fibres, the colums of GoU alone show signs of degeneration. 2. On the left side the degeneration of the nerve fibres produces alterations in the posterior column not only of the left but also of the right side ; this fact will explain the symmetry of the lesions in tabes notwith- standing the number and independence of the centres in which the degenera- tion commences (spinal ganglia and posterior roots). TABES dorsdaLis. 359 of the lesions in these organs is attended by some difficulties, and as, when an autopsy is made it is not habitual to keep 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 caiTying on the examination), describe and figure considerable lesions in the cells and fibres of the spinal ganglia in tabes. For my part I 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 unaifected 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 neuritis. 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 trunks. 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 trunks themselves. I am convinced, for my part, that the degeneration of these * During the publication of these lectures I learn from an article by Darier (Gaz. hebdona de Me'd., 30th January, 1892) that M. BabinsM also expressed the opinion in 1891 that the origin of the lesions in tabes should be attributed to the spinal ganglia. Babinski is of opinion that a fumctional derangement in the functions of the cells in these ganglia would be sufficient to produce the morbid process which occurs in tabes. 860 LECTURES ON DISEASES OF THE SPINAL COKD. peripheral ganglion cells, of which I have frequently spoken to yon, is greatly concerned in the origin of the peripheral neuritis which occurs in tabes. Nor can I possibly admit that simple organs of transmission, such as the nerves, can be primarily and spontaneously the seat of such a decided lesion as that which is known by the name of peripheral neuritis, and I am as decidedly opposed to this theory, as I have already explained, as I am to that which supposes primary affections to exist in the columns of the spinal cord. Now that you understand, gentlemen, how and why changes Fig. 187. — Diagram of the course and termination of the posterior root fibres. The dark fusiform patch (G) placed below and on the right side represents a spinal ganglion, the line which crosses it indicating the point of division of the posterior root which issues from it. This posterior root divides into three branches, each of which gives off secondary descending branches. + + + + .Short fibres passing to the apex of the posterior horn ; . . . . fibres of moderate length which join the posterior horn at its base after passing upwards in the column of Burdach ; long fibres passing into the nucleus of the column of Oroll (nucleus of the class) D after passing upwards in the column of GoU. The nucleus of Burdach exists in C. TABES DORSUALIS. 361 occur in the fibres of the posterior roots, I must show you how it is that the degeneration of these fibres which penetrate into the cord in order to form the posterior column, producss sclerosis Kigs 188 -190. —The meaning will be explained directly. 362' LECTURES ON DISEASES OF THE SPINAL CORD. in this part. But in order that you may be able to understand completely what is meant, I must first remind you of the course which the posterior root fibres pursue in the spinal cord (vide Explanation of the figa. 188—190. This diagram is meant to show that the seat of the lesions of the spinal cord in tabes varies according as the posterior roots connected with one or other pair of spinal nerves are affected, or as one or other group of fibres in that root is specially involved. A, section of the cord in the lumbar region; B, section of the cord in the cervical region ; C, section of the medulla oblongata at its lower part. The left half of the figure represents a spinal cord in which the posterior roots are only affected in the lumbar region (a, b, c). The right half represents a cord in which the posterior roots are affected not only in the lumbar region (a, b, Cj but also in the cervical region (a', V , c'). Upon the left side the change in the short fibres (o) of the posterior roots produces in the lumbar region a zone of degeneration in the external bandlet; the change in the fibres of ■moderate length (J) produces degeneration in the column of Burdock ; the change in the long fibres (c) produces degeneration in the column of Goll. In the dorsal and more still in the cervical region (B), however, it is no longer the same, and the roots which pass into the cord being here in a healthy condition the external bandlet is completely unaffected by degeneration ; the fibres of moderate length and the long fibres affected in the lumbar region alone reach- ing to this height in the cord produce degeneration" in the columns nf Burdach and of Goll. While other affected fibres of lumbar origin do not reach the cervical region, the long fibres extend to the medulla oblongata and produce at that part a zone of sclerosis in the column of Goll.' On the kioht side of the DiAQRAm the zones of degeneration produced by the change in the posterior roots of the lumbar nerves (a, b, c) are the same as on the left side ; the external bandlet, column of Burdach, column of Goll, in the section of the spinal cord in the lumbar region (A) ; the column of Burdach and column of Goll in the section of the cord in the cervical region (B) ; the column of Goll in the section of the medulla oblongata (C). On account of the posterior roots, however (a', 4', c'), being also affected on the right side which did not happen on the left side, the zone of degeneration exists in the right external bandlet; in addition to this (which is not marked in the diagram) the degeneration in the column of Burdach is much more extensive than on the left side, since upon that side it receives in addition the fibres of moderate length and long fibres which are affected. Lastly, in the medulla oblongata upon the right side these fibres of moderate length and long fibres coming from the posterior roots, which are affected in the cervical region in joining the nucleus of Burdach, produce in the column degeneration which does not exist upon the left side. It is also very probable that in any pair of spinal nerves one group of fibres can be alone affected at first, and that this group is specially that of the fibres of moderate length or of the long fibres. This explains the frequent predominance of the lesions in the column of Burdach and the column of Goll. fig. 187). We divided them into three groups in the same way as_Singer and Munzer : 1. The group oi short fibres which pass into the posterior horn almost immediately after their entrance into the cord. 2. The group of fibres of moderate length which pass upwards TABES DORSUALIS. 363 for a certain distance in the cord, being inclined towards the posterior fissure and then curving outwards and passing into the posterior horn nearly at its middle part ; a certain number of these fibres pass into the columns of Clarke. This 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 suflfered 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 fibres 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. It 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 through- 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 (vide 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 364 LECTDEES 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 coining from the roots at a lower level, that is to say, the fibres which are exclusively seated in the column of Goll and the column of Burdach. It may again be supposed that the groups of fibres in the different posterior roots are not sim altaneously 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 in sections of the cord which are made in parts where. the lesions 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 column of Goll ; the external bandlets properly so-called are found to be almost Fig. 191. — Section of the cervical region of the cord in a case of tatioa (the parts are of a lighter colour in proportion as they are more affected by sclerosis). If the figure is turned round in such a way that the posterior horns are at the upper part, it is seen that the part which is chiefly affected by sclerosis has clearly the form of the letter M. The band of healthy tissue which separates the outer from the inner stroke of the letter seems to occupy precisely the same region as that in which the comma- shaped degeneration of Sohultze is found to occur in some cases of descending degeneration. The posterior part of the column of Goll and the middle portion of the column of Burdach are diseased in this case ; the external bandlet, properly so-called, is unaffected. TABES DORSUALIS. 365 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, 183), it will be observed, gentlemen, that in the secbions of the cord above the lumbar region the lesions are not seated in the external bandlets but in the column of Burdach and in the column of GoU. There is then, in my opinion, one theory without which the *■ pathological anatomy of tabes cannot be described ; this is the important fact 'that the lesions of the spinal cord in tabes occur hij Fig. 192. — Section of the spinal cord (upper part of the cervical region) in tabes. Tho anterior half of the column of Goll is alone affected by sclerosis in this case ; the general form of the region affected is that of a Y reversed. sei/me'iits, 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 different segments of the cord, it is specially in those consti- tuting the lumbar and cervical enlargements that the lesions of tabes occur. 366 LECTURES ON DISEASES OF THE SPINAL CORD. understood ; the same pair of nerves has not been affected by the primary lesion in the two cases. In the one tahes has occurred in the lower limbs and abdominal viscera ; in the other, cervical tabes exists ; in a third, the chief signs presented are those of cephalic tabes. This difference results from the fg,ct that in the first the roots of the cauda equina, in the second those of the cervical enlargement, in the third those of the cranial nerves were specially and primarily afiected. These three principal segments often suffer in almost a parallel manner, though at the same time it must be remembered that one or other may be affected, if not quite exclusively, at any rate with such a predominance of the symptoms, both from a clinical and anatomical point of view, that the constitution of the three forms, viz., the lumbar, cervical, cephalic, which have been just mentioned is undoubtedly legitimate. When the existence of this extreme difference, when the origin of the morbid process is remembered, you will be perhaps surprised that the lesions in tabes are usually symmetrical upon one side of the cord or the other. This symmetry can un- doubtedly be in some degree explained by the admission, as I have already had occasion to show you in connection with the spinal cord of patients in whom an amputation had been per- formed, that a portion of the root fibres on one side passed into the posterior column of the opposite side. This symmetry again should not surprise you, since we are accustomed to meet with it in afiections having an anatomical substratum of a most dissociated and peripheral nature, namely, toxic forms of paralysis (lead poisoning, alcoholism, arsenical poisoning, &c.). The exposition of all these facts, gentlemen, is now concluded, and, I may add, of all the hypotheses connected with them. Allow me to state rapidly, at the end qf this .lecture, the idea which I have myself formed as to the nature of tabes. From an anatomical point of view : The lesions of the spinal cord in tabes are by no means the result of primary myelopathy and disease of the posterior columns. They are merely the result of degeneration which has occurred in the posterior nerve roots. This degeneration of the posterior root fibres is due to change in the cells of the spinal or peripheral ganglia. TABES DORSUALIS. 367 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 aetiology of tabes as to the role played by syphilid. It is to the syphilitic infection that the cellular changes must be attributed. But by what means ? This is no longer a case of new syphilitic growths, presenting or not the appearance of circumscribed 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, at any rate to propose a rational solution of the problem. This solution I shall borrow from Strtimpejl. In my opinion, we cannot help admitting, in accordance with that distinguished physician, that syphilis acts as a real organic poison, a toxine. 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 hj'pothesis, 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. * It will be observed that although the nerve cells of the sensory organs (the skin, tendons, &c.) and of those connected with special sensation (the eye, ear, &c.) are specially involved in the toxo- syphilitic process which causes tabes, the nerve cells of the motor system seem in some cases to undergo similar change (some forms of amyotrophy, hemiatr^hy of the tongue, &c.). The cells of the brain may also be affected, as is shown by the degeneration of the nerve fibres of the convolutions which occurs in some cases (general paralysis of the insane, oases observed by M. Jendrassik). 368 LECTUKE XXX. Friedreich's disease. HiSTOKY. — The first cases were deaoribed by Friedreich in 1861 ; in England, the case of Carpenter (1871), of Gowers (1880); in France, the thesis of Brouase (1882) ; Lecture of Charcot ; thesis of Soca. Symptoms. — A. Motor dis- orders : — (a) Disorders of gait, (b) Difficulty in maintaining the upright position. (c) Atactic tremor,* (d) ChorfAform moaemenls. (e) Paralytic symptoms. B. Sensory disorders : — (a) Pains, (b) Ancestliesia, analgesia, (c) Disorders of the muscular sense. C. Disorders of the reflexes : — (a) Cutaneous rejhxes. (b) Tendon reflexes. D. Disorders in the organs connected with the SENSES: — Ocular disorders; nystagmus. E. CebebraIj DISORDERS, vertigo; cephalalgia; state of the intellect; disorders in speech. F. Genito-itrinary DIS- ORDERS. 6. Trophic and vasomotor disorders: — Clvb-foot; mtiscular atrophy ; curvature of the spine. Course of the disease : Progressive, remissions at times occur ; recovery impossible. Diagnosis from : — Tabes, insular sclerosis, chorea of Sydenham, a pseudo-disease of Friedreich recently described by Nonne. Gentlemex, — Having studied in detail both insular sclerosis and tabes, we are now in a position to consider the description of another disease of which some of the symptoms exist in both these affections, and which, while it is termed by some heredi- tary ataxy, and considered by others as a part of insular sclerosis, actually bears the name of " Friedreich's disease." A simple glance at the disease will enable us at once to recognize its similarity to these two affections. It consists in the existence of ataxy, which is slowly pro- gressive. Its onset occurs at an early age, sometimes in many mem- bers of the same family ;' the motor trouble exists first in the lower limbs, the muscles seated in the upper extremities or at a higher level only being affected at a later period. In con- firmed cases there is increasing difficulty in walking, and the * This form of tremor, which only occurs when a movement is attempted, is called by the French " tremblement intentionnel." Gowers (Dis. of Nerv. Syst., Vol. II., p. 549, Note) truly says that this expression cannot be used in English "on account of the meaning of 'intention,'" and that "atactic tremor " would be, on the whole, the best term. {Translaior.) FRIEDREICH'S DISEASE. 369 gait resembles that due to cerebellar disease' and to tabes, in addition to which choreiform 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 clinical 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, Friedreich 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 facts 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 cases ; 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 I 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 I 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, 24 370 LECTURES ON DISEASES OF THE SPINAL CORD. and Brousse proposed to call the affection by the name of " Friedreich's disease." The first case observed, or at any rate recognized, in Paris, was that of a young lad who had been admitted into the service of Charcot in 1884 at a time when I had the honour of being his clinical assistant. I still remember the circumstances con- nected with his admission, which I shall mention in order to show you what was known at that time about the disease. The boy was brought to Charcot by a physician attached to one of the hospitals, who was most interested in medical progress, well-informed, and, if I may use the expression, quite on the watch for any new discovery. This distinguished physician had 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 scrupiilousness which can.^ 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 brought the lad to the Salpetriere 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 so 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 Freid/reiclis disease. Since that time many works and reviews (Rutimeyer, 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 Cilles de la Tourette, Blocq and Huet, the very remarkable thesis of Soca (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 SYMPTOMS of the disease of Friedreich affecting several different systems should be described in somewhat methodical FRIEDREICHS DISEASE. 371 order, and I shall follow that which is usually employed, it being at the same time understood that this classification is purely artificial. A. Motor dism-ders. — These may be said to constitute the chief Fig. 193. — Boy aged six years, brother of the patient who forms the subject of the next figure, suffering from the disease of Friedreich. (After E. Massalongo.) Inoo-ordination very pronounced, the attitude of the legs, 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 gait. — In confirmed cases these troubles are 3T^ LECTURES ON DISEASES OF THE SPINAL CORD. vei'y pronounced, and the patient reels in walking, the legs being wide apart, the steps irregular, awkward, and often re^ Pig. 194 —Girl aged ten years affected by the disease of Priedreioh. (After E. Massalongo.) The attitude of the patient clearly indicates the inco-ordination and difBoulty in maintaining the upright position which exists. sembling those of an intoxicated person ; it does not resemble the gait which exists iii 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 asso- 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, which are very analogous to those which occur in insular sclerosis. (J}) Difficulty inmaintainirigtlie xtpright position. — This difficulty was well perceived by Friedreich, and described by him as static ataxy. 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. (c) 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 unnecessary to repeat what was said upon that occasion. (cZ) Choreiform movements. — These movements are " abrupt;" 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 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 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 occurs dn 374 LECTURES ON DISEASES OF THE SPINAL CORD. tabes, are rare, and but slightly pronounced wlien they do exist : — (ft) Pains. — These are not felt in Friedreich's disease ; Charcot, however, has shown that they exist in certain cases, and that their character may resemble that of the lightning pains in tabes. (h) Anaesthesia and analgesia similarly but seldom occur ; at the same time some authors, specially Soca, believe these dis- orders to be somewhat frequent, and that the reason for their being so seldom observed is that they pass unperceived from being but slightly accentuated. In some cases, on the other hand, absolute hemianaesthesia exists, the cause of this symptom being hysteria. This affection is sometimes associated with Friedreich's disease, in the same way as we have seen it occur in the course of insular sclerosis or tabes ; in such a case care must be taken not to attribute to disease of the spinal cord what is really the mere effect of this neurosis. Dejerine, how- ever, observed cases in which, as he says, the sensory disorders could not be attributed to hysteria. (c) Muscular sense. — According to most authors the muscular sense is usually unaffected, at any rate as regards the knowledge of the posture of the limbs, since the symptom of Eomberg, when it exists, seems due to the choreiform instability, rather than to any disorder of the muscular sense. At the same time Soca, owing to some facts observed by him, is of opinion that the latter is sometimes affected. These symptoms, however, are of such a complex nature that in my opinion it is very difficult to apportion them correctly, and nothing definite can be asserted as their cause. C. Disorders of the reflexes. — (a) The cutaneous reflexes are usually retained ; in some cases they have been found excessive. It does not seem, however, that there is ever anything character- istic in their nature. (6) The tendon-reflexes are almost always lost, and it is this loss whi<;h conduces to give such a singular clinical appearance to Friedreich's disease, which consists in a mixture, as it were, of the symptoms of tabes with those of insular sclerosis. Simple diminution of the tendon-reflexes occurs in some cases. In others they are observed to be increased. Is it possible that some mistake has occurred with regard to these cases which are Friedreich's disease. 375 not really examples of iViedreich'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 usually 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 connected lui.th the senses. — These, properly speaking, do not affect the organs themselves, but 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 infrequency of sensory disorders during the course of Friedreich's disease. (a) Ocular disorders. — The most frequent and interesting dis- order is : — Nystagmus. — 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 onlj- meet with it in fact two, three, or even more years after the onset of the disease. 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. Parcdysis of the ocular muscles 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 nerve 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. Cerebral disorders. — Vertigo occurs somewhat frequently, existing at times almost permanently, while in other cases it comes on in well-marked paroxj'sms. 376 I-ECTUKES ON DISEASES OF THE SPINAL CORD. Cephalalgia has been observed in some cases, and may present the same characters as in migraine. The intellect may be considered as almost unaffected ; this fact must be well remembered since it will surprise you, gentle- men, when j^ou are in the presence of these young patients. On account of their strange, unstable, and frequently stupefied appearance you will be tempted to believe them endowed with much less intelligence than a healthy child. In this, however, you will be mistaken, since if you question the parents or the children themselves you will find them to be almost as advanced in knowledge as other children of the same age. Such is the case in childhood. As regards the adult, those suffering from this disease who survive childhood are perhaps somewhat less intelligent than their healthy companions. My own observations have led me to think that their mental development is more or less imperfect, although they cannot be said to suffer from idiocy, or even backwardness. Some of them can never learn to write, but the cause of this is really the difficulty which exists in using the muscles. The speech presents considerable changes in confirmed cases which are due to derangements in the motor innervation of the muscles by means of which articulation occurs. The speech is slow, hesitating, and at the same time unequal (in the same phrase some words may be spoken by the patient more rapidly than others). In addition to this the pronunciation is thick, and at times indistinct. At the same time it is not the spasmodic utterance which exists in most cases of insular sclerosis. If I had to make a comparison, I should rather say that the speech resembles the " cerebellar gait ' since, like that symptom, it is heavy, uncertain, and wavering. F. Oenito-urina/ry disorders. — These are very slightly pro- nounced, this being another difference between Friedreich's disease and tabes ; at most nocturnal incontinence of urine exists to a slight degree in very rare cases. As regards genital disorders, properly so-called, Soca, who has carefully sought for them, states that they do not exist, while impotence is never observed either in the male or female, the only derangement being more or less marked delay in the existence of the sexual instinct in the male, or of menstruation in the female. fkiedreich'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 suffering 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 anterp-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 a 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 a 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 believed by 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 g' 5=s >=3 s ai 1-^^ a ri2 ^ 03 '^ ^ ^ fl ID "3 _o ■I 0) o o ■y-i '^ =tH O i^ ,Q d .2 S f- *^i^ ^ >. bo OB « -*J -t^ ■J 5 n 1 o ia s p( .2 o -4A 1 ! 0) s a ^ ^ o d 1 1 ^ ■s B 1 1 OS 3 OQ 1 ^ fi- ■+3 Pi o PI n c3 ►^ s CO 1 ta" "S 1 Oi o ,■■) 'bn ■4-4 -f9 (0 «!-■ I— 1 -*a (D in -^ ,J3 a> (U *tt ^ E-i r" ^ O d -S 1 1 ITS i 1 S dD u (» -H we have already observed, nothing analogous is found to occur in infantile paralysis. Myopathic 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. '128 LECTURES ON DISEASES OF THE SPINAL COED. A form of muscular atrophy must be also mentioned which occurs in early life and which is known, at any rate in France, Fig. 227.— A girl affected during the first year of her life by infantile hemiplegia of cerebral origin affecting the left side. The left arm is shorter and less developed, pronounced club-hand existing. The eame remarks may be made about the left lej. (.Patient under the care of Charcot. Stereotype of Londe.) by the name of the Charcot-Marie form, in which both the lower and upper extremities are thus affected by pronounced atrophy. What distinguishes this form of wasting from the atrophy which exists in infantile paralysis is its onset, whicli INFANTILE PARALYSIS. 429 is essential and progressive; the almost perfect symmetry in tlie 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 cases, which though certainly rare, have un- doubtedly been shown to exist (A. Ohauffard), children who have been long affected by hysterical 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 hj-sterical symptoms prevent the occurrence of any mistake. The aetiology 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. I 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 setiological 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 occurred 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, 430 LECTURES ON DISEASES OF THE SPINAL CORD. overwrought, weakened, either by the pain suffered, or loss of sleep, or the diarrhcsa which is so often associated with the eruption of teeth. The organism which is thus fatigued will on that account be specially exposed to the different forms of infection. Such is the part which I believe dentition to play in infantile paralysis. This is a time of life during which the organism being depressed, in consequence of its existence, is specially liable to suffer from the different infectious diseases which may be followed by infantile paralysis. Dentition is not an actual cause, but a period during which this liability exists. My firm conviction, in fact, is, gentlemen, that infantile paralysis invariably, or almost invariably, depends upon the existence of some general affection, usually of an infectious nature. This has been my opinion for many years : and though I do not believe that it is confirmed as yet by many adherents in Prance, this is of little consequence, and certain presentiments lead me to believe that " the time approaches " when this will be the case. I hope, in, fact, that I shall be able, if not to convince you all, at any rate to obtain some converts to my opinion, while reserving until another occasion a statement of the arguments which are famished by its pathological anatomy, I would now place before you those which are founded upon its clinical features alone. As regards the onset of infantile paralysis I dwelt at sufficient length upon the different general symptoms which occur at this time ; it is unanimously agreed that they are quite analogous to those which exist in acute febrile general diseases. It is possible that the acute disease may be of indeterminate nature resulting from some " unknown affection," as so often occurs in practice. In a certain number of cases, however, which you will perhaps think more suitable for the demonstration of this theory, the nature of the infectious disease is known, one of what may be called the fundamental infectious diseases existing. It is during the course of measles, scarlatina, diphtheria, small- pox or whooping cough, that the onset of infantile paralysis occurs. Lastly, one of the arguments which should, in my opinion, have the most weight, is that which is based upon the epidemic nature of infantile paralysis. There are certain important observations upon this subject which I must mention to you. INFANTILE PARALYSIS. 431 Cordier* in a communication made to the Societe des Sciences Medicales of Lyons in 1887 states that he saw in two months (June and July 1885), at Sainte-Foye-l'Argentifere, 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. Leegardf mentions on his part the fact that in the small town of Mandal (Norway), Oxholm and Iiis colleagues observed 8 cases of infantile paralysis between the end 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, these cases formed but a small minority which could not seriouslj'- invalidate the comprehensive statistical observation made by Medin. That author also recalls that in 1881 Bergenholtz had already observed a small epidemic of 13 cases at Umea, and that G. Colmer,§ having inquired of the parents of a child suffering from infantile paralysis, learnt from them that in their district, within the distance of a few miles, and in 3 or 4 months, from 8 to 10 children had been similarly affected. As regards the 5 cases observed by Briegleb || in his clinical service at lena, during the months of June and July, it should be remarked that, as the cases came from places which were so distant, that one or two hours were occupied by the train in passing between them, they seem of less value in connection with the demonstration of this fact than the preceding. * Cordier, Lyon M^dioale, 188S. f Leegard, On poliomyelit njed demonstration af mikr. prep. ; d'aprfes I'analyse dn Neurolog. C'entmlblatt. 1890, p. 760. J Medin, Epidemisk optraeden af infantil Paralyse. Hygeia, Septembre, 1890. § G. Colmer, American Journal of Medical Sciences, 1843. II E. Briegleb, Ueber die Prage der infeotiosen Natur der aouten Poliomyelitis laaujur. Dissert. lena, 1890. 432 LECTURES ON DISEASES OF THE SPINAL CORD. Owing to these facts, the epidemic nature of infantile paralysis, at any rate in some circumstances, seems to me definitely estab- lished, and in my next lecture we shall see how this fact may be understood. Such are the arguments which I consider of value in connec- tion with the infectious origin of this disease. I would not terminate my observations about its aetiology without reminding you that in most cases hereditary transmission plays a very im- portant part. For my part, as I have already said, I have no difficulty in believing that the neuropathic tendency communi- cated by transmission to the organism of a child may cause its nerve centres to be less able to resist the causes of disease. At the same time, I find it quite impossible to look upon hereditary predisposition as the effective and direct cause of the disease. The age at which the affection usually exists is, as I have already said, the earliest infancy. In most cases it occurs during the first 2 years of life, specially between the ages of 1 year and 18 months. At the same time it may occur later, as in the 3rd or even the 4th year. We shall have upon another occasion to study a form of poliomyelitis which occurs in the adult, which so much resembles the infantile form that, properly speaking, no precise age can be mentioned which separates these two affec- tions, which seem to occur at any age from infancy to the adult age, and even longer. At the same time, from a clinical point of view, infantile paralysis should be considered a distinct disease, on account of its being specially frequent in early infancy, and of the singular aspect which the affected limbs present which are in full process of development. As regards sex, there is nothing special to be said, the two sexes being equally affected. 433 LECTURE XXXVI. INFANTILE PARALYSIS (continued). Pathological anatomy. — History of the discovery of changes in the anterior horns ; Vnlpian and Prevost (1865), Lookhart Clarke, Charcot and JofEroy, Parrot and Jeffrey, Eoger and Damaschino. (a) Character of the lesions when tlie affected focus is of old date, lesions in the anterior horns, nature of these lesions many foci usually exist which are rarely quite symmetrical ; atrophy of the half of the spinal cord which corresponds to the paralyzed side, and in some cases of the cerebral hemisphere upon the same side (Rumpf , Colella, Pomario) ; lesions but slightly pronounced in the anterior roots and trunlcs of the mixed nerves, explanation of Joffroy and Aehard : alterations in the muscles ; altera- tions in the hones, diminution of the diameter of the Haversian systems} alterations in the blood vessels, (b) Character of the lesions when the affected focus is of recent date : every appearance of a true inflammatory focus then exists, this focns of acute myeUtis may extend to the adjoining white matter ; the reason of this fact probably connected with the distribution of blood vessels in that region. Nobmal Anatomy of the blood vessels supplied to the anterior horn. Anterior spinal system. — Anterior spinal artery; anterior median artery ; anterior radicular branches ; each of these arteries may be the seat of the intra medullary vascular lesion which constitutes infantile paralysis. Identity of infantile spinal paralysis with infantile cerebral hemiplegia ; my disagreement with Tizioli and Strumpell. Observation of P. J. Mobius. This explanation of what occurs enables the late " renewal " of the clinical course of infantile spinal paralysis, which has been considered to be understood. Therapeutics. — Electricity, massage, hydrotherapy, orthopsedic apparatuses. The pathological anatomy of infantile paralysis deserves full consideration, not only on account of its own merits, but also because 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 aetiology 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 essential to childhood." At one of the first autopsies made by Cornil and Laborde, the 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 28 434 LECTURES ON DISEASES OF THE SPINAL COED. authors stating for the first time atrophy of the anterior horns, the disappearance of a certain number of cells in them, the diminished size of the white columns upon the same side of the cord, and the fact that the lesions only extended to a certain height. In the same year, Lockhart Clarke, in England, described granular disintegration of the anterior horn. It was in 1870, that these facts relating to the pathological anatomy of the disease were made of definite value, on account of the discovery by Charcot and Jofiroy of the connection which existed between amyotrophy and the lesions of the cells in the anterior horns, since these authors deserve the whole honour of having discovered, and clearly indicated this relation which is one of the bases of medullary pathology. In 1871 and again in 1881, Roger and Damaschino attempted to show that the lesion in the anterior horns is clearly of inflammatory nature, usually consisting in the focus of softening. The fact is that these authors being in a children's hospital, observed the disease in conditions which were quite difierent from those in which neurologists usually do so; they could study the disease in fact from the time of its first onset, whilst the latter, in most cases, only come in contact with the disease when the paralysis has already existed for many years. The character of the lesions is very different, when the disease has only existed for 1 month, from that which it has 5, 10 or 20 years later. A. We will first consider the latter case which is most usual, ihe autopsy only being made after a certain number of years. The fact must obviously be remembered that an old cicatrix now exists, and that the lesions have also affected an organ which was in lull development ; this will explain the perhaps consider- able degrfe to which wasting and atrophy exist in the spinal cord in which the primary changes are seated. In the spinal cord the grey substance is recognized by means of the naked eye and in preparations which are properly stained to be more translucent at the seat of the affection than in other parts of the grey matter. At these points a glass of slight magnifying power shows that the minute structure of the grey substance has changed its character, that the large nerve cells of the anterior horn, which is affected, have disappeai-ed, some 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 Fig. 228. — Lumbar cord in a case of infantile paralysis. (A.) Poous of old date Eeated in the antero-extemal part of the left anterior horn ; it will bo observed that the left half of the cord taken as a whole is Emaller 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 which both sides are involved the diseased parts are never quite symmetrical, the changes occur 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 have 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 486 LECTURES ON DISEASES OF THE SPINAL CORD. found not only in the spinal cord but also in the medulla oblongata and pons varolii. This, however, occurs more rarely, or at any rate the clinical symptoms which are due to this condition are not usually ascribed to the ordinary form of infantile paralysis. Such are the principal points which should be noted in con- nection with the lesions in the grey matter of the anterior horns ; whether the changes which occur are quite confined to the grey substance or extend into the white matter is a question which must still be answered. It is difiicult to decide this point in such cases as those which we are now considering, namely, when the affection has already existed for many years, since, on account of the retraction which takes place in the cord, the limits of the grey and white matter are not always very clearly defined; more information is gained by the examination of lesions which have recently occurred ; this point will be recon- sidered upon a future occasion. On the other hand the examination of lesions of old date gives the most precise information as to the nature and seat of the degeneration which occurs after lesions in the grey substance of the anterior horns. If a section of the spinal cord, which is made at the seat of one of these foci, is examined with the naked eye, it is easily seen that the corresponding half of the cord is of much smaller size than that of the opposite side ; this diiFerence in size occurs not only in the antero-lateral but also, and to a very pronounced degree, in the posterior columns ; the posterior horn is also of smaller size than that on the sound side, and a certain number of observers (Parrot and Joffroy, Charcot, Schultze, Roger and Damaschino, &c., &o.) have found atrophy to exist in Clarke's column upon the side of the disease. This extensive atrophy in different parts of the spinal cord upon one side is probably due to many causes, on the one hand to arrest of development in these parts in consequence of a certain number of cells and nerve fibres being destroyed, and on the other to degeneration or an ascending form of atrophy analogous to that which we considered in detail in the lecture which was devoted to the changes which occur in the nervous system after amputations of the limbs. Lastly, there is a lesion, the occurrence of which has been INFANTILE PARALYSIS. '437 noted by certain authors (Rnmpf, Colella,* Fornariot), who observed that the motor convolutions of the brain, which rig. 229.— Section of the spinal cord from a case of old infantile paralysis. ' (Alter Charcot.) It will be observed that the white snbstanoe and grey matter tipQn the right side, which is the seat of the lesion, are affected 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 (?). Theoretically this is by no means improbable ; but if, gentlemen, you ever undertake a research of this kind you will soon perceive how extremely diflBcult it is' to recognize so small a difference between the two hemispheres. The anterior roots are recognized by the naked eye to be clearly diminished in size, though under the microscope scarcely an'y lesion, according to Joffroy and Achard,{ is found to exist, and islets of sclerosis are not 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 * Colella, La paralisi spinale atrofica infantile in rapporto con i centri corticab , &c. La Psichiatria, anno VII. f Fornario, La paralisi infantile atrofica in rapporto alle Tie sensitive del midollo, &e. Gim-nah di Neropathologia , May-Jnne, 1880, p. 131. X Joffroy and Achard. Archives di Midecine Experimentale, 1891. 438 LECTURES ON DISEASES OF THE SPINAL COKD. number of authors, on the other hand, have stated that verj- characteristic lesions exist in the anterior roots. As regards the trunks of the mixed nerves I need only repeat, gentlemen, almost in the same words the observations which I have just made with respect to the anterior roots; the same remarks and explanation on the part of JofFroy and Achard, the same divergence of opinion on the part of other authors. Veiy pronounced changes exist, as you would expect in the muscles, which are affected by atrophy, and may even in certain cases completely, or almost completely disappear; or again a certain number of fibres may disappear while the others are unaffected. It often occurs that the fibres which disappear are replaced by an extensive interstitial lipomatosis, which is un- doubtedly of much the same origin as the subcutaneous lipoma- tosis, of which I had occasion to speak in one of the preceding lectures, and the description of which was given by Landouzy. As regards the hypertrophy of certain fibres in the affected muscles, to which Dejerine has called attention, it would be due, according to some authors, to compensatory excess of action ; in Erb's opinion it constitutes the first stages of the morbid process which ends in atrophy ; lastly, Joffroy and Achard believe it to be a degenerative change, possibly connected with the incomplete lesion of a certain number of cells in the anterior cornua. The trophic disorders of the limbs affected in infantile paralysis extend even to the bones. Even at first sight one oo Fig. 230.— Sections of the two humeri in a case of infantile paralysis. (A.) Section of the hnmerns on the sound side ; (B.) Section of the humerus upon the side affected by infantile paralysis ; the dimensions of the latter are much smaller, and the shape is more rounded. (Damaschino collection.) recognizes that the outline of the affected bones, instead of presenting, as in the normal condition, ridges and depressions, is irregularly rounded, and does not present the irregular surface which normally exists. In addition to this the thickness of the compact tissue is far more uniform than in a healthy bone at INFANTILE PARALYSIS. 439 different points of the circumference 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 size should be partly ascribed to this cause. Such, gentlemen, are 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 I have already said, they are of but slight value 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. B. The autopsy takes place at the end of a few days, or at most five or six weel's : In 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 inflammatory 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 440 LECTUEES ON DISEASES OF THE SPINAL CORD. already alluded when speaking of the lesions of old date. It must be well understood that the nerve fibres are also affected, a certain number of them having the characteristic granular appearance. As regards the blood vessels, the changes which occur in them are most obvious ; in the parts of the grey matter where the affection exists they are fotmd to be dilated. At times, in recent cases, obstruction is even found to exist in consequence of thrombosis. Proliferation occurs in the nuclei of their walls Pig. 231.— Tief b half of the lumbar cord in a case of infantile paralyaia. In the left anterior horn a focal lesion is seen to exist at (a) in which the blood vessels are much developed. (Spinal cord from a patient under the care of Arch- ambault. Damaschino collection.) which are themselves thickened. In every case the changes in the blood vessels are very clearly marked. Thus an inflammatory process clearly occurs in this disease, a .form of acute myelitis which produces, as I have said, focal lesions. Though there is, perhaps, little to say with respect to the seat of these foci, as regards their height in the cord, since the distance to which they extend is quite variable, the same is by no means the case as regards their seat in the antero-posterior or transverse direction. In the antero-posterior direction these foci are clearly seated in the anterior horn and usually do not extend beyond the neck of the cornu. In the transverse direction the lesions have apparently a certain tendency to exist in the antero-external and antero- internal portions of the anterior horn ; but owing to the fact that they have a tendency to occur at its outward part, they often encroach upon the antero-lateral column where it adjoins INFANTILE PAEALYSIS. 441 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 is 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 I 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 union, 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 fissure, and can be traced as far as the cornus meduUaris ; 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 lai-ge branches at right angles to its course which pass backwards in the anterior fissure and constitute the anterior median arteries. Each of the anterior median arteries 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 serious lesion, the corresponding anterior horn will be also affected. It 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 as to supply these horns on both sides ; this explains the fact that the lesions in infantile paralysis are nearly always unilateral. 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 SPINAL CORD. median fissure are known to pass into the white substance -iX'hich adjoins the anterior horn. It may also be suppcsei that the primary lesion in infantile paralysis, which is constituted by a focus of rcute polio- Fig. 232.— Diagram intended to show how lesions of the anterior horns are produced in infantile paralysis. A, anterior spinal artery; B, anterior median artery ; C, commisanral artery ; D, D', D", anterior radicular arteries ; E, anterior lateral artery ; F, middle lateral artery. Two foci of disease existing in Infantile paralj'sis are represented, each of which has a different origin ; one (M) is dne to a primary lesion in the branch of the commissural artery which passes into the anterior horn, it ia entirely comprised within the horn ; the other (L) is dne to a primary lesion seated in one of the anterior radicuhir arlerief, it extends into the white substance of the antero-lateral column. myelitis, does not result from an affection of the anterior median artery, but of one of the anterior radicular arteries. The seat of these arteries enables the fact that the antero-lateral column is affected, and the assymmetery of the lesions to be explained. It is possible that these arteries are equally liable to be the seat of the vascular lesion which now interests us, and this difference of seat explains certain varieties which exist in the symptoms. What, however, is the nature of this lesion of vascular origin ? It is in the setiology of the disease, gentle- men, that we shall find the answer to this question. You will not certainly have forgotten all the facts which plead in favour of the infectious nature of infantile paralysis : the acute onset — • the febrile symptoms — the epidemic nature of the disease ; on INFANTILE PARALYSIS. 443 account of these facts, solely from a clinical point of view, we decided that the disease 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 nen'ous 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 thi-s 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 Striimpell,* pre- ceded me, as I am happy to acknowledge, in having this opinion, both of ihem clearly expressing the opinion that the two diseases of childhood, which I have just named, were closely analogous * Vizioli only mentions the similarity which exists in the course of the two affections and the development of the symptoms ; he makes no aUnsion to the data furnished either hy general pathology or pathological anatomy, and, from a clinical point of view, carefully distinguishes infantile spinal paralysis " from the other forms of paralysis which are liable to occur at an early age (in consequence of infectious disease or some other cause)." — Emiplegia cerebrals spastica. II Morgagni, 1880. — This bibliographical indication had preriously 444 LECTURES ON DISEASES OF THE SPINAL CORD. to eaeh other. At the same time, singularly enough, with the exception of this idea as regards the analogy of the diseases, we all three have very different opinions about the nature of these two diseases. Contrarily to Vizioli, I believe both- these affections to be of an infectious character ; while on the other hand I cannot agree with Striimpell that they are systemic diseases of the grey matter. Whatever may be thought of these two opinions, the fact that the two affections are identical seems to me undeniable, and if you have any doubt about this, I need merely quote an observation of Mobius which I have quoted several times ; a brother and sister, whose respective ages were three years in the former, and a year and a half in the latter case, after suffering from general symptoms (fever, gastric derangement, &c.) during a few days, were both attacked simultaneously, the sister by atrophic spinal paralysis, the brother by spastic in- fantile hemiplegia. I will go even farther, gentlemen : I am convinced that a favourable opportunity will occur, and that infantile hemiplegia, due to a cerebral cause, and infantile spinal paralysis will some day be found associated together in the same patient, and I am confidently awaiting the publication of this typical observation which will conclusively prove the identity of these two affections. One question still remains unanswered : what is the infectious agent which determines the existence of this morbid process in the spinal cord ? In reply, suppositions can alone be made. Is it the micro-organism which specially occurs in e:^anthemata or other diseases, in the course of which we have found infantile paralysis? or are other micro-oi'ganisms associated with these, combined infection existing? and in this case is the pathogenic micro-organism specific or common ? These are questions which cannot yet be answered, at the same time increased knowledge escaped the notice of the authors interested in this question ; it is taken from the remarkable work of Sigm. Freud and Oso. Eie upon infantile hemiplegia, Vienna, 1691. Striimpell, whose memoir upon this subject was written in 1884, starts with the idea that infantile hemiplegia, due to a cerebral cause, is for the same reason as infantile spinal paralysis an essentially systemic affection, sea/ted in the grey matter of the cOnTolutions j hence he applies the name of poHo-enoephalilia to this form of hemiplegia, in the same way as the term poliomyelitis was adopted for infantile spinal paralysis. According to M. Striimpell again both these affec- tions are by nature infectious. INFANTILE PARALYSIS. 445 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 recwrrence due ? One might be tempted to suppose that this was an additional proof that the affection was due to the effect of micro-organisms. The microbes existing in the cicatricial foci becoming again active 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 furunculosis ; 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 not 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 epilepsy occur, often accompanied by a recurrence of the paralytic symptoms, a recurrence of paralysis which exactlj^ resembles that which occurs in the cases of infantile 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 less beneficial than might have been expected. During 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 4-46 LECTURES ON DISEASES OF THE SPINAL CORD. quinine and other internal antiseptic agents, or at times when hyper-pyrexia exists, the use of cold baths are specially indicated. At a somewhat later date different counter-irritants may be applied to the spine, such as tincture of iodine, diluted croton oil, &c. Lastly, when the period of regression is definitely established, but not previously, in order that a spinal cord which is already affected by inflammation, may not be much subjected to ill-timed irritation by the electrical current employed in the treatment, a weak, a very weak current should be used that the child may not be much disturbed ; and whether the galvanic or faradic current be employed, it must be remembered that but slight contraction of the muscles need be produced, and that violent contractions should not occur. --- During this period of regression, massage, muscular exercises, hydro-therapy (douches, saline baths, sulphurous baths), the patient should also be watched, in order that when deformities threaten to occur, this may be prevented by the use of an appropriate orthopcedic apparatus. At the same time, care must be taken, gentlemen, not to allow the children to be covered by a too heavy casing of iron, under pretext of preventing deformity in a paralysed limb which must be always useless ; they would then be unable to take the exercise which is necessary for their development and good health, and you would thus be preventing what you wish to promote. 447 LECTURE XXXVII. AMYOTROPHIC LATERAL SCLEROSIS. History: — Disease of Charcot. Symptoms: A. Spasmodic symptoms: excess of tendon reflexes in the lower and upper limbs, foot-elonns, tendency of the limbs to pass into a condition of contracture, or at any rate of spasmodic rigidity. B. Paralysis. C. Muscular atnophy, its seat, existence of fibrillary contractions; electrical reactions. D. Bulbar symptoms ; paralysis and atrophy of the muscles of the lipa, tongue, and soft palate ; difficulty in mastication ; loss of the lateral movements of the lower jaw ; distorted action of the heart ; examination of the masseter tendon reflex (jaw jerk) ; examination of the pharyngeal reflex. No disorder of the sphincters or trophic derangement exist, frequent diminution of intellectual power, liability to emotion ; the exist- ence of symptomatic neurasthenia. Couese of the disease ; diiferent modes of onset : A. By atrophy of the upper limbs ; B. by bulbar symptoms ; C. by spastic paraplegia. Duration of the diseass. 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 Jeffrey, 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 in a logical manner. Thus, lateral sclerosis signifies the existence, not only of paralysis, but also of spasmodic symptoms, while the term ^■amyotrophic" indicates 148 LECTURES ON DISEASES OK THE SPINAL COKD. the occurrence of progressive muscular atrophy, so that the name is a short but exact statement of the clinical symptoms which exist in the disease of Charcot. This affection, in fact, is one of the rare forms of amyotrophy which are associated with spastic phenomena. With your permission we will commence by studying these, which is the most natural course to pursue, owing to the fact that these symptoms usually occur at the onset of the disease. The tendon reflexes are excessive, not only in the lower and upper limbs, but in all parts of the body, such is the case both in the foot and knee, in the wrist and elbow, and even the jaw jerk may be most excessive in degree. The excess of the tendon reflexes is carried to a degree such that associated phenomena as the periosteal reflexes (specially in the fore-arm, in which move- ments of flection and pronation are produced) and foot-clonus, the latter perhaps existing to a pronounced degree, may occur. But, as you know, the knowledge of tendon reflexes has been acquired at a comparatively recent date, and yet before the discovery of Erb-Westphal, Charcot had clearly indicated the spastic nature of amyotrophic lateral sclerosis; he based this idea upon the fact, recognized by him, that more or less pro- nounced contractures existed in the limbs, specially the lower limbs. Some, who were opposed to the idea that amyotrophic lateral sclerosis should be considered a distinct disease, did not recognize the existence of these contractures. With regard to this difierence of opinion it must be said that if we expect to meet with contractures as pronounced as those which occur in hemiplegia or spastic paraplegia, we shall often be disappointed ; but if, on the other hand, we look carefully for some sign of rigidity in the upper or lower limbs this will be easily found in the great majority of cases. This tendency to spasm has a real eflect upon the attitude of the limbs ; thus the lower limbs are often seen, when the patient is confined to bed, to be rigidly extended, with more or less pro- nounced rotation of the inner border of the foot inwards ; again, when the patient walks the lower limbs may present the appearance which I have already had occasion to describe as c'haracteristic of the spasmodic disorder of gait. The upper limbs are usually in the position described by Charcot as follows : " the arm hangs by the side of the body, AMYOTROPHIC LATERAL SCLEROSIS. 449 and any attempt to separate them is opposed by the action of the shoulder muscles. Pig. 233.— Woman affected by amyotrophic lateral sclerosis. This figure shows the atrophy of the hands and the position in which they were usually placed by the patient ; the legs, in which the atrophy is much less pronounced, are close to each other, the feet being slightly rotated inwards, in short the characters of slight spasmodic rigidity are observed in the lower limbs. (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 semi-flexed, whilst the fingers are also strongly flexed into the palm of the hand." As an annex to these spastic symptoms tremor has been mentioned in some cases to occur in the limbs when voluntary (?) 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 difierence 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 29 450 LECTURES ON DISEASES OF THE SPINAL COED. 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 occur on account of the small degree to which atrophy exists in their muscles. This muscular atrophy, which constitutes the second phase in the symptomatic history of amyotrophic lateral sclerosis, presents a certain number of special characters. It occurs gradually, affecting muscle after muscle, fasciculus after fasciculus, and, so to speak, fibre after fibre. As regards its seat, the upper limbs are affected, specially the hands, in which, in fact, it commences. The thenar and hypo- thenar eminences are first involved ; the interossei are also aifected at an early date ; the consequence of these muscular disorders is that the hand becomes " claw-like " (" en patte de singe ") in its most characteristic form, the palm of the hand being flattened, with the first phalanx extended, whilst all the others are flexed. The fore-arm is affected in its turn, and then, as Charcot says, the upper limbs remain in pronation. As regards the arms above the elbow they are only affected later, and usually to a less degree. The muscles of the neck suffer also from atrophy and paralysis, and the head is then bent forwards over the sternum, and sometimes, specially at the onset, becomes rigid in this position. In the lower limbs the atrophy only occurs in many cases at a very late period, after being preceded, during a somewhat long time, by spastic paralysis. Briefly, gentlemen, in order to understand the different ■deformities and attitudes which the limbs have in amj^otrophic lateral sclerosis the following elements should all be considered : paralysis, contracture, atrophy ; to this triad tendinous con- traction must also be added. It may, in fact, happen that the contracture ceases to exist, whilst the muscular atrophy is sufficiently pronounced to have destroyed the greater part of the muscle, and yet the deformity which is thus produced persists ; AMYOTROPHIC LATERAL SCLEROSIS. 451 this is due to the fact that tendinous retraction has occurred during the occurrence of these different processes. Fig. 231. — A man affected by amyotrophic lateral sclerosis, in whom extreme spastic rigidity existed, inyolving all the muscles of the body. This spastic condition is indicated in the figure by the attitude of the patient, who, although the muscles of the leg 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 fibrillary 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 cases, 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. 452 LECTUEES ON DISEASES OF THE SPINAL COED. 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 symptoms connected with the hulh are, in fact, of special interest in the disease which we are now considering, constituting, as they do, the special gravity of the aifection. The muscles connected with the lips (the orbicularis oris, quadratus menti, depressor labii inferioris, and levator menti) Pig. 235.— A patient affected by amyotrophic lateral sclerosis, with most pro- nonnced bulbar symptoms. The lips can scarcely be moved, and the month is always kept open ; the pronounced character of the nasal fnrrow, the wrinkles in the frontal region, the expression of wonder in the face will be observed. It is clearly seen in the figure that the hands are affected by atrophy and spasms. (Collection of Charcot.) are sometimes involved early in the disease ; the fibrillary con- tractions are sufficiently pronounced in the region of these muscles to indicate the changes which have occurred in them, specially as regards the chin, which presents a series of. movable depressions and elevations, which cause it to have exactly the appearance which it presents in a young infant who is about to cry. "When the changes in the muscles are more pronounced, 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 furrow, which drags the angle of the mouth downwards ; the saliva dribbles constantly from the mouth, and the patients " slaver." "When they laugh Pig. 236. — Woman affected by amyotrophic lateral sclerosis. The appearance of the mouth and chin, and the prononnced character of the nasal furrow will be 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, on account of the paralysis and atrophy of the lips the patients can neither blow, M-histle, pout, or pronounce the vowels e and i. The tongue, even when it still retains its normal size, presents from the time that it is first affected fibrillary movements, which do not escape the notice of an experienced eye. When the atrophy is very pronounced the surface of this organ has an undulating appearance, and presents a series of rounded eminences 4ol. LECTUKES ON DISEASES OF THE SPINAL CORD. and depressions, which resemble depressed cerebral convolu- tions. Lastly, when the paralysis reaches the most pronounced 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 labials i, r, s, 1, 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 veiy pronounced, hangs down in a relaxed condition, being often Fig. 237. — Pace of the patient affected by amyotrophic lateral sclerosis, who is the snbjeet of fig. 233. The appearance of the month will be observed, which is drawn outwards, as also the depth of the nasal farrows, the wrinkles in the frontal region, and the expression of astonishment in the face. (Collection of Charcot.) 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 oesophagus, 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 vacjus nerve becomes also affected, and, in consequence, troubles connected with the respiration (attacks of suffocation, dyspnoea) 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 pneumonia 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 affection, and what valuable information was derived from them as regards the state of the different segments of the spinal cord. As regards the medulla oblongata,, similar information is given by the con- dition of the masseteric tendon 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 456 LECTURES ON DISEASES OF THE SPINAL CORD. slight blow is then given to the paper-knife between the left hand and the patient's teeth with a percussion hammer. Which- ever method is employed, the result is that the masseter contracts, that is to saj^, the jaws approach each other. After but little experience it can easily be recognized whether this contraction is more or less strong, and, consequently, whether the masseteric reflex is increased or not. In amyotrophy, specially when the medulla oblongata is involved, this reflex is very excessive. Another reflex which it is interesting to observe in cases of amyotrophic lateral sclerosis complicated by very pronounced bulbar symptoms is the pharyngeal reflex. This reflex is, as you know, the movement of deglutition which occurs when the back of the throat is tickled, either by the finger or a roll of paper. In cases of amyotrophic lateral sclerosis in which bulbar symp- toms do not exist, or are still but slightly pronounced, the in- tensity of this reflex is unchanged ; when the bulbar symptoms are obviously more accentuated it may be apparentlj^ diminished to a slight degTee. but, whatever may be said to the contrary, it only ceases to exist, if it does so, quite in the last stage of the disease, when the muscles of the soft palate and pharynx are, so to speak, entirely destroyed. The sphincters remain unaffected during the greater part of the affection. The cutaneous sensibility and special senses are quite unaffected.- As regards sensory symptoms, the only ones that can be men- tioned are the very slight and also transient pains which occur in some cases ; most frequently tingling alone exists. There are no trophic derangements of any importance. Are ihe psychical functions altered in the course of amyotrophic lateral sclerosis ? Most authors deny that this is the case. I cannot, for my part, agree with them, and should not only be inclined to consider such a change as very frequent in the disease, but even as constituting one of its ordinary symptoms. Properly speaking, this change in the psychical functions does not produce any startling symptoms which must be noticed by the observer. If, however, one takes the trouble to seek them, a tendency to laugh or weep without any sufficient reason will almost certainly be found to exist ; the liability to emotion is decidedly excessive, besides which the whole intellectual and moral condition of the patient are childish — the credulity, the AMYOTROPHIC LATERAL SCLEROSIS. 457 simplicity of the patient, are at times quite astonisliing. Lastly, the symptoms of neurasthenia are sometimes observed, specially at the 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 afiects 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, shoi\ld be examined. Amyotrophic lateral sclerosis is thus sometimes found to be accompanied and, so to speak, preceded by the symptoms of neurasthenia. The course and symptoms of the disease vary much, both as regards its duration and mode of onset. T^he onset, in fact, occurs in three waj's which are 'quite dif- ferent from each other. A. The first symptoms which occur are in the uyper 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. B. The first symptoms are connected with the medulla ob- longata ; 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 spastic 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 distinct 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 458 LECTUKES ON DISEASES OF THE SPINAL COED. invariably the case. Does death occur at an earlier date in the case of patients in whom the onset consists of bulbar symptoms ? This is usually so, but the rule is by no means without excep- tions, and it is quite impossible to connect the length of time which a patient suifering from amyotrophic lateral sclerosis has to live with the mode of its onset. The duration of the disease is also, as I have already said, somewhat variable ; that of the ordinary forms may be fixed at from 18 months to 2 years. In some cases, in which the pro- gress has been very slow, it lias been known to attain and even to exceed 3 or 4 years. Lastly, the course of the disease is not infrequently very rapid, and in 6 or even 3 months the disease produces a fatal result. Death results from different causes. Some disorder of the resioiratory system may produce it; the dyspnoea becomes ex- treme and, notwithstanding their great anxiety to breathe, the necessary amount of air cannot be made to enter the lungs, probably on account of some disorder in the functions of the vagus nerve. Death may, again, be due to some derangement connected with the heart : syncope occurs once or several times from which the patient recovers, and then takes place for the last time and the patient succumbs. Lastly, death is not in- frequently due to intercurrent disease, either unconnected with amyotrophic lateral sclerosis or, more often, due to its existence. Such is the case, for instance, as regards the pneumonia which is connected with the digestive process. This is due, as I have alreadjr said, to some derangement in the process of deglutition, owing to which some particles of food penetrate into the bronchi, and even into the parenchyma of the lungs. To whatever cause it may be due, the termination of the disease is inevitably death, and, whether amyotrophic lateral sclerosis is treated or not, it invariably has a fatal termination. 459 LECTURE XXXVIII. AMYOTROPHIC LATERAL SCLEROSIS (continued). Patholooical anatomy. I. Spinal cokd. A. Changes in the grei/ matter of the anterior horns : atrophy of the large ganglion cells, inflammation of the whole anterior horn. B. Changes in the white substance ; (a) lesions of the pyramidal trace (direct and crossed) ; (b) lesions of the whole aniero-lateral columns., while at times the lesions are apparently seated in the portion of these tracts which is between the anterior horn and surface of the cord, or in the part adjoining the anterior horns ; lesions in the part occnpied by the columns of Gall. II. Medulla oblongata. A. Changes in the grey matter affecting the nuclei of the hypoglossal, 5th nerve, portio dura, &o. B. Changes in the white matter : the pyramids, the posterior longitudinal tract (faisceau longitudinal posterieur), the band of Eeil (Muratof). in. The pons tabolii. IV. The cbus cbbebei. V. The brain. The presence of granular bodies in the internal capsule in the motor convolutions. Atrophy of the large pyramidal cells of those convolu- tions. These lesions at the same time are not constant. The method of seeking the granular bodies. Theories which exist as to the natube of amyotrophic lateral sclerosis. Lesions of the anteeioe boots, the motob NEKVE-TBxrNKS, the intea- MTT CULAE NEBVES, and the MUSCLES. Diagnosis. A. Of the amyotrophic form from : the muscular atrophy of Duohenne-Aran {?) ; muscular atrophy due to polyneuritis ; the different forms of myopathy ; syringo-myelia ; hypertrophic cervical pachy meningitis ; arthri- tic muscular atrophy. B. Of the form with spastic paralysis from : transverse myelitis; spastic paraplegia ; insular sclerosis. C. Of the form with bulbar symptoms from : acute bulbar paralysis ; chronic bulbar paralysis ; pseudo- bulbar paralysis. Gentlemen, — 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 sclerosis — changes in the grey matter of the anterior horns. I. — Spinal Cord. 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 460 LECTURES ON DISEASES OF THE SPINAL COKD. of the anterior horns. These cells diminish in size, lose their processes, and before long almost or quite disappear. Fig. 238. — Normal anterior horn, with its ganglion cells, and the network of nerve fibres which when coloured by Weigert's hsematoxylin give a dark hue to this horn (aemi-diagranunatio). All the cells in the anterior cornua, at anj- rate in those of the cervical cord, seem equally liable to be affected by the atrophic process, both those of the anterior and those of the antero-lateral group. Possibly, by studying cases of recent development it may be possible to recognize which of these groups are first affected. In the lumbar cord many more cells as a rule remain in a healthy condition than in the cervical region. (&) It vrould be a mistake to think that the large ganglion cells of the anterior horns are alone involved to the exclusion of the adjoining parts. The grey substance is, in fact, severely affected throughout the whole extent of the anterior horns, and the change can be traced as far as the base of the posterior horns, and perhaps even in these parts. This alteration in the grey substance is characterized by increase in the number of nuclei, a proliferation of the interstitial tissue, and very pro- 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. The absence of most of the nerve cells, and the atrophy of some others will be observed : on account of the fact that a large proportion of the nerve fibres in this part have disappeared the deep colour which Weigert's hsematoxylin gives normally to the anterior horn is not produced (vide fig. 238) ; on the other hand this deep hue is produced in 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 itself, and without the use 462 LECTURES ON DISEASES OF THE SPINAL COED. of the microscope, to justify the diagnosis of amyotrophic lateral sclerosis in a living patient. Eig. 240.— Section of the spinal cord (lumbar region) in a case of amyotrophic lateral sclerosis. The part affected by sclerosis seems to be almost entirely seated in the crossed pyramidal tract, being represented by a clear triangle, which exists in the lateral colnmn. (h) In addition to the degeneration in the direct and crossed pyramidal tracts lesions undoubtedly exist in the other parts of the antero-lateral column. You remember, gentlemen, that when we studied the second- aiy descending degenerations of the spinal cord I remarked that when the degeneration was due to a transverse lesion the extent of sclerosis in the region of the crossed pyramidal tract was far greater than when this effect was due to a lesion seated in the cerebral hemisphere. In amyotrophic lateral sclerosis the same is the case, and the islet of sclerosis in the region of the crossed pyramidal tract extends far beyond the limits of that column. The fact is, that in the same way as when descending de- generation occurs after a transverse lesion of the cord, so also in amyotrophic lateral sclerosis changes exist which extend to the extra^pyramidal poi'tion of the antero-lateral columns. I would add that the seat of these changes is at times somewhat analogous in the two cases. This is to say, gentlemen, that the lesions in other parts of the antero-lateral column are specially seated in the portion of that tract which is intermediate between the grey matter of the anterior horns and the surface of the cord. The sclerosis pro- duced by them is, on the one hand, less pronounced, and on the AMYOTROPHIC LATERAL SCLEROSIS. 463 other less extensive than when degeneration occurs after a transverse lesion as already mentioned ; for all these reasons it Fig. 241. — Section of the spinal cord (the dorsal region) in a case of amyotrophic lateral sclerosis. The parts affected by sclerosis are whiter in colonr in propor- tion to the. degree of sclerosis. B. Sclerosis in the region of the crossed pyramidal tract ; T>. sclerosis in the intermediate regions of the antero-lateral column ; C. sclerosis in the region of the direct pyramidal tract ; A. sclerosis affecting GoU's columns to a slight degree. L. Lissauer's tract, which, althongh of grey colour in the the diagram, is quite free from disease. 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 GoU'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 464 LECTURES ON DISEASES OF THE SPINAL CORD. presents when coloured by carmine, or by the yellowish-brown tint, which it assumes when coloured by Weigert's hsematoxylin. This condition of GroU's columns is mentioned by many observers, and in 1885 Charcot and I myself specially noted it in connexion V Fig. 242. — Section of the spinal cord (cervical region) in a case of amyotrophic lateral sclerosis. The parts affected by sclerosis are whiter in colour in pro- portion as they are more severely involved. B. Sclerosis In the lateral column, extending far beyond the limits of the crossed pyramidal tract, above which a transverse band (inore dark in colour) of sound tissue is found, and then, between this and the direct pyramidal tract C, wldeh is much diseased, the whole of the antero-lateral column is affected by sclerosis to a slight degree ; A. sclerosis of moderate intensity in the region of Goll's columns. with two autopsies in which it was more clearly found to exist. I am aware, gentlemen, that in spinal cords which are free from disease Goll's columns may often be distinguished from the rest of the posterior columns by their special hue, but in that case there is no appearance of sclerosis, whereas, on the contrary, the existence of this condition is obvious in amyotrophic lateral sclerosis. I must add that in this disease the change in Goll's column is in no way accompanied by the production of granular bodies. II. — Medulla oblongata. We iind changes of the same kind in this part as have already been found to occur in the spinal cord. A. Changes in the grey matter. — The motor regions are specially altered in this part, but not all of them, since, as you AMYOTROPHIC LATERAL SCLEROSIS. 465 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 possess. Amongst these nuclei it is that of the hypoglossal nerve in which change is most often found to have occurred (atrophy and disappearance 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 condiiion (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 number 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. Muratoff 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 should be observed that in some cases lesions in

, granular bodies seated in the middle part of the crus. These granular bodies existed also in the crus upon the right side but were not indicated in order that the diagram might be more simple (half dia- grammatic). seated in the part through which the fibres of the pyramidal tract pass; the zone which they occupy is usually of very small extent. IV. — Crura cerebri. When the morbid process extends to this region it again affects the part through which the fibres of the pyramidal tract pass, that is to say, the middle part of the lower portion of the AMYOTROPHIC LATERAL SCLEROSIS. 467 crus. The lesions of the grey matter in this part, if they exist, are also unknown. V. — Brain. M. KoschewnikofF first noted the existence of changes in the internal capsule and white substance of the motor convolutions. The Moscow professor showed in fact, by means of a series of patient investigations, that granular bodies were found in some- what large number in the two parts which I have just named. A short time afterwards (December, 1883), having 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 these 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. b. 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 nerTons centre is placed in MuUer's fluid at the temperature of the surrounding air during from 10 to 20 days. (During the last part of this period Muller'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 loss due to evaporation.) "When the part is sufficiently consistent (it must not be really hard, since when this is the case disaggregation of the sections is apt to occur) it is placed during from 15 to 30 minutes in a, somewhat thick solution of gum arable ; after this time it is placed upon a microtome adapted to frozen tissues, and sections are made which are received into a vessel containing Muller's fluid, which is diluted and has been boiled some hours previously and then allowed to cool. A few sectionB are then separated and placed in a vessel containing boiling water, Muller's fluid and the gum with which they are impregnated being thus removed from them. The sections are then placed upon slides and may either be examined at once or, what is preferable, be stained by one of the following processes ; — A. — Mount the section in glycerine coloured by the QuinolcSiue (prepared by dissolving the bleu de Quinoleine in a few drops of alcohol and mixing it with glycerine, the Pierre Marie and Huet process) ; at the end of a few minutes it is covered by the cover glass, and under the microscope the granular bodies are found to be of a deep blue colour, which is clearly distinct from the turquoise 468 LECTURES ON DISEASES OF THE SPINAL CORD. lesions of lateral sclerosis are found in the brain, and not in the spinal cord alone). a. Changes in the wJiite siAstance ; this consists in the exist- ence of numerous granular bodies disposed in radiating series like the fasciculi of fibres in that part, and directed from the centrum ovale to the cerebral cortex; in sections parallel to the large axis of the motor convolutions this disposition is very clearly seen, and it may be supposed that these series of granular bodies take the place of the nerve fibres which are afiected by degeneration. /8. The change in the grey matter which I noted in 1883, and of which Charcot and I reported a fresh example in 1885, consists in atrophy of the large pyramidal cells of the cerebral cortex. In the two cases, in fact, to which I allude the section of the motor convolutions showed a much smaller number of these cells in the cortex of the motor convolutions than in a healthy person ; in addition to this the large pyramidal cells which remained presented the appearance of being aifected by atrophy. A short time later Koschewnikoff also stated in one of the cases of amyotrophic lateral sclerosis which he had observed the existence of these changes in the large pyramidal cells of the cortex. Such, gentleman, are the lesions of the nervous centres which blue tint of the rest of the section. This oolonr, however, is not retained for an indefinite time. B. — The section having been withdrawn from the water, and placed upon the slide is coloured by carmine, and after being washed, the slide is turned round so that the surface upon which the section rests is directed downwards • it is then placed upon a small cup or watch-glass which contains a few drops of a solution of osmic acid, and left exposed to the osmic vapours until it turns of a slightly brown colour ; it is then again washed and mounted either in glycerine or Canada balsam. By means of the latter process of colouring the section, which I have employed since December, 1883, permanent specimens are obtained. It consists in reality of the same elements as the " colouring of Marchi " (1885) = fluid of MUller + osmic acid. The difference between these two methods consists in the fact that in the plan which I recommend a section of the frozen tissue is made which is only coloured subsequently, whereas in the method of Marchi the section is hardened and coloured simultaneously by the combined action of the bichromate and osmic acid. I readily admit that the latter method is the most rapid and easy for those persons who are not accustomed to the freezing process, but on the other hand I believe that the plan which I recommend is not exposed to the same causes of error and should be preferred in certain examinations. AMYOTROPHIC LATERAL SCLEROSIS. 469 exist in amyotrophic lateral sclerosis. How may these be con- nected together and their existence be explained ? After what has been said of the preference which these changes show for the whole length of the pyramidal tract this Fig. 244. — Section of a oonvolution (motor region) in a case of amyotrophic lateral sclerosis. This section was made in a direction parallel to that of the fibres of the white substance. Black granulations are observed in the white substance disposed in diverging linear series. The granulations represent the granular bodies which resulted from degeneration of the nerve fibres. A, Grey matter ; B, white substance. 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 varolii, and medulla oblongata as far as the spinal cord. At the same time the motor cells of the cord are aifected 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 470 LECTURES ON DISEASES OF THE SPINAL CORD. are by no means strictly confined to the seat of the direct and crossed pyramidal tract. In order to explain the extension of the lesions to other parts of the antero-lateral column, it has been said : 1. That fibres are afiected which have been separated from the pyramidal tract. 2. That the additional fibres which are affected are commissural fibres of which the degeneration was secondary to lesions in the grey matter. 8. It may again be supposed that these fibres belong to a system which is analogous to those of which we have studied the degeneration in the antero-lateral column after transverse lesions in the cord. 4. As regards the opinion which I formerly held that the exten- sion of the lesion is due to direct propagation of the inflammation to difierent parts of the cord, this I hasten to retract. The truth is that we know nothing certain about this point ; in other respects the existence of these extra pyramidal islets of sclerosis cannot be regarded as proving that columnar degenera- tion of the pyramidal tract throughout its whole length is im- possible. In certain cases, however — and this is more serious — the lesion of the pyramidal tract, instead of existing throughout its whole length from the motor convolutions to the spinal cord, only occurs in the cord and ceases in the medulla oblongata, the crura cerebri containing no traces of degeneration. It might be supposed that in these cases the change in the large pyramidal cells of the cortex and the secondary degenera- tion, which occurs in consequence in the pyramidal tract, are first indicated by the alteration in the lower part of those fibres (intra-medullary portion of the pyramidal tract) ; this, however, is really most improbable and quite different from what we are accustomed to observe in cases of hemiplegia with secondary degeneration ; in these, on the other hand, the changes are seen to extend from above downwards in the same direction as the fibres of the pyramidal tract. Clinical observation again shows us that the affection may as well follow a descending (onset in the bulb, secondary affection of the upper and lower limbs) as an ascending path (onset in the limbs, secondary affection of the medulla oblongata). Lastly, gentlemen, it seems to me impossible that a fasciculus of fibres can degenerate of its own accord and independently of the cells from which it takes origin (trophic cells), nor do I AMYOTROPHIC LATERAL SCLEROSIS. 471 believe that the pyramidal fibres can be altered in an inter- mediate segment of their path. It seems so impossible to explain this difficulty that in my 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 to 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 case. Before ending these remarks upon the nature 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 insane, to which I have just alluded, and in which degeneration of the pyramidal tracts, which is limited to the cord, is observed to occur (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. * I cannot admit, as some authors have maintained, that amyotrophic lateral sclerosis, progressive muscular atrophy, and the different forms of myopathy are varieties of one process alone, rings, as it were, of the same chain. Important differences exist between them while very few analogies bring them together. 472 LECTURES ON DISEASES OF THE SPINAL CORD. The description, of the lesions which occur in that disease will now be continued, especially those which occur in the peripheral pait3 of the neuro-muscular system. The nerve fibres which compose the anterior roots are usually affected by atrophy to a somewhat pronounced degree. As regards the trunks of the motor nerves which supply the wasted muscles, their lesions seem in complete discordance with the changes in the anterior horns on the one hand, and on the other with the alterations in the muscles, which are the con- sequence of these lesions ; in some cases, in fact (Kronthal), these nerve trunks are apparently quite unaffected, although the muscles supplied by them may have in great part dis- appeared. It should be observed, gentlemen, that as Kronthal has remarked, this discordance seems only to exist as regards the nerve trunks of medullary origin ; in the nerves connected with the bulb, on the contrary, very pronounced changes occur, changes which are quite analogous to those observed in the bulbar nuclei, and in the muscles which they innervate. What is the cause of this singular anomaly ? A fresh problem exists which is no less insoluble than those which have already been men- tioned.* The small iatra-muscular nerves, according to Babes and Marinesco, are affected by sclerosis to such an extent that they are no longer, so to speak, constituted by a solid cord or fibrous tissue ; thej'' contain at the same time fibres which are very narrow and fibres of large size in varying numbers. The muscles involved in the morbid process are usually affected by simple atrophy; it must not, however, be supposed that all their fibres disappear at the same time, since by the side of some fibres which are diminished in size others exist which have retained their normal dimensions. In some cases again It is an abuse of general pathology to join together what olinioal features have so clearly separated. On account of the failure of the different theories which have been proposed to explain the origin of amyotrophic lateral sclerosis I should be somewhat inclined to believe that vascular changes have some effect, in connection specially with the grey matter, and producing secondary changes in the white substance j it may again be supposed that one or several anatomical systems in the cord or medulla oblongata are primarily " dissolved " (??). * Joffroy and Achard also recognized in one case an obvious want of corre- spondence between the degree of change in the cord and that in the peripheral nerves. {Arch, de Med, Experim. et d'Anat, Path., 1890, II. 434.) AMYOTROPHIC LATERAL SCLEROSIS. 473 slight lipomatosis exists between the different fasciculi, this tendency being far more pronounced in the tongue than in other parts. Diagnosis.- — The clinical features of amyotrophic lateral sclerosis are so numerous, that it will be well to consider them separately when considering the affections which simulate this disease in making the diagnosis. A. Diagnosis of amyotrophic lateral sclerosis of the form in which muscular atrophy occurs as regards other kinds of amyotrophy : — (ft) The amyotrophy is combined with diminution or loss of the tendon reflexes : Progressive muscular atrophy of Buchenne-Aran (?) ; Muscular atrophy supposed to be due to peripheral neuritis ; Myopathies. In 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 each other ; the seat of the atrophy, the age at which the onset occurs, the presence or absence of fibrillary contractions, the electrical reactions, &c. (6) The amyotrophy is combined with excessive tendon reflexes. 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, &c.). Cervical hypertrophic pachymeidngitis is also often accompanied by muscular atrophy in the upper limbs, and excess of the tendon reflexes; but not only are bulbar symptoms 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- 474 LECTURES ON DISEASES OF THE SPINAL CORD. men, to recognize the existence of lesions in the joints in order to avoid any mistake being made in consequence of the excess of the reflexes. B. Diagnosis of amyotrophic lateral sclerosis of the bulbar form, as regards other affections in which similar symptoms occur : — Acute bulbar paralysis is distinguished by its more abrupt onset, more rapid course, by the absence in most cases of any very pronounced symptoms connected with the limbs, and the exaggeration of the tendon reflexes. Pseudo-bulbar paralysis of cerebral origin is not accompanied by amyotrophic symptoms, but most often by a varying degree of hemiplegia affecting one or both sides ; in this form of dis- ease the pharyngeal reflex is usually much diminished or lost, whereas in true bulbar paralysis (amyotrophic lateral sclerosis) it is retained. The analogies between subacute or chronic bulbar paralysis and amyotrophic lateral sclerosis are of the remotest kind, and, in my opinion, need not be discussed at greater length. At the same time I must warn you against the opinion of Ley den, according to which a form of bulbar paralysis exists which is associated with lesions of the pyramidal tract, but at the same time is quite distinct from amyotrophic lateral sclerosis. These cases in reality belong purely and simply to the latter disease, and to separate them is to inflict an injury both upon clinical medicine and pathological anatomy. jSjTIOLOGY. — Though amyotrophic lateral sclerosis is not, properly speaking, a rare disease, it cannot be included within those which we observe daily ; this will partly explain, gentle- men, some of the doubts which are connected with it. The age at which it supervenes is variable, since the onset occurs at any age between 25 and 50 years, or to be more exact it may be said between 35 and 50 years ; if in fact I base my observations upon cases which I have myself seen, it should be said between 35 and 45 years. It is thus a disease which occurs in the second part of adult life ; thus you should really mistrust, gentlemen, the so-called cases of amyotrophic lateral sclerosis in which the onset occurs in adulescence or even in childhood. The female sex seems more liable to this disease than 'the male, but without its being possible to state the exact proportion. AMYOTROPHIC LATERAL SCLEROSIS. 475 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 aifections 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, gentlemen, if the empty sound of a word will satisfy us, the power of stating that amyotrophic lateral sclerosis is a disease of involution. What shall I say of the treatment of amyotrophic lateral 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. INDEX. Abnormal sensations in tabes ; tingling and numbness, &o., 170 — 178. Abortive forms of spastic paraplegia, not rare, 94; of insular sclerosis, 125; diagnosis of, 126; one symptom exists in an isolated manner, 126; or predominates much over the other, 126. Absence of knee-jerk, possible in insular sclerosis, 129; the case being then difficult to distinguish from one 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. Absorption of the osseous material near the Haversian canals in tabes, 216. ■Acute adtjlt spinal paralysis, 420. This affection is quite analogous to infantile spinal paralysis previot sly studied, and occurs when childhood is ended, 420; the symptoms, setiology, and pathological anatomy are so identical with those of acute infantile paralysis that the two affections should be considered simultaneously, 421 ; works written abont the origin of acute adult spinal paralysis, 421 ; onset of adult spinal paralysis, 421 ; symptoms of the disease, 421 ; paralysis, 421 ; and period of regression, 421 ; amyotrophy, 422. 'Acute intoxication, as by chloroform, may prevent the patellar tendon reflex from occurring for a time, 189. Adult spinal paralysis, quite resembles the infantile form, and occurs when child- hood is ended, 420 ; the symptoms, aetiology and pathological anatomy are identical with those of acute infantile paralysis, 421. Advanced cases of tabes, the lesions in, 338. .aetiology of insular sclerosis, 131 — 134. .S)tiology of tabes, 298 — 300 ; the only true cause of tabes is syphilis, a fact first stated by Fonrnier in 1876, 300 ; Erb confirmed this idea, 300 ; number of tabid patients in whom syphilis exists, 300—303 ; aetiology of combined lateral and posterior sclerosis, 399 ; of infantile spinal paralysis, 429. Age at which the onset of tabes occurs, 228 ; age of onset of Friedreich's disease, 385 ; at which most cases of combined lateral and posterior sclerosis occur in adults, 399 ; age at which adult spinal paralysis occurs, 420 ; age at which infantile paralysis occurs is the earliest infancy between 1 year and 18 months. Age most affected by insular sclerosis, 132 ; age and sex specially affected in amyotrophic lateral sclerosis, 474. Alterations in spinal cord in amyotrophic lateral sclerosis, 459 (in the grey matter, 459 ; white substance, 461 ; lesions of the pyramidal tract, 461 ; and antero-lateral column, 462). Alteration in the size of the pupil in tabes, 199; mydriasis, myosis, 199 obliquely oval shape, &c., 199. Amblyopia in insular sclerosis noted by Charcot to be always more excessive than lesions in the optic papilla, 115. 478 INDEX. Amputation of a limb, secondary degeneration after, 62 ; apparent difficnlty in its occurrence, 62, Hayem's opinion (1875) ; seats of the atrophy aa supposed by Dickinson and Tulpian (1868) ; work of Priedlander and Krause, 63. Amyotrophic lateral sclerosis, 447 — 175. Charcot remarked that sclerosis existed in the lateral columns at some autopsies, 447 ; symptoms are indicated by the name of the disease, 447 ; ttus lateral sclerosis indicates not only paralysis, but also spasmodic symptoms, while the term amyotrophic indicates the occurrence of progres- sive muscular atrophy, 447, 448 ; to these spastic symptoms tremor has been known to occur in the limbs when voluntary movements are made, a mitigated form of the spasmodic tremor which exists in insular sclerosis, 449 ; variety of spastic symptoms in amyotrophic lateral sclerosis, 449 ; paralysis exists in this disease, 450 ; in addition to wasting, 450 ; and contracture, 450 ; muscular atrophy in the bulbar nerves, 452 ; symptoms connected with the bulb, 452 ; the muscles of the lips are sometimes involved early in the disease, 452 ; fibrillary contractions, 453 ; other symptoms of the disease, 453—456; course, onset, 457; and duration of the disease, 458; causes of death in the disease, 458. The name enumerates the symptoms and lesions which exist in this disease, 459, lateral sclerosis, changes in the grey matter of the anterior horn, alterations in spinal cord in this disease, 459 (changes in the grey matter, 459 ; and white substance, 461 ; the lesions of the pyramidal tract and antero-lateral column, 461) ; condition of GoU's columns, 463 ; medulla oblongata, 464. A. Changes in the grey matter, 464; B. Changes in the white substance, 465; pons varolii, 466; crura cerebri, 466; brain, 467; diagnosis of this disease, 473 ; aetiology, 474 ; age and sex specially liable to this disease, 474 ; if the empty sound of a word will satisfy us the complaint may be termed a disease of involution, 475 ; treatment, 475. Amyotrophy, 119, 120 ; may be the predominating symptom in abortive insular sclerosis, and be followed by bulbar palsy, 129 ; these oases may resemble amyotrophic lateral sclerosis and thus cause an error in diagnosis, 129 ; the course is but relatively progressive in tabes, changes rarely extending to more than one group of muscles, 254. Amyotrophy in adult spinal paralysis, 422. Amyotrophy, may be associated with secondary degeneration of the pyramidal tracts 27 ; hemiplegia may cause it to appear in the joints of the hand, 28 ; and of the arm and lower extremities, 28 ; supposed causes of, 28 ; Charcot recognized a lesion in cells of anterior cornua, 28 ; an explanation confirmed by Carri^re, Hallopeau, Pierret, Pitres, Brissaud, Ac, 28 ; Quincke, Eoth, Muratow, Darkschewitsoh, observed similar cases while Dejerine in 4 cases of atrophy, after hemiplegia found peripheral neuritis without lesions in the cornua or anterior roots, 28, 29. Ansemia of the spinal cord, diminishes the patellar tendon reflex, 189. Analogy between infantile spinal paralysis and infantile hemiplegia due to a cerebral cause, 443 ; both probably being of an infectious character, 444. Anatomical and clinical existence given to insular sclerosis by Charcot and Vulpian in 1866, 103. Anatomy of the posterior roots, 321, 322 ; 3 groups of fibres contained in them, 322. Angina pectoris not infrequently occurs in tabes, 272. Anidrosis, or absence of perspiration, 249 ; may also occur in tabes upon one or both sides, 249. Anterior column, 33 ; some fibres affected by descending degeneration after transverse lesions of the cord, 33 ; fibres of anterior and lateral columns, pathology of, 34 ; origin of, 35. INDEX. 479 Anterior horn— reduced in size in the Inmbar region in amputation of lower extremity, 72. Antero-lateral column, 30, its composition, 30; supposed by Vulpian to be specially affected by atrophy, 71 ; seemed smaller than on same side after amputation of thigh, 74. Antero-median artery passes into the anterior horn upon one side of the cord, explaining why the lesions in infantile paralysis are nearly always unilateral, 441. Apoplectiform or epileptiform attacks, in insular sclerosis, 123 ; in hysteria, 130 ; apoplectiform attacks in tabes may be of a mild or severe form, 291 ; being probably due to haemorrhage, softening, some process of long duration in the meninges or ependyma, or vasomotor disorders due to change in the medulla oblongata or pons varolii, 291 ; such lesions are probably connected with syphilis, 292. Area of traumatic degeneration of Sohiefterdeoker, 50. Argyll-Robertson, pupil of, does not exist in tabes, 314. Arthropathy of left hip-joint in tabes, 222 ; other example in hip and knee-joint, 223, 224 ; frequency of arthropathic cases, 228 ; according to Erb 2 in 56 cases, 228. Articulation of words difficult in Friedreich's disease, 376. Artifices of Fonrnier to disclose the inoo-ordination in insular sclerosis, 160, 161. Ascending degeneration after transverse lesions of spinal cord, &c., 50 ; observed after division of half the spinal cord in dogs and cats, 77. Ascending degeneration consecutive to lesions of nerve roots, 41 ; et seq. Ascending degeneration in the posterior column, 50. Ascending root of the lateral mixed system, or slender column, in tabes, 283. Astasia-abasia, in the diagnosis of tabes, 310. Asymmetrical action of right and left pyramidal tracts, 10. Ataxic gait not precisely the same as that due to cerebellar disease in abortive insular sclerosis, 129. Ataxy of the vocal cords seems rare in tabes, 281. Ataxy, the existence of, constitutes Friedreich's disease when slowly progressive, 368 ; occurring at an early age, 368 ; and sometimes in many of the same ^family, 368 ; increasing difficulty in walking follows, 368. Atrophic changes, which are pronounced, may exist behind the globe of the eye, 188. Atrophy of cerebral convolutions, is not within the scope 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 tongue on the same side, 282. Auditory organs, frequency with which they are involved in tabes, often during preataxic period, 204. Aural vertigo (Meniere's disease) in tabes occurs in two groups, 205. It differs from M^nifere's disease, properly so-called, in not occurring in true paroxysms, 205, as stated by Pierrot, Charcot, &c., 205. Autopsy, difference in after one month or 5, 10, or 20 years, in infantUe paralysis, 434. Awkwardness of movement greatly contributes to the spastic paresis existing in insular sclerosis, 106. Axis cylinders are retained, which affects the whole history of insular sclerosis, 145 ; this many authors have verified, 145, 146 ; as may be done by the micro- scope, 146. Babinski, at an autopsy at which amyotrophy was associated with degeneration of cord found no lesion incornua, cord, or nerves, 28, 480 INDEX. Band of Eeil, 36, 45. Barbacci, 0., after division of half the spinal cord in the dog and cat observed ascending degeneration of the posterior column on the opposite side, 77. Bathing them presses the legs closely together in spastic paraplegia, 95. Beohterew, observations on development of pyramidal tract in animals, 11. Bilateral degeneration of pyramidal tract exposes the troubles on the sound side in hemiplegia, 27. Bladder and rectum, usually unaffected in spastic paraplegia, 93. Blastoderm, external layer the origin of the nervous system, 41 ; neural plate, 41, 42. Blood vessels are often found at the centre of the islets, 149 ; close to which the most serious changes in sclerosis occur, 149 ; cardiac changes in tabes, 272. Bone changes possibly due to peripheral neuritis in tabes, 218. Bone lesions in tabes found to be precisely those found at the commencement of arthropathy as if fractures and arthropathy were similar pathological condi- tions, 218. Bones most liable to suffer from spontaneous fracture in tabes, the femur, the leg below the knee, the bonep of the forearm, 212 ; these are the bones which are most exposed to injury, 213. Boumeville, Guerard, the memoir upon insular sclerosis appeared in 1869 ; 103. Brain, focal lesions of in tabes, 348 ; disappearance of nerve fibres from convolu- tions, 348 ; as possibly occurring in general paralysis of the insane, 348, 349 ; is according to Jendrassik the seat of the most important lesions in tabes, 350 those in the spinal cord being not improbably due to secondary degeneration caused by the lesions in the brain, 351. In my opinion the cerebral lesions have no direct connection with sclerosis in the cord, 352. Brissaud, inaugural thesis of, about degeneration in spinal cord, 15 ; affection of sound limbs in a hemiplegia patient suffering from contracture, 24 ; opinion as to cause of amyotrophy being associated with degeneration of pyramidal tracts, 28. Bulbar symptoms, in insular sclerosis, 120; viz., difficulties connected (a) with deglutition, (A) with mastication, &c. , 240. Burdach, posterior and exterior portion of column that from which the indications of ascending degeneration most quickly disappear after transverse lesion of cord, 51 ; anterior part remains changed over a longer portion of its path, 51. Burdach, rest of column of, extent to which sclerosis exists in it, &c., 337, 341. Canal, central, changes occurring in from tabes, 346. Canals, Haversian, absorption of the osseous material near the, 216. Carri&e, opinion as to a lesion in the anterior cornua following degeneration of the pyramidal fibres, 28. Cases described by Nonne in which symptoms occurred resembling those in Friedreich's disease, 381. Causes of death in amyotrophic lateral sclerosis, 458. Cells of ganglia of spinal cord are centres of origin, 46 ; cells in grey substance of cord or bulb centres of termination, 46. Cerebellar affections in the diagnosis of tabes, 310. Cerebellar peduncles, their degeneration after removal of part of or the whole cerebellum, 35. Cerebello-spastic form of insular sclerosis, 104. Cerebellum, supposed by Marchi to contain the trophic centre of fibres in the antero-lateral columns, 37. Cerebral disorders in Friedreich's disease, 375. Cerebral lesions followed by degeneration of pyramidal tract, 15 ; necessary seat of lesion in brain, its destructive nature, 16. INDEX. 481 Cerebral rheumatism seemed to Charcot to be followed by insular sclerosis, 135. Cerebral symptoms in insular sclerosis, 121 ; in Friedreich's disease, 375. Cerebral tumour often indicated by vomiting headache, character of optic disc, and thus distinguished from the faltering speech, &c., of insular sclerosis, 128. Changes supposed to exist in the nerve filament which enters the nutritive orifice of the tibia which is to suffer from spontaneous fracture, 217 ; Siemerling having observed an analogous case, 217, 218. Character in spastic paraplegia always capricious and odd, 94. Charcot and Bouchard, use cf secondary degeneration of pyramidal tract in its description by them, 2. Charcot, opinion of that bilateral degeneration of crossed pyramidal tracts may occur after lesion in one cerebral hemisphere, 26 ; opinion of regarding cause of amyotrophy accompanying degeneration of pyramidal tracts, 28, 29. Chemical changes in the bones in tabes, 217. Chorea, cannot be mistaken for insular sclerosis, 127. Choreiform movements in Friedreich's disease, 373. Circulation in the cord, causes which affect it, 404 ; I. Extra-medullary branches, 404 ; II. Intra-medullary branches, 406. Clarke's columns, effect of division of posterior roots in Cauda equina upon Clarke's columns, 49 ; the origin of the direct cerebellar tract, 65 ; their structure, 55 ; how they end, 56 ; a lesion in the cells produces ascending degeneration in the fibres of the direct cerebellar tract, 56 ; smaller after amputation of a limb, 72 ; healthy condition of the cells in Clarke's columns in tabes should be joined with the corresponding state of the direct cerebellar tracts of which these cells are the trophic centres, 345. " Claw-like " attitude of hands in tabes, 252. Club-foot, tabid club-foot, not to be confused with tabid foot, the former being due to atrophy of the muscles of the leg, the latter to osteo-arthropathy, 255. Collaterals, emergence of, 12 ; formation of, 35. Columns of Clarke, vide Clarke's columns. Combined Lateral and Posteeiob Sclbeosis. The symptoms of this disease attract less notice than its pathological anatomy, 394 ; Westphal first mentioned it, 384 ; works written upon this subject, 394, 395. A. In the posterior columns: degeneration of columns of GoU and Burdach, 395. B. In the lateral columns : the crossed pyramidal tract not wholly involved, 396 ; direct pyramidal tract usually unaffected in combined lateral and posterior sclerosis, 396 ; direct cerebellar tract always affected, 396 ; Gowers' ascending antero-lateral tract usually less affected, 396. In the grey matter cells of anterior horns may be affected, and the same may be said of the posterior horns, 397 ; certain cells at times suffer from atrophy in this disease, 397 ; symptoms of tabid nature may exist in this disease, viz., inco-ordination (awkward movements, ataxic gait and symptom of Romberg, &c.), 397 ; conjointly with which excess of the knee-jerk, foot clonus, paresis or paralysis, cramps, &c., may exist, 398. Diagnosis of disease as regards [a) tabes, [b) the principal forms of spastic paraplegia, 399 ; aetiology of the disease, 399 ; most oases occurring in adults between the ages of 20 and 40. I. The classification is much the same as that formed by Ballet and Minor, and contains four groups, 400; (a) some forms of general paralysis of the insane, (4) systemic sclerosis of the columns of Goll, columns of Burdach, and direct cerebellar tracts, 400. 482 INEEX. Combined Lateral and Postbriob Sclerosis {continued) — II. Some of the lesions in the diffuse form of myelitis may be associated with sclerosis in the lateral columns, 402 ; being really a diffuse form of sclerosis alone, 402. III. B^jfuse wyelHis may give rise to ascending and descending secondary degeneration, the lesions being then in the columns of Goll or crossed pyramidal tract, 402. IV. Certain forms of diffuse sclerosis seem to indicate the existence of this disease, a group which might well be termed false combined lateral and posterior sclerosis, 402. Comma-shaped degeneration of the posterior columns, 38; Barbacci denies its existence, 38. Commissural cells, described by Eamou y Cajal in many points of the grey matter, 35. Confirmed ataxia is only recognized by obserrant patients in tabes, 291. Congenital spastic rigidity of limbs. Little's name for spastic paraplegia, 85. Connections of crossed and direct pyramidal tracts, 18. Contraction of the visual field, 202 ; opinions of different authors as to its nature, 202 ; the author's opinion coincides with that of Berger that no form of con- traction is characteristic of tabes, 202. Contracture may occur in 2 lower limbs when the tendency to spasm is increased in degeneration of pyramidal tract, 24. Convulsive attacks, probably epileptic, and not due to the disease in spastic paraplegia properly so called, 97. Cornil, confirms observations about degeneration of spinal cord, 15. Cornu commissural zone is the name given to commissural fibres joining the grey substance at different heights, 323. Cortex of brain, the changes in this part after the onset of tabes, 353. Course of insular sclerosis, 124, &c. ; variations in, 125 ; of tabes, 293 ; of Fried- reich's disease, 379. Crepitation may occur in the diseased joint at the onset or subsequently in tabes, 221. Crisis, gastric, usually an early symptom in tabes, 268 ; is liable to recur and gradually to diminish and cease, or to persist during the whole disease, 268 ; death at times occurs during the collapsed condition of one of the crises, 268 ; abnormal form,s of the crisis, 269 ; its duration, 269. Crossed pyramidal and direct cerebellar tracts affected by sclerosis would indi- cate a " vascular form of sclerosis," according to Dejerine andLetulla, while others opposed this thesis, 391 ; arguments used by these opponents, 391. Crossed pyramidal tract, length of, 6 ; seat of, 6, 7 ; passage of fibres from one side to the other (according to Charcot), 26 ; according to Hallopeau, 26. Crura cerebri, in amyotrophic lateral fclerosis, 466. Crus cerebri, seat of pyramidal tract in, 6. Cruveilhier, first observed atrophy of pyramid upon one side in hemiplegia, 15. Darkschewitsch, observed amyotrophy with degeneration of pyramidal tract with- out lesion in the cord or nerves, 2:j. Deafness, mode of ascertaining the cause of in tabes, 204 and note. Death, causes of, in insular sclerosis, 125. Debove supposes that the appearance of the limb in tabid arthropathy is due to rupture of the capsule and effusion into the articular cavity, 220, 221. Decussation of the pyramids, 6, 9, 10. Degeneration at central extremity of amputated nerve involves sensory fibres alone and those whose function is afferent, according to Friedliinder and Krause, 77. INDEX. 483 Degeneration of neryes, change in, after amputation, 64. Degeneration of pyramidal tract, 15, &o. ; vide pyramidal tract, degeneration of ; of cerebellar peduncles, 35. Degeneration of the nerves and spinal cord after amputation of a limb, 62 et s(q., 71 et seq. Descending degeneration conEecutive to transverse lesions of the cord, 30 et seq. ; may affect fibres in the antero-lateral columns, 30 ; or posterior column, 37. Descending system of suloo-marginal zone, effect of degeneration upon, 34. Destruction of the sheaths of myelin, within the islets in insular sclerosis, 145 ; those outside being sound, 145. Development of pyramidal tract in animals, 12 ; in the human embryo, 12. Diagnosis is not always of typical form in insular sclerosis, 130. Diagnosis of amyotrophic lateral sclerosis, 473, 474. Diagnosis of combined lateral and posterior sclerosis as regards (a) tabes, (6) the principal forms of spastic paraplegia, 399. Diagnosis of infantile spinal paralysis as regards birth-palsy, 426 ; syphilitic pseudo-paralysis, 427 ; myopathic atrophy, 427 ; hysteria, 429. Diagnosis of spastic paralysis only made later when movements are not found to be free, 95. Diagnosis of tabes, 306, &c. ; of Friedreich's disease, 380. Diagram : if the diagram of a section of the normal spinal cord in which the course of the vessels is indicated be placed over one representing a section of the cord affected by combined lateral and posterior sclerosis, the parts affected by sclerosis (combined lateral and posterior) are fonnd to correspond to the parts which receive blood from the system of the posterior spinal artery, 407, 408. Diffuse sclerosis occurs at a later age (40) than insular sclerosis, and does not present the classical symptoms of insular sclerosis, 152. Dignat, afieotion of sound limbs in ahemiplegic patient suffering from contracture, 24 ; diminution of muscular force (Dignat), 24. Direct cerebellar tract, its degeneration, 53 ; its origin, pathology, and termina- tion, 53, 55 ; affected from its origin in Friedreich's disease, 387 ; viz., from lower part of dorsal region to medulla oblongata, 387 ; always affected in combined lateral and posterior sclerosis, 396. Direct pyramidal tract usually unaffected in combined lateral and posterior sclerosis, 396. Direct pyramidal tracts 8 — 12 ; lesion in, said to produce more pronounced paralysis than one in the crossed tract, 13 ; while according to Gowers the function of the direct tract is specially connected with the movement of the upper limbs, 14. Discovery of Friedreich's disease, 369. Disease of Graves is not infrequently associated with tabes, 273. Disorders of general nutrition in tabes, 207 ; most suffer from consumption or medullary phthisis, as it used to be termed, 207. Disorders of sight in insular sclerosis, their difference in form and intensity, 117. Disproportion between the slightness of the injury and the fracture of the bone in the " spontaneous fractures " of tabes, 210. Disseminated ; this word indicates that the islets are of small size in insular sclerosis, 141. Dissociated anaesthesia, 175. Division of peripheral nerves, secondary degeneration after, 62. Double vision is the natural consequence of such ocular paresis, if pronounced, occurring in certain positions of the eye, and corresponding in fact to the paresis producing it, 114. Drink : how the patient is able to drink in insular sclerosis, 108, 109. 48t INDEX. Daration of insular sclerosis very variable (1—20 years according to the form of the disease), 125. Dyschromatopsia, as regards red and green exists in one form of insular sclerosis •which can only be recognized by the photometer, 117 ; none exists in another form, 118 ; dyschromatopsia, said by Charcot to be pronounced in insular sclerosis, 118, being analogous to that condition when it occurs in tabes, blue and yellow persisting longest, 202 ; whereas in hysteria the red does so, 118. Dystrophy of the nails sometimes occurs, 247; whiohpresentthe same appearance as in some cases of eczema or psoriasis, 248. Early age : the early age of the patients must be regarded as the true cause of the pronounced changes which occur in infantile disease, 417; not the paralysis and atrophy which affect the muscles, 417 ; but the atrophy of the bones, 417 ; the • paralysis is flaccid, the tendon-reflexes lost in both extremities, and the condition of the extremities, 417. Eddies composed of long fibrils in Friedreich's disease, seen microscopically, 390 ; lesions similar to those observed in epilepsy, 390 ; considered by Chaslin as due to the neuroglia being affected by sclerosis, 390 ; as of a glioma, 390. Edwards, Blanche, Miss, description of hemiplegia in insular sclerosis, 107. Efferent motor nerve : any lesion in tabes prevents the motor impulse from being carried to the muscle and produces cessation of the reflex, 188. Electric current, effect of, in tabes, 177. Electrical irritability much diminished, the response to the electric currents by the muscles being otherwise unchanged in tabes, 253. Embryonic transformation of the medulla in bones in tabes, 217. England : in this country many, and amongst them Gowers and Waller, believe the patellar tendon reflex to be an example of idio-musoular contraction, 192 ; reasons for this belief, 192. Epidemic nature of infantile spinal paralysis, 430, 431. Epiglottis : the epiglottis may be much lower in position in tabes, 277. Epiphora in tabes, 198. Equilibrium in the upright position, becomes the object of special attention on the part of the patient in tabes, 159 ; sight is important enabling any bad position to be rectified, 159; the swaying movements which occur when a screen is interposed between the eyes and feet are obviously due to a similar cause, 159. Eqninus is a position often assumed by the feet from contraction of the gastroc- nemii in spastic paraplegia, 87. Erb was the first to describe the phenomenon of patellar tendon reflex, 192 ; and to indicate that it was a tendon reflex, 192 ; whilst Westphal showed its absence in tabes, which has been termed the sign of Westphal, 192. Errors possible in detecting and observing the patellar tendon reflex, 182. Excessive tendon reflex, its causes, 190. Exophthalmos in tabes, 198, 273. External bandlets, and zone of Liasauer, the initial seat of lesions in tabes, 363. Extremities of the bones present in tabes aspects which may be referred to two large types : — A. The atrophic type, 231. B. The hypertrophic type, 232. Eace, position of part of pyramidal tract destined to the face in the internal capsule, 5. Facial nerve, unilateral paralysis is often glosso-labial hemispasm of hysteria, 166. Faltering speech may be the predominating symptom in insular sclerosis, with or without nystagmus and optic neuritis, 128 ; symptoms which often indicate a cerebral tumour, 128. INDEX. 485 Fatigue has the same effect as age, in tabes, upon the patellar tendon reflex, causing it to cease when trembling of the limb occurs from fatigue, 189. Feet often cross each other in spastic paraplegia, 88. Fibres of posterior root, short, 42 ; of mediumlength, 42, and long, 44. Fibrillar contractions usually but not always absent in the muscular atrophy of tabes, 252; usually existing when amyotrophy occurs early in the disease alone, 253 ; their presence in amyotrophic lateral sclerosis, 452. Field of vision, normal in insular sclerosis, 117. Final condition and death in Friedreich's disease, 379 ; recovery being unknown, 379 ; though remissions may occur, or sudden aggravations, 379. Fleohsig re-opened question of secondary degeneration of pyramidal tract in cord, 2 ; section of in brain, 5 ; mode of performing, 4, 5 ; tract of, 53 ; middle zone of — shown by Strlimpell to correspond to a small fusiform tract of fibres in a healthy condition, 840. Focal lesion in the grey matter of the cord, may it possibly be associated with changes in the posterior columns, when paraplegia occurs suddenly in tabes, 165, 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 may occur upon sound side, when the tendency to spasm is increased in degeneration of the pyramidal tract, 24 ; when a child advances in spastic paraplegia, 87 ; often so pronounced as to occur spontaneously whenever the ball of the foot rests upon the ground during the efforts to walk in insular sclerosis, to which the condition presents an additional obstacle, 104. Forms of amyotrophy in tabes, 264; what varieties of amyotrophy are contained in each, 264. Forms of tabes, superior or cervical form, 295 ; cerebral form, and mild form of Charcot, 296 ; tabes dolorosa, 296 ; severe form, 296 ; morbid processes, pro- ducing the severe forms, 296; consumptive tendency often produces the severity, or the coincidence of general paralysis of the insane with tabes, 297. Fournier, artifices of, to disclose the inoo-ordination in insular sclerosis, 160, 161. Fracture, all or nearly all the bones in tabes are liable to fracture, 218. Fractures of the vertebrae may occur in tabes, 214. Frequency of insular sclerosis, 132. FriedlSnder, affect of upper limbs in a hemiplegic patient suffering from con- tracture, 25. Friedbeich's disease, histoet, 368—370. A. Motor disorders constitute the chief group of symptoms, 371 ; disorders of the gait, 371, &c. B. Sensory disorders may exist, but are rare, 374. C. The disorders of the reflexes, 374. D. Disorders in the organs connected with the senses, 375—382 ; nature of the disease, 382. This is a disease which tends to affect many members of the same family, 383 ; Friedreich noticing this proposed the name of hereditary ataxy, 384 ; aetiology, 383—385 ; age of onset, 385 ; sex most affected, 385. Patholooical anatomy, 385 ; this branch is the least advanced, 385 ; the tenuity (graoilit^) of the spinal cord should be first mentioned ; it can be recognized by the naked eye, 385 ; possible causes, 386 ; microscopically sclerosis of the cord can be recognized, 386. A. In the posterior column. Degeneration of the columns ofGoll, 386 : these lesions disappear in the lower part of the medulla oblongata, 386 ; the columns of Burdach also involved throughout their whole extent, but in varying intensity according to the region of the cord, 386. 486 INDEX. Feiedkeioh's disease, histokt, 3£8— 370 (continued) — B. Direct cerebellar tract, affected from its origin, 387 ; viz., from lower part of dorsal region to medulla oblongata, 387; C. lateral colnmrs, 387 ; D. marginal zone of Lissaner, lesions in, 389 ; grey matter in the cord, different changes in, when this disease exists, 389; peripheral nerves, 390; crossed pyramidal and direct cerebellar tracts affected by sclerosis would indicate the " vascular form of scleros^'s," according to Dejerine and LetuUa, while others opposed this thesis, 391 ; arguments used by these opponents, 391 ; other supposed meaning of the eddies, 391. Many of its symptoms exist in insular sclero^tis, 130; viz., the nystagmus, slowness of speech, disorders of gait, and tremor when voluntary movements are made, &c., 130; in Friedreich's disease the parts are relaxed, being affected by spasm in insular sclerosis, 130 ; the tendon reflexes are diminished or lost in Friedreich's disease, 130 ; first case recognized in France, 370. Its diagnosis as regards tabes, insular sclerosis, and the chorea of Syden- ham, 380, 381 ; this is a disease which tends to affect many members of the same family, 383 ; Friedreich noticing this proposed the name of hereditary ataxy, 384 ; it seems unconnected with syphilis, 381 ; at times it follows an infectious disorder, such as measles, scarlatina, or smallpox, and it is pro- bably connected with an infectious disease of childhood, 384. Fundus ocnli, changes are frequent, but moderate in degree in insular sclerosis, 118 ; the visual loss due to them being usually slight in degree, 118 ; changes in fundus oculi dissimilar in insular sclerosis and tabes, 129. Fiirstner, degeneration of the pyramids of cord produces diminution in size of anterior cornua upon the same side, 29. Gait, difficulty in maintaining the upright position was perceived in Friedreich's disease by Friedreich and termed static ataoci/, 373 ; whilst the head if affected gives rise to a long series of slightly pronounced movements of salutation, 373 ; lateral curvature is usually observed in the confirmed disease, 379; disorder in the gait or of the fpeech or sclerosis being usually the first symptom observed, 379. Gait, disorder of, in insular sclerosis, 103; form, clearly spastic in character, 103 ; consists of paraplegia with tonic contraction and strongly marked rigidity, 103 ; the spastic gait already described to occur in spastic paraplegia and other diseases, 103 — 105 ; disorder of the gait may predominate, 128 ; characters of, 128 ; one form of gait is cerebellar in insular sclerosis, 129. Gait, in Friedreich's disease resembles that due to cerebellar disease and to tabes, besides which, choreiform instability exists, 373. Ganglia connected with the nerve roots after amputation of the thigh, 70. Ganglia of the spinal nerves, mentioned by many authors, have never been fully described in tabes, 347 ; but some lesion appears to exist in them, 348. Gangrene in tabes towards the end or beginning of the disease, 246 ; from the presence of some micro-organism, 246, 247. Gastric crises in insular sclerosis, 119. Gastric crises in tabes, 265—269 ; certain facts given by Fournier, 266; on the pain and vomiting, 266 ; sudden onset and abrupt cessation, 268. General conditions in tabes, some producing diminution and some cessation of the patellar tendon reflex, 189. General conditions which may make the patellar tendon reflex excessive are of different kinds, 190; strong emotions, as music, may have this effect accord- ing to Lombard, 189. General paralysis of the insane, distinctions from insular sclerosis, 128. INDEX. 487 Generative system in the male, 286 ; (a) impotence, {b) genital excitement, 286; (c). reflexes connected with the genital organs, viz., cremasteric reflex, its description, 287; bnlbocaverncsus reflex, 288; atrophy and anaesthesia of the testis, 288.' Derangements connected with the genital organs in the female, 289 ; alm-ost analogous to those in ma'es, 2E9; (») genilal depresEion, ^£9; (A) genital excitement, 289; the clitoridean criies, 289; (c) pains in the genital orgars, 290. Genital disorders are a complication in insular sclerosis, being a mott important symptom in tabes, 129 ; genito-urinary disorders in tabes, 309. Genito-nrinary disorders in tabes, 376. Giving way of the legs in tabes, 161. Glycosuria in insular sclerosis, enumerated in two memoirs by Eichardiere and Miss Blanche Edwards, 120 ; may occur in tabes, 249, and other changes in the composition of the urine, 249. Golgi, the corpuscles of, 184 ; connection of nervous filaments in the corpuscles with the muscular fpindles of Kuhne, or with the Pacinian corpuscles 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 fibres which form it, 51 ; pass to the nucleus of the column of Goll, 51 ; degeneration of column of Goll not always the same, 51 ; when affecting only the inferior roots of the cauda equina 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 the pre- ceding case, 52. The sclerosis of these columns is directly due to the degeneration which occurs in the long fibres, 363 ; degeneration of in combined lateral and posterior sclerosis, 395 ; condition of these columns, 463. Gowers', ascending antero-lateral tract little affected in combined lateral and posterior sclerosis, 396. Gowers, tract of, 53, 57, &c. ; its other name, 58 ; its origin, 58 ; pathology, 58 ; its termination connected with the nucleus lateralis, 58 ; other fibres, according to Tooth, passing into the cerebellum, 59; distinct from direct cerebellar tract, 59 ; by its later development, 60. Graniilar bodies are seen bclh in islets of old date and of recent formation, 144 ; in insular sclerosis, vide, "infectious disease after which sclerosis has appeared," 150. Grey matter of the cord, secondary descending degeneration within, 40. Different changes in, when Friedreich's disease exists, 389 ; cells of anterior horns may be affected in combined lateral and posterior sclerosis, 397 ; the same may be said of the posterior horns, 397. Gustatory organs, strange sensations of ta-te, a persistent taste of sugar, or complete has of taste may exist in tabes probally en account of some change in the nerves of taste, 206. Hallopeau, opinion as to how ihe crossed pyramidal tracts communicate, 26; opinion as to how the decussation of the pyramids may be accompanied by degeneration, 26 ; little tendency to produce diffuse inflammation around it when secondary degeneration exists in pyramidal tract, 27 ; opinion of, as to cause of amyotrophy accompanying degeneration of pyramidal tracts, 28. Head and upper part of trunk inclined forwards and moved as if inseparable in spastic paraplegia, 85. Hemiatrophy of the tongue, description of, by different authors, 258 ; present at times in tabes, it may occur without any connection with this affection, 258 ; description of, 258 ; all its movements still possible, except that of hoUowing 488 INDEX. itself into the form of a grooTed surface, 259 ; also observed in general paralysis of the insane and syphilis, 261, affecting the medulla ohlongata and pons varolii, 26 1 . Hemiplegia often observed in insular sclerosis, 106 ; hemiplegia, in tabes has been specially studied by Debove, 164; and Miss Blanche Edwards, 165, three forms in which it shows itself, viz., permanent, transient, and transient or permanent hemiplegia with anasstbesia in the skin and special senses, 165 ; as regards pathological anatomy a focal lesion may be found in the cerebral hemispheres or pons varolii (htemorrhage, softening), or possibly no appreci- able lesion, 165 ; as when hysterical hemiplegia is combined with tabes, 165 ; if there is loss of tendon reflex before hemiplegia, 165 ; it remains usually absent, 165 ; it may return and even be then excessive, 165 ; Charcot called attention to its existence, 103 ; one pronounced example having been described in the Thesis of Babinski, its characters described by Miss Blanche Edwards, 107 ; parts affected in hemiplegia, 107 ; crossed hemiplegia may occur, 107. Hemiplegic patients suffering from spasm, attitude of, 19 ; diminution between the length of time which passes between the blow being given and the movement, 19. Hereditary disease, spastic paraplegia not so, 96. Hereditary predisposition tends to produce tabes, 304. Hereditary syphilis may be the cause of tabes, 305. History of infantile spinal paralysis by Prevost and Vulpian, Charcot and Joffroy, Eoger and Damasohino, 413. History of tabes, 154 — 156 ; external bandlets shown by Charcot and Pierret to be first affected, 335 ; of Friedreich's disease, 369, 370. Hitzig's belief as to the cause of the contracture in hemiplegia, 19. Hyperassthesia (hyperalgesia) in tabes. Its character and form, 173; relative hypersesthesia, 173, 174. Hyperidroais, or increased function of the sudoriparous glands is not very rare in tabes, 249 ; it may be uni- or bilateral, 249. Hypoglossal nerve, changes in, when lingual hemiatrophy exists in tabes, 261, 262. Hysteria may be the predominating symptom in insular sclerosis, 126 ; may exist in insular sclerosis, 130 ; disorder of speech, diplopia may exist in hysteria, 130 ; its possibility should never be forgotten, or that it may be associated with insular sclerosis, 131 ; may be the cause of paralysis of different muscles in tabes, 167. Impairment of sight is often unilateral in insular sclerosis, 118. Impressions, loss of power to respond to in tabes, 177 ; the reverse of the " sum- mation" of impressions, 177 ; the sensibility being diminished by continuation of the same exciting force, 177. Inco-ordination may be taken to mean the failure to maintain the upright position, 159. Inequality of the pupil in insular sclerosis, 114. Infantile spinal pabaltbis. Names of the disease, 412 ; viz., essential paralysis of children, acute anterior polio-myelitis of children, anterior tephromyelitis of childhood, 412 ; infantile spinal paralysis described by Heine, 1860, and Rilliet and Barthey, 1883. History of infantile spinal paralysis by Prdvost and Vulpian, Charcot, and Joffroy, Roger and Damaschino, 413 ; symptoms of infantile spinal paralysis, 413 ; onset by gastro-intestinal derangements and fever, 413 ; symptoms associated with the nervous system, 413 ; paralysis, its character and extent, 414 ; the period of regression continues during one or two weeks, the motor nuclei of some but little affected regaining power, their paralysis being due to the shook of the disease, 415; when the nuclei of the muscles are destroyed, the muscles INDEX. 489 remain paralysed and atrophy occurs, 415 ; the period of deformities now commences, such as clnb-foot, club-hand and various deformities in the trnnk from simple scoliosis, 415 ; to what is termed by the expressive name of cul-de-jatte, 416 ; when neither the lower part of the trunk or lower extremities can be used by the patients to maintain the upright position or for locomotion, 416 ; this deformity is rare, scoliosis being more frequent, 416 ; the early age of the patients must be regarded as the true cause of the pronounced changes which occur in this disease, 417 ; not only the paralysis and atrophy which affect the muscles, 417, but the atrophy of the bones, 417 ; the paralysis is flaccid, the tendon reflexes lost in both extremities in the affected muscles, and the condition of the extremities, 417 ; changes in the electric currents in infantile spinal paralysis, the idio-muscular con- tractility is usually unaffected as regards sensibility, 418; reflex action in infantile paralysis, 419 ; trophic disorders which occur, 419 ; in the growth of the limbs subcutaneous adiposis, coldness of the paralysed limbs, colour and thinness of the skin, 419 ; callosities, 420 ; chilblains, 420 ; other changes in the skin, 420 ; fragility of the bones, 420 ; intellectual state owing to the life led and inherited tendency, 420. Onset often unperoeived, 423 ; but one limb is seen not to move, 424 ; nothing having occurred during the night, 424 ; onset during convalescence after an acute disease, 424 ; termination of the disease usually consists in paralysis associated with atrophy of those muscles which remain affected during the period of regression, 424; transient paralysis, 424; improvement may occur after some months or years, 424 ; paralysis possibly ends in death when the lesions are very extensive and seated in the upper part of the spinal cord or medulla oblongata, 424, 425 ; course and termination, 425 ; of the disease according to different authors, 425; such as the progress of amyotrophy, 425 ; in the muscles of the limbs or trunk, 425 ; second onset of amyo- trophy, 426 ; may be afterwards observed in the acute spinal paralysis of the adult, 426 ; diagnosis, 426 ; birth-palsy, 426 ; syphilitic pseudo- paralysis, 427; myopathic atrophy, 427; hysterical paralysis, 429; setiology of the disease, 429 ; usually depends upon the existence of an infectious disorder, 430; infectious diseases after which infantile spinal paralysis is liable to occur, measles, diphtheria, scarlatina, small-pox or whooping cough, 430 ; epidemic nature of the disease, 430 ; age at which the disease occurs, in earliest infancy between one and eighteen months, 432 ; sex, 432. Pathological anatomy of the disease, 433; atrophy of the anterior horns first stated by Provost and Vulpian in 1865, 433, 434 ; diminution of cells in them and diminished size of the lateral^ columns on the same side of the cord, 434 ; connection of amyotrophy and the lesion of these cells, 434 ; difference in autopsy after one month or 5, 10, or 20 years, 434 ; cells of Deiters, 435 ; antero-median artery passes into the anterior horn upon one side of the cord explaining why the lesions in infantile paralysis are nearly always unilateral, 411 ; normal and pathological anatomy show that it is through the vascular system that this infectious disease involves the spinal cord, 443 ; probably an infectious form of embolism or thrombosis occurs in one or several arteries of the anterior median fissure, 443 ; the anterior median and anterior radicular arteries are the only vessels affected by the morbid process, 443 ; analogy between the disease and infantile hemiplegia, due to a cerebral cause, 443 ; believed by the author to be both of an infections character, 444 ; character of the infectious agent cannot be stated, 444 ; recurrence of the infectious agent, 445 ; similar cases in other diseases, 445 : treatment of the disease, 445, 446. Inffectious diseases after which acute infantile spinal paralysis is liable to occur, are measles, diphtheria, scarlatina, small-pox or whooping cough, 430. 400 INDEX. Infectious diseases after which insular sclerosis has occurred, names of diseases, 135. Inferior cerebellar peduncle, possible degeneration of, 35. Inflammatory affections in foetal life, or during the first few dajs after birth, giving rise to meningitis or encephalitis interfere with the development of the pyramidal tract, and seem scarcely to have a place in this book, 97. Inflammatory process, when it diminishes in insular sclerosis the function of the nerves becomes almost restored, 147. Inheritance, that of tabes may be indirect, 305 ; epilepsy, hysteria, and other diseases may be found as Charcot says in the near relations of tabid patients, 305 ; as may diabetes, 305, and perhaps hereditary syphilis, 305. Inhibiting action of pyramidal tracts, suspension of, 23. Injury or pressure affect the pyramidal tract which is the last to develop, 97 ; probably due to infection, 135. Insular sclerosis in the diagnosis of tabes, 310 ; insular sclerosis how distinguished from spastic paraplegia, 152. Insulae scleeobis. Lesions of, 102 ; history, Charcot and Vulpian described it, 103 ; division of symptoms into spinal, cerebral and bulbar, 103 ; spinal symptoms, 103 j motor symptoms, 103 j (a) spastic gait, 103. The purely cerebellar form (/3) indications of, 104. The cerebello-spastic form (y) is the most frequent and usual form, the characters of the cerebellar and spastic form being associated together, 104. Symptoms, 104—110. Effects of the disease upon sensation, 111, &c. A. Common sensibility, 111 ; usually absent. 111, subjective disorders of sensibility (numbness, tingling, &c.), and at times pain. 111 ; of a somewhat diffused or lightning character. 111 ; or a sense of constriction round the trunk may eiist quite analogous to that which occurs in tabes, 112. B. Special senaihility, impairment of, 112. Atrophy of the optic nerve was noted by Charcot in some cases, 115; the amblyopia was also noted by him to be always in excess of the lesions in the disc, 115. Varieties of change in the visunljkld mentioned by Uhthoff, 118 ; dyschrom- atopsia mentioned by Charcot as sometimes pronounced in insular sclerosis, being analogous to that condition when it occurs in tabes, blue and yellow persisting longest, whereas in hjsteria the red does so, 118. The onset was found by Uhthoff to be sudden in half, and gradual in the other half of the cases, 118 ; the visual changes occur at different periods in the disease, 118 ; lastly they have at times been of an intensity parallel with that of the other symptoms of the disease, becoming more pronounced when the other symptoms increase in severity, 118, 119. C. ViscEEAL DI80EDEKS, 119 ; incontinence or retention of urine or foeces, impotence, or gastric crises, 119 ; seem but rarely to occur, 119. D. Trophic disorders, 119 ; changes in the rails, 119; sloughing in the glutccal region, &c., as complications, 119 ; amyotrophy, 119, 120. II. Bulbar symptoms: the following symptoms should be classed amongst those of bulbar origin, 120 ; difficulties connected (a) with deglutition, (i) with mastication, tremor in the tongue, &c., 120. Islets of sclerosis probably in the floor of the 4th ventricle, where a puncture causes the presence of sugar in the urine, unless diabetes really exists, 120 ; polyuria at times exists, being due to a similar cause, 120 ; bulbar, or so- called bulbar symptoms, except perhaps the tremor in the tongue, so rarely occur in insular sclerosis, that they need merely be mentioned, 120. KnDex. 491 Insulak sclerosis [continvd)— and spasmodic, 121; otservation of a patient speaking, 121; preparations to speak, 121 ; actual speech, 121 ; vertigo, 121 ; that of M&i6re's disease sometimes imitated by the vertigo, 122 ; mental charge, 122 ; attacks of spasmodic impulsive laughter, 122 ; apoplectiform or epileptiform attacks, 123 ; in which the temperature usually rises to 102° and 104° F., the reverse occurring in apoplexy due to cerebral disease, 123. Course, Diagnosis, iETioLOGT. Onset, 124 ; at times an attack of apoplexy, alone or complicated by hemi- plegia signalizes the onset, or at times, hemiplegia without apoplexy, 124 ; at times vertigo and giddiness, or sudden disorders of sight are the first symptoms, 124. Possibility of combined lateral and posterior sclerosis must be remembered, 129 ; spastic symptoms are then combined with certain symptoms of tabes, 129. The gait is cerebellar, 129. The predominating symptom is hemiplegia, 129 ; whether hemiplegia occur or not after an attack of apoplexy, the diagnosis between a focal ctrebral lesion and insular sclerosis may be, specially during the first moments after its onset, of great difficulty, 129. The predominating symptom is amyotrophy, which may or may not he accom- panied by lulbar palsy, 129 ; these oases, as mentioned, may resemble amyo- trophic lateral sclerosis, and thus give rise to an error in diagnosis, but are so rare as possibly never to be seen. In such a case the atrophy never attains a pronounced degree, or produces spasm in the upper limbs, or affects the chin, lips, or tongue, 129. Diagnosis oe the typical eobm. a. Friedreich's disease. Many symptoms of this disease exist also in insular sclerosis, 130 ; such are nystagmus, slowness of speech, disorders of gait, &c., 130. b. Eysteria. Vertigo, apoplexy, hemiplegia, intentional tremor, disorder of speech, diplopia may exist in hysteria, 130 ; the possibility of hysteria should never be forgotten, or the two diseases may be associated together, 131 ; aetiology of insular sclerosis, 131 ; frequency of insular sclerosis, 132 ; male sex suffers slightly more than female, 132 ; age most affected is the first half of adult life, viz., between 20 and 30 years, 132 ; after the age of 40 insular sclerosis need scarcely be considered, 132 ; it may, however, occur in child- hood, as shown by the authors, XJnger, Nolda, though rare at this time of life, 132 ; causes mentioned, 132 ; the truly effective cause, in my opinion, is infection, or rather infections, 133. Pathological anatomy, nature, treatment, 137, &c. Intelligence, disordered at times with fits, inability to sit, &c. , indicates condi- tions termed by author tabid spasmodic conditions, 96 ; their causes, 96. Intermediate tract of lateral column, 32, 37. Internal capsule, localization of pyramidal tract in, 5 ; limbs of, 5 ; knee, 5 ; tracts for face, tongue, limbs, 6. Interval, Tschirjew, in his observations upon spastic paralysis, showed that the interval of the tendon reflex was in this disease shorter than in healthy persons, 193. Intoxication by ergot of rye may produce lesions analogous to those of tabes, 304 ; also by fermented maize (pellagra), 304. Intra-mednllary afferent system proceeding from posterior roots, 41 ; anatomists who studied it, 41. 492 INDEX. Iodide of K. or Na. does much good in vascular sclerosis for the sclerotic element and should be administered for a long time in small doses, 153 ; mercury may be given as a disinfectant in the same way, 153 ; some similar disinfectant will probably at some future time totally prevent the evolution of insular sclerosis, 153. Iris, disordered action of, in insular sclerosis, 114; liability to many disorders in tabes, 199 ; persistence of accommodation reflex associated with loss of light reflex termed sign of Argyll-Robertson, 200. Islets, of sclerosis, probably in the floor of the 4th ventricle, where a puncture causes the presence of sugar in the urine, may produce glycosuria unless diabetes already exists, 120. Islets, primary, 67 ; of degeneration, 68, 69 ; evolution of, 68, 69. Jackson and Bastian believe the action of the cerebellum to be indispensable that the tone be maintained, which enables the tendon reflexes to be produced, 192. Joffroy and Brissaud were also interested in patellar tendon reflex, 192. Jurgens showed that in tabes all or almost all the joints, even when no arthro- pathy exists, present dilatation of the capsule and elongation of the ligaments, 234 ; most of them being in a condition of " virtual arthropathy," 234. Knee and thigh, swelling of, in tabes, lateral movements very pronounced, and can be at times produced by slight pressure of the hand upon the foot, 223 (fig. 127). Knee-jerk, increase of, may occur, when there is degeneration of pyramidal tract, upon the sound side, 24 ; the interval for it being longer than ^ to ,]g of a second (Jj to ig) (Brissaud), 193 ; other arguments support the theory that the knee and other tendinous phenomena are reflex in nature : (a) The loss of the patellar tendon reflex when the cord is an^mio (as from aortic compression), (6) Its loss in diseases during which the direct irritability of the muscles is scarcely modified at all (tabes, general paralysis of the insane), (c) Its production by the summation of irritations as long as the small shocks take place at least one in the second, (d) Its production produced by percussion of the periosteum, 193. Knee-jerk not absolutely lost in tabes, 314 ; completely lost in Friedreich's disease, 369. Knee phenomenon, Westphal's article on, 191 ; he believed contraction of the quadriceps to be due to the irritation produced in this muscle by the tendon which is struck, and in which all the fibres of the muscle end, 191 ; stating also that the muscle must be in a certain state of tore, the nature of which he does not explain, 191. Knoblauch and Fiirstner called attention to the fact that degeneration of the pyramids produced diminution of size in anterior cornua upon same side, 29. Lactic acid may be conjoined with hydrochloric acid in small but varying quantity, in the gastric juice in tabes, 267. Laryngeal and other disorders in the diagnosis of tabes, 309. Laryngeal paralysis, posterior cricoarytenoid muscles most often affected, 280 ; the thyro-arytenoid or lateral crico-arytenoid muscles may be paralysed but less often, and to a slighter degree in tabes, 280. Laryngismus, tabid, chronic symptoms of, 280. Larynx, tabid laryngismus, laryngeal crises, 277 ; authors who mentioned laryngeal crises, 277 j description of this condition, 277 ; laryngeal ictus, 278; descrip- tion of 278, 279 ; prognosis and frequency, 279 ; pathology, 279 ; treatment, 279. INDEX. 493 Lateral and posterior sclerosis (combined), possibility of, must be remembered in the diagnosis of insnlar sclerosis, 129 ; spastic symptoms are then combined with certain symptoms of tabes, 129. Lateral columns, and lesions of, in Friedreich's disease, 387. Lateral columns, crossed pyramidal tract not wholly involTed in combined lateral and posterior sclerosis, 396. Lateral limiting layer, seems not to degenerate in either degeneration, 61 ; but really degenerates, consisting of short commissural fibres, 61 ; and only within the zone of traumatic degeneration, 61. Lateral curvature in Friedreich's disease, 379. Lateral movements easily produced in the knee joint in tabes, example of, 222. Legs, extended and pressed against each other, and the feet adherent to the soil, so that the patient walks with difficulty in insular sclerosis, 103. Lesions in the tendons in tabes, 243. Lesions of insular sclerosis, 102. Lesions seen in a section of nerves stained by the haematoxylin of Weigert, 65 ; sound and diseased fasciculi, 65, 66. Lesions which accompany the clinical varieties of the fractures in tabes, 216 ; the worm-eaten appearance of some parts, or ulceration, 216. Leyden, comfirms observations about degeneration of spinal cord, 15. Limbs of internal capsule, seat of pyramidal tract destined to the internal capsule, 5. Lips, muscles connected with, sometimes involved early in the disease, 452. Lissauer, zone of, 325, 326 ; remarks by upon the lesions met with in tabes, 346. Locomotor ataxy of Duchenne of Bonlogne is now replaced by the term tabes, 154 ; notwithstanding the barbarism and solecism to which the use of this word forcibly condemns ns, 154. Long fibres of posterior roots, 44 ; their pathology, 44. Longitudinal commissural fibres, 35. Loss of patellar tendon reflex in tabes, 193 ; except in some cases when it persists although the signs of tabes are quite characteristic, 193 ; the lesions then most often occupy almost exclusively the upper part of the cord, 193 ; its preser- vation in these cases is not constant, on account of the fact that the external bandlets of the posterior column at the upper part of the lumbar region of the cord are unaffected, 194. Loss of the sense of posture, 157 ; of differences in weight, 157 in tabes in which disease a difference of J, J, or even more may not be distinguished, 157 ; plans to decide whether differences in weight are recognized, 157. Loss of teeth, 248 ; has been observed since 1?68, 248 ; description of 248 ; mechanism of, 248 ; or its dependence on trophic disorder, 248 ; or on periostitis and osteitis affecting the alveoli and teeth, 249. Lower limbs rotated inwards, and rigid in a state of slight flexion at the hip and knee-joints in spastic paraplegia, 86. Marginal tract, degeneration of, 34. Marginal zone of Lissauer, lesions of in Friedreich's disease,- 389. Mastication, unilateral paralysis of muscles of (Schnltze) occurs in tabes, 166. Medulla oblongata, atrophy of the nuclei of the vagus, glosso-pharyngeal, and spinal accessory nerves mentioned by many authors, 282 ; changes in the grey matter, 464 ; changes in the white substance in amyotrophic lateral sclerosis, 465. Mental condition, in spastic paraplegia, 93, 94 ; in insular sclerosis, 122. Mercurial tremor, a similar tremor may be the predominating symptom in insnlar 494 INDEX. Microscopical appearance, in insular sclerosis, islets seen clearly, as if almost punched out, with a slight magnifying power, 142. Monkey, duration after division of one of the posterior roots in such animals, of 3, 4 weeks or more, a small tract of degeneration is then found in the posterior column above the lesion, 46. Motor convolutions, origin of pyramidal tract in, 2. Movement, the extent of in insular sclerosis, and effect of emotion upon it, 109. Movements, involuntary in tabes, resembling those termed athetoid, 162 ; and muscular tremor occurs in some cases of tabes, 162. Muscle, the atrophic muscular, or other changes diminishing their contractility, first cause diminution and then cessation of the patellar tendon reflex, specially in idiopathic muscular atrophy, 188, 189. Muscles, number involved in spastic paraplegia, 89, 90 ; of face involved in ditto: 91 ; secondary lesions occur in the muscles in tabes, resembling those which exist in the muscular atrophy of tabes, 281. Muscular atrophy in tabes, 251 ; many authors published observations about it, 251 ; the term denotes muscular emaciation , flaccidity , and weakness, 252 ; true muscular atrophy will be now considered, 252. Muscular atrophy in the parts supplied by the bulbar nerves, 452 ; in amyotrophic lateral sclerosis, 452 ; symptoms connected with the bulb most important, 452. Muscular atrophy may be due to peripheral neuritis, the spinal cord, anterior roots, and large nerve trunks being unaffected, 256 ; according to many authors muscular atrophy is due to a lesion iu the grey matter of the anterior horn, consecutive, according to Charcot and Pierret, to extension of the change in the posterior columns, 256, 257 ; Condol^on found changes in the anterior comua, anterior roots, nerve trunks, and intra-muscular nerves, 257 ; the facts of hemiatrophy of the tongue are opposed to this, 258. Muscular exertion notably increases the knee-jerk, 190. Muscular exhaustion, paroxysms of, first mentioned by Pitres in 1884, have been at times met with since that time, 170 ; description of, by Pitres, 170. Muscular force diminished in upper extremity of sound side when degeneration of pyramidal tract occurs, 24. Muscular sense, disorders of, in insular sclerosis, 156, 157; usually unaffected in Friedreich's disease, 374. Musculo-spiral nerve, paralysis observed by numerous authors in tabes, 166. Musculo-tendinous bodies in close relation to the Pacinian corpuscles, 184 ; also found in the tendinous laminae, 184. Myelin, the cause of the clear appearance of the islets is the decided manner in which the sheaths of myelin disappear throughout the whole islets, whereas outside they are preserved in insular sclerosis, 142. Myosis, inequality of the pupils, &c., in insular sclerosis, 114 ; Parinaud believe.s that a nervous affection in which myosis with retention and perhaps excess of the light reflex, as regards the rapidity of its production, exists, should be regarded as insular sclerosis, rather than tabes, 115. Nature of Friedreich's disease (unknown), 382. Nature of tabes, 366 ; anatomically the lesions are merely the result of degener- ation which has occurred in the posterior nerve roots, 366 ; which is due to change in the cells of the spinal or peripheral ganglia, 3C6; tbe change in these cells being due to syphilis acting as a toxine, 367. Nerve, auditory, the excessive irritability to electric currents is not rare in tabes, 205, 206 ; and this is supposed to possibly explain the relative frequency of vertiginous sensations in some patients suffering from tabes in whom the ear is but slightly affected, 206, Nerve fibre, peripheral sensory coming from the spinal ganglion, 42. INDEX. 495 Nerve fibres in central organs differ from those in the peripheral nerves by not containing a sheath of Schwann , 145 ; being only composed of an axis cylinder and sheath of myelin, 145 ; that a nerve fibre may degenerate it must be separated from the cell from which it takes origin and which is its trophic centre, or the cell itself must disappear, 355. Nerves, changes in when degeneration occurs, 65; changes in nerves, their roots, nuclei, and the medulla oblongata, in tabes, 282. Nerves, peripheral, most pronounced peripheral neuritis, was first stated by Westphal (1878) in tabes, 348. Nervous affection in which myosis with retention and perhaps excess of the light reflex, as regards the rapidity of its production, exists, is probably insular sclerosis and not tabes, 115. Nervous system, origin of, 41. Neural plate of blastoderm divided Into three segments, from one of which the central nervous system, notably the motor portions, and a lateral segment whence the sympathetic and spinal ganglia are formed, 41, 42. Neurasthenic pseudo-tabes, 313 ; how to recognize it (?), 313. Neuroglial is more affected in insular sclerosis than in Friedreich's disease, 144. Non-correspondence between the number of sensations and that of the impres- sions, 176. Non-development of pyramidal tract the ordinary lesion in every case of spastic paraplegia produced by premature birth, Ac, 96. Normal and pathological anatomy show that it is through the vascular system that this infectious disease (infantile paralysis) involves the spinal cord, 443 ; probably an infectious form of embolism or thrombosis occurs in one or several arteries of the antero-median fissure, 443 ; the antero-median and anterior radicular arteries are the only vessels affected by the morbid process, 443. Nucleus of Burdach (or nucleus cuneatus) joined by long fibres of posterior root, 44. Nystagmus, important as regards the diagnosis of insular sclerosis, the movement occurring in a horizontal direction, though Uhthoff has once seen the move- ment occur in a, vertical direction, 113; in two cases quoted by Uhthoff the patients thought that they saw the objects move, 113. Nystagmus in Friedreich's disease, 375. Ocular disorders may exist in insular sclerosis, 129 ; in Friedreich's disease, 375. Ocular muscles, paralysis of, frequent, 166 ; paralysis of organs external to the globe of the eye in tabes, 195 ; in Friedreich's disease, 375. Ocular paralysis, in the opinion of oculists may be due to a peripheral or central cause of nuclear origin, in tabes. This however is doubtful, 198. Ocular tone, diminution of, in tabes, 199. Olfactory organs, disagreeable odours or anosmia may exist, being probably due to a lesion of the olfactory nerves special to tabes, 20B. Onset dates from time of birth in true spastic paraplegia, 94 ; in insular sclerosis, sudden in half, gradual in half the cases, 118 ; the onset may occur in an attack of apoplexy alone or complicated by hemiplegia, 114 ; other forms of onset occur in different ways, 124. Onset of tabes : this may occur by ocular paralysis, 294 ; or some alteration in the micturation, or genital functions, or perhaps the larnygeal or gastric crisis, &c., 294. Ophthalmoplegia, external, may occur, but the 6th and 3rd nerves are prin- cipally affected in insular sclerosis, 114. Ophthalmoscopic appearances of the visual disorders in tabes, 202 ; the papilla is at times of a uniform red colour, and the nasal portion of the papilla becomes usually of a somewhat grey colour, instead of being more red than the temporal portion it becomes of almost the same hu3, 203. 496 INDEX. OpMhalmosoopio changes, remark of Uhthoff as to their frequency in insular sclerosis, 115. Optic nerve, acute atrophy of, well marked in insular sclerosis, 118 ; islets are analogous to those in the brain and cord, 147. Optic nerve, affected in 10 or 20 per cent, of the cases of tabes being more frequent in those who have previously suffered from paralysis of the ocular muscles, 201. Optic nerve [continued) and acuity of vision unaffected in Friedreich's disease, 375 ; and no pupil symptoms exist, 375. Optic neuritis is only produced according to Uhthoff in insular sclerosis, owing to the existence of recently formed and extensive islets in the optic nerve im- mediately behind the globe of the eye, 148. Optic neuritis is rare in cases of some years' duration, being usually found during the prseataxic period, 201 ; Gowers states that he has never seen it as the first evidence of tabes, 201. Optic papilla, different degrees of alteration occurring in the optic papilla according to Uhthoff in insular sclerosis, 115. Ordenstein, thesis of, upon insular sclerosis, 103. Organs, external to the globe of the eye in tabes ; paralysis of external muscles very frequent occurring in 39 per cent. (Moeli, Berger), and according to Gowers 80 per cent, of the cases suffering from tabes at some time in the disease, 195. Origin, that of the primary interstitial process in the islets of sclerosis is in the blood vessels, 149 ; which is not surprising after what has been said of the infections nature of insular sclerosis, 149. Osteoplasts, changes in, in tabes, 217. Pain is far more pronounced in tabes than in insular sclerosis, 129. Pain reflex, irritation produced by pain causes the pupil to dilate momentarily in tabes, 201 ; though the reflex is often lost at an earlier date in tabes, than the light or accommodation reflex, 201. Pain often occurs in paroxysms, 169, between which the patient is at times free from pain, 169. Palpebral opening, narrowing of, in tabes, 199. Paraesthesia in tabes, 174. Paralysis agitans may be the predominating symptom in insular sclerosis, 126. Paralysis exists in amyotrophic lateral sclerosis, 450 ; in addition to wasting, 450. Paralysis is one of the motor symptoms in tabes, 163 ; due to inoo-ordination, 30 ; that the dynamometer often indicates numbers representing great muscular strength, 163 ; not only inoo-ordination, but independently of it certain paralytic symptoms may appear, 164. Paralysis, merely the effect of the cerebral lesion, not indicating degeneration, 18. Paralytic symptoms in Friedreich's disease, 373. Paraplegia may exist in the lower limbs in insular sclerosis, 106 ; extensor spasm of the lower limbs being usually associated, 106. Paraplegia spastica cerebralis, name given by Heine to spastic paraplegia, 85. Paraplegia with sudden onset may occur in tabes, perhaps the first symptom noticed by the patient, 165, 166. Patellar tendon reflex, generally obtained by the students, though few arc interested in what it really means, 180 ; description of, 180 — 188. Pathological anatomy of insular sclerosis, 137, &c. ; from macroscopic point of view, affected parts present, changes in the meninges, 137 ; and nervous centres, 137 ; islets in cerebral hemispheres, 138 ; irregularly placed, and INDEX. 497 fissures, 141 ; they may occupy any part of the cerebro-spinal axis rarely occupying the large ganglia of the brain or olivary bodies, 141. Pathological anatomy of tabes, 321 ; infantile spinal paralysis, 433 ; atrophy of anterior horns first stated by Prevost and Vulpian, 433, 434 ; diminution of cells in them, 431 ; connection of amyotrophy with the lesions of these cells, 434. Pathological anatomy of tabid arthropathy, 230, and loose bodies in the articula- tion, 230 ; of Friedreich's disease, 385, 886, &c. Pathology of the reflex may be compared with the practical jokes or games of children, 191. Patients suffering from tabes in whom arthropathy has existed for some time, 221. Perforating ulcer which may occur, 2 J4 ; mild or perforating form, 245 ; seats of, . 245. Period, prodromic of tabes, 293; the ataxic do., the period of confinement and that of paralysis, 293 ; the prodromic period that in which the lightning pains occur, 293 ; often thought rheumatic, 293. Periosteal reflex in tabes, 193. Peripheral sensory nerve fibres and posterior root fibres, trophic centre of, 42. Peripheral nerves in Friedreich's disease, 390. Persistent pain in tabes, as the " girdle pain " (said in one case by Fournier to be so severe as to produce dyspnoea) or the feeling of a bracelet, or " gaiter," or painful sensations in the trunk, 170. Pharyngo-laryngeal system in tabes, A. Pharynx, 276 ; B. Larynx, 277. Physical influences in tabes which may modify the sensibility, the anaesthesia being increased by cold, whilst heat, the electric current, &c., cause its effect to cease for a time or diminish its intensity, 177. Pierret, opinion as to the cause of amyotrophy accompanying degeneration of pyramidal fibres, 28. Pitres, bilateral degeneration of pyramidal tracts after a unilateral cerebral lesion, 24 ; investigation of as to degeneration of the two crossed pyramidal tracts due to a unilateral cerebral lesion, 25. Pitt, believes Friedreich's disease to be due to some defect in the development of the cord, 392, Plantar reflex, like many other cutaneous reflexes, may be preserved during a long period or lost, 194 ; in the latter case more or less anaesthesia often exists in the sole of the foot, 194 ; this being the case, the presence or absence of this reflex is of slight importance in connection with the diagnosis of tabes, 194. rneumogastric and spinal accessory nerves, no changes found similar to those in the nucleus of the hypoglossal nerve in tabes, 262. Polysesthesia, explanation of, 176. Polyneuritis, in rare cases much excess is observed (Strilmpell and Mohius), 190. Polyuria may exist from a similar cause, as diabetes in insular sclerosis, 120. Pons varolii, path of pyramidal tract in, 6 ; in amyotrophic lateral sclerosis, 466. Posterior column, in tabes, 335 ; the external bandlets, 336 j the column of Goll, 336. Posterior columns in Friedreich's disease, 386. Posterior columns, secondary degeneration occurs in it, 37 ; comma-shaped de- generation of, 88 ; posterior commissure, 323 ; to which is given the name of anterior zone of the posterior oohmms, 323 ; fibres affected by degeneration through the whole breadth of the posterior columns, 38 ; and at a lower point more at the posterior part, 38. Posterior horn, anatomy of, 329 (vide fig. 165, p. 327) ; posterior horn properly so called, 329 ; and columns of Clarke, 331 ; one would expect the grey matter in this horn to be involved in the tabid lesions, as many authors have maintained. 498 INDEX. Poaterior root fibres, division into 1, short fibres ; 2, fibres of moderate length ; 3, long fibres constituting the columns of Goll, 362, 363. Posterior roots, contain three varieties of fibres described by Singer and Munzer, 42 — 45 ; their description, 42 — 45. Posterior roots, which part of the cord usually suffer from their atrophy in tabes, 347 ; a certain number of nerve fibres being microscopically found to suffer thus, 347 ; the cervical nerves suffer like the poaterior roots, 347. Pott's disease as distinguished from spastic paraplegia, 99. Preparations to speak in insular sclerosis, 121. Profession of thoae affected by tabes, 293. Professions most liable to tabea, 308. Prognosis in insular sclerosis, 125. Psychical derangements in tabes, 292 ; frequently connected with general paralysis of the insane, 292 ; mental condition often good, 292. Ptosis in tabes, 198. Pupil symptoms, absence of in Friedreich's disease, 375. Purely cerebellar form of insular scleroaia, 104. Pyramidal cells in cortex, partly or wholly the origin of the pyramidal tract, 2. Pyramidal ti;aot, anatomy of, 1, et seq. ; origin of term, 2, &o. ; secondary degenera- tion of utilized in works of Tlirck, Charcot and Bouchard, 2 ; Flechsig's description of, 2 ; course of, 2, 3 ; seat of in internal capsule, 4 ; in cms cerebri and its relations therein, 5 ; seat of in pons varolii, 6 ; in medulla oblongata, 6 ; decussation of the pyramids, 6 ; crossed pyramidal tract, 6, et seq. ; direct pyramidal tracts. 8, et seq. Degeneration of consecutive to cerebral lesions, 15, &c. ; Cruveilhier observed such degeneration of the pyramid upon one side, but not further into the cord, Turck discovered degeneration of pyramidal tract in the cord in, 1851 — 55, 15 ; other allusions to such degeneration, 15 — 30. Pyramidal tract, fibres have their trophic centre in the cerebral cortex, 31 ; degeneration of, has a special character according as a lesion in the brain or cord is the cause of its existence, 31 ; degeneration may affect the fibres of the crossed, and direct pyramidal tract, 32. Eaoe, Jewish, have a special immunity as regards tabes, 307. Rapidity with which the nervous impulse passes in tabes is not exactly known, 193. Reaction of degeneration usually absent when healthy and diseased fibres are associated in tabes, 256. Recovery, when it occura, the sheaths of myelin which had disappeared in the islets of sclerosis, and the nerves may return to their normal condition, 147. Rectum and bladder, affection of, in spastic paraplegia, 93. Recurrence of sensations in tabes, 175. Recurrence of the amyotrophic process in infantile paralysis, 445 ; similar cases in other diseases, 445. Reflex action, disorders of, in tabes, 179 ; reflex movements may be obtained throughout the whole cord, and even by means of the brain, so much re- sembling each other that they may be classed together, 179 ; such are the knee-jerk, that of the tendo-Achillis, wrist, elbow, &c. ; as to the cutaneous, plantar, and abdominal reflexes, these are specially tested, the others being less considered, 179 ; description of certain reflex movements by Erb in 1875, attention being paid to that which takes place in connection with the knee, 191. _ Reflex action (patellar) was shown in the Salp6tri6re Hospital by Charcot scon after the works of Westphal and Erb were published, 192. INDEX. 499 Eelative anaesthesia in tabes, 174. Retardation of sensations in tabes, 174, 175. Rigidity of leg preventing flexion of the knee in insular sclerosis, the progression of each foot is accomplished by elevation of the corresponding half of the trunk and pelvis, 103, 104. Root zones of the posterior columns, &c., 323 ; anatomy of, 323, 324, 325. Rubbing of the foot against the ground in insular sclerosis produces a noise enabling the character of the gait to be known afar off, 104. Rupprecht mentioned works of Little, 85. Rupture of the tendons in tabes, 243 ; examples of the rupture, 243, 244. Sohiefferdeoker, " zone of traumatic degeneration of," 30. Sclerosis, after amputation of a limb, 75 ; if in an irregular form and disseminated, the diffuse form exists, and the type of sclerosis in foci, 143. Seats of pain in tabes, 168, 169, 170. Seat of sensations in tabes, inability to recognize, 175. Seats of tabes, viz., the lower limbs, abdominal viscera or cervical enlargement (cervical tabes), or in the cranial nerves (cephalic tabes), 366, &c. Seated position in spastic paraplegia, 88. Second period of tabes, 294 ; the ataxy of movement is almost always confined to the lower limbs, 294; and several years pass before the upper limbs suffer, 294 ; visceral symptoms and the ocular affections may now be observed, 294. Secondary degeneration of pyramidal tract, 15, &c. ; time of indication noticed by Pitres, 17 ; secondary degeneration of the posterior roots in the spinal cord, 46 ; not associated with insular 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 sclerosis. 111 ; usually absent, 111 ; and at times pain of a somewhat diffused or lightning character, 111, or a sense of con- striction round the trunk may exist analogous to that occurring in tabes, 112 ; objective disorders according to Freund, 112, do not form part of the clinical features of insular sclerosis, 112; they should be regarded as a clinical curiosity, 112. Sensory disorders and lightning pains are absent, 369. Seyisort/ disorders in Friedreich's disease, 373 ; are rare, 374 ; though pain, anses- thesia and analgesia may exist, 374. Sensory metamorphosis, 175. Seneobt symptoms amenable to objective contkol in TiBES. (a) Anses- thesia (analgesia). Its occurrence, and consequences, 171; accidentally recognized to exist by the patient, 171 ; its seat, 171 ; its intensity, 172. Sensory symptoms of a purely subjective nature in tabes, 168. Sensory tetanus, 176. Sex, male suffers more than female in insular'sclerosis, 132 ; most Uable to tabes, 228, 306 ; the latter sex as supposed being less Kable to syphilis, 307. Sex most affected in Friedreich's disease, 385 ; in infantile spinal paralysis, 432. Sheath of Schwann, rupture of, in degeneration, 69. Shedding of the nails, 247 ; often observed in tabes, 247 ; mode of their being shed, 247 ; a batch of ecchymosis appears beneath the nail before it is shed, usually without pain, 247. Shooting, wrenching, or burning pains in tabes, 169. Short fibres of posterior roots, 42. Special sensibility in insular sclerosis, 112. Sight, disorders of, frequent in insular sclerosis, and often much accentuated, 112 ; 600 INDEX. Small object, if held in hand and the eyes closed daring the existence of tabes, the object is at once dropped, usually without the patient perceiving it, 161. Soft palate, paralysis of one side of the soft palate may co-exist with hemiatrophy of the tongue, and corresponding inferior vocal coVd, 260. Softness of the brain of newly-bom children causes it to be specially affected by injury or pressure, 97. Spasm often produced by secondary degeneration, 18 ; cause of in secondary degeneration, 19 ; Brissaud's mode of showing the cause, 20 ; Vnlpian's belief as to the cause, 21. Spasmodic impulsive laughter in insular sclerosis, 122. Spastic paraplegia, history of discovery, 84; names given to, 84; age of its occurrence, 84 ; symptoms in childhood, 84 ; to which the name spasmodic tabes dorsalis should be applied, 85 ; other names, 85 ; symptoms, 85— 98 ; gait, 85—87 ; muscles involved, 89 ; difficulties in deglutition and disorders of spefeoh, 90 ; disorders of other muscles, 90 ; reflexes, 92, 93 ; common sensibility and muscular sense unchanged, 93 ; electrical irritability and vaso-motor disorders, 93 ; affection of rectum and bladder, 93 ; mental con- dition, 93 ; character odd, tendency of disease to improve, 94 ; cannot walk until age of 18 months or 2 years, 95 ; disease not hereditary, 96 ; non- development of pyramidal tract, the ordinary lesion in every case, 96 ; distinction from so-called tabid-spasmodic conditions, 98 ; diagnosis, 99, &c. ; treatment, 101. Spastic paresis, often observed in upper limbs, in insular sclerosis, 106. Spastic symptoms, to which tremor is at times added in amyotrophic lateral sclerosis when voluntary movements are made, 449, being a mitigated form of spasmodic tremor which exists in insular sclerosis, 449 ; variety in the intensity of spastic symptoms in amyotrophic lateral sclerosis, 449. Speech, disorders of, frequent in spastic paraplegia, 90 ; in insular sclerosis, 121 ; observations of a patient speaking, 121. Spinal cord, changes in, perceptible with the naked eye in tabes, 335 ; changes at the onset, 335 ; changes may occur throughout its whole length from the filum terminate to the medulla oblongata and even higher, 347. Spinal ganglia, form trophic centre of peripheral nerves and posterior columns of the cord, 42. Spinal symptoms of insular sclerosis, 103. Spontaneous eoohymoses in tabes seemingly connected with paroxysms of lightning pain, but when they cease, and at the moment of their cessation, 247 ; nor are they seated at the same place as the pains but somewhat above their seat, 247 ; they are often unperoeived, 247. Spontaneous fractures in tabes, occur somewhat frequently, 208 ; entire absence of pain, 208 ; though premonitory pain may occur previously, 209 ; the swell- ing considerable, 209 ; tendency to consolidation rapid, 209 ; a false articulation is in some cases formed, 209 ; swelling of part considerable, 209 ; much callus forms owing to the immobility not being complete, 209 ; and the occurrence of reciprocal friction, 209 ; such fractures are liable to be associated with short- ening of the limb, 210. Standing, difficulty in (the sign of Eomberg), one of the first symptoms recognized, 158 ; especially if the eyes are closed or the room dark, 158 ; difficulty in passing any article of dress over the head, 158 — 9. Strabismus in spastic paraplegia, 90. Straus, his belief that contraction after hemiplegia was but an increase of the muscular tone, 20. Subjective sounds often heard in tabes, 205 ; their various character, 205. Rnlon.ma.rtrmn.l tract. descendinsT. 37. , INDEX. . 501 Summation of impulses, explanation of, 176. Superficial Teflexes, may be increased or diminislied, 93. Symmetry, absence of, found in the cord frequently, but less accentuated than after amputation, 72 ; supposed by Flechsig to be congenital absence of symmetry, 71, 72. Sympathetic nerve, changes in, supposed by some to be the cause of tabes, 353 ; either functional or organic, 353. Symptoms of amyotrophic lateral sclerosis, 447, et srq. Symptoms of infantile spinal paralysis, 413; onset, gastro-intestinal derange- ments, symptoms associated with the nerrous system, 413 ; paralysis, its character and extent, 414 ; period of regression continues during 1 or 2 weeks, the motor nuclei of some muscles but little affected regaining power, their paralysis having been due to the shock of the disease in the cord, 415 ; when the nuclei of the muscles are destroyed the muscles remain paralysed and atrophy occurs, 415 ; the period of deformities now commences, such as club-foot, club-hand, and various deformities in the trunk from simple sclerosis, 415, to what is termed by the expressive name of cul-de-jatte, 416 ; when neither the lower part of the trunk or lower extremities can be used 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 ; trophic disorders which occur in the growth of the limbs, subcutaneous adiposis, coldness of the paralysed limbs, change of colour and thinness of the skin, 419 ; callosities, 420 ; chilblains, 420 ; other changes in the skin, 420 ; fragility of the bones, 420 ; intellectual state owing to the life led and inherited tendency, 420. Symptoms of tabid nature exist in combined lateral and posterior sclerosis such as co-ordination, awkward movements, ataxic gait, and the symptom of Eomberg, &c., 379 ; conjointly with which excess of the knee-jerk, foot clonus, paresis or paralysis, cramps, &c., may exist, 393. Symptoms and pathological anatomy of insular sclerosis described by Charcot and Vulpian, 103. Symptoms divided into spinal cerebral and bulbar, 103. Symptoms indicating the existence of optic neuritis in tabes, 202. Symptoms of combined lateral and posterior sclerosis attract less notice than pathological anatomy, 394. Syphilis, in which the blood vessels are specially affected, may be at times the cause of combined sclerosis, whose effects are localised in certain regions of the cord, 411. Syringo-myelia, in the diagnosis of tabes, 311. Systemic sclerosis (combined) 400. With regard to the forms of this disease an interesting observation of Stenson should be recalled, 401 ; in this the blood was arrested and then re-established in the abdominal aorta, and the degeneration which occurred in the grey matter and antero- lateral columns was observed by Ehrlich and Briger, 401 ; Singer and Milnzer observed that a marginal zone corresponding to the direct cerebellar and Gowers' tract was in a healthy condition, 401. Tabes. Teeminologt. History. Symptoms, 154, et seq. Locomotor ataxy of Duchenne of Boulogne, now replaced by the term tabes, 154 ; " tabes " means " consumption," Eomberg having described the disease in 1851 as "tabes dorsualis," Duchenne de Boulogne in 1858 gave to it tie name progressive locomotor ataxy, 155 ; names given of grey degeneration of the posterior columns, posterior leuoomyelitis, sclerosis of the posterior columns, 155 ; progressive locomotor ataxy has fallen into disuse because the 502 , INDEX. Tabes {continued)— 155 ; and because inoo-ordination and ataxy of movement do not necessarily occur as symptoms, 155 ; the term tabeshas thus prevailed, 155 ; since dorsualis is bad Latin the word used should be dorsalis, 155 ; since " tabes " is itself an anachronism, the anachronism should be preserved in its entire state, and the term " tabes dorsualis " used as before, and as Charcot does now, 155 ; claims of Duchenne, 156. A. Motor symptoms, 156. Tho6e which exist are (1) disorders of the muscular sense ; (2) involuntary muscular movements ; (3) paralysis, 156. 1. Involuntary muscular movements resembling those termed athetoid, 162. 2. Paralysis. Lastly paralysis, quite a different symptom, is one of the motor symptoms occurring in tabes, 163. I. Sensory symptoms op a pttbely subjective nature, 168. II. Sensory symptoms amenable to objective control, 171. (a) AncestJieaia (analgesia). Anaesthesia occurs at the surface of the skin, and also in the deeper parts, viz., the muscles, bones, and articulations, 171. (b) ffypercBSthesia, or rather hyperalgesia (Leyden) never causes the tactile sensibility to be more acute in tabes than in the normal state, the sensibility to pain being alone increased, 173. A. Sensory tetanus, meaning of the term, 176 ; B. Polycesthesia, explanation of term, 176 ; summation of impulses, 176, 177. C. Disorders of refiex action, 179. Eeflex movements may be obtained throughout the whole length of the cord, and even by means of the brain, some so much resembling each other that they may be classed together, 179. Patellar tendon refiex, 180. Pseudo- tendon-reflex is the name given to a blow upon the skin of the knee producing a reflex movement of the leg analogous to that occurring in the patellar tendon reflex, 183 ; presence of nerves in tendons, 183 (musculoten- dinous bodies), 184 ; in close relation to the Pacinian .corpuscles, 184; which are also found in many tendinous laminae, 184 ; the corpuscles of Golgi, 184 ; the impulse having reached the nerve fibres of the tendon passes with them into the cord, 186 ; cause of loss of reflexes, 186. General conditions which may influence the production of the patellar tendon reflex, 189. Age, its effects upon the reflex, 189. Fatigue, sleep, some infectious diseases, and forms of acute intoxication, 189, have a similar effect, 189. The conditions which may make the patellar tendon excessive, 189. Muscular exertion notably increases the knee-jerk, 190 ; method of Jendrissik for obtaining the knee-jerk, 190. Lesions of the pyramidal tract in any part much increases the different tendon reflexes— such as many cases of transverse myelitis, compres.aion of the spinal cord, and amyotrophic lateral sclerosis, 190. Tabes, rapidity of nervous impulse unknown, 193. The rapidity with which the nervous impulse passes is not exactly known, 193; the interval for the knee-jerk being longer than Jj to ^ (really J,— il,) of a second (Brissaud), 193. I. Organs external to the globe of the eye, 195 et seq. Paralysis of external muscles very frequent, occurring in 39 per cent, of the cases (Moeli, Berger), and according to Gowers |ths of his cases suffering from tabes presented this symptom at some time, especially during the period of inco-ordination, 195 ; tlhthoff met with this paralysis in 20 per cent, of his cases, only INDEX. 503 Tabes {^continued) — 195 ; epiphora in tabes, 198 ; ptosis in tabes, 198 ; narrowirg of palpebral opening, 199 ; diminntion of ocular tone, 199 ; liability of the iris to many disorders, 199 ; alteration in the size of the pupil in tabes, myosis, 199 ; mydriasis, 199 ; obliquely oval shape, 199 ; absence of reflex contraction in the iris when a light is brought near the eye in from 80 to 90 per cent., 200; persistence of accommodation reflex associated with loss of light reflex termed the sign of. Argyll-Eobertson, 200 ; the patient when examined should be made to look at a distant object otherwise the pupil may contract from accommodation, 200 ; the accommodation reflex usually retained daring the first stages of tabes usually ceases after a time, 200 ; the optic nerve is affected in 10 or 20 per cent, of the cases, being more frequent in those who have previously suffered from paralysis of the ocular muscles, 201 ; Gowers states that he has never seen it as the first evidence of tabes, 201 ; usually bilateral in tabes its onset may not be simultaneous in the two eyes, the left eye being most often first affected, 201. Auditory Okgans. Frequency with which auditory organs are involved, 20 i ; often during the preataxio period, 204 ; diminution in the acuity of hearing, 204 ; deafness absolute, and auditory neuritis may occur, 204. Anral vertigo (Menifere'a disease) occurs in two groups, 205 ; A. In one there is diminution of the acuity of hearing, and different lesions may exist in the twmsmitting portion of the ear, 205 ; B . In other cases neither diminution of the acuity of hearing nor lesions in the transmitting portion of the ear exist, a lesion probably existing in what is termed the nerve of spnce, a lesion apparently directly due to the existence of tabes, 205 ; the excessive irritability of the auditory nerve to electric currents is not rare in tabes and this is supposed to possibly explain the relative frequency of vertiginous sensations in some patients suffering from tabes in whom the ear is slightly affected, 206. Olfactory ORflANS. Disagreeable odours or anosmia may exist, being probably due to a lesion of the olfactory nerves, which is special to tabes, 206. GnSTATOKY Oksans. Strange sensations of taste, a persistent taste of sugar, or complete loss of taste may exist probably on account of some change in the nerves of taste, 206. .Trophic disorders in tabes, 207 ; disorders of general nutrition in tabes, 207 ; most suffer from consomption or " medullary phthisis " as it used to be termed owing to which they are thin with flaccid muscles, sunken eyes and pinched features, 207 ; while some arc stout and of healthy appearance. Spontaneous fractures occur somewhat frequently, 203 ; entire absence of pain, 208 ; though premonitory pain may occur, 20D ; the swelling considerable, 209 ; tendency to consolidation, 209; much callus forms owing to the immobility not being complete, 209 ; and the occurrence of reciprocal friction, 209. Period at which these fractures occur varies, 212 ; these fractures are far more common in the female sex, 212 ; bones which suffer most from spon- taneous fracture in tabes, are the femur, the leg below the knee, the bones of the forearm^212. Tabid arthropathy also called Charcot's joint disease, Charcot having dis- covered its existence and described it clearly in 1868. Clifford, Albutt, Buzzard, and Volkmann also mentioned it, 219. Description of tabid arthropathy, 220. Tabid arthropathy, also called "Charcot's joint disease," Charcot having .liannTp.rerl its existence and described it clearly in 1868 ; Ball, Clifford, 504 INDEX. Tabes (continued)'^ saoker, alludes to 53 writings by Frenoli, 36 by English, and 18 by German authoi:s, 220 ; absence of pain in tabid arthropathy, 221 ; course of its two forms recognized by Charcot, 221 ; I. a mild, 11. a serious form, and con- siderable change in the articular surfaces, 226 ; and other complications may occur as spontaneous fractures, pain, the passage of the articular extremities through the skin, 227 ; suppuration, 227 ; theories as to the nature of tabid anthropathy, 234 ; A, as to its being a nervous lesion, 234 ; B, as to its cause not being a nervouS' lesion, 235 ; cannot be solely the result of syphilis, 236 ; peripheral neuritis can scarcely be the cause, 237 ; change in the spinal cord seems more probable, 238. Tabid foot may proceed either from tabid arthropathy or from spontaneous fractures, 239 ; first description of, 239 ; its onset, 239 ; description of, 239, 240 ; nature of swelling, 239, 240 ; thickening of inner border of foot, which may produce a prominence due to the tuberosity of the scaphoid and internal cuneiform, 240 ; character of arch of foot, flattening of sole of foot, 240 ; the plantar curve may however be exaggerated, 240 ; displacement of metatarsus outwards is sometimes observed, 240 ; shortening of foot, enlarged malleoli, 241 ; mobility of the' segments of the foot diminished, 241 ; crepitation when spontaneous or passive movement in the joint occurs, 241 ; absence of pain on pressure, 241 ; anaesthesia on the back, while sensation exists in the lower part of- the foot, 241 ; pathological anatomy of the condition, 241 ; changes in the OS calois, 242 ; the swelling gradually subsides at length, but the defor- mities persist, 243 ; suppuration does not occur more than in other forms of arthropathy, 243. Trophic disorders in the fibrous tissue in tabes, 243 ; distention and perfora- tion of the articular capsules, 243 ; intra-articular ligaments, alteration and disappearance of, 243; lesions in the ttndons, 243; rupture of the tendons, 243; disorders of the skin, list of eruptions which may occur, 244 ; perforating ulcer, 244 ; the mild or perforating form, 245 ; seats of, 245 ; cause of, 246 ; gangrene in the disease, 246 ; description of, 246 ; from the presence of some micro-organism, 246, 247 ; shedding of the nails, 247 ; often observed, 247 ; mode of their being shed, 247 ; dystrophy of the nails, 248 ; loss of teetk, 248 ; has been observed since 1868, 248 ; description of, 248 ; hyperidrosis is not rare, 249 ; it may be uni- or bilateral. Anidrosis or absence of perspiration may also occur in tabes upon one or both sides, 249 ; glycosuria may occur and other changes in the composition of the urine, 249 ; diarrhoea may occur, 249; do glycosuria tabid diarrhoea, &c„ depend on peripheral neuritis or lesions in the spinal cord ? This question it is impossible to decide, 250. Muscular atrophy, 251 ; many authors published observations about it, 251 ; the term denotes muscular emaciation, flaccidity and weakness, 252 ; true muscular atrophy alone to be considered, 252 ; usually in the lower limbs, especially below the knee and in the foot, 252. The tabid club-foot has only been described recently by Joffrpy, 254 ; the affection having before been attributed to tonic contraction combined with the weight of the bedclothes on the feet, 254 ; description of Jeffrey, 254 ; hemi- atrophy of the tongue, description of by different authors, 258 ; it may occur without any connection with tabes, 258, description of, 259^ paralysis of one side of the soft palate may co-exist with hemiatrophy of the tongue and corre- sponding inferior vocal cord, 260 ; this hemiatrophy has been also observed in general paralysis of the insane and syphilis, 261 ; affecting the pons varolii and medulla oblongata, 261 ; patients suffering from tabes or general paralysis. of the insane have been frequently affected by syphilis, and it may thus be asked whether hemiatrophy of the tongue is due exclusively to tabes or to its usual INDEX. . 505 Tabes (continued) — ' exists, 261, 262 ; changes in the hypoglossal nerve when lingual hemiatrophy exists, 262 ; no change in the nucleus of the pneumogastrie or spinal accessory nerves found similar to that in the nucleus of the hypoglossal nerve, 262 ; the muscles of the larynx and soft palate receive fibres also from the hypoglossal nucleus, 262 ; something similar occurs in the eye the internal rectus receiving fibres from the nuclei of the 3rd and 6th nerves, 262 ; the same is the case as regards paralysis of the soft palate and larynx which result from this charge in the hypoglossal nucleus, 263. Digestive system in tabes. (a) Gnslria crisis, we owe the knowedge of this symptom to Charcot (1872), 265 ; certain facts were also exposed by Foumier, 266 ; what characterises these are the pain, and the incontroUable vomiting, 266 ; the gastric crisis is usually an eariy symptom, 268 ; being liable to occur and gradually to diminish and cease, or to persist during the whole disease, 268 ; death at times occurs during the collapsed condition of one of the crises, 268 ; abnormal forms of the crisis, 269; its duration, 269; tabid anorexia, 269. The intestines 1. Intes- tinal tenesmus or strong desire to evacuate the bowels ; 2. tabid diarrhoea, with little or no pain and no obvious cause, 270 ; may persist from two to four years, 271 ; treatment is ineffectual. II. Vasoulae system in tabes heart, lesion of the aortic are more frequent than those of the mitral valve, 271 ; cardiac lesions occur somewhat frequently in tabes, 271 ; vascular lesions, 272 ; arterio sclerosis not infrequent, 272 ; the base of certain theories about the nature of tabes, 272 ; and of the cardiac changes just mentioned, 272 ; angina pectoris not infrequently occurs, 272 ; presenting the usual symptoms of that complaint, 272, 273 ; and at times associated with gastric crisis, 273 ; disease of Graves is not infrequently associated with tabes, 273 ; opinions as to this coincidence, 274 ; tachycardia may occur, 275 ; absence of knee-jerk, 275. III. The Phabynqo-laknyqeal system in tabes. A. Pharynx, 276 ; hyperaesthesia or anaesthesia of the soft palate may exist, 277 ; the sensibility of the pharynx be diminished, as well as that of the larynx, 277 ; the epiglottis may be much lower in position, 277 ; pharyngeal crises of Oppenheim, 277. B. Larynx, tabid laryngismus ; laryngeal crisis, 277 ; authors who men- tioned laryngeal crises, 277 ; description of this condition, 277 ; laryngeal ictus, 278 ; description of, 278 ; treatment, 279 ; course and frequency, 279 ; pathology, 279 ; treatment, 280 ; chronic symptoms of tabid laryngismtis, 280 ; laryngeal paralysis, posterior cryco-arytenoid muscles most often affected, 280 ; the thyro-arytenoid or lateral cryco-arytenoid may be paralysed less often and to a slighter degree, 280 ; ataxy of the vocal cords seems to be rare, 281 ; secondary lesions occur in the muscles resembling those which exist in the muscular atrophy of tabes, 281 ; changes in the nerves, their roots, nuclei, and in the medulla oblongata, 282 ; atrophy of the nuclei, of the vagus, glosso-pharyngeal, spinal accessory nerves mentioned by many authors, 282 ; atrophy of the vocal cord may coincide with hemiatrophy of the tongue, 282 ; lesion in the ascending root of the lateral mixed system or slender column, 283. Derangements of the urinary system, 283 ; glycosuria, diminution in the amount of urea and of phosphoric acid with a proportionate increase of earthy phosphates, 283 ; Albert Eobin remarks upon the secretion of phos- phorus, 283 ; excess of urine secreted, urinary crises^ 283. This flux is far from rare in tabes, 284 ; derangements of urinary excretions may occur at the onset, 284; the patients usually consult a surgeon who aT^nnia.llv trp.a.tH a.fFep.ti nn R nf the ilrinarv Dn.Asao'es. and are tftrmed bv (tUvoti 506 INDEX. Tabes {continued)— pass water, 284; complete retention of urine may oocnr though usually of temporary duration, 285 ; absolute (quite at the end of the disease) or relative incontinence are frequent, 285 ; anaesthesia may occur in the mucous membrane of the bladder and urethra with perhaps the occurrence of pain at the neck of the bladder or in this organ, 285 ; gastric or nephritic colic may occur, 285. Genekative system in the male, 285, 286 ; (a) impotence, (b) genital excite- ment, 286 ; (c) reflexes connected with the genital organs, viz., cremasteric reflex, its description, 287 ; bulbo-cavemosus reflex, 288 ; atrophy and anses- thesia of the testis, 288. In the FEMALE, derangements connected with the genital organs in the female, 289 ; almost analogous to those in males, 289 ; (o) genital depression, 289 ; (6) genital excitement, 289 ; the clitoridean crisis, 289 ; (c) pains in the genital organs, 290. CeEEEEAL system in TABES, 291. Apoplectiform attacks of a mild or severe form, 291 ; probably due to hgemorrhage, softening or some morbid process of long duration in the meninges or ependyme, or vasomotor disorders due to change in the medulla oblongata or pons varolii, 291 ; such lesions are probably connected with syphilis, 292 ; psychical derangements, 292 ; frequently connected with general paralysis of the insane, 292 ; profession of those affected by tabes, 293 ; course of the disease, 293 j the prodromic period, the ataxic, the period of confinement, and that of paralysis, 293 ; the prodromic period is the time at which lightning pains occur often considered rheumatic, 293 ; onset of dis- ease by ocular paralysis, 294 ; or some alteration in the micturition, or genital functions, or perhaps the laryngeal or gastric crises, Ac, 294 ; confirmed ataxia is only recognized by observant patients, 294 ; no great change may occur for some time, 294 ; second period, £94 ; the ataxy of movement is almost always confined to the lower limbs, 294 j several years pass before the upper limbs suffer, 294 ; visceral symptoms and the ocular affections may now be observed, 294 ; third period, almost absolute loss of control over the movements of the lower limbs, aggravation of the urinary disorders. 295 ; cystitis with pyuria, 295 ; forms, superior or cervical form, 295; cerebral form, mild form of tabes (Charcot), 296; ia Vinriir 110 • flip f.T*p.nnnT mn.v nnmir iti Tnnanloa nt \\nA-tr 510 INDEX. backwards when the patient stands in an upright position or walks, or carries something to the mouth, 110 ; in pronounced cases the tremor tends to occur in an antero-posterior direction and to produce as it were a movement of circum- duction, 110; in some observations the tremor has been said to be unila,teral, which is rare, although it may predominate on one side, 110 ; the tremor in the tongue does not seem quite analogous to that in the limbs, but rather to consist in a difficulty in protruding the tongue and in keeping it out of the mouth, 120 ; tremor may be the predominating symptom in insular sclerosis, 126. Trophic centre of peripheral nerves and posterior columns of the cord formed by spinal ganglia, 42. Trophic disorders in insular sclerosis, 119 ; changes in the nails, 119 ; sloughing in the glutoeal region, &c., amyotrophy, 119, 120. Trophic disorders in tabes, 207, 243 ; distension and perforation of the capsules, 243 : disorders of the skin, list of eruptions which may occur, 244. Trophic disorders of the articular ligaments take part in the production of muscular atrophy, 255, 256 ; appearance of muscles and muscle fibres, 256. Tiirck, use of secondary degeneration of pyramidal tract in description of the latter by him, 2 ; described extension of degeneration in 1851 —55, 15. Upper limbs, tendon reflexes excessive in sound upper limbs when pyramidal degeneration occurs, 24 ; upper limbs in tabes are as exposed to inco-ordination as the latter, 161 ; but from the difference in the functions the symptoms produced are also different, 161. Urinary system, A. derangements of the urinary system in tabes, 283 ; glycosuria diminution in the amount of urea, and of phosphoric acid with a proportionate increase of earthly phosphates, 283 ; Albert Eobin's remarks upon the secretion of phosphorus, 283 ; excess of urine secreted, "urinary crises," 283; this is one of those fluxes which are far from rare in tabes, 284 ; derangements of urinary excretion, 284 ; very common and may occur at the onset of tabes, 284 ; the patients usually consult a surgeon who specially treats affections of the urinary passages, and are termed by Guyon " false urinary patients," 284; the patients may have to strain in order to pass urine, 284 ; complete retention of urine may ooopr though often of temporary duration, 285 ; absolute (quite at the end of the disease) or relative incontinence are frequent, 285 ; anaes- thesia may occur in the mucous membrane of the bladder and urethra, and perhaps pain at the neck of the bladder or elsewhere, so that vesical or nephritic crises may occur, 285. The sheaths of myelin being destroyed, the axis cylinders are not snffi- . ciently isolated as indicated by the tremor, 147 ; others attribute it to islets in the thalamus opticus, others to an excessive degree of reflex action in the spinal cord, 147 ; the nerve cells are but little affected, though when con- tained in the islets they diminish in size, lose their prolongations, and present clear indications' of atrophy, 147. Urine, incontinence or retention of in insular sclerosis, 119. Vague expression owing to ocular paresis in insular sclerosis, 114. Vascular origin, that insular sclerosis is of vascular origin similar to that which attends embolism is certain, 151 ; many believe the sclerosis which exists in the posterior columns of the spinal cord to be of vascular origin, 353 ; peri- vascular inflammation, 353. Vascular system, heart, lesions of the aortic are more frequent than those of the mitral valves in tabes, 271 ; cardiac lesions occur somewhat frequently in tabes. 271. INDEX. 511 Vertebral fractures in tabes 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 insular sclerosis, 121, 122 ; in hysteria, 130. Visceral disorders in insular sclerosis, 1 19 ; visceral pains in tabes, 170. Vision, organ of, nearly all the structures in, may be affected in insnlar sclerosis, 113. Visual disorder, development of, in insular sclerosis, 118 ; improvement may occur, 118 ; disorders bilateral, 118 ; changes corresponding to pronounced decoloration of the optic nerve, 118 ; varieties of change in visual field mentioned by UhthofF, 118. Visual function, symptoms connected with, well known but again discussed recently, 112. Voluntary movements, pre-eminently made by pyramidal tract, 13; this tract has an excitomotor or inhibitory effect upon the cells of the cord, 13. Vomiting of gastric crisis, 266 ; its composition, 266, 267 ; gastric juice secreted in excess, 267 ; and more acid in reaction, 267 : explanation of this change, 267. Vulpian, confirmed observations about degeneration of the pyramidal tract in spinal cord, 15. Walking, mode of, in a little girl with spastic paraplegia, 87 ; and leaping almost impossible when spastic paraplegia is severe, 88. Weariness is produced by severe pain and repeated vomiting in the gastric crisis of tabes, 267 ; an indifference to all which surrounds the patient, 268 ; and if the affection is severe a state of algidity may occur, and the colour of the patient become livid as in cholera or sea-sickness, 268. Westphal, bilateral degeneration of pyramidal tracts after a unilateral cerebral lesion first 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, 186; showed the absence of knee-jerk after Erb had done so in tabes, 192 ; 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 these lectures, 103 ; upon combined lateral and posterior sclerosis, 394. Wrist semi-flexed and fingers strongly flexed into the palm of the hand in amyo- trophic lateral sclerosis, 449. Zone of Lissauer, 325 ; marginal zone, 326 ; external segment of zone of Lissauer, 326 ; inner segment of zone of Lissauer, 326 ; constituted almost entirely by small fibres, 328 ; which insinuate themselves between the large fibres coming in fasciculi from the posterior root, &c., 328 ; other small fibres accompany the large fibres of the posterior roots and pass with them into the gelatinous substance of the posterior oornu or into the interior part of that oornu, 328 ; this zone not increasiiig in size from below upwards, 328; in the lumbar region this group of fibres is most developed, its smallest size being in the dorsal region, 329; small nerves which constitute the zone of Lissauer almost entirely disappear in tabes, 337; lateral column in no way affected in these oases, 338 ; degeneration of the zone of Lissauer an eariy change in tabes, 363. rrintod by Jas. Trcscott & Sox, Suffolk Lane, E.G.