Kitabı oku: «Tics and Their Treatment», sayfa 21

From youth she had at intervals been stricken with pains in the limbs, and with recurrent bilious attacks. Two years ago the death of her husband was the occasion of great mental strain and distress. Sixteen months ago she noticed a curious sensation in the right eye, not painful, accompanied from time to time by blinking of the lids. Very gradually the convulsive movements spread over the whole of the right face, and for the last month their frequency and intensity have been such that rest is an impossibility.

When she came under observation what impressed the mind first was the remarkable asymmetry of her figure: the right side of the face was smaller than the left, the right eye appeared to be at a lower level than the other, while the mouth was strongly deviated to the right and the chin twisted in the same direction. For a minute or two the facial contortion held sway, disappearing only to reappear quickly.

Not solely to the old torticollis was the facial asymmetry attributable, but also to the convulsive movements of the right half of the face. The effect of these was to close the right eye, deflect the nose to the same side, drag the mouth in a similar fashion, and wrinkle the skin of the chin and neck. Hence was evolved a unilateral grimace quite unlike any ordinary expression, resembling rather the facies in contracture secondary to facial paralysis.

During the next few months there was a gradual change from this tonic to a clonic stage, in which the movements were of less frequent occurrence, but more rapid. In repose there was no further indication of the old facial palsy than the flattening of the facial lines on the right. Under the influence of any emotion, or any passing contrariety, or in the course of an animated conversation, or if circumstances call for their repression, the spasms increase in number and degree, whereas solitude and tranquillity favour their subsidence.

A recent development has been the discovery of a means of checking the spasm – viz. by compressing the larynx with the fingers of the two hands. Madame L. admits the illogical nature of the manœuvre, but extols its efficacy. As a matter of fact, it sometimes fails of its object.

How, then, is this localised convulsive movement to be designated? Is it a tic or is it a spasm?

The march of the disease, its painlessness, the absence of any reaction in sleep, the success of the little laryngeal trick, the inhibitory effect of the will, the definite influence of attention, distraction, in short of the psychical condition of the moment – all plead in favour of its classification in the former category. On the other hand, we cannot shut our eyes to the fact of the pre-existence of specific organic disease, and, moreover, the spasm is strictly confined to the anatomical distribution of the facial nerve. Even in periods of repose there is a certain amount of fibrillation on that side. On these counts are we to hazard the diagnosis of facial trophoneurosis?

A subsequent opportunity of examining the same patient served to confirm the diagnosis of spasm secondary to facial dystrophy, and treatment failed to make any impression on the condition.

Our object in giving these cases has been to point out the difficulties in the way of diagnosis, especially where spasm is superadded to a mental state that itself predisposes to tic. The wisest plan in many instances is to confine oneself to a description of the symptoms and to tabulate the arguments for and against a particular view, without perpetrating the error of committing oneself.

Many cases labelled convulsive tic might be quoted where the expression of so definite an opinion ought to have been reserved, as in one reported by Mayer¹⁷¹ under the title of convulsive tic consecutive to infraorbital neuralgia:

A man, thirty-two years of age, had suffered from a severe infraorbital neuralgia of some weeks' duration, apparently attributable to a chill. The pains recurred at intervals till their substitution five years later for slight spasmodic twitches of the left eyelid, which gradually developed into violent convulsions of the whole of the left half of the face. These spasms were preceded by a sensation of numbness in the left ear, while during repose no modification of facial expression was to be remarked.

Further, there was a history of exactly similar neuralgia and spasm in the mother of the patient, although in her case the latter had been the first to appear, and had been replaced after a six years' interval by left facial neuralgia, which resection of the nerve failed to relieve.

In these cases the condition is undoubtedly one of painful facial spasm, inaccurately and unfortunately styled "tic douloureux."

Bruandet¹⁷² has recorded a typical example of right facial hemispasm consequent on facial neuralgia, in which, however, no certain macroscopical or microscopical lesion was detected, in either cortex or bulb. But the mere fact that no structural alteration was discovered post-mortem cannot invalidate the diagnosis; the imperfection of our methods of investigation suffices to explain the negative results of such researches.

B. Tic or Spasm of the Neck – Torticollis Tic and Torticollis Spasm

To make a diagnosis of torticollis, it is essential to satisfy oneself of the integrity of the bones, muscles, and articulations of the cervico-scapular region, previous to directing attention to the psychical state of the patient. In regard to the latter point, the question of heredity must not be neglected. If personal and hereditary defects are prominent, the presumption is in favour of mental torticollis; and if the convulsive movements present the characters of tic, the diagnosis is practically certain.

In three cases under the observation of Fornaca,¹⁷³ for instance, there is no room for doubt. Not merely was there no sign of irritation from peripheral sources, but also no one of the three was psychically normal.

Nevertheless we frequently find ourselves confronted by the question: is the movement a tic, or is it a spasm? For, strictly speaking, there are both a torticollis tic and a torticollis spasm, and their separation one from the other is often a matter of the greatest perplexity.

We must refer the reader to the chapter devoted to mental torticollis for a consideration of the features of that condition, and we need not dwell on those cases of spasmodic torticollis that are obviously occasioned by irritative lesions of nervous centres or conductors. In this latter category may be placed the case put on record by Oppenheim, where torticollic spasms were produced by pressure of a cerebellar tumour on the cranial nerves.

But in the affection known as hyperkinesis of the accessory of Willis we have little doubt both tics and spasms have been included. Apart from the cases of spasmodic torticollis, so called, which Babinski has published and to which reference has already been made, we may be allowed to cite one or two more, in order to exemplify the differences of interpretation to which they are liable.

At the Congress of Toulouse two patients were shown by Desterac,¹⁷⁴ both of whom had suffered since the age of eight from a disease akin either to Friedreich's disease or to hereditary cerebellar ataxia.

They presented the spastic gait of the former with the involuntary movements of the latter, in addition to spasm of the hand in writing, spasmodic movements of the trunk, and spasmodic torticollis. Both had club foot and scoliosis, and one was afflicted with spasm of the face and left arm. In his case, further, there was nystagmus, together with loss of reflexes and difficulty in articulation, while fibrillary contractions were to be observed in his muscles. The other patient's reflexes were exaggerated, and he showed a double extensor response.

In Desterac's opinion their spasmodic torticollis was dependent on this congenital constitutional affection, which might be regarded as a fruste form of one of the diseases above mentioned.

Through the kindness of M. Desterac the opportunity has been granted one of us of examining the two patients, and we should like to point out why we think his interpretation of their symptoms must be considered with reserve.

Speaking generally, we thought the cases closely resembled those in which a long-standing mental torticollis is accompanied with convulsive movements of the limbs. The scoliosis was not permanent, the deformation of the foot could be overcome, and at the same time we failed to convince ourselves of the presence of nystagmus and the absence of the knee-jerks. Moreover, we happened to observe one of the patients in the street unawares, and remarked how between two phases of bizarre contortions his vicious attitudes and convulsive gestures almost entirely vanished. In fact, the clinical picture seemed to us to be quite other than that associated with organic disease such as Friedreich's disease or hereditary cerebellar ataxia.

Another case recently brought before the Neurological Society of Paris by Marie and Guillain¹⁷⁵ serves even better to illustrate the intricacies of diagnosis.

The patient was a man of fifty-eight, who for years had exhibited certain movements apparently of an athetoid nature. His head was extended and rotated to the right synchronously with elevation and eversion of the left shoulder, then it passed into flexion. Except for a few odd movements of the tongue, the face conserved immobility. In the arms the localisation of the contractions was mostly proximal, though there were alternating flexion and extension movements of the fingers which suggested athetosis. Flexion, inversion, and adduction of the thighs also occurred. The recti abdominis were similarly involved.

Under the influence of emotion the movements were increased, but they could not be inhibited by an effort of attention. Their rate was too slow for chorea. Ordinary voluntary movements were performed without apparent trouble; the patient was able to dress himself, and to drink without spilling the liquid. Diminution of the knee-jerks was noticed, with what seemed to be an extensor response. Slight scoliosis of the vertebral column and a misshapen right foot recalled Friedreich's ataxia. There was nothing to justify a diagnosis of hysteria.

This curious condition dated from the year 1874, when the patient had a febrile attack, in the course of which pain and tingling appeared in the toes of the right foot, followed by involuntary movements of the same member. Analogous symptoms were not long in appearing in the left arm. Two months later the condition had become general, but from that time no special modification took place.

In the subsequent discussion it was remarked by Souques that the case resembled one recorded by Chauffard¹⁷⁶ as Friedreich's disease with athetotic attitudes, where the patient was a child with club foot, diminution of the knee-jerks, and generalised athetotic movements.

Notwithstanding our inability to assign a definite nosographical position to examples of this kind, we think it desirable to make some reference to them, in the hope that further observations will aid in their diagnosis. They at least remind us that convulsions occurring in the course of organic disease may be simulated by the manifestations of certain motor neuroses.

TICS AND CHOREAS

A. Sydenham's Chorea

It would be difficult to find a better description of chorea minor than that given originally by Sydenham himself:

The dance of Saint Guy, chorea Sancti Viti in Latin, is a sort of convulsion whose incidence is greatest, in both sexes, between the age of ten and puberty. Its onset is characterised by weakness of one limb, which the patient drags behind him, and soon the arm of the same side is affected in the same way. He finds it impossible to maintain the same position of the arm for two consecutive moments, however great be his efforts to attain this object. Before he can bring a full glass to his lips he makes innumerable gestures and antics, as the convulsive moments of the limb deviate it from one side to the other, until at length he has piloted the glass opposite his mouth, when he empties it at a gulp.

If we were to confine ourselves to this description by Sydenham, which so far as typical cases of the disease are concerned is perfectly accurate, differentiation between tic and chorea would not be a matter of any complexity. Unfortunately, however, the varieties of this form of chorea are legion, and in practice one constantly meets with conditions suggesting alike the gesticulations of chorea and the convulsive reactions of tic. Moreover, it has been pointed out by Oddo¹⁷⁷ that the fact of the habitual exaggeration of tic during the very years when chorea is liable to appear is calculated to confuse the issue.

He has attempted, however, to specify certain factors in the differential diagnosis. In the first instance, the form of the movements is of significance: there is no co-ordination in the muscular play of the choreic; it is amorphous, indefinable, and erratic, whereas the gestures of tic are purposive, and may be said to have a shape. One never sees in chorea a succession of similar movements, but though a patient be suffering from several tics, each of them is reproduced always in the same fashion. Unilaterality of distribution is more common in chorea than in tic; in other words, chorea, more or less, follows anatomical lines in the regions it affects, whereas the incidence of tic is physiological.

Both are arhythmic in their manifestation; nevertheless the repetition of tic is noteworthy for its regularity as compared with the changing mode and rate of the other. Noir emphasises the diagnostic value of its frequency, abruptness, and reiteration of identical movements. In a majority of cases the interference of the will is futile as far as chorea is concerned, while the victim to tic is usually capable of restraining his muscular activity at least for a space. The choreic exhibits his movements in public, but the tiqueur seeks the seclusion of his own room. The association of tic with obsessional ideas is frequently encountered, but there is no similar connection between obsessions and chorea. In addition, the myasthenia, pains, and alterations in the reflexes that often characterise chorea are awanting in the other affection.

It cannot be gainsaid, however, that the frequency with which atypical varieties of chorea occur is inimical to a ready diagnosis, and the onerous nature of the task is not lessened by the circumstance that many choreics are the offspring of neuropathic parents and reveal psychical anomalies comparable to those of the subjects of tic.

In a disease such as variable chorea, which has features in common both with tic and with chorea properly so called, the problem of diagnosis is still more complicated, though excellent hints for its solution have been furnished by Brissaud.¹⁷⁸

However frequently and warmly the theory of the origin of chorea in a neuropathic predisposition was advocated by Charcot, the fact of its usual evolution consecutive to some toxic or infective process is no less certain. Its incidence is greatest in children and the adolescent; it runs a regular course of increase and decrease; and the circumstances which cause the symptoms to vary during this cycle are never sufficiently potent to bring about even transitory suppression of them.

It is true that changes in the intensity of the symptoms seem to confer a remittent character on the affection, but there is nothing at all comparable to the sudden and unexpected waxing and waning of the form of chorea at present under consideration. None of the pathological attributes just mentioned concerns variable chorea, which, in addition, differs from Sydenham's chorea in two points – the multiplicity of the types of movement, and the fact that the patient can voluntarily check his involuntary actions. For these reasons, assimilation of the two clinical varieties is impossible, and the confusion of the two in practice need never occur.

A form of chorea entitled "habit spasm" by Gowers, and "habit chorea" by Weir Mitchell, has been the subject of further study by Sinkler,¹⁷⁹ but in all probability the cases of this description reported are instances of the variable chorea of Brissaud.

B. Huntington's Chorea

In spite of the preponderating etiological significance of heredity and the constancy of psychical imperfections in the chronic chorea of Huntington, its confusion with tic is not at all likely to occur. Difficulties might arise in distinguishing chorea major from variable chorea, however, and here we have the views of Brissaud to help us.

True chronic chorea is an incurable neurosis, of life-long duration. We have no trouble in pronouncing a diagnosis of chronic chorea if the symptoms date back five, ten, or twenty years, but they must have had a commencement, and the whole problem is to foretell the course of a chorea as yet only a few weeks or months old.

The involuntary movements of chronic chorea, like those of Sydenham's chorea, are illogical, but they are combined in a co-ordinate manner – that is to say, certain functionally associated muscular groups act simultaneously as for a particular end: the patient shrugs his shoulders, closes his fists, cracks his fingers, utters cries, he swallows, sniffs, sucks in his breath, makes the sound of kissing, etc, in all of which actions orderly participation of the musculature in a foreordained way is evident. Slight twitching of individual muscles and parts of muscles also occurs.

There is no limitation of the movements to a special division of the body; on the contrary, they spread from one muscle to another, and from one segment to another, rapidly and arhythmically. The gait is by turns skipping, dancing, or stumbling, interrupted by falls or by abrupt jerks of the loins. Speech is uncertain or monotonous; writing is incorrect and badly formed, sometimes illegible. A fact of the utmost importance is that all these involuntary movements may be modified, abated, relieved, so to speak, by voluntary movements in an inverse direction. In some cases the power of willing is still sufficiently developed to permit of the patient's following his occupation.

The steadily progressing increase in the seriousness of the motor trouble, paralleled by progressing mental deterioration, is one of the most significant factors in the differential diagnosis. It is precisely the variability of the symptoms that distinguishes variable chorea.

C. Hysterical Chorea

The conditions to which the name of hysterical chorea is applied may assume two forms, the commoner being known as rhythmical chorea, the other as arhythmical chorea. In the former case the convulsive movements are usually unilateral, being confined sometimes to a single limb, and reproducing, for instance, the actions of dancing (saltatory chorea), or of swimming (natatory chorea), or such professional movements as those of the blacksmith (chorée malléatoire). Occasionally there is a more or less faithful reproduction of deliberate and purposive acts in the form of attacks of varying duration, recurring, moreover – and this is their cardinal feature – at equal intervals.

Under the title of disease of the tics two cases have been published by Nonne,¹⁸⁰ the first consisting of rhythmical twitches in a man of forty years, secondary to a head injury, the other presenting similar appearances, but concerning a young girl of eighteen years who had sustained a shock. In neither was there any sign of hysteria. The reporter animadverts on the designation "rhythmical chorea," and protests that the systematisation and co-ordination of the movements are very different from the clinical picture of Sydenham's chorea, while their rhythmical nature does not allow of their being classified as tic.

Sometimes hysterical chorea is arhythmical – that is to say, the movements are irregular and contradictory, as in ordinary chorea. True chorea in cases of hysteria comes under this heading, as well as those cases where hysterical patients imitate the movements of chorea. The presence of the distinctive characters of hysteria makes a diagnosis of tic improbable.

The separation of hysterical from variable chorea may be peculiarly perplexing, as in one of Brissaud's cases, where the patient's extraordinary mental instability was such as is encountered only in advanced hysteria, while her disorders of motility were highly characteristic of what is known as variable chorea.

The condition described as chorea gravidarum may be placed at one time in the category of hysterical chorea, at another in that of ordinary chorea. In it there is intense motor restlessness, and accompanying mental symptoms are not awanting in a majority of instances.

D. Electric Chorea, Bergeron's Chorea, Dubini's Chorea, Fibrillary Chorea of Morvan

To render the study complete, we may remind ourselves of those still imperfectly differentiated forms known as electric chorea (Hénoch-Bergeron) and Dubini's chorea.

Bergeron's chorea affects children chiefly, and is characterised by the suddenness of its onset and the rapidity with which it attains its maximum. The movements are abrupt and brief, as though produced by an electric discharge at regular intervals, but their intensity does not hinder the execution of voluntary acts. They are sometimes confined to the head and limbs, most commonly they are generalised, and during sleep they disappear.

In the opinion of many, Bergeron's chorea is secondary to gastric disturbance. A cure may be regarded as certain, and indeed frequently follows the administration of an emetic. Sometimes the effect of the latter seems to be purely psychical.

Pitres thinks that this condition, as well as the electrolepsy of Tordeus, is simply a manifestation of infantile hysteria. According to Noir, there is an affinity between tic and electric chorea, and Ricklin is inclined to consider the two identical, but further study of the question is desirable.

Dubini's chorea is ushered in by pains and aches in the region of the head, neck, and sometimes the loins, and these are succeeded by electric-like twitches in the segment of a limb, which quickly become general. Severe convulsive attacks also occur, without loss of consciousness, entailing actual paresis of the limbs. The duration of the disease may be days or months, and 90 per cent. of the cases have a fatal issue. Confusion with tic is impossible.

We need not concern ourselves with so-called paralytic chorea, or with the fibrillary chorea of Morvan, which is a disease of adolescence, characterised by fibrillary contractions in the calves and thighs, passing thence to the trunk muscles and even to the arms; the face and neck, however, are spared, and during voluntary movement the fibrillation vanishes. Probably it is merely a variety of the paramyoclonus of Friedreich.