ANTERO-LATERAL SCLEROSIS; POSTERIOR SCLEROSIS; PATHOLOGY AND TREATMENT OE LOCOMOTOR ATAXIA BY SUSPEN- SION AND BY APPARATUS. BY DE FOREST WILLARD, M.D., SURGEON TO THE PRESBYTERIAN HOSPITAL, PHILADELPHIA', CLINICAL PROFESSOR OF ORTHOPEDIC SURGERY IN THE UNIVERSITY OF PENNSYLVANIA ; GUY HINSDALE, M.D., AND ASSISTANT PHYSICIAN TO THE ORTHOPEDIC HOSPITAL AND INFIRMARY FOR NER- VOUS DISEASES AND OUT-PATIENT PHYSICIAN TO THE PRESBYTERIAN HOSPITAL. THE MEDICAL NEWS, FROM November 24, 1894. [Reprinted from The MEDICAL News, November 24, 1894 ] ANTEROLATERAL SCLEROSIS; POSTERIOR SCLEROSIS; PATHOLOGY AND TREATMENT OF LOCOMOTOR ATAXIA BY SUSPEN- SION AND BY APPARATUS.1 SURGEON TO THE PRESBYTERIAN HOSPITAL, PHILADELPHIA J CLINICAL PROFESSOR OF ORTHOPEDIC SURGERY IN THE UNIVERSITY OF PENNSYLVANIA J DE FOREST WILLARD, M.D., GUY HINSDALE, M.D., AND ASSISTANT PHYSICIAN TO THE ORTHOPEDIC HOSPITAL AND INFIRMARY FOR NERVOUS DISEASES AND OUT-PATIENT PHYSICIAN TO THE PRESBYTERIAN HOSPITAL. The degenerations of the spinal cord, or the system- diseases of the cord, constitute the most hopeless class of affections in spinal pathology. Insidious ofttimes in their onset, relentless in their advance, cutting off one by one the means of progress, the exercise of the senses, and the control of the mind, these diseases have long been treated in vain. Why should we be content to calmly listen to the funeral march as these patients are borne, one by one, to their final resting place? In the lan- guage of a distinguished teacher, we are saying to them as they enter our consulting-rooms : " My dear sir, you have a strip of fibrous tissue in the back part of your spinal cord; it is very small, but it will get bigger, and you will grow worse. Why it comes there I do not know, 1 One of the Mutter Course of Lectures delivered before the College of Physicians of Philadelphia. 2 and why I cannot stop it I cannot understand; but I have triangulated your nervous centers, located the lesion, and if I ever get your cord I can dye the affected part a beautiful orange-yellow or a bright carmin-red." The whole range of the scleroses and palsies of various types has waited patiently, despairingly, at the fountain of science. We can pity the patients, but our outstretched arms seem powerless to save. We are en- couraged, nevertheless, to find some faint streaks of light in the East that are beginning to dispel the gloom that has overshadowed us and that are ushering in a day of new things, Although, as a rule, the scleroses of the cord are gradual in their onset, and although it is customary to ascribe to slowly-operating causes their production, yet cases of acute locomotor ataxia are not infrequently ob- served. It is quite likely., also, that traumatism is the initial cause oftener than is generally supposed. Care- ful inquiry will sometimes elicit the fact that the disease dates from an injury to the back, or that a slight acci- dent has hastened or developed an attack. It has hap- pened that a man has received a blow upon the back producing myelitis with paraplegia and sensory disturb- ances of the lower extremities; the disturbances, let us say, were chiefly on the left side; a second injury was sustained, and ataxia and loss of knee-jerks supervened. It is probable that in persons of a neurotic predisposi- tion, locomotor ataxia is precipitated by traumatisms of even a slight degree. Spillman and Parisot have an- alyzed fourteen cases of tabes associated with peripheral injuries of various kinds, and they cite fractures, contu- sions, and wounds of the extremities, the extraction of teeth, or even the operation for cataract, as precipitating the disease. But here, as elsewhere, the danger is that post hoc is not always propter hoc. Acute locomotor ataxia, according to Leyden, may follow acute diseases; it may succeed to diffused neuritis. 3 It may begin as traumatic myelitis or as meningo- myelitis. We are not without evidence that there is a tendency toward secondary degeneration of a particular set of fibers after a local accident to the cord, such, for example, as compression-myelitis. In a recent case of Preston's, of Baltimore (Medical News, March 18, 1893), this was well shown. In this instance a man, aged twenty-three, was crushed by a bank of earth. There was no external injury of note. There was entire loss of sensation and of motion below the level of con- trol over the bladder and rectum. The superficial and deep reflexes were abolished. Bedsores of a trophic nature formed. Death occurred on the fiftieth day, and examination showed a fracture of the last dorsal and first lumbar vertebrae, with compression of the cord. A microscopic examination showed degeneration of the posterior columns reaching into the cervical region. On the other hand, there may occur a degeneration also of the lateral and direct cerebellar tracts. Following an accident an ascending degeneration ot the posterior columns and direct cerebellar tracts may ensue, and at the same time a descending degeneration of the crossed pyramidal tracts of the lateral column. Such a case was reported to the College of Physicians of Philadelphia, four years ago by Meigs (Trans. Col- lege of Physicians, 1890). In this instance a sailor was struck by a wave and dashed against the bulwarks of a vessel, causing him to strike the back of his head and neck against the rail. He at once lost all sensation and power of motion from the clavicle downward, and from the time of the accident he had retention of urine and incontinence of feces. Post-mortem examination thirty-six days after the accident showed that there was neither luxation nor fracture of the spine, but there was a small extra-dural hemorrhage into the spinal canal at the level of the seventh cervical vertebra. Microscopic sections of the cord above the seat of injury showed de- 4 generation of the posterior columns (columns of Burdach and Goll), of the whole of the direct cerebellar tract, and of a portion of the antero-lateral ascending tract, while below the seventh cervical vertebra the crossed pyramidal tracts of the lateral column were degenerated. (See illustrations in article alluded to.) In the lumbar region the lateral limiting layer and the direct cere- bellar tracts, together with the enclosed crossed pyra- midal tract, were degenerated, though in less degree than the latter. Cases of this kind and other cases in which the traumatism is not so severe show that after the primary lesion has cleared up a system-lesion may be left as a residuum in the cord. The relation of the different forms of myelitis to the various scleroses has not yet been investigated as it should be, and it offers an excellent subject for special study. How far these affections are a part of the pro- cess of inflammation is a matter of dispute. The term is broad enough to embrace the most flagrant febrile process as well as the chronic succession of changes pervading an organ or certain tracts of tissue, and dis- closing, after years of existence, simply a hyperplasia of fibrous tissue without any febrile symptoms having been observed. At the foundation of all inflammatory processes lies the principle of infection, and it is not altogether un- likely that all cases of myelitis and all the scleroses, whether primary or secondary, may in the future be harmoniously related to their respective infections. How far we may be carried by the present all-pervad- ing belief in the microbic origin of these diseases we cannot now say, but I note that no less an authority than Dana has recently published a paper entitled "The Microbic Origin of Chorea, with a Case,'' {Trans. Amer. Neurolog. Assoc., 1893). Dana found diplococci in the superficial layer of the cortex and in the deep layer of the pia, in which situation there were cellular infiltra- 5 tion and degenerative changes, and in the medullary and upper part of the cord there were evidences of men- ingeal irritation, vascular disease (arteritis) and nerve- root irritation. At the Congress of American Physicians and Surgeons, in 1891, Dana discussed the nature and causes of the scleroses of the spinal cord, and announced his faith in the newer pathology. The old belief that sclerosis of the cord is a parenchymatous inflammation with secondary growth of connective tissue is giving way. It is a degenerative process, showing under the microscope a gradual decay and death of the nerve-fiber and cell. In some sclerotic processes, like locomotor ataxia, this decay is accompanied by the development of irritating products, leukomains or tox-albumins, which may produce so active a change in the connective tissue as to lead to something resembling a secondary or reac- tive inflammation. Just what is the ultimate cause of these degenerative processes is not known. As yet no microbe has been discovered. We have simply the toxin theory-a working hypothesis-a disturbance that may be likened to that which has been observed upon the solar system, and which the French astronomer knew was due to an undiscovered planet. Some believe that this is a steadily secreted poison in the human body, and that it is constantly acting on diseased tissue; they bring in evidence the fact that many degen- erative processes follow syphilis and the infectious fevers and they account for it in the suggestion " that patho- genic germs have poured into the system a poison or have so modified the cellular nutrition that there is a poison constantly thrown out which irritates and de- stroys certain areas of nerve-tissue." Then, again, in Friedreich's ataxia, the hereditary form of sclerosis, it has been shown that certain strands of the spinal cord were never endowed with vitality enough to carry on their functions for more than a decade or two. With this premise some have enunciated the view 6 that by the presence of the poisons of certain infectious organisms the nerve-cell is stunned and its growth stunted; it follows that nutritional equilibrum is destroyed and premature senility and death are brought about. Sanderson in his Croonian Lectures on the " Progress of Discoveries in Relation to Infectious Diseases " {Brit. Med. Journ., 1891) states, that whereas degenera- tive diseases do not follow those infections which do not confer long immunity, such as diphtheria, sepsis, ery- sipelas, tuberculosis; and whereas on the other hand, diseases that do confer long immunity, like typhoid fever, measles, scarlet fever, smallpox, syphilis, etc., are most likely to set up degenerative changes, it would, therefore, not seem unlikely that the very thing which protects against recurrence of infection may be the cause of some internal degenerative change ? It is probable that immunity is secured through the modification of cell-nutrition and through the continued presence of some antitoxin in the liquor sanguinis. It is important to bear in mind in connection with researches in the pathologic anatomy of the nervous system that there are important and often misleading changes, both gross and minute, produced in the spinal cord by injury during its removal from the body and its preparation for microscopic study. Attention has been called to this subject by Van Gieson (TV. Y. Med. Journ., 1892), who has compared the lesions produced by bruises with the conditions found in a number of reported cases of malformation of the cord. The lesions artifi- cially produced are reduplications of the gray matter, irregularly shaped horns, canals, cyst-like spaces, and misplaced gray matter, and they may be produced any time at an autopsy by careless technique or bruising. It has been shown that just such artefacts have been erroneously described as congenital malformations or as the result of pathologic processes, and have given occa- sion for extensive and interesting speculations as to 7 their supposed relations to the production of disease- processes. Among the surgical measures that have been adopted in the treatment of lateral and posterior spinal sclerosis, suspension has been the most widely employed. The apparatus used was brought to the notice of the pro- fession by Motschutkowski, of Odessa, Russia, and about the same time Charcot reported encouraging results from its use in France. Three or four years previously Dr. Weir Mitchell liad adopted the same measures in two cases of sclerosis, but failure to obtain good results prevented further trial at that time. The measure in itself is not by any means a new one, having been used as long ago as 1826 by Prof. J. K. Mitchell in cases of Pott's paralysis; in fact, it has been in use for centuries for various conditions of the spinal column. Motschutkowski made his observations first on a tabetic patient to whom he was applying Sayre's plaster jacket for the treatment of spinal curvature. He noticed a remarkable improve- ment in certain symptoms. Raymond, of Paris, who was in Russia on a scientific mission called Charcot's attention to the Russian publication. Without going into the history of this procedure it will be enough to say that an enormous amount of litera- ture appeared upon this subject in 1889. Five-hundred- and-sixty cases are reported in which suspension was adopted, and several valuable papers on this subject were published in the Transactions of this College in that year. The treatment has been extensively adopted in Philadelphia since that date, but with varying success. For a time almost every large hospital in this and in other cities gave the plan a fair trial. The various re- ports claimed improvement in a proportion varying from 30 to 87 per cent., the highest being Motschutkowski's original record, but no cures were noted. In 1890 Dr. Guy Hinsdale, of Philadelphia, reported twenty-three cases treated at Dr. Weir Mitchell's clinic 8 at the Orthopedic Hospital and Infirmary for Nervous Diseases. Of the twelve cases which had fifty or more suspensions, in seven there was reported a lessening of pain ; in two there was no change ; one had no pain to start with and none at the end of treatment; while in two the pains were worse. As to sexual power five re- ported increase; one, lessening; one had always been good ; and one not stated. In no case was there such a gain in sexual power as to make a man competent who had been incompetent before the commencement of the treatment. The gait was improved in four cases ; in seven it was unchanged. Station improved in six ; was unimproved in four, and was good to start with in two. Knee-jerk was unchanged in all the cases. Bladder- control was impaired in four; rectal control was unaf- fected. In general it was found that many cases feel an indescribable sense of gain from suspension. They feel better, eat better, sleep better, and wish to continue the treatment. Now that the novelty of the treatment has worn ofif, it is natural to find fewer cases at present being treated by this method. At the University Hospital suspension has yielded fair results, and by the kindness of Dr. C. S. Potts I am enabled to give the results in the following cases hitherto unpublished. In a total of eleven cases four were very much improved, and the remaining seven, while not sustaining any marked improvement, invari- ably felt better for some time; and several of them after the treatment had been stopped returned request- ing to have it continued. The special symptoms relieved were the pain, incontinence of urine, sexual weakness to some extent, and several in whom muscular weakness of the legs was present felt much stronger after the suspension. The apparatus used was that of Charcot, as modified by Mitchell, in which the elbows are displaced in slings, a band around the waist preventing the arms from get- 9 ting away from the chest. In this way the force applied to the head may be easily regulated. The duration of suspension was one-and-a-half minutes at first, gradu- ally increasing to four minutes. The only bad symptoms reported were subsequent attacks of vertigo ; one fainted the first time, but bore it well afterward; the patient, however, was in an enfeebled condition. It is proper to state that all but three of these patients took, in addition, internal remedies, potassium iodid, mercury, etc. The treatment was certainly palliative if not curative. The following are synopses of three cases in which improvement was observed. Case I.-Mrs. S. was in the first stage of locomotor ataxia, probably of specific origin. She had been treated with anti-syphilitic remedies for some time with benefit, except that severe burning pains in the feet were not re- lieved. She was then suspended every day for six weeks, and the pains almost entirely disappeared in that time. Case II.-T. P. had been ataxic three years, and un- able to walk without a person supporting him. He suf- fered from incontinence of urine and gastric crises. After the third suspension he felt better; after the fifth he could walk without assistance, and the bladder-symp- toms were much improved. After two weeks' treatment he had an erection of the penis, the first for over a year. This recurred every morning throughout the remainder of his treatment. At the end of a month of suspension he returned to his home, able to control his urine and to walk much better; and his attacks of vomiting and pain were much less frequent. In addition to the treatment by suspension he took silver nitrate for two weeks. Case III.-The disease was of specific origin. The man had had internal treatment for seven months with- out much improvement. He suffered pain in the legs, sexual weakness, and was unable to walk a block with- out sitting down. After suspension his pains ceased, his legs grew stronger, and after treatment on alternate days 10 for four months he returned to his work as a shoemaker. One year later he was married, and since that time a child has been born. Stewart has reported (The Medical News, Sept. 12, 1891), from the Jefferson Medical College, a case of postero-lateral sclerosis which showed remarkable im- provement as to gait, station, and tactile sensation. The patient was suspended three times a week for five months, suspension being made as frequently by the head alone as with the arm and head supports. The duration of each treatment was about five minutes. That suspension produces a change for the better in many of these refractory cases has been definitely proved, but just how it acts upon the living spinal cordis still a matter of conjecture. Measurements show an elongation of the spinal column amounting in some instances to one- and a-half inches. This stretching involves in its ten- sion the surrounding structures, the muscles and tendons, and also the spinal cord and dura mater, and spinal nerve-roots. Adhesions from chronic meningitis are broken down, and it is possible, but not proved, that this allows a freer transmission of nervous influence along the nerve-tubes, more especially those which run on the surface of the posterior columns. Hegar's post- mortem studies show that in the dorsal region the dura of the cord is lengthened 5 mm. by moderate flexion of the spinal column. He produced this by placing blocks under the chest and abdomen, and bending the neck, the legs being free. Strong flexion was found to increase this length to 7 mm. The dura returned to its normal length on placing the cadaver in a straight position. Hegar laid bare both sciatic nerves, inserted threads at different levels and made a strong pull. The distance increased one millimeter; the spinal column was again flexed and the distance increased six millimeters, and in this position the sciatic nerves were strongly pulled and the distance increased eight millimeters. The spinal 11 column was next exposed, and the two threads were stitched into the substance of the cord at a distance of 15.35 c. m. from each other. Moderate flexion length- ened the cord 0.75 c.m., and strong flexion over 1.05 c. m. Thus it was shown that the cord itself allows greater ex- tension than does the dura.-(Douglass Graham, Hegar, Wiener med. Blatter, 1884, No. 3.) Cattani has shown that stretching of nerve-trunks is accompanied by tearing of the axis cylinder, stretching of the medullary sheaths, with subsequent degeneration and regeneration. Such manipulation of the nerve tissues doubtless makes a great change in their vascularity. Just as Swedish movements and massage renovate the mus- cular system, so it is highly probable that flexion and extension of the cord and the accompanying stretching that occurs combine to alter and improve the nutrition of the tissues affected. The first surgeon to apply stretching of peripheral nerves in a case of ataxia was Langenbeck. This oper- ator stretched the sciatic, and improvement at once followed; the pains were diminished in a remarkable degree, and the incoordination became less. The expe- rience of other operators has not been satisfactory, how- ever, and it is only in cases in which the pain is intense and referable especially to the sciatic nerve that nerve- stretching should be considered. All theories proposed to explain the action of this procedure have failed to satisfy the profession, and " it would seem now to be passing into merited disuse " (Gowers). One of the remarkable features of the surgical pathol- ogy of spinal scleroses, particularly of locomotor ataxia, is an occasional trophic alteration of skin, teeth, bones, and joints. Prof. Charcot's name is identified with the knowledge of these spinal atrophies, while other French writers, notably Ball, Oulmont, Bouchard, and Dejerine, and in this country Dr. H. C. Wood and J. C. Shaw have rendered important contributions to the subject. 12 Charcot depicts on the one hand cases of extraordinary wasting of the articular ends of the bones. In a femur it was found that the head and most of the great tro- chanter had disappeared; the edge of the acetabulum had atrophied to a corresponding degree. On the other Fig. i. Fig. 2. hand, the changes to which attention was first attracted are those of swelling of the joints and thickening of the bones. (Fig. I.) While it is possible for any of the joints to become affected, the arthropathy is more com- mon in the knees, the ankles, and the small bones of the foot. (Fig. 2.) This typical swollen foot has been charac- 13 terized by Charcot as "la pied tabetique." The tabetic hand (Figs. 3 and 4), as well as the tabetic foot, may occur, and the example which is shown in the drawing from the publication of M. Ball is characteristic; it is extremely rare, and Dr. Wood, who has portrayed it in his work, says that he has never seen an example. Fig. 3. Fig. 4. Tabetic bones in the stage of hypertrophy have been carefully analyzed in France; the phosphates were found to be greatly diminished, but the fatty matter in the dilated Haversian canals was enormously increased. "The histologic and chemic results show a strongly marked resemblance between the bones of locomotor ataxia, of osteomalacia, and of general paralysis" (Wood). An atrophy secondary to hypertrophy suc- ceeds in advanced cases of tabes, and in such cases fractures of the long bones are liable to occur. When 14 this happens there is apt to be rapid and excessive formation of callus; or again, it is possible to have atrophy in one limb and enlargement in the other. Thus, in locomotor ataxia, as in general paralysis and allied diseases of the nervous system, the changes in the bones are of a trophic character, tending to atrophy and hardness; or, on the other hand, there may be permanent alterations related to those found in osteo- malacia. Much light has been thrown on the occurrence of atrophy in tabes by the discovery that in some cases of locomotor ataxia the nerves themselves are the seat of the disease. Investigations of the peripheral nerves in tabes by Dejerine, Goldscheider, Shaw, and others show that the ganglion-cells of the anterior horns of the spinal cord are not always accountable for the atrophy, but that the peripheral nerves as far as the atrophied mus- cles have undergone disease, in cases in which atrophy is the marked symptom.1 Atrophy in tabes usually affects the lower extremities, and as it progresses the muscles and tendons undergo shortening, giving rise to con- tractures and fixation of the neighboring joints. Much of this contracture and fixation is due to want of use, as well as to spinal arthropathy. Attempts have been made to produce these bone-changes artificially in ani- mals by operations upon the nerve-centers, so that, if possible, degeneration might occur in the spinal cord with a resultant lesion of the joints. Giacomo (Soc. de Biologie, March, 1885, p 156, quoted by Wood), in a very large dog cut the posterior roots of three lumbar nerves between the ganglia and the cord. After some months the joints of the left foot became enormously swollen and edematous, without increase of sensibility or temperature. At the autopsy it was found that sec- ondary degeneration had occurred in the spinal cord at a level corresponding with the joint-changes. 1 J. C. Shaw, International Clinics, 1892, vol. iii, p. 176 15 In cases of tabes which extend through a compara- tively long period of time, degeneration of the peripheral nerves may involve the patient in another very serious change, to wit: symmetrical gangrene of the toes. I am not aware that such cases have been reported in Phila- delphia, but a recent case of Pitres, of Bordeaux, pre- sented this condition in the following history : The first symptoms of tabes were observed in 1875; five years later lancinating pains in the legs began, and the chief clinical features of the case were well estab- lished ; ten years later the patient felt as though his toes were dead during cold weather, while in hot weather they became blue and swollen and pained greatly. In the sixteenth year from the onset of this train of symptoms gangrene became fully developed in all the toes of the right foot and in the great toe of the left foot. The patient dying by accident shortly afterward, the usual condition of the spinal cord was found, together with a very marked de- generation of the corresponding peripheral nerves. Nevertheless, numerous apparently normal nerve- fibers existed. (Revue Neurologique, May 15, 1893.) Another case of symmetrical gangrene of the toes occurring in a tabetic patient has recently been re- corded by Kornfield (Wiener med. Wochenschr., No- vember 5, 1892.) An interesting feature of the case was a certain resemblance to cases of syringomyelia, viz.: in the characteristic dissociation of sensibility; or, in other words, symptoms of posterior poliomyelitis, anes- thesia to pain, to heat, and to cold, with preservation of tactile sensibility and of the muscular sense. But, on the other hand, there was no characteristic muscular atrophy. Post-mortem examination showed, besides the sclerosis of the posterior columns, an acute inflammation of both peroneal nerves, fully explaining the occurrence of gangrene. (Fig. 5.) Those who have investigated the peripheral nerves in 16 tabes concur in finding the most decided changes in the peripheral ends. The microscope shows the myelin broken up into irregular masses of various sizes ; later on absorption of this granular-looking mate- rial occurs, and the sheath of Schwann is found empty and shrivelled. The axis-cylinder frequently remains Fig. 5. for a long time unaffected, and in some specimens it can be seen very sharply stained by carmin in the sheath with very little myelin around it. (Shaw, International Clinics, vol. iii, 1892.) The view is gaining ground that tabes is a more dif- fused lesion of the cerebro-spinal axis and of its branches 17 than has hitherto been realized. It would be much better in post-mortem studies of locomotor ataxia if attention were not riveted too closely to the spinal cord, but that more attention be given to the peripheral nerves and to the arterial blood-supply of these and of the spinal cord. Gowers has called attention to this, and lately Preston (Medical News, July 8, 1893), attributes to arterio-sclerosis of the intercostal vessels a diminished amount of blood in the spinal cord, and in consequence of this imperfect supply of nutriment an atrophy and degene- ration of the nerve-elements. As a consequence of this degeneration of nerve-matter there starts partly from the sheath of the nerve, partly from the bloodvessel, an outgrowth of connective tissue, which in its turn encroaches upon this often less resistant nervous matter, giving the characteristic picture of sclerosis. Preston shows that the etiologic factors, by common consent conceded to be the most important in the causation of tabes, viz : syphilis, exposure to cold and wet, or rheu- matism, and alcoholism, are the exact conditions most favorable to the development of arterio-sclerosis. Acting on this supposition, he has suggested the use of nitro- glycerin in order that the small branches of the spinal arteries may be dilated and a larger supply of blood be afforded the cord. In the surgical treatment of these pathologic condi- tions it is obvious that but little can be done. Contrac- tures occur so late, marking the hopeless progress of the affection, that interference by the surgeon can only avail little. The condition of the limbs can, however, some- times be benefited by tenotomy when the existing de- gree of incoordination does not present an insuperable obstacle to locomotion. The Medical News. Established in 1843. A WEEKLY MEDICAL NEWSPAPER. Subscription, $4.00 per Annum. The American Journal OF THE Medical Sciences. Established in 1820. A MONTHLY MEDICAL MAGAZINE. Subscription, $4.00per Annum. COMMVTA TION RA TE, $7.50 PER A NNUM. LEA BROTHERS & CO. PHILADELPHIA.