ON THE PROGRESSIVE LOCOMOTOR ATAXIA: ITS HISTORY, SYMPTOMATOLOGY, PATHOLOGY, AND TREATMENT. BY ROBERTS BARTH GLOW, A. M., M. J).. Professor of Physics anil Medical Chemistry in the Medical College of Ohio ; Lecturer on Clinical Medicine and Physician to St. John’s Hospital ; formerly Assistant Surgeon (Captain) U. S. Army, etc. CINCINNATI: ROBERT CLARKE & CO., 55 WEST FOURTH STREET. 1866. ON THE PROGRESSIVE LOCOMOTOR ATAXIA: ITS HISTORY, SYMPTOMATOLOGY, PATHOLOGY, AND TREATMENT. BY ROBERTS BARTHOLOW, A. M., M. D., Professor of Physics and Medical Chemistry in the Medical College of Ohio; Lecturer on Clinical Medicine and Physician to St. John’s Hospital; formerly Assistant Surgeon (Captain) U. S. Army, etc. CINCINNATI: ROBERT CLARKE & CO., 55 WEST FOURTH STREET. 1866. Neque enim credunt, posse eum scire, quomodo morbos curare con- veniat, qui, unde fei sint, ignoret. A. Corn. Celsi Medicince Liber I. Non minus ccrto etiam a ruinutissimis morb'i circumstantiis indiea- tiones curativas possit Mcdieus desumore, quam ab iisdem sumpsit diagnostiea. Sydenham. Ob. Medicce Prcef. Ed. TerU PREFACE. In the preparation of this little essay, I have made liberal use of the elaborate work, De L'Ataxie Locomotrice, et en particular de la maladie appellee Ataxie Locomotrice Progressive, by Dr. Paul Topinard. The sections on the history and symptomatology have been condensed from that work chiefly. I have added some historical facts and references; and have contrasted the symptomatology of my patient with that de- scribed by Dr. Topinard. Whatever aid has been derived from other sources is duly acknowledged. This essay was written for the Cincinnati Journal of Medicine, and appeared in the numbers for April, May, and June. I had the convic- tion, in preparing it, that the readers of that periodical would not be unthankful for some recent information on a rare form of nervous dis- ease, which, as it happened to me, might also happen to them. I do not urge the novelty and importance of my labor in justification of its republication in this form. It is reprinted now for distribution among such of my personal friends and of the professional public as may be interested in studies of this character. 344 Race Street, Cincinnati, 0. R. B. PROGRESSIVE LOCOMOTOR ATAXIA: ITS HISTORY, SYMPTOMATOLOGY, PATHOLOGY AND TREATMENT. Nomenclature.—The Ataxie Locomotrice Progressive; Duchenne’s Disease ; Progressive Spinal Paralysis ; Tabes Dorsalis ; Progressive Locomotor Ataxia, etc. Stewart Kelch, by occupation a gilder, was admitted to St. John’s Hospital, service of Prof. Bartholow, Feb. 10, 1866. He gave the following history : About the 15th of December last he began to experience some troubles of vision; objects were confused and indistinct; soon after he had double vision. He noticed that his food had no taste about the same time. His hearing and smell were unaffected. In consequence of these ocular troubles he consulted a female doctor who had some reputation as an oculist, who prescribed an eye-wash and some medicine. Finding that no improvement took place he consulted a well-known oculist in this city, who, after a prolonged ophthalmoscopic examination, directed glasses and gave him some internal remedy. His eyesight, soon after this, suddenly improved. Whilst he was in attendance upon the female doctor, he began to experience numbness and lack of voluntary control in the inferior ex- tremities, and the apparent loss of power and the difficulty of locomotion were well marked during the period he was under the care of the oculist, who, probably, did not recognize the nature of the malady. Soon after troubles of the same character appeared in the upper extremities. 6 PROGRESSIVE LOCOMOTOR ATAXIA. During the whole period he suffered from nocturnal emissions, saty- riasis and at length complete impotence. He had had some troubles in micturition and defecation, the desire coming on suddenly and the evacuation occurring immediately. He had no rheumatic pains. Pricking and tingling in the extremi- ties and a dull pain in the loins and limbs, which appeared about the time his ocular troubles began, were the only symptoms of this kind. Symptoms on admission.—Kelch is 36 years old, 5 ft. 6 in. high, light complexion, lymphatic temperament, full face with prominent lower jaw ; very protuberant blue eyes. He has no troubles of vision at present except some dimness of sight; his eyes are more prominent than natural. His face i» express- ionless. His taste has returned, but his tongue is protruded to the right. Sensibility, both tactile and muscular, diminished in the upper extremities, especially the right. Power of co-ordinating movements not completely lost, but diminished, in consequence of which his attempt to grasp any object is uncertain and vacillating. He can not use his knife and fork. He complains especially of a sensation of numbness in his hands and forearms, and a sense of pricking. They are, .5° F., colder than natural. There is no wasting of the muscles, but a contraction of the central tendon of the extensor communis digit- orum. His grasp is as strong nearly as in health. When requested to stand with his feet together and his eyes closed, he can not do so without the support of a chair. He walks as an un- practised youth on stilts. When requested to walk across the room, he rather runs than walks, and evidently can not control the movements of his feet, which are thrown up and down and laterally, in the most irregular manner. The chief sensation in his feet is that of numbness. Tactile sensibility and myotility are more diminished in lower than upper extremities. He perceives the galvanic shock when a strong current is passed through his limb, but feebly. The muscular sense is almost abolished. In walking, he can scarcely feel the floor, and when his eyes are not fixed upon his feet, he can not direct his movements. He has been for some weeks entirely impotent. His intellect appears to be unimpaired. Treatment.—He was put upon the crystallized nitrate of silver, 1-6 of a grain ter in die. After ten days’ use of this remedy, I gave him 30 grains of the iodide of potassium daily. His improvement under this treatment has been most marked. He is very rapidly regaining the power of co-ordination of muscular movements, his cutaneous and muscular sensibility are returning ; he walks with considerable vigor PROGRESSIVE LOCOMOTOR ATAXIA. 7 and certainty, and tie begins to experience sexual desire with erections. The iodide of potassium seemed to be indicated by the fact that the patient had for ten years followed the profession of a gilder, and had during that period of time, absorbed more or less of the mineral mat- ter used in the prosecution of his trade. -As M. Melsens has demon- strated that this remedy makes soluble combinations with these min- eral poisons, thereby facilitating their excretion, it was applicable to the treatment of this case on the theory that the alteration in the spinal cord might be due to the slow and long continued action of the miner- als. Moreover, the iodide of potassium is one of the most reputable remedies in this affection.* History. The name, ataxie locomotrice 'progressive, was given this malady by Duchenne of Boulogne, who described it with more partic- ularity than any of his predecessors, but he is not entitled to the merit of discovery. It had been recognized in England in 1847, and was well described in Germany in 1834, but under other names, as paraplegia with disordered co-ordination of movement, tabes dorsalis, gray degen- eration of the posterior columns of the spinal cord, progressive spinal paralysis, etc. The most ancient of these terms, tabes dorsalis, originated with Hip- pocrates, who applied it to the accidents produced by the abuses of venery. The passage in which the term occurs is found in De Morbis, usually ascribed to Hippocrates, but by Hr. Adams, translator of the the Sydenham Society edition of the works of Hippocrates, is assigned to the Cnidian school: Tabes dorsalis a spinali medulla oritur, maxime vero recentes sponsos et libidinosos corripit. Sauvages, a long time after, described a group of symptoms, pro- duced by the same cause, which closely correspond to the disease now known as progressive locomotor ataxia. Lallemand also, under the title of the “ dorsal consumption,” described a set of symptoms of the same character.f The term tabes dorsalis was, by the Germans, changed in its signifi- cation, and applied to a disease of the cord characterized by atrophy of this organ. The first autopsy, clearly establishing this condition of the cord, was made so long ago as 1679. The disease was very clearly described in Hufeland’s Practice of Medicine, under the name tabes dorsalis, in 1834. Steinthal, in 1844, was equally explicit in his details concerning this disease, describing, as had Hufeland, the difficulties of * See reports of two cases in the Medical Times and Gazette, treated with this re- medy, Jan. 27, 1866, They were only improved, however. f Dee per ten seminales involontaires. Paris et Montpelier, 1869-41. 8 PROGRESSIVE LOCOMOTOR ATAXIA. locomotion, tlie loss of power of co-ordinating muscular movements, the amaurotic amblyopia, etc. Romberg,* in 1851, under the same name of tabes dorsalis, and Wun- derlich, the following year, .under the title of progressive spinal paraL ysis, particularize with great precision the essential phenomena of this disorder. The morbid anatomy of progressive locomotor ataxia had been studied with the naked eye only, prior to 1857, in which year Ludwig Turck published a memoir at Vienna, in which he described the mi- croscopic appearances of the degenerated posterior columns of the spinal cord. Virchow and Raciborski confirmed these observations, which had established that the alteration proper to tabes, consisted in an atrophy of the nervous elements with hypertrophy of the interme- diate connective tissue. Whilst these observations were accumulating in Germany as to the nature of tabes dorsalis, attention was being called in England to defects of co-ordination, dependent upon an affection of the cord. Todd, es- pecially, influenced by the theoretical views which he entertained as to the functions of the posterior columns of the spinal cord, and having had two cases in which defects in the co-ordination of voluntary move- ments existed with integrity of the muscular force, diagnosticated during life and confirmed by post mortem observation, a lesion limited to the posterior columns. “ Two sorts of paralysis of movement,” says Todd, “ are found in the inferior extremities ; one consists in feebleness or loss of voluntary movement; the other is distinguished by diminution or complete abolition of the power of co-ordination of movements.” He says nothing, however, of ocular troubles. In 1856 and 1858, Gull published in Guy’s Hospital Reports a long series of observations, relative to diseases of the cord. In one case he observed trouble in the co-ordination of movements and alterations of the posterior columns of the cord. Gull, ignoring the labors of the Ger nans in the same field, ant. the investigations of Duchenne not having yet seen the light, did not hesitate to ascribe these phenomena to chronic myelitis. In Trance various observations were made and recorded in the pro- ceedings of the anatomical society, and in the immortal work of M, Cruveiihier. These observations extend from : 1828, those of M. Hutin; 1830, those of M. Cruveilliier; 184-5, those of M. Fredault; 1847, those of Monod; 1856, those of M. Luys; to 1858, those of M. Laborde. * Manual of Nervous Diseases. Syd. Soc. Trans. PROGRESSIVE LOCOMOTOR ATAXIA. 9 It is subsequent to these observations that M. Duchenne announced in the Archives de Medicine of 1858, the existence of a new disease which he proposed to call “ ataxie locomotr ice progressive,” describing it in these terms : “ Progressive abolition of co-ordination of movements and apparent paralysis, contrasting with integrity of the muscular force.” This able observer ignored the ideas of Todd and the labors of Hufe- land, Steinthal, Romberg and Ludwig Turck. His merit consists in this : that he comprehended the relation of the symptoms, their habit- ual succession, and insisted more than Romberg had done, upon the ocular troubles. This was certainly the view of the merit of Duchenne entertained by Trousseau, when he baptized the new disease by the name of its presumed discoverer. Symptomatology.—The duration of this malady is from one to thirty years; hence, it is useful to make some divisions to facilitate the description. We adopt the division of M. Duchenne : “Tiie ataxie locomotrice progressive is divisible into three periods : the first charac- terized by three symptoms—pains, ocular troubles and anaplirodisia; the second, by disorders of muscular sensibility and of the sensibility of the inferior extremities; the third, by the extension of the same troubles to the superior extremities.” Pains.—These are in general the first symptoms to attract the atten- tion of the patient. In one hundred and four observations made with reference to the frequency of the pains and the period of their appearance relative to the ataxic symptoms, they were present in forty-four before the locomo- tor ataxia; in twenty, after; in eighteen, present, but time not definitely fixed ; in twenty-two, absent. They were present in the case of Kelch after the locomotor ataxia. These pains are observed in all diseases of the cord, but are more frequent in progressive locomotor ataxia. At their origin, these pains are fugitive and distributed over the sur- face of the body or one of its lateral halves, or they are limited to one region, to one extremity, especially to the feet. If, after having been generally distributed, they disappear in the superior half of the body to concentrate in one limb, this part, in which a primitive or secondary localization of the pains has taken place, is destined to be the first at- tacked by the ataxic disorders. They are sometimes superficial, some- times deep-seated, in the muscles or in the neighborhood of the large joints. They are encountered in the head, jaws, orbital cavities, exter- nal auditory canal, the urethra, the deeper parts of the pelvis. In the globe of the eye they give rise to congestive phenomena, identical with 10 PROGRESSIVE LOCOMOTOR ATAXIA. tlio&e that accompany neuralgia of the fifth; injection of the conjunc- tiva, lachrymation, heat, dilatation of the pupil. These pains are resolvable into two classes: the one, dull and nearly continuous, exceptional; the other, short and sharp, habitual. The first have been compared to the sensation produced by the con- striction of a limb by a cuirass. The second, are sharp pains in the extremities shooting toward the trunk; more rarely, they are likened to flashes of fire, puncture of an awl, strokes of a hammer, or vibrations flowing along in the direction of the muscles, or more commonly, as a tic tac in some point like that of a watch. Their fundamental character is their mobility and intermittence. They may appear and disappear many times a day, and the attacks vary in duration from some hours to seven days or more. It is exceptional for them to increase at night. Sometimes the pains are so horrible as to justify the expression of Remak : tabes dolorosa. Their analogy with certain rheuipatismal pains is remarkable. They are influenced by all those atmospheric causes which exasperate rheu- matic pains. Hence it is that winter is a bad season for the ataxic. Functional troubles of the cranial nerves.—These functional derange- ments of the cranial nerves were well marked in our case. He had am- blyopia, diplopia, protrusion of the eye, paralysis of taste, etc. The comparative frequency of these functional troubles of the cranial nerves—optic, motor oculi, hypoglossal, auditory, facial, glosso-pha- ryngeal, etc., without regard to period is as follows : present in ninety- seven cases ; absent in twenty-eight. The cranial nerves are divisible into two or three orders: the first preside over movement; the other over special sensibility, but some of their filaments have general sensibility. What is the nature of the .dis- order proper to each ? It is evident that the alterations of the optic, auditory, lingual, trige- minal (sensitive part), produce but two orders of phenomena: hyper- esthesia (the neuralgic or rheumatic pains already alluded to) ; special anesthesia (amblyopia, paralysis of taste). In regard to the motor oculi, facial, inferior maxillary, hypoglossal, that portion of the pharyngeal plexus which presides over the muscu- lar sensibility of the vail of the palate, and of the pharynx, and the recurrents, their functions will be exalted, or abolished, or perverted; the muscles to which they are distributed will be .either paralyzed or ataxic. PROGRESSIVE LOCOMOTOR ATAXIA. 11 The phenomena proper to the motor oculi are constantly paralysis* except two cases in which Friedreich saw double bilateral nystagmus* and one in which M. Beau speaks of defects in the co-ordination of the movements of the eyes. Facial hemiplegia, when there exists greater or less irregularity in the two sides of the face, has been noticed. Embarrassment of speech and pronunciation has been observed in sixty-seven cases. The time when these functional troubles of the cranial nerves appear Varies. The ocular troubles in more than half of the cases appear in the first period, a little after the pains and sometimes in their absence ; the feebleness of vision and the embarrassment of speech appertain to the second period; and the dysphagia and the paralysis of the vail of the palate to a still more advanced period. The most ephemeral and intermittent of the ocular troubles are the diplopia and strabismus. The temporary character and spontaneous disappearance of these acci- dents give rise to a therapeutic illusion. Thus, the subject is surprised by them in good health; the pains which precede or accompany them are referred to rheumatism; the connection between the two is not un- derstood. He goes to an oculist who diagnoses congestion of the papilla Or paralysis of the third pair, orders an unguent and collyria, electrizes the orbital cavities, and as if by enchantment the malady disappears. These examples are very common. It is the history of our own case. Did the oculist effect a cure? The symptoms would have disappeared as well with cold water. The disorders pertaining to the orbital cavities are paralysis of motor oculi, producing ptosis, external strabismus, diplopia, and dilatation of the pupil; paralysis of the motor externus, producing internal stabis- mus and diplopia; paralysis of the patheticus, producing also diplopia and strabismus and other vague symptoms. Finally, kopiopia, ambly- opia, and amaurosis occur. The diplopia is generally attributed to defect in the convergence of the two visual axes upon the object examined. The condition of the retina, according to the ophthalmoscopic ex- aminations, consists in congestion or atrophy, more or less advanced, of the optic papilla. This is confirmed by some post-mortem observa- tions; in others, however, no alteration is found after death. The absence of lesions, remarked by various authors, in the cranial nerves affected with functional troubles, is in conformity with the temporary character of the different symptoms. It is this fugitiveness which 12 PROGRESSIVE LOCOMOTOR ATAXIA. enables the distinction to be made between them and similar maladies arising from other causes. Troubles in the genital organs.—In respect to importance, the disorders of the genital organs take place after the pains and ocular derange- ments. In Germany especially, where the ancient views on the subject of tabes dorsalis are not yet forgotten, a particular interest attaches to these symptoms. That venereal excesses are causative can hardly be admitted. As it is difficult to question females, all our observations are silent with regard to them. Moreover, nocturnal pollutions may occur without constituting a morbid state. Finally, the subjects who are attacked by this malady have generally attained the age of from thirty-five to forty years, the period of natural decline in the sexual desire. Four symptoms present themselves: spermatorrhoea, anaphrodisial and impotence. The first occurs among the earliest symptoms of the first period. In the case of Kelch, spermatorrhoea had existed for six months prior to the occurrence of the ocular troubles. He admitted to venereal excesses. The nocturnal pollutions, accompanied at first by erection and pleasurable sensations, finally became passive. After the spermatorrhoea, or sometimes preceding it, there is a progressive dimi- nution of the venereal desires, difficulty in satisfying them, and at length complete impotence. Vesical disorders are observed in some cases. The trouble con- sists in slow emission of the urine, incompleteness in emptying the bladder, and inability to determine when the emission of urine has ter- minated. Incontinence has been observed, but retention rarely. Among the disorders of micturition is found anaesthesia of the vesi- cal and urethral mucous membrane, in consequence of which the indi- vidual is unconscious of the emission of urine. The dysuria, as the ocular paralysis, is temporary and intermittent. Rectal troubles are rare. Constipation is habitual. When the mat- ters are liquid they escape before the patient has time to reach the closet. Incontinence does not occur until about the commencement of the second period. These vesical and rectal troubles occurred in our patient Kelch. PROGRESSIVE LOCOMOTOR ATAXIA. 13 Duchenne's Second Period.—The boundary between the first and second period is not so clearly marked as the arbitrary division of Du- chenne would indicate. It is by very gradual and sometimes almost imperceptible steps that the patient passes from one to the other, al- though but a few months may be occupied in the transition. It is by accident, in the street, at night or morning, that the patient perceives suddenly a numbness in the bottom of his feet, heaviness and feebleness in the inferior members, or some difficulty in walking. Our patient, Kelch, described these very characteristically. On rising in the morn- ing, he first began to perceive that his feet were “ asleep,” that it was necessary to stamp them upon the floor for sometime before he was able to walk with any degree of certainty. This symptom was at first only experienced in the morning, but it gradually increased so as to be felt at all times. He attributed the uncertainty of his steps entirely to the sensation of numbness* The progress of the malady is not always gradual; sometimes the symptoms occur suddenly and by leaps, as it were. When the symp- toms of the first period have not appeared, or should they be incom- plete, the beginning of the second period may be still either insidious or well-marked. Numbness, ataxia, anaesthesia, dysuria, and the same ocular phenomena may appear at the same time or at short intervals. Numbness.—The ataxic allude under this name, to an internal sensa- tion of heaviness, seated in a part or whole of a member, as if it were asleep. The numbness is a phenomenon, connected with the general sensibility, but distinct from the sense of touch, of heat, of pain, and from the muscular sensibility. It is a perversion of the functions of the sensitive nerves, as is the pains or the tingling. Some patients employ the words numbness and tingling as convertible terms. Kelch, so em- ploys them in describing his sensations. The tingling, however, be refers to his forearms and hands, especially the right, more than to the inferior extremities. It corresponds to the distribution of certain fila- ments of the radial nerves and is accompanied by uncertainty of touch and inability to use his hands properly. As remarked in the history of his case, he had been unable for sometime to use his knife and fork. This sensation, however, did not extend to the trunk as it often does. Cutaneous Anaesthesia.—Anaesthesia is one of the habitual symptoms of the progressive locomotor ataxia. In 109 observations, this symp- tom, complete or incomplete, was present in 76 cases, very lightly in 15, and not at all in 18 cases. It was very manifest in our patient, Kelch. A diminution, perversion, or exaltation of sensibility is found in this malady in the skin and in the mucous surface accessible to our investi- gation ; but we have here in view the cutaneous anaesthesia, chiefly. PROGRESSIVE LOCOMOTOR ATAXIA. 14 M. Beau recognizes two modes of sensibility in the integument—one special, or sensorial, as the sense of touch; the other general, as the sense of pain. M. Landry distinguishes a third, the sensibility to temperature, When the anaesthesia is complete there is no difficulty; but if one or two of the three kinds of sensibility, only, is involved and that lightlyy it is important to apply the best and most delicate tests. An examina- tion of the ataxic to be complete should be extended to the four mem- bers, to the trunk and face, and to many points in the various regions,, which habitually, are very unequally and differently affected. The prick of a sharp pin, pinching with the nails and with an artery forceps,- plucking the hairs, tickling, the successive application of one or more fingers, of cold or hot bodies, the electric shock, may all be applied to- determine the extent of the anaesthesia. When it is difficult to deter- mine the modification of sensibility, comparison should be made between two symmetrical points, as the plantar surface of the feet, the inner faces of the thighs, the forearms, etc. Incomplete anaesthesia, as the numb- ness, advances step by step, from the plantar surface of the foot toward- the pelvis and trunk. Its intensity is stronger in- the part primitively affected, and its superior limit is not well defined. For example, the plantar surface of the foot, is without sensibility to touch, to pain or to- tickling, the calf of the leg is somewhat more sensitive, the thigh is slow in transmission of the impression only, and the belly is normal. In the superior extremities the anaesthesia is experienced in the fingers, espe- cially the ring and little fingers, diffuses itself over the rest of the hand and forearm, rarely passing above the elbow; but it reappears in the neck, in the inferior half of the face, and extends to the tongue, to the internal face of the jaws and to the vail of the palate. In our patient Kelch, this partial anaesthesia was confined to the hands and forearms— the ring and little fingers being more affected than the rest of these parts. The two halves of the body are not necessarily symmetrically affected. Thus one arm, one side of the face, of the tongue, is affected,, while the other side may not be. The sensibility to pain is diminished or abolished in places, in regions or in the whole extent of a member. The part affected may be pricked and the blood drawn without evidence of pain, but it is indis- pensable that the point of the instrument be exceedingly fine, for an ordinary pin will arouse the sense of touch. Pinching with the nails,, which is easily graduated, has not this inconvenience. The sense of touch is habitually altered. The patient is deceived as to the real cause of an impression and as to the point at which it is made. He does not dis- 15 PROGRESSIVE LOCOMOTOR ATAXIA. tinguish the touch of one or more fingers, from that of some other object. He confounds a small roll of paper with a pencil. The con- sequences of tactile anaesthesia may be, thus, attributed to ataxia. Trousseau and Duchenne have observed that the thermoscopic sensi- bility continues in the ataxic. It was hence supposed that this consti- tuted a means of differential diagnosis between progressive locomotor ataxia and other affections of the cord, but subsequent investigations have shown that this view is erroneous; the thermoscopic sensibility may exist in various diseases of the cord. Muscular Anaesthesia.—The sentiment of muscular activity, the mus- cular sense, the muscular conscience, are synonymous terms, clearly separable, however, from the muscular sensibility. Several methods may be adopted to ascertain the state of the muscular sensibility. The electrical method is mathematically accurate. It is applied as follows : the skin having been dried with some absorbent powder to neutralize the superficial effects of the electricity, a current of variable intensity is passed through a muscle in the direction of its length. In the physiological state this current determines a contraction, but in addition thereto, a dull sensation if it is feeble, and a pain if it is strong. This sensation—as was the case in our patient Keleh—what- ever may be the intensity of the current, is in the ataxic, either very feeble or null. We thus measure the electro-muscular sensibility as it is called. The second method is more easy of execution. This consists in ascertaining the degree of sensation produced by pinching with the pulps of the fingers a sufficient quantity of the skin; afterward of a por- tion of healthy muscle, the sterno-mastoid, or biceps for example. This pressure graduated at will, may be made to produce acute pain. The suspected muscles are afterward explored in the same fashion. It is rare that the responses of the patient, how little intelligent soever he may be, leave any room for doubt. The third method is more especi- ally to determine the state of the muscular sense. It is based upon this : In the physiological state, without having recourse to our sense of vision, we are concious of the direction which the will imposes upon our members, and of the precise point at which a movement is arrested. The use of the eyes is necessary to the ataxic, to conduct his movements It is also useful to ascertain the degree in which the patient possesses the faculty of appreciating the weight of bodies, or the knowledge of the amount of muscular force necessary to raise a given weight. With this view different weights are placed in his hands, and his appreciation of them noted. Locomotor Ataxia.—The locomotor ataxia is described in general 16 PROGRESSIVE LOCOMOTOR ATAXIA. terms by Topinard, as a disorder of the function which presides over progression, equilibration, and other voluntary muscular acts, not caused by paralysis, muscular atrophy, deformity of the skeleton, etc., and different from paralysis agitans, chorea, or general or partial convulsions tonic or clonic. The phenomena of the locomotor ataxia are to be studied as they manifest themselves in the inferior extremities, in the superior extremi- ties, in the face, etc. Those partaining to the inferior extremities are most interesting and important. In our patient Kelch, as indeed in the greatest number of cases, the uncertainty of locomotion was most mani- fest in the morning in dressing. Sometimes it is felt in the street, more often in the evening and in darkness. They experience pain in mounting the stairs, or in stepping up on to the pavement. They con- tinue to attend to business, but find they grow more readily fatigued than was their wont. A strong emotion, a slippery pavement, or the exertion to avoid an approaching carriage causes them to trip and fall unless there be some ready support at hand. Already they appreciate the utility of vision, and say that the disorders of locomotion are ex- aggerated because their attention is withdrawn from their own move- ments. To give them assurance in walking, they must have the idea that some one is near to succor them. The fears which they display, come of the experience which they have acquired of the instability of their movements and insufficiency of their support, and do not prove pusillanimity. Some authors have imagined that the defects of control of the voluntary movements are only experienced by the patient in the erect position ; but they are also observed when he is in the recumbent position. When the patient gets under the bed clothes he extends him- self by a quick and rough movement. If he be desired to execute a given movement, the muscles become rigid, the foot is extended upon the leg, the leg upon the thigh, and the limb, all of one piece, launches out at a single bound, or by irregular jerks, in the desired direction. The motions of the pelvic-femoral muscles, are most visibly ataxic. In- telligent patients explain the rigid extension which they instinctively give to the foot and leg in executing a given movement, by saying that their muscles are more rebellious to the will in intermediate attitudes. When the patient is required to assume the erect posture, and place his feet together, he is seen to oscillate from side to side, betrays a cer- tain anxiety, stretches out his arms like a ballancer, in spite of himself, and finally falls. In walking the ataxia assumes considerable proportions, in many cases ; in others, however, it is almost inappreciable. To facilitate the 17 PROGRESSIVE LOCOMOTOR ATAXIA. description, we distinguish arbitrarily, in respect to intensity, three degrees of ataxia. In the first degree the patient raises his feet roughly, one after the other; they describe the arc of a circle with the convexity turned out, without dragging upon the floor; the toes elevate themselves a little in advancing, and the heels turn in toward each other, striking the ground with force. He walks without decision, and can not place his feet where he desires. In the second degree, the walking has been compared to that of a man inebriated, or to that of a landsman on a sailing packet, or to that of a rope dancer. An examination of the muscles discloses the fact that the flexors and extensors are hard and stiff. The triceps forms a considerable protuberance, which gives to the limb a special and forced configuration. The walk is carried on by the aid of the pelvic-femoral muscles. The feet are raised quickly, are jerked forward, the toes being elevated. In the third degree of ataxia the patient is unable to step without holding on to a chair, to the bed post, or without being supported by two attendants. The sole of his foot seems in perpetual search of a point of support which continually escapes. Sometimes one of the feet turns into such a position that its outer side rests on the ground, and its dorsum appears in advance; or one leg, describing a great circle, becomes locked behind the external malleolus of the opposite limb. It is difficult to describe the precautions and the stratagems which are used by those patients who have retained the use of their arms, in getting out of bed on to a chair and in pushing up to the stove. Their attention is applied to avoid the most feeble voluntary movements, they dare not abandon their caution for an instant, knowing that the first effort of muscular contraction will be the signal of an agitation which all their efforts can not repress. In the superior extremities the ataxic disorders are less characteristic, and, also, less frequent. In Kelch they were marked and came on early. In 118 cases collected by Topinard, the ataxic disorders extended to the upper extremities in 46 cases, and in one only, were limited exclusively to these members. There are some differences, also, in the phenomena observed, due to certain differences in the physiological properties of the lumbar and cervical enlargements of the cord. To discover the troubles of co-ordination of the superior extremities, many methods are employed. They were disclosed in Kelch by an ina- bility to use his knife and fork. The patient may be desired to make the sign of the cross, to carry his index finger to the extremity of his 18 PROGRESSIVE LOCOMOTOR ATAXIA. nose, to pick up some small object, etc. His movements may be ex- amined when lie eats, dresses, sticks a pin, ties a cravat, etc. These ataxic disorders appear in the upper extremity usually after they attack the lower; sometimes in the two extremities simultaneously. The first indication of them is seen in the awkwardness of the patient in perform- ing the various acts above mentioned. lie does not touch directly with the index finger, the point of his face which is designated to him. In all those acts in which the sense of touch is employed, and in picking- up small objects, the ataxia becomes plainly visible. In the third period, all of the symptoms of progressive locomotor ataxia have become general; the signs of the first and second period are united, much more intense and occupy a more extended surface. The movements are frightfully irregular and disordered in the erect position; prehension is impossible; cutaneous anaesthesia extends to impressions of heat and cold ; the muscular anaesthesia is absolute ; the muscular sense is abolished; there is incontinence of urine and fames, and lastly, a true paralysis supervenes. State of the Muscular force.—The ataxies are in general vigorous sub- jects, well nourished, whose muscles are hard and prominent, and limbs are voluminous. A small number, on the contrary, are irritable charac- ters, hypochondriacs and feeble. Before using the dynamometer to ascertain the state of the muscular force, the physician must study the constitution, temperament, idiosyn- crasies of the subject, the lowering of his vital forces by mental depres- sion, insomnia, bad hygiene, by the sojourn in hospital, etc. A simpler method than the use of the dynamometer, is that employed by Topinard, as also by Duchenne. For the muscles of the arm, shoulder, thigh, leg or foot, they should be placed in the attitude to insure their maximum contraction, and the patient should be directed to maintain, with all his force, this position; the degree of force necessary to overcome this re- sistance, will be a measure of the muscular force. It is sometimes necessary in the same subject to compare sound with affected muscles, or one side of the body with the other. The fundamental condition of the ataxic paraplegia of Todd, and the progressive ataxia of Duchenne is the integrity of the muscular force. This proposition of Duchenne, is a little too absolute. Ordinarily the ataxia exists with persistence of a sufficient degree of muscular force to preclude the idea of paralysis; but, also, in a great number of cases there is a progressive feebleness, more apparent in the regions where the evidence of the other affections is most prominent, due to the influ- ence of the cord upon nutrition and to an alteration of the motor parts PROGRESSIVE LOCOMOTOR ATAXIA. 19 of this organ. Finally incomplete, and sometimes, complete paralysis is a remote termination of the malady. The anatomy and physiological pathology of this disease agree perfectly with this view. Considered with reference to the periods, it is certain that integrity of the muscular force is a character more constant in the second, although its diminution is an indication of aggravation of the lesion in the cord. Course and Duration.—The course of the malady has been suf- ficiently indicated in the preceding pages. The duration of the first period only, can be approximatively fixed. We have before remarked that it oscillates between some months and twenty years; it averages from four to five years. From the origin of the disease, indicated with more or less clearness by the pains, the ocular troubles and the ataxia, to the second period—in 4 cases the duration is spoken of as months; in 6 cases as one year ; in 27, as from two to three years ; in 47, as from four to seven years; in 17, as from eight to fourteen years; in 18, as from fifteen to thirty years. The average is seven years. We have seen that in our patient Kelch, the duration of the first period, was somewhat less than one year—taking as the point of departure from the state of health, the sudden occurrence of spermatorrhoea. If, however, we assume that the ocular troubles were the first symptoms in his case, six months will in- clude the whole course of the malady, so far as it had developed itself. There can he no question that the sexual disorder was the first symptom. It will be perceived that the phenomena observed in his upper extremi- ties, were those of the first period, and not of the second. We have consequently no means of determining the duration of the first period if its evolution had not been interfered with it. As Topinard’s statis- tics show, somewhat more than four per cent, had a duration less than one year. Complications.—These are divisible into two classes: those pertaining to the cerebro-spinal axis; those of other parts of the body. Among the first are spinal meningitis, congestion and softening of the cord and of the brain, general paralysis, and progressive muscular atrophy. Cephalalgia, dizziness and intellectual disorders which sometimes ac- company the ocular derangements, must be viewed as complications.. Some patients complain of loss of memory, and forgetfulness of certain words, certain events, etc. Others present various hallucinations. Mania or dementia, is found at an advanced period—the termination of the second or in the third period. The complications of the second class are either acute or chronic. Of the acute affections, cystitis, enteritis and erysipelas are the most fre- quent. Cystitis usually results from the prolonged retention of the 20 PROGRESSIVE LOCOMOTOR ATAXIA. urine—the bladder expelling it but in part. The symptoms first ob- served, are those of sub-acute, afterward of chronic cystitis. The in- flammation extends along the ureter to the kidneys, and finally gives rise to urinous fever and putrid infection. The occurrence of enteritis, Topinard is disposed to ascribe to the prolonged use of the nitrate of silver, the remedy so much vaunted by Wunderlich in the treatment of the progressive locomotor ataxia. Of the chronic affections, phthisis pulmonalis is most common. In 44 deaths, 13 were attributed to this cause. common form presents two distinct periods—the first and the second—or begins by locomotor ataxia, and has f The first symptom in our patient, Kelch, was a remarkable increase in the activity of his sexual desires) this, however, was quickly followed by profuse spermatorrhoea. Was there any thing in the occupation or external conditions of our patient, Kelch, to account for the production of his symptoms? In his business, as a gilder, gold foil is the substance chiefly employed, and a small quantity of white lead in the preparation of the frames. The symptoms have been ascribed, by some persons unacquainted with the essential phenomena of the progressive locomotor ataxia, to lead poison- ing. We might adduce, in answer to this doubt, two cases quoted by Topinard, in the first of which (ob. xxxiii) the symptoms were clearly those of lead poisoning (intoxication saturnine), but in the second, a painter (ob. cxcviii), all of the phenomena were those of the progres* sive locomotor ataxia. Kelch had never suffered from colic, paralysis, nor did he present any of the evidences of mineral poisoning, except the symptoms of the disease under which he labored, which may or may not be attributed to his occupation ; most probably not. There is no evidence of hereditary tehdency in his case. Treatment.—The treatment of the progressive locomotor ataxia is a most unsatisfactory subject. The term progressive is unfortunately too often most fitting) for no matter what therapeutical expedients we may adopt, the disease continues on its course. Much depends, how- ever, as we have already seen, upon the period of the disease in which we find our patient. The very unfavorable prognosis ordinarily ex- pressed, heretofore, must now be somewhat modified. The cases which we have adduced show that appropriate treatment may accomplish much, if employed during the stage of functional derangement. JDuchenne relies upon the iodide of potassium and faradisation. We have quoted two cases from the Medical Times and Gazette, in which the use of the iodide was followed by improvement. Dr. Huglilings Jackson says that this drug was of considerable benefit in one case un- der his charge, and without service in others. Our own case is a strik- ing example of its value. The nitrate of silver, proposed by Wunderlich, has been much em-- ployed with apparent advantage in some cases, but in many more it has failed utterly. The propriety of prolonged administration of this remedy is very questionable; at most the benefit is very doubtful, and 27 PROGRESSIVE LOCOMOTOR ATAXIA. its ill results are certain. Topinard attributes to this remedy the ritis which has been observed in some cases. Ergot and belladonna in combination proved very successful in the hands of Dr. Charles Taylor. Trousseau thinks favorably of the latter, Arsenic, oil of turpentine, and strychnia have also been used without any very decisive results. The favorite remedies with the Drench are sulphur baths, douches, and electricity faradisation chiefly. An improved hygiene is very important. The subjects of this mal- ady are principally those who have been exposed, by reason of poverty and unhealthy occupations, to the common causes of disease. A more abundant diet, a purer atmosphere, warmer clothing, and cleanliness have in many instances apparently arrested the progress of the disease. These hygienic means should not be neglected in any case. Without their aid remedial agents will probably prove useless. Certainly the facts which we have presented in the course of this paper give us en- couragement to persevere in the treatment, for, if not curable, we have every reason to hope for an arrest of the morbid action, an improve- ment in the symptoms, or a decided amelioration in all of the attendant phenomena,