Chapter M: M——, was a stout Irish woman about forty years of age. She had (21)

{817} Aside from those variations due to the distribution, extent, and intensity of the lesion there are others which depend on the rapidity of its invasion. There are three types in this respect—the apoplectiform, the ordinary acute, and the subacute. The term apoplectiform has been used in two different senses, one being clinical, and referring to the rapidity of onset of the symptoms; the other anatomical, and referring to the nature of the lesion. It is, however, doubtful if a distinction in the latter sense is practically valuable. The presence of a blood-clot in a myelitic focus is itself secondary to the softening, and the intensity and rapidity of the process must have shown itself in the development of the latter.[95] The term ought, therefore, to be used in a clinical sense only.

[Footnote 95: I am able to recall but a single case in which, even clinically speaking, a primary hemorrhage into the cord-substance was plausible. In this instance a sudden arrest of menstruation had occurred nearly simultaneously with a combined strain and exposure in a young girl.]

The constitutional symptoms of acute myelitis—which, however, are often absent—are its most distinctive features in one sense. Disorders of motion and sensation and perversions of nutrition are signs common to all destructive affections of the cord, whether of a traumatic, neoplastic, or a chronic inflammatory character. But fever, headache, and delirium,[96] associated with gastric and visceral disturbances of acute development, are not found to be initial and intrinsic symptoms with them as with acute myelitis. In their absence the mode of onset is characteristic. A high degree of paralysis, motor or sensory, is developed with a rapidity unequalled in any chronic affection of the cord. Sometimes there is a prodromal period in which formication, numbness, and disorders of movement are observed in the same part of the body which are destined to become paralyzed at a later stage of the malady. Within a few hours, days, or at most weeks, complete paraplegia may become developed. The prodromal symptoms may include any form of disturbed sensation. Lancinating pains, tingling, a feeling of the limbs falling asleep, peculiar and indescribable sensations attending the acts of micturition and defecation or of placing the feet violently on the ground, are common. Occasionally they are found in one limb only, although motor and sensory paralysis may ultimately occupy the symmetrical member as completely as the one first involved. In some cases it has been noted that the patient is unable to lie down, or, if lying, to occupy the dorsal recumbent position. This feature has been utilized to support the theory of an initial congestion, which is supposed to be relieved or aggravated according as the cord is kept elevated or depressed.

[Footnote 96: These symptoms are to be regarded as actual parts of the myelitic symptoms only when they accompany the prodromal or initial periods. The later complications, uræmia and septicæmia, the latter arising from pyelitis, cystitis, or decubitus, often lead to constitutional disturbance which is not due to the myelitis as such.]

In most cases of transverse myelitis, when the anterior cornua are destructively involved, we possess in the electrical tests valuable and unerring means of determining the altitude and extent of the lesion. Whenever we find the atrophy of a paralyzed muscle accompanied by qualitative electrical changes in myelitis, we must conclude that the cell-group from which that muscle receives its nerve-supply is destructively involved.[97] These changes are particularly well demonstrable when the {818} cervical or lumbar enlargements are affected. They are not as readily ascertainable in the case of a transverse myelitis in the upper dorsal region, on account of the situation of the muscles supplied by the upper dorsal nerves, and the consequent difficulty of application of the necessary tests.

[Footnote 97: The proposition, originally, I believe, made by myself, that there are distinct cell-groups in the spinal cord which are constant with certain animal species, and correspond in relative development to ventral, dorsal, and appendicular muscular groups (“Architecture and Mechanism of the Brain,” _Journal of Nervous and Mental Diseases_, April, 1880), appears to be confirmed in a general way by the researches and cases of Edinger, Kahler-Pick, Dejerine-Major, Genzmer, Von Monakow, Sahli, Prévost-David, F. Schultze, Remak, and Parrot-Joffroy; for the knowledge of the first and last of which I am indebted to the review of the subject by Starr (“Localization of the Functions of the Spinal Cord,” _American Journal of Neurology and Psychiatry_, August, 1883).]

The disturbance of the deep reflexes with very few exceptions affects the same peripheries as are represented in the destroyed and impaired gray nuclei. Thus, if the lower part of the lumbar enlargement be affected, the reflexes of the Achilles tendon and the gluteal muscles will disappear; if the upper lumbar enlargement, the knee-jerk disappears.[98] Disease of the lower part of the cervical enlargement is in like manner associated with absence of the wrist-tendon reflexes, while the disappearance of the elbow-tendon reflex suggests a higher involvement at the levels of the fifth and sixth cervical nerve-roots.

[Footnote 98: The disappearance of the knee-jerk and similar reflexes was originally supposed to be a phenomenon exclusively pertaining to spinal disease and to a destructive lesion anywhere in the track of the centripetal and centrifugal nerves connected with that segment of the cord in which the reflex is supposed to be translated. But aside from a number of physiological observations[99] which prove that cerebral conditions may influence the intensity of the jerk, there are pathological ones which show that it may be permanently abolished in disease of the pons and cerebellum, and temporarily abolished or diminished immediately after capsular and ventricular hemorrhage. The associated symptoms in the former case, and the history of the onset and total hemiplegia in the latter, serve to distinguish them from destructive spinal lesions should the occasion for discriminating between them ever arise; which is not likely.

[Footnote 99: S. Weir Mitchell and M. J. Lewis found that voluntary effort increases the jerk at first, but if continued diminishes its excursiveness (_The Medical News_, 1886, Feb. 13th and 20th).]

In complete transverse acute myelitis of the cervical region high fever is a constant symptom. In unilateral myelitis of this region flushing of the face and unilateral sweating are produced, together with iridoplegia, sometimes preceded by dilatation, owing to initial irritation and succeeding paralysis of the sympathetic branches originating at this level of the cord.

Just as the disturbed reflexes and the belt sensations enable us to distinguish at what levels of the cord the myelitis is situated, so the distribution of the motor and sensory paralysis affords corroborative evidence of such location and additional proof of its extent and intensity.

Transverse myelitis at and above the level of origin of the phrenic nerve is almost immediately fatal, through its interference with the innervations required in respiration. In the upper part of the cervical enlargement it produces complete paraplegia of motion and sensation in the trunk and all four extremities. In the lowest part of the cervical enlargement it produces paralysis of the same parts, but the serratus magnus and scapular muscles escape. The nuclei of origin of the muscles moving the upper extremity are situated so that those which are farthest removed from the axis of the body when the arms are extended are situated lowest in the cord. The sensory paralysis is distributed in harmony with the motor paralysis; that is, when there is paralysis of motion in the hand {819} and forearm the anæsthesia or subjective numbness is also in the hand and forearm. The same correspondence is not found in affections of the lumbar enlargement, for anæsthesia of the gluteal region accompanies paralysis of the crural muscles when the lesion is low down at the level of the lower lumbar and upper sacral nerves. The distribution of the anæsthesia, in other words, is not by segments of the limb, but by surfaces. The gluteal, posterior femoral, gastrocnemial, and outer pedal surfaces are affected together with the muscles moving the foot, while the thigh and inner side of the leg and foot become anæsthetic, with lesion of the upper part of the lumbar enlargement accompanying paralysis of the quadriceps and deep muscles. It is not difficult to understand this discrepancy when we bear in mind the different plan of distribution followed by the brachial plexus as compared with the lumbar and sacral plexuses. It is not, in my experience, found that the anæsthesia affects that surface which covers the part moved by the paralyzed muscle; which is characteristic of associated paralysis and anæsthesia from cortical disease.

One of the most dreaded occurrences in acute myelitis is the malignant bed-sore. The ordinary decubitus which results from the protracted sojourn of the patient in bed, coupled with the prominence of his trochanters and sacrum resulting from general or atrophic emaciation, is also common, but is comparatively benign and easy to prevent or to manage when established. The malignant bed-sore, on the other hand, is a spontaneous occurrence, due to the same obscure but undeniable trophic influences exerted for good by the normal and for evil by the diseased nerve-centres, which play so large a part in the symptomatology of tabes dorsalis. It cannot be avoided; it is not due to pressure alone, or, as some have claimed, to the macerating influence of the dribbling and decomposing urine. The development of this lesion is exceedingly rapid, and it may be regarded as a sort of local gangrene. The skin shows a livid color; vesicles appear, then burst; the part becomes denuded; and within a few days a deep ulcer with a dark border and base appears, discharging a sanious fluid. The subsequent history is that of a rapid extension and destruction of the neighboring tissues, even down to the bone, and if situated over the sacrum opening into the spinal canal through the necrotic arches of the sacral vertebra, thus leading either to general septicæmia or to putrid infection of the spinal meningeal sac. Occasionally, gangrenous spots coexist on other parts of the body, notably the lower extremities, where neither pressure nor maceration can be accused of playing a part, proving that the process is primarily due to the spinal affection.

Acute central myelitis, as described by Dujardin-Beaumetz, Hayem, Hallopeau, and Erb, usually runs its course very rapidly. Indeed, all of these observers speak of it as the most violent and quickly fatal variety of spinal inflammation. I have, however, seen one case with T. A. McBride at the Presbyterian Hospital in which all the characteristic symptoms of acute central myelitis were markedly developed and present in their characteristic groupings, and yet the patient had been suffering from progressing symptoms of myelitis for one year and a half before that time.[100] {820} Usually, complete anæsthesia and paralysis of the lower half of the body occur in this form. But the most characteristic feature is a rapidly progressive atrophy not only of the paralyzed muscles, but also of some which are still partially under the dominion of the will. With this there is extreme vesical and rectal trouble, the sphincters being paralyzed. As a rule, the deep and superficial reflexes are destroyed—they are always diminished—and trophic disturbances of a malignant type, such as acute decubitus, joint-changes, and œdema, are common. The paraplegia is characterized by the flaccid condition of the limbs; the contractures and spastic symptoms found with other forms of myelitis are entirely absent, and qualitative electrical changes, beginning with disappearance of farado-muscular contractility, are found in the atrophying muscles. There are marked constitutional symptoms with this form; the tendency to an ascent of the process and successive involvement of one segment after another of the gray matter is great, and a fatal issue, as far as known, is inevitable.

[Footnote 100: At the time the patient had undergone such a profound change in appearance that I failed to remember him, and it was only by accident I learned that I had seen him in private consultation with his family attendent, F. A. McGuire, a year previous. On the latter occasion I had made the diagnosis of subacute myelitis chiefly limited to the posterior columns; there were ataxia, both static and locomotor, slight incontinence, belt sensation, and ocular symptoms, with abolition of the deep reflexes in the lower limbs.]

DIAGNOSIS.—The principles governing the determination of the affected region of the cord in acute myelitis are exactly the same as those detailed in the later sections on Chronic Myelitis or Sclerosis, the acuteness of the onset, and the relapses which sometimes occur, and the predominance of irritative spasms—which, however, is an inconstant criterion—serving to distinguish between the acute and chronic form of spinal inflammation. In the present state of our knowledge it is impossible to always differentiate between acute central myelitis and syringo-myelus—a condition in which the formation of a periendymal neoplasm, and its subsequent breaking down in the axis of the cord, lead to the formation of a tubular cavity.[101] The neoplasm in this instance is classified among the gliomatous new formations. The symptoms depend, exactly as do those of myelitis, on the distribution of the destructive lesion. In some cases the posterior cornua and columns are chiefly involved, and extreme anæsthesia is found; in others the anterior columns are affected, and the symptoms of a poliomyelitis or an imperfect transverse myelitis may be imitated.[102]

[Footnote 101: This cavity, unlike that of hydro-myelus, is not a dilatation of the central canal, but, lying to one side of it, is excavated in the cord-substance.]

[Footnote 102: Repeated fractures have been noted in cases marked by profound analgesia. It is believed that they are not always due to trophic changes, but may be the result of muscular action, exaggerated on account of the patient's inability to gauge his efforts. Still, in the majority of cases the presence of positive trophic disturbances of the skin seems to indicate the probability of some textural change facilitating the fracture.]

As a rule, the sensory disturbance in syringo-myelus is out of proportion to the muscular atrophy developed; that is, it involves a far more extensive province. It is usually of a peculiar character: some forms of sensation are involved but slightly, or even escape, and others may be nearly destroyed. Commonly, it is the pain and temperature-sense which suffer most, while the cutaneous space and pressure, as well as the muscular sense, are not materially disturbed. These peculiarities are not commonly found in cases of myelitis, and when present, and particularly when the paralytic or sensory affections involve all four extremities {821} alike, they suggest the existence of syringo-myelus. As yet we are unable to make more than a probable diagnosis between the two diseases.

DURATION AND PROGNOSIS.—The duration of the disease varies. Cases of the apoplectiform variety are mentioned, in which the process reached its height in a few minutes, or where the patient, having retired in good health the night before, awoke finding himself paralyzed in the lower half of his body. Death may terminate such a case in a few days or weeks. In another class of cases, complicated by serious involvement of the bladder, the fatal termination is often precipitated by putrid cystitis, pyelitis, or uræmic poisoning, and even in cases which have passed the dangers of the early period in safety these ominous complications may develop with the usual result many years after the beginning of the illness. In a number of cases the first period, that in which the morbid process becomes developed, is followed by one of comparative quiescence, in which the paralyses of sensation and motion then established remain stationary for months and years. A number of authors, Erb, Leyden, Strümpell, and Ross, speak of such a case as one in which chronic myelitis has followed an acute myelitis. It seems improper to use the terms acute or chronic in this way. As it is generally understood that the term acute applies to myelitis in which disintegration of the nerve-elements predominates over interstitial proliferation, and in which the secondary sclerosis is rather like the cicatrix of an acute inflammation and necrosis, it should not be confounded at any period, no matter how similar the clinical signs may be, with a process which is essentially an interstitial one from the start. If chronic amaurosis results from an acute glaucoma, we do not change the latter designation to chronic glaucoma.

In cases where the symptoms at the acme indicate rather an involvement of the peripheral than the central paths of the cord, and in which an incomplete motor and sensory paralysis develops, the patients often regain a considerable amount of motor power and sensation, so that they may reach a good age, suffering at most from a paresis of some one muscle or muscular group, occasional bladder trouble, and pains. It has been laid down as a rule that where paraplegia and other signs remain stationary for years, there is no hope of even partial recovery. The following remarkable and well-attested case proves that this rule is not without exceptions:

Isidor K——, æt. forty-four years, worker in tobacco. After over-exertion in the fall of the year 1879 he had numbness, tingling, dorsal pain, and paresis rapidly developed, which induced him to consult the physicians at the clinic of the University of the City of New York. After some slight improvement a relapse occurred, and several others followed, usually provoked by over-exertion, till he became completely paraplegic. He was, according to his account, several times exhibited to a medical class by William A. Hammond, and remembers that this authority spoke of a possible ascent of the affection and ensuing involvement of the arms. His bladder was at no time seriously disturbed. For four years and three months he was totally paraplegic; his lower extremities were without life; and for the greater part of the time he could not move his toes. The limbs were cold and pale, but underwent little atrophy. The only exercise obtained during this time was in a roller carriage. His sexual power was abolished throughout the whole {822} four years and over. Nothing can be learned as to his reflexes in this period.[103] The paralysis of sensation was as complete as that of motion, and the lower limbs never perspired. The arms remained free. There was a dorsal belt sensation.

[Footnote 103: Hammond has no notes of the case, having discontinued the clinic, and Ludwig Weiss, the physician in charge, saw him only occasionally in behalf of a benefit society.]

On July 23, 1884, shortly before mid-day, while lying on the bed, in which he had lain a helpless cripple for over four years, except when lifted into the roller carriage, he felt a sudden rush of warmth. Surprised at this first sensation he had felt for years in limbs which had been quasi-foreign appendages, he raised up the bed-clothes and saw that they changed color. There was some tingling for about three minutes, and a perspiration broke out in the affected members. With this he found he could move his feet: half alarmed, half exulting, he sent for his physician, L. Weiss, who found that the patient could stand and walk with considerable freedom. I was then consulted, and found the patient presenting a picture of incomplete transverse myelitis. He could walk, turn about, stand with closed eyes with slight swaying, and his knee-phenomenon was of short excursiveness, but exceedingly spasmodic, and this symmetrically so. He was carefully watched, and against the advice of his physician engaged in peddling cigars, and subsequently took a position as attendant at the pauper asylum on Ward's Island. Here he was on his feet fully twelve hours a day, and his motion, which had continued improving until it was to all practical intents and purposes normal, aside from a slight stiffness, again became impaired, and a joint trouble in the metacarpo-phalangeal articulation of the right little toe, which had troubled him a week after his partial recovery, recurred.[104] On January 15th of the present year I again examined him. His knee-phenomenon was greatly exaggerated, cutaneous sensations scarcely impaired, gait paraparetic, but he could walk great distances, and claimed to suffer less from the exertion than from the tenderness accompanying the joint trouble referred to. There had, therefore, occurred, without any assignable cause—for the patient was not under treatment for a year or more before the event—an almost instantaneous restoration of sensation, locomotion, and sexual power; all of which faculties, notwithstanding the infraction of every medical direction given, remained established for two years, with prospects of so continuing a longer period.

[Footnote 104: This was a trophic joint trouble.]

TREATMENT.—Most of the therapeutic propositions relating to the treatment of the acute myelitic process are based on the theory that it is of a congestive character or associated with congestion. Accordingly, the internal administration of such drugs as ergotin, which diminish the calibre of the blood-vessels, and local measures, such as depletion, wet cupping, and counter-irritation, intended to act in the same way by derivation, are unanimously recommended by authorities. The suggestion of Hammond, that the patient occupy a ventral or lateral, and not the dorsal, position, is based on, and entirely consistent with, this same view. It is difficult to say what effect is attributable to these measures. Remarkable spontaneous changes—retrogressions as well as advances of the morbid process—occur equally under expectant as under active treatment. I have never seen any improvement in the active phase of simple myelitis which {823} I felt confident I could attribute to any special remedy employed with a view of acting directly on the morbid process. Indeed, improvement has been claimed by Jewell as a result of the use of strychnia—a drug which under the very dogmas governing the orthodox treatment of the disease might be expected to do positive damage.[105]

[Footnote 105: Jewell gives very large doses of this alkaloid. L. C. Gray, in a discussion held before the American Neurological Association, cited numerous observations directly conflicting with those of Jewell. I cannot, in view of a recent observation in a typical case of acute anterior poliomyelitis, in which by accident the toxic effects of strychnia were obtained, consider this dispute as at all settled. In direct connection with the toxic symptoms the abolished patellar jerk returned in an exaggerated form; motion also returned, and rapid improvement ensued.]

In relapses of acute myelitis which had been brought on by chilling of the feet I have obtained good results by derivation to the lower extremities, and on many grounds think that the morbid process in the cord, if it can be affected at all, can be affected by treating the periphery where the symptoms are noted more readily than by applying the cautery or bleeding over the proven site of the disease. Exposure of the dorsal region to cold has not yet been noted as a cause of acute myelitis, whereas such exposure of the lower extremities is a frequent one. This seems to show that the spinal cord is more vulnerable to influences affecting its nervous distribution than to those which are topographically nearer. If this is true as regards morbid influences, it may be urged that it is plausible as regards remedial influences if these are to drive out the disease tendency by the same door it entered.

Rest is imperative during the active progress of the disease. It may be stated as a canon that the earlier the patient takes to his bed, and the more thoroughly he obeys the injunction to attempt no motion of the affected members, the better, cæteris paribus, will the result obtained be. Countless cases are on record where a relapse was directly traceable to a walk undertaken prematurely or carried farther than was wise. As convalescence or partial restitution advances, gradually increasing exercise is to be attempted, not waiting for the danger-signal of a tired feeling to discontinue it; for that feeling, developed, means positive harm already done. It is therefore necessary to allow the returning function to be utilized only within small limits at first, and extending them slowly.

In all cases of severe myelitis where the formation of bed-sores is to be apprehended the water-bed should be employed. Owing to the low temperature which the rubber sac constituting it has, it is necessary to cover it with some well-warmed and non-conducting material and to have the room well warmed. If bed-sores are already formed, they are to be treated according to ordinary surgical principles. Antiseptic means should be in the foreground in the case of the malignant bed-sore. It is to be remembered that the latter is a gangrenous process, and, in so far as the formation of a line of demarcation between the necrotic and the conserved tissue is concerned, the ordinary expectant rules of surgery govern the case: the water-bath appears to yield the best results. Ordinary bed-sores yield readily to mechanical protection and stimulating ointments or the balsam of Peru. Iodoform is recommended, but it produces granulations of an indolent character as compared with those obtained through the use of resinous ointments.

The warm bath is probably the most useful measure in acute myelitis. {824} In cases due to exposure I do not believe it can be applied too soon. Its temperature should, in the beginning at least, not be higher than about 88° F., and the duration about seven minutes. The continued administration will depend on the immediate effect on the patient, and the sittings can be ultimately prolonged to half an hour or even longer. It should be administered once daily, and, when the patient is not disturbed by the manipulation connected with its use, even oftener. Cold baths are recommended by the Germans, but it seems rather in the passive period of the disease than during its active phase.

The management of the bladder trouble is one of the most critical points in the treatment of most cases. The results of retention of urine are more frequently the cause of a fatal issue than any other single complication. Where there is complete retention continuous catheterization is to be employed, as recommended by Strümpell.[106] In other cases the bladder should be emptied thrice in the twenty-four hours. The catheter is to be thoroughly disinfected, and if the slightest sign of cystitis is noted the bladder should be washed out with astringent and disinfectant solutions. The continuous irrigation with a solution of corrosive sublimate, 1:2000, appears to give the best results.

[Footnote 106: A. Nelaton's catheter is introduced into the bladder, and kept in place by strips of adhesive plaster fixed to the inner aspect of the thighs. A perforated condom fixed to the catheter, and then fastened to the inguinal region, is in my opinion a better measure, especially in patients who have some motor power left. The catheter is connected with a rubber tube, which should run on a decline in order to effect complete drainage.]

If there be a syphilitic taint present—and more particularly is this true of cases where no other cause can be assigned—large doses of iodide of potassium[107] should be administered, and mercurial ointment energetically applied, even to the point of salivation.

[Footnote 107: Here, if anywhere, the so-called heroic doses are applicable.]

I am unable to say anything with regard to the local use of electricity in the active period of the disease. Ascending currents are recommended by Erb, weak currents of about five minutes' duration being employed over the part supposed to be affected. In order to secure sufficiently deep deflection of the current large electrodes are applied. The use of galvanism and faradism on the affected periphery, the former being preferred where atrophic, the latter where anæsthetic conditions preponderate, is to be instituted early and continued through the duration of the disease. The rules stated in the section devoted to electro-therapeutics are to be followed. In cases where the bladder or rectum are paralytic, these organs should have galvanism applied to them by means of electrodes shaped like catheters or sounds.

After the acute phase of myelitis is passed, the secondary period is to be managed according to the principles laid down in the section on the Treatment of the Sclerotic Processes.

{825}

THE CHRONIC INFLAMMATORY AND DEGENERATIVE AFFECTIONS OF THE SPINAL CORD.

BY E. C. SPITZKA, M.D.

The intimate dovetailing of parenchymatous and connective substance in the spinal cord renders the determination of the precise origin of a morbid process often difficult if not impossible. Whatever the mode of origin, the typical termination of inflammatory and degenerative spinal disorders is sclerosis—a condition in which the essential nervous elements suffer diminution or destruction, while the connective substance is either absolutely or relatively in preponderance. The clinical result is nearly the same in all cases, and with few exceptions depends less upon the histological character than on the topographical distribution of the lesion. It is for this reason that we shall follow rather the topographical than the morbid histological principle in classification.

The chronic disorders of the spinal cord here considered have as a common character the slow and progressive displacement of the normal conducting nerve-tubes by a grayish, reddish, or otherwise discolored tissue, which is firmer in texture than normal nerve-substance. Minute examination shows that it is above all the myelin of the nerve-tubes that is wasted or destroyed, while the axis-cylinders may share in this dissolution, or, as in one form of sclerosis, they may persist and even continue in their conducting function. This increased consistency is found to be due to the increase of connective-tissue trabeculæ and of the neuroglia. In some cases this increase is active and absolute, like the increase in certain phases of hepatic cirrhosis; in others it is rather relative and passive, being merely a condensation of the connective framework after the intervening elements have wasted away, just as the texture of a compressed sponge is more compact than that of an expanded one.

Crudely, the affections of the cord characterized by this change may be divided into two great groups—the regular and the irregular. The former are characterized by their limitation to special nerve-tracts. Thus, in primary lateral sclerosis we find a special tract in the posterior part of the lateral columns occupied by the lesion. A number of spinal diseases with well-defined symptoms are due to active morbid processes which similarly follow the normal distribution of the great nerve-tracts of the cord, with the same, almost mathematical, regularity with which the medullary white develops in the fœtal and infantile cord. In another class of cases we find that the same tracts are involved in like manner—not in the way {826} of primary disease, but through the interruption of the physiological continuity of the nerve-tract by some other disease, to which the sclerosis is therefore secondary. These are the so-called secondary degenerations.

The irregular affections of the cord comprise cases in which the sclerotic foci are either disseminated through the cerebro-spinal axis in numerous foci, or diffusely involve a greater or lesser length and thickness of the medullary cylinder, or, finally, extend in the transverse plane completely or nearly so across the section area of the cord at a definite level.

As the symptoms of the regular affections of the cord are by far the most readily recognizable, and a preliminary knowledge of them will facilitate the better understanding of the irregular forms, we shall consider the former first. They may be subdivided into two groups. The largest, longest known, and best studied consists of acquired, the other, containing less numerous cases and varieties, and rendered familiar to the profession only within the last decade, comprises the spinal disorders due to defective development of the cerebro-spinal and spinal-fibro systems.

Tabes Dorsalis.

SYNONYMS.—Locomotor ataxia and ataxy, Posterior spinal sclerosis; Graue degeneration der Hinterstränge (Leyden), Rückenmarksdarre (popular), Rückenmarksschwindsucht; Tabes dorsualis (Romberg); Ataxie locomotrice progressive (Duchenne).

HISTORY.—Undoubtedly, this disease must have been observed by the ancient masters of medicine, but their descriptions group together so many symptoms of other organic as well as functional diseases of the cord that it is impossible to obtain a clear idea as to what special form they had in view when they spoke of tabes dorsualis. This term was adopted by the German writers of the last and the earlier part of this century as designating all wasting affections of the cord; and it was not till Romberg in 1853 and Duchenne in 1858 differentiated the characteristic clinical signs, and Cruveilhier and Todd (1847) determined the distribution of the lesion in the posterior columns, that the medical profession began to recognize the distinctness of this the most common form of organic spinal ailment.

At this time, when tabes dorsalis became recognized as a clinical entity depending on sclerotic degeneration of the posterior segment of the cord, the inco-ordination or ataxia manifested in the peculiar gait of the patient was regarded as the most characteristic, essential, and constant symptom of the disease. It was regarded as an axiom in physiology that the posterior segment of the cord was subservient to centripetal and sensory conduction, and nothing appeared to follow more naturally than the conclusion that the patient exhibited inco-ordination because, his sensory conduction being interrupted or destroyed, he had lost the necessary gauge for judging of the required extent and force of a given movement. Since then both spinal physiology and pathology have undergone a profound revolution. So far are we to-day from regarding anterior and motor or posterior and sensory as convertible terms that we locate the {827} most important centrifugal tract of the cord behind the plane which divides it into anterior and posterior halves, while the most recently differentiated centripetal tract is represented by Gowers as lying in front of it. It has been determined, through Türck, Bouchard, and Flechsig and his pupils, that the posterior white columns of the cord contain several systems of fibres, whose functions—where determinable—are of an exceedingly complex nature. The elaborate investigations of Charcot, Schultze, and Strümpell, made during the past decade, have shown that the distribution of the diseased field in the posterior segment is not uniform, but is most intense in special areas. These were found to be affected with a remarkable constancy and regularity. Almost coeval with this discovery came the recognition of symptoms found in the earliest phases of the disorder by Westphal, which, attributable as they were found to be to the involvement of the special reflex mechanisms of the cord, enable us to demonstrate the existence and extent of the disease at periods which previously were not within the ken of the clinician. The reproach which Leyden was justified in making, that most tabic patients pass through the pre-ataxic phase of their disorder under the diagnosis of rheumatism or some similar affection, can now no longer be made. Our ability to recognize the advent of this disease has reached such a degree of refinement that the question arises whether we are always justified in alarming a patient who has a prospect of remaining free from invalidating complications for many years with the announcement of a disease which, above all other spinal affections, is looked upon with dread as equally hopeless, insidious, and distressing.

CLINICAL HISTORY.—The development of tabes dorsalis is typically slow and its precise commencement usually not determinable. In some cases this or that one of the characteristic symptoms of the disease preponderates from the beginning, and continues throughout the illness as a prominent feature; in others distinct stages can be recognized, each marked by one or several symptoms which were absent or slight in the other stages. In some cases the progress of the affection is marked by episodes which are absent or rare in other cases. As a rule, however, it may be stated that tabes is a progressive affection, marked by pronounced temporary changes for better or worse, which are often developed with astonishing rapidity, and subside as quickly, terminating fatally unless its progress be arrested by treatment or interrupted by some other fatal affection. The latter is by far the more frequent termination in the well-to-do class of sufferers.

For purposes of convenience we may consider the symptoms of the earlier period of the illness as pre-ataxic, and those of the later as the ataxic. It is to be remembered, however, that in the strictest sense of the term there is usually some disturbance of co-ordination even in the early period, while the symptoms of the pre-ataxic period continue over the ataxic period, and may even become aggravated with it. Often the patient does not himself suspect a spinal, or indeed any nervous, disorder, and consults the physician either on account of rheumatic pains, double sciatic neuralgia, bladder disturbance, diminished sexual power, amaurosis, diplopia, or even gastric symptoms, which on closer examination are revealed to be evidences of tabes. The determination of the initial symptoms is retrospective as a rule.

{828} Of the subjective signs, one of the commonest, if indeed it be ever entirely absent, is a tired feeling, particularly noted in the knees and ankles. This sensation is compared to ordinary muscle-tire, but is provoked by slight exertion, and not as easily remedied by rest. Often a numb feeling is associated with it, although no objective diminution of cutaneous sensation be determinable. This combined feeling of tire and numbness, described as a going-to-sleep feeling in the ankle, has indeed been claimed by one observer[1] to be pathognomonic of early tabes. Next in frequency, and almost as universal, are peculiar pains: these are manifold in character and distribution, but so distinctive as to alone suggest the existence of the disease from the manner in which the patient describes them. One variety, the lightning-like, is compared to a sudden twinge of great intensity shooting through the limb. The sciatic and anterior crural branches are the lines usually followed by this pain, but there are cases where perineal and abdominal regions are affected. Often the pain is so severe that the patient cries out or the limb is violently contracted under its influence. It differs from rheumatic pain in the fact that it is distinctly paroxysmal and that the intermissions are complete; that it is not greatly aggravated by motion nor relieved by rest, while the rheumatic pain is; that tabic pain is usually relieved, and rheumatic pain aggravated, by pressure, while hyperæsthesia is present with the former, and either absent or barely indicated with the latter. The lightning-like pains are sometimes combined with another form, which is even more distinctive in character. This form does not affect the distribution of special nerves, but is found limited to a small area which the patient is able to localize definitely: it may not exceed a centimeter or two in diameter, and within this area the pain is excruciating. It is either of a burning character or compared to the firm pressure of a vise or heavy weight, or to the tearing, boring, and jumping of a violent toothache.

[Footnote 1: Canfield, _Lancet_, 1885, vol. ii. p. 110.]

While some patients escape these pains almost entirely,[2] others are tormented with them at intervals for years, their intensity usually diminishing when the ataxic period is reached. There is little question among those who have watched patients in this condition that their pains are probably the most agonizing which the human frame is ever compelled to endure. That some of the greatest sufferers survive their martyrdom appears almost miraculous to themselves. Thus, in one case the patient, who had experienced initial symptoms for a year, woke up at night with a fulminating pain in the heels which recurred with the intensity of a hot spear-thrust and the rapidity of a flash every seven minutes; then it jumped to other spots, none of which seemed larger than a pin's head, till the patient, driven to the verge of despair and utterly beside himself with agony, was in one continued convulsion of pain, and repeatedly—against his conviction—felt for the heated needles that were piercing him. In another case the patient, with the pathetic picturesqueness of invalid misery, compared his fulminating pains to strokes of lightning, {829} “but not,” he added, “as they used to appear, like lightning out of a clear sky, but with the background of a general electrical storm flashing and playing through the limbs.”

[Footnote 2: I have at present under observation two intelligent patients (one of whom had been hypochondriacally observant of himself for years) who experienced not a single pain, as far as they could remember, and who have developed none while under observation. Seguin mentioned a case at a meeting of the Neurological Society with a record of but a single paroxysm of the fulgurating variety. Bramwell (_Brit. Med. Journ._, Jan. 2, 1886) relates another in which the pains were entirely absent.]

Either while the pains are first noticed or somewhat later other signs of disturbed sensation are noted. Certain parts of the extremities feel numb or are the site of perverted feelings. The soles of the feet, the extremities of the toes, the region about the knee-pan, and the peroneal distribution, and, more rarely, the perineum and gluteal region, are the localities usually affected.[3] In a considerable percentage of cases the numbness and tingling are noted in the little finger and the ulnar side of the ring finger; that is, in the digital distribution of the ulnar nerve. The early appearance of this symptom indicates an early involvement of the cord at a high level. Some parallelism is usually observable between the distribution of the lightning-like pains when present and the anæsthesia and paræsthesia if they follow them. With these signs there is almost invariably found a form of illusive sensation known as the belt sensation. The patient feels as if a tight band were drawn around his body or as if a pressure were exerted on it at a definite point. This sensation is found in various situations, according as the level of the diseased part of the cord be a low or high one. Thus, when the lower limbs are exclusively affected or nearly so the belt will be in the hypogastric or umbilical region; if the upper limbs be much involved, in the thoracic region; and if occipital pain, anæsthesia of the trigeminus, and laryngeal crises are present, it may even be in the neck. Correspondingly, it is found in the history of one and the same patient: if there be a marked ascent—that is, a successive involvement of higher levels in the cord—the belt will move up with the progressing disease. This occurrence, however, is less frequently witnessed than described. In the majority of cases of tabes disturbances of the bladder function occur very early in the disease. Hammond indeed claims that in the shape of incontinence it may be the only prodromal symptom for a long period.[4]

[Footnote 3: In the exceptional cases where the initial sensory disturbance is marked in the perineal and scrotal region I have found that the antecedent fulminating pains had been attributed to the penis, rectum, and anal region; and in one case the subjective sense of a large body being forcibly pressed through the rectum was a marked early sign.]

[Footnote 4: _New England Medical Monthly_, 1883.]

I have under observation a patient who has been compelled to use the catheter daily for years, who has gradual disappearance of the knee-jerk and reflex iridoplegia, but who has presented no other evidence of tabes during the year and a half he has been watched. Among the exact signs of tabes, reflex iridoplegia and abolition of the knee-jerk are probably the earliest to appear. It may be assumed with safety that in ninety-nine out of a hundred cases both the inability of the pupil to respond to light and the absence of the knee-jerk will be found long before ataxia is developed. Cases are recorded where no other positive signs were found, and no other signs of the disease developed for a number of years,[5] and others where disappearance of the knee-jerk was the very first indication.

[Footnote 5: Westphal, also Tuczek, _Archiv für Psychiatrie_, xiii. p. 144.]

The opinion of observers as to the frequency of double vision as an early symptom of tabes is far from being unanimous. The majority of writers speak of it as rare, but it is probable that this usually transient symptom is forgotten by the patient, or because of its apparent triviality {830} escapes notice. The patient while looking at an object sees a double image of the latter. This may last for a few seconds, minutes, or hours, and rarely for a day or week.[6] A distinct history of this symptom was given by 58 out of 81 patients in whom I recorded the oculo-motor signs. By far the most important of the exact prodromal signs of tabes are two symptoms—one involving a special faculty of co-ordination, the other the reflex movements of the pupil. One or both of these must be present to justify the diagnosis of incipient tabes.[7] The disturbance of co-ordination consists in an inability of the patient to stand steadily when his eyes are closed. The majority of healthy persons when tested in this way may show a little swaying in the beginning, but eventually they stand as steadily as they do with open eyes, and there is no subjective feeling of uncertainty as to falling. But the tabic patient exhibits oscillations, and makes efforts to overcome them which, instead of neutralizing, usually aggravate them and betray the great disturbance of his equilibrium. It is not as if he swayed merely because he is uncertain of his upright position, but as if some perverted force were active in throwing him out of it. It is found to be a pronounced feature even in cases where the patient with open eyes is able to walk nearly as well as normal persons, and experiences no trouble in performing intricate evolutions, such as dancing, walking a line, or even walking in the dark—faculties which the patient is destined to lose as his disease progresses.[8]

[Footnote 6: It has been asserted that the severer and more persistent diplopias are found with tabes dependent on syphilis.]

[Footnote 7: Not even the absence of the knee-jerk ranks as high as these two signs. Aside from the fact that this is a negative symptom, it is not even a constant feature in advanced tabes.]

[Footnote 8: It does not seem as if the disturbance of static equilibrium were due merely to the removal of the guide afforded by the eyes, for it is noted not alone in patients who are able to carry out the average amount of locomotion in the dark, but also in those who have complete amaurosis. Leyden (_loc. cit._, p. 334) and Westphal (_Archiv für Psychiatrie_, xv. p. 733) describe such cases. The act of shutting the eyes alone, whether through a psychical or some occult automatic influence, seems to be the main factor.]

In most cases of early tabes it is found that the pupil does not respond to light; it may be contracted or dilated, but it does not become wider in the dark nor narrower under the influence of light. At the same time, it does contract under the influence of the accommodative as well as the converging efforts controlled by the third pair, and in these respects acts like the normal pupil. It is paralyzed only in one sense—namely, in regard to the reflex to light; just as the muscles which extend the leg upon the thigh may be as powerful as in health, but fail to contract in response to the reflex stimulus applied when the ligamentum patellæ is struck. For this reason it is termed reflex iridoplegia.[9] It is, when once established, the most permanent and unvarying evidence of the disease, and is of great differential diagnostic value, because it is found in comparatively few other conditions.

[Footnote 9: It is also known as the Argyll-Robertson pupil. Most of the important symptoms of tabes are known by the names of their discoverers and interpreters. Thus, the swaying with the eyes closed is the Romberg or Brach-Romberg symptom; the absence of the knee-phenomenon, Westphal's or the Westphal-Erb symptom; and the arthropathies are collectively spoken of as Charcot's joint disease.]

In a number of cases ptosis of one or both eyelids is noted at an early stage of the disease. It is usually temporary, and coincides as to time with the diplopia, if present.

Patients presenting some or all of the subjective and objective {831} manifestations of tabes mentioned may continue in a condition of otherwise comparative health, enabling them to attend to their vocation for from one to twenty years, and it is not improbable that the pre-ataxic period may extend over nearly a lifetime. In a less fortunate minority of cases some of the most distressing evidences usually marking the last stages of the disease are found developed at the onset. Thus, cases are known where optic nerve-atrophy preceded the true tabic period by ten or more years; others in which trophic disturbances, manifested in spontaneous fractures of bones[10] or violent gastric crises, or even mental disturbance, inaugurated tabes dorsalis, instead of closing or accompanying the last chapter of its history, which is the rule.

[Footnote 10: Berger, _Deutsche medizinische Wochenschrift_, 1885, 1 and 2.]

The disturbance of co-ordination above spoken of as manifested in the inability of the patient to stand well with his eyes closed is the first step in the development of the characteristic ataxia which marks the full-blown affection. The patient finds that he tires more and more on slight exertion—not because his muscles are weak, but because he has to make more voluntary effort than a person in health. He finds that he stumbles easily—is unable to ascend and descend at the curbstone or to walk over an irregular surface with ease. Going down stairs is peculiarly irksome. “I would rather,” aptly said one patient, “troubled as I am in walking, go a mile in the street than walk up three flights of stairs; but I would rather go up six flights of stairs than walk down one.” Soon the patient notices that walking in the dark becomes more a feat of relative skill than the easy, almost automatic, act it was in health. His vocation, if it was one involving the use of the feet, becomes irksome, difficult, and finally impossible, and in a number of cases the upper extremities are also involved.[11] Delicate motions, such as those required in needlework, in writing, and by watchmakers, musicians, opticians, and lapidaries, are clumsily performed; even coarser movements, such as buttoning the clothes and carrying a glass filled with water to the mouth, are performed in an uncertain and clumsy manner. Meanwhile, the disturbance of motion in the lower limbs progresses. Difficult as it formerly was for the patient to stand on one foot or with both feet together while the eyes were closed, he is now unable to do either with the eyes open. He straddles in his walk, or, in order to overcome the element of uncertainty involved in moving the knee-joint, keeps this joint fixed and walks with short, stiff steps. If ordered to halt suddenly while thus walking, the patient sways violently, and makes movements with his hands or arms to recover his balance, in some cases staggering and even falling down. He shows a similar unsteadiness when told to rise suddenly from a chair or to mount one, and it becomes impossible for him to walk backward. Later on, it will be found that his feet interfere in walking. He has lost the power of gauging the extent and power of his motions to such a degree that he may actually trip himself up. To neutralize in some way this element of uncertainty of his steps, the patient is compelled to exaggerate all his ambulatory movements, and there results that peculiar gait which was the first symptom directing attention to the {832} disease to which it is due. The feet are thrown outward, and violently strike the ground; the heel touches the latter first, and the patient appears as if he were punishing the ground and stamping along instead of walking. The reason for his adopting these tactics are twofold. In the first place, he has a subjective sense of walking in a yielding substance, as if on a feather bed, air-bladders, cushions, or innumerable layers of carpet, and he therefore makes efforts to touch firm bottom. In the second place, his motor inco-ordination, in so far as it is not the result of anæsthesias, is greatest in those segments of his limbs which are farthest removed from the trunk, and which, enjoying the greatest freedom of combined motion, are also most readily disturbed. His uncertainty is therefore greater in the toes than in the ankle, greater in the ankle than in the knee, and greater in the knee than in the thigh: he prefers to touch the ground with the heel to touching it with the toes, and to move his limb in the hip than in the knee-joint. As the patient advances in life even this limited and clumsy form of locomotion becomes impossible: he takes to his bed, and it is found that he loses all sense of the position of his lower and occasionally of his upper limbs. He is unable to tell which limb overlies the other when his leg is crossed—unable to bring one limb in parallel position with another without the aid of his eyes. If told to touch one knee or ankle with the toes of the other side, his limb oscillates around uncertainly, and makes repeated unskilful dashes at the wrong point, and ultimately all but the very coarsest muscular co-ordination appears to be lost, even when the supplementary aid of the eyes is invoked.