Kitabı oku: «Tics and Their Treatment», sayfa 18

VARIABLE CHOREA OF BRISSAUD

If the disease of Gilles de la Tourette, by reason of the uniformity of its symptomatology and the regularity of its evolution, justifies its differentiation as a separate entity among the tics, a comparison of it with another type, of polymorphic manifestation, irregular evolution, and uncertain duration, may prove instructive. We refer to the affection described by Brissaud as variable chorea.

The form of the motor reactions in this condition warrants the application to it of the term chorea, but the analogies the disease presents to tic are very close, nevertheless, and sometimes the two occur in the same individual. Patients suffering from variable chorea reveal the same mental abnormalities as are found among those who tic, while the troubles of motility are sometimes so similar to what we meet with in the latter that Gilles de la Tourette regarded the condition simply as one form of convulsive tic, the more so that it is occasionally accompanied by explosive utterance and even coprolalia.

This view, however, is calculated to obliterate the distinctive characters of the two affections, and ought not to be entertained. We cannot do better than repeat Brissaud's original description:

The use of the word chorea need occasion no ambiguity: the chorea consists in the appearance of meaningless and apparently idiopathic involuntary movements, whose repetition during rest and action alike is proof of their irrationality and incongruity; the duration of the symptoms may be limited as in chorea minor or Sydenham's chorea, or unlimited as in chorea major or Huntington's chorea. "Variable" is the epithet we apply to the chorea because of the lack of uniformity in its exteriorisation, the irregularity of its development, and the inconstancy of its duration. It comes and goes, waxes and wanes, vanishes abruptly to reappear unexpectedly; it is a neurosis without a characteristic march.

Notwithstanding the fact that we are dealing with a chorea – that is to say, with a disease which is almost as readily recognisable by the public as by any professional – the difficulty of fixing its onset is paralleled by the difficulty of knowing when it has ceased. This uncertainty is explained by the facile and changeable nature of the patient; until the condition is revealed by unmistakable signs it passes for an insignificant muscular caprice of no pathological importance, while its disappearance is not associated with any particular modification of the patient's ways.

There is a natural tendency to identify all "nervous movements" with myoclonus, but the conception is a remarkably nebulous one, and means nothing more than "muscular twitch." On the other hand, it is well understood that "nervous movements" are more or less sudden movements of limbs, shoulders, face, always involuntary and generally increasing in force and frequency with the nervous state of the patient.

Parents say, for instance, that their child has become more restless and irascible, and at the same time that he has had "more movements of the nerves." The coincidence is unfailing. Is the expression "nervous movement" lacking in precision? Yet it signifies what it is intended to signify. We are concerned neither with tonic convulsions nor with clonic spasms, nor yet with tics of habit; what the term stands for is a complex contraction, brisk but not violent, closely allied to the simplest of automatic acts, such as a step in advance, a shrug of the shoulders, a frown, a sigh, a moan, a crack of the fingers, an exclamation – in any case usually a gesture of impatience. The whole thing, however, is so variable and fugitive, that it cannot be said to constitute a definite convulsive phenomenon. The contractions, further, in spite of their complexity, escape the notice of their originator, who is quite surprised at being asked the meaning of the movement he has just made, as he is almost entirely ignorant of it.

Briefly, the "nervous movements" of which we have been speaking do not belong either to myoclonus or to tic, but owe their distinctiveness to their multiplicity and inconstancy. At the same time they are always grafted on a certain neuropathic diathesis akin to that of chorea; in fact, they are nought else than a form of chorea themselves.

The psychical peculiarities of the patient with variable chorea may be summed up in instability of thought and action, combined with mental infantilism. Hence the terms "polymorphous chorea" and "chorea of degenerates" are used synonymously for variable chorea.¹³⁸ Sometimes the disorders of the mind include hallucinations, and various forms of phobia or mania.

One or two examples may be given:

A microcephalic youth of sixteen, a monorchid, developed what appeared at first to be an ordinary chorea subsequently to an orchidopexy. The movements, however, varied from day to day and from hour to hour. Sometimes they disappeared for days at a time, to reappear suddenly just when the neurosis seemed cured. The influence exerted on them by the will was both mild and transient. They constituted, in short, a particular kind of chorea, changing and changeable, and differing from intermittent chorea in that neither remissions nor relapses were ever wholly complete. Further, the condition was implanted on a basis of mental and physical degeneration, and seemed likely to become established as a permanent functional stigma.

In another case a peculiar chorea gradually supervened, for no obvious reason, in an adult female of tardy and imperfect physical and intellectual development. It was difficult to decide whether the psychical or the somatic phenomena were preponderant; but to the material, tangible, and visible signs of constitutional inferiority was superadded a choreiform instability of the whole voluntary muscular system, consisting in agitation, gesticulation, and incorrigible motor restlessness, coupled with a conspicuous incapacity for rational action.

The steps in the evolution of this functional defect were very slow, and coincided with final confirmation of the intellectual insufficiency. As for the chorea, its localisation and its intensity, its increase and its decrease, its extension and its limitation, seemed to vary, in a way that could not be foreseen, at the call of certain undetermined circumstances.

In a third instance we meet with many of the symptoms already noted among those who tic:

X. is a well-developed boy of fifteen, but there is something peculiar about his physiognomy which defies analysis. If his mother's statements can be trusted, he is intelligent, quick, witty, sound in judgment, and blessed with an excellent memory. From the very first he has been eccentric, timid, and hypersensitive, and is to-day as tender-hearted and affectionate to his people as ever. He has various little "manias" of his own; he must have a knife, fork, and spoon for himself, and cannot take his food in comfort if they have been set before some one else. Each morning he dresses himself with extreme deliberation, then comes down to breakfast, of which, however, he will not partake unless he has touched all the door handles on his way. This little matter has developed into an obsession. His loathing of cold water is so pronounced that his morning toilet is rather a stormy proceeding, and as he is too old to be washed by his mother, the inevitable result is that his face and hands are never clean. At school he is both attentive and docile, finding pleasure in his study of the classics, but evincing a perfect passion for German. Anything German is a source of ineffable joy, so much so that he hugs his dictionary with childish exuberance. He listens deferentially to his teachers, but takes no note of what he hears. In German, Greek, and Latin he is at the head of his class, whereas in history and mathematics he is at the foot.

The "nervous movements" for which he has been brought to the consulting-room consist of a series of gesticulations akin both to tic and to chorea. Some are much more frequent than others, meaningless gestures executed spontaneously, one might almost say unconsciously. As he walks to school with his books under his left arm, his right hand roams over his person; and in the class-room the movements are repeated. At table he rubs his back against the chair, and alternately flexes and extends his right leg. Apart from these "habit tics," he exhibits actual twitches of his muscles generally, and evidence of the consequent disturbance of his movements is furnished by a glance at his untidy bedroom, his disarranged books, his blotted papers, his slovenly clothes. When he goes out with his parents, he is never at their side, but lounges along in his own way, then suddenly hurries to regain his place by them, falling back again and occupying himself by crossing his legs, knocking his ankles together, shrugging his shoulders, grimacing, etc. All the movements can be arrested for a time by an effort of the will. At any one's behest he can maintain tranquillity for a minute, but the strain is too severe, and the muscular dance recommences sooner or later.

The movements are highly variable in type and degree, nor can the mother specify the date of their appearance. It is only during the last three years that her attention has been more particularly drawn to them, and their increasing gravity occasions her some anxiety. The boy has become the laughing-stock of his companions at school, hence he limits his stay there to the actual hours of his classes.

Three years later the choreic symptoms vanished. X. is to-day a stalwart youth, though still timid and eccentric. It is evident that in his case the variable chorea has been but an episode in adolescence, to be added to the numerous stigmata of degeneration enumerated above.

Notwithstanding its slow evolution (says Brissaud), the neurosis, in so far as it was a disorder of motility, seems to have completely disappeared. The importance of this for prognosis is fundamental, but from the point of view of diagnosis it is no less significant, seeing that the nature and form of the movements suggested chronic or Huntington's chorea.

A case described by Gilles de la Tourette¹³⁹ as disease of the tics seems really to have been one of variable chorea.

A woman of twenty-two, who had never been very strong, had an attack, at eight years, of involuntary movements of face and arms which prevented her feeding herself, and at the hospital a diagnosis of chorea was made. Two months later cessation of the movements allowed of her return to school, but a second attack followed after two years, and a third a year later. At the time of observation she was in the throes of her sixth relapse. Every one who had seen her considered the condition as chorea.

Tourette, however, was dissatisfied with the diagnosis. There was no suggestion of its being Sydenham's chorea, or hysterical chorea, still less of its belonging to Huntington's variety. According to the author, the muscular twitches were amorphous and indefinite, and characterised by extreme variability in form, expression, and intensity.

In our opinion the clinical picture is that of variable chorea, and we are confirmed in our opinion by a consideration of the patient's mental condition.

She comes of a pronounced neuropathic stock. One of her two sisters is nervous and impressionable, and probably a neurasthenic, while the other is subject to hysterical attacks. She herself is of a profoundly nervous temperament; she cannot go to bed without assuring herself several times that no one is concealed beneath it; she suffers from fears and dreads and obsessions of all sorts; she is, in fact, an "unstable," a degenerate.

In one of our patients the symptoms were unilateral, constituting a variable hemichorea.

It is a matter of some difficulty to furnish an adequate description of the movements of the right arm. We note, first of all, that their activity depends on whether the arm is free or held in a fixed position. Voluntary movements are carried out stiffly, but are interrupted by sudden deviations, sometimes of rather a wide range, and highly irregular in distribution. Notwithstanding these breaks, the end to which the movement is directed is always attained with precision.

While L. was an apprentice dressmaker, she occasionally used to make various contortions with her arm, though if her attention was diverted they did not occur, and as a matter of fact she did her work well enough. Once she became familiar with the mechanical act of sewing, the involuntary performances ceased. Before her disease asserted itself, she had commenced to learn the piano, and she continued to make unimpeded progress, as her teacher discovered a method of holding her elbow which checked all convulsive twitches.

The involuntary movements of the right leg were so insignificant as to be almost negligible; they united to produce a sort of irregular tremor which became appreciable only when the patient was very tired or very annoyed. Sometimes a long walk was followed by a certain hesitation in putting the right foot to the ground, and by defective inhibition of the antagonists of the desired movement. Sometimes one foot was knocked against the other, and sometimes the right appeared to assume an equinovarus position. On the other hand, we have seen L. walking in the street with her father, when no anomaly could be detected in her gait. The distraction of any occupation such as dancing or playing a game has the effect, for the time being, of banishing the greater part, if not all, of the spasmodic phenomena.

This is undoubtedly a case of Brissaud's variable chorea of a unilateral type, and a consideration of the symptoms confirms the intimate relationship between it and tic.

Various intermediate forms have been noted. In one of Brissaud's cases, variable chorea and multiple tics co-existed. Féré¹⁴⁰ reports a case of variable chorea preceded by tic, and Bernard another in which starting, trembling, facial tic, variable chorea, etc., were associated.

Tics of phonation are often superadded to the gesticulations of variable chorea. Brissaud refers to the case of a girl of sixteen in whom involuntary movements resembling those of this type of chorea were coincident with a sort of hiccough, and a more or less inarticulate cry; at a later stage the movements became very infrequent, the hiccough was more constant, and the cry developed into a coprolalic ejaculation.

Variable chorea and variable tic are obviously very closely allied. The movements of the latter, however, are distinguished by their greater abruptness and smaller variety. They are tics by reason of their systematisation and co-ordination; they are variable because they pass from one region of the body to another. There is no necessary relation between them; each has an individuality of its own and is independent of the rest. In variable chorea, on the other hand, one movement passes insensibly into another, and the variants of any particular one are legion.

However easy it is, then, to separate the two clinically, it is none the less true that they spring from the same soil of mental defect. Variable chorea differs in nature from other choreas, though its form is the same; it may be distinguished from tic by the type of movement, but in essence it is identical.

CHAPTER XII
ANTAGONISTIC GESTURES AND STRATAGEMS

HOWEVER harmless and insignificant a tic may be, it is a source of annoyance to its subject of which he constantly seeks to disembarrass himself. But the feebleness of his will militates against any sustained effort, and if for a brief space he can conserve his immobility, victory eludes his grasp, for his tics resume the offensive and increase in violence. More than ever convinced of his helplessness, he resorts to measures that serve but to accentuate the mischief. Thus it comes to pass that he desists from his attempts at repression and admits himself vanquished.

Some there are, nevertheless, whose inventive faculty leads them to adopt singular attitudes, to execute curious gestures, to utilise elaborate apparatus – proceedings always more or less childish, whose employment is usually followed by success, but only for a time. The history of O. acquaints us with a whole series of these subterfuges, for which the expressive name of para-tics was invented by him, tricks intended to mask or to modify existing tics, but they soon themselves became as involuntary and as inevitable.

Not all who tic are imaginative enough to conceive such plans, and many have no thought of showing fight at all, but it is worth while dwelling on this point for a little, especially in view of the frequency with which certain tics are accompanied by methods of correction evolved by the patient.

To begin with, we may quote the case of mental torticollis. The sufferer's head is irresistibly driven to the right, say, yet he replaces it immediately by the mere application of his right forefinger to his chin, and the correct attitude is maintained so long as the finger is applied. Of the variants of this efficacious antagonistic gesture the most common is the grasping of the head in the hands, or its support in the palm, or the simple contact of the fingers with chin, or cheek, or temple. In some cases the mere threat of this gesture suffices for the purpose. S. approximates his hand to his left ear, but before he has actually touched it his head turns spontaneously to the right. It would be difficult to find more conclusive evidence of the purely psychical value of such corrective acts.

Sometimes the resources at the patient's disposal are confined to one measure, though more frequently he avails himself of several, as in a case recorded by Sgobbo.¹⁴¹ The antagonistic gesture may fail of its object if some one other than the patient put it to the test. Even with the expenditure of considerable force he may make no impression on the tonic contraction; this rule, however, is by no means general.

One of our patients, whose head used to be strongly tilted on to his elevated right shoulder, while his right arm was flexed, his left shoulder depressed, and his whole trunk deviated to the former side, was able instantaneously to resume his normal attitude by merely placing his thumbs one on either side of his head. If any one else sought to correct his vicious position he could do so by applying his fingers to two well-defined spots on the occiput, towards the base of the mastoid processes.¹⁴²

Occasionally the antagonistic gesture is of the nature of a paradox. We may cite an example from Raymond and Janet.¹⁴³

If we ask the patient whether she cannot sometimes prevent her head from rotating, she declares she can, and demonstrates how it is done by lightly touching her forehead with her finger tips. Now, in view of the fact that her head is deviated to the left and backwards, it will be seen that no pressure exerted in front could obviate this. What really happens is that at the moment of contact not only does she inhibit the movement by the aid of her will, but she also makes a slight forward inclination of her head to rest it on the point of support. No performance of this description could have any efficacy in the case of a genuine spasm due to irritation on a reflex arc.

At length the day arrives when the hand is unequal to the task, and the patient endeavours to utilise more resistant bodies, such as the back of a chair or the wall of the room, as in a case of retrocollis reported by Brissaud. These devices in their turn prove insufficient, and relief is obtained only in the recumbent position. Fournier¹⁴⁴ has seen a case of convulsive twitching of the right sternomastoid and trapezius arrested when the head was reclining on a pillow.

Even in bed, however, there is usually something to complain of: the pillow is too high, or too low, or too soft; the rustle of the packing is disagreeable, the sheets are too rough, etc., etc. It is then that all sorts of unlikely arrangements are adopted, and the patient puts his head under the bolster, or lets it hang over the edge of the bed, or piles up additional cushions and mattresses calculated to retain it in the desired situation.

Frequently the stratagems are highly ingenious and complicated.

Madame K.,¹⁴⁵ forty-three years of age, suffers from clonic movements of the head which disappear with the adoption of a torticollic attitude, the face looking to the left. Nothing is easier than voluntarily to correct this attitude, but the clonic movements at once reassert themselves, although they may momentarily be kept in abeyance by placing the hand on the chin.

Numerous and ingenious have been the devices framed by this lady, but in no instance has their success been other than transient. Her latest invention is a stiff high collar fashioned of several thicknesses of a heavy material. At the risk of strangling herself she has so compressed her neck that no movement is possible, but the right arm has now become the seat of action.

A patient of Grasset¹⁴⁶ used to promenade in the grounds of the hospital holding a cane in his teeth and maintaining his head in position by keeping one finger on the end of the stick.

Another patient, under the care of Noguès and Sirol,¹⁴⁷ whose head was fixed in irresistible anteroflexion and rotation to the left, had invented a most elaborate piece of apparatus, the adoption of which was followed by perfectly satisfactory results. On the frame of a pair of pince-nez deprived of the glasses he fixed a piece of iron wire ten centimetres long in such a way that it stood out from the spring at right angles to the plane of the pince-nez. It was sufficient to wear this thing on his nose to inhibit the spasm, and to be able to talk, walk, do anything unhampered by his torticollis; it was not even necessary to concentrate his gaze on the extremity of the rod.

In the case of one of our patients, N., whose head we had on several occasions succeeded in keeping straight while he was writing by directing a pin towards his left cheek, the idea was entertained of utilising this procedure out of doors, and accordingly a long pin was fixed in the collar of his overcoat. There never was the slightest prick on his cheek, but we strongly dissuaded him from the continuation of this objectionable practice.

Antagonistic stratagems of this kind are met with in other tics.

A curious case of mental trismus is reported by Raymond and Janet,¹⁴⁸ where the patient always spoke through his clenched teeth, but opened his mouth widely enough when showing his tongue or when eating. To overcome the tonic contraction of his masseters he used to insert a minute piece of cork between his jaws, though he could also open them to articulate properly by holding his chin with his hand.

Chatin's patient¹⁴⁹ nullified the permanent contraction of his masticatory muscles by insinuating his little finger between the dental arches.

In this connection reference may again be made to the fixation attitude adopted by young J.¹⁵⁰ for his left arm, a subterfuge of his own invention which he considered a sovereign remedy. In essence it was nothing else than an efficacious antagonistic gesture, inspired by a tic and become its indispensable complement. Of other ingenious ideas of his brief mention may be made.

Convinced of the necessity and possibility of checking the movements of his shoulder, he sought the aid of his "immobilising mattress," an ordinary mattress spread in a corner of the dining-room, on which he flung himself and reclined from morning to night, making the wretched thing his companion, solace, and confidant, who alone understood and could alleviate his tics. In his anxiety to find some point of resistance for his left arm to work against, he had a second and much narrower mattress put under the first, so that prodigious efforts were required on his part to maintain equilibrium on the cylindrical surface. This was exactly what he desired, and for a time he ceased to tic.

An equally curious case is that of one of Raymond and Janet's patients afflicted with multiple tics.¹⁵¹

He was a man thirty years old, who denied having had tics for more than four years; he had always been eccentric, however, and came of a family some of whose members were dullards and others hysterics. His career at school and college was brilliant, but his vain and erratic disposition had prevented him from realising his boundless ambitions, and carrying into effect many ingenious schemes. For that matter, a prominent trait in his character was a curious scrupulousness that led him to seek an impossible perfection for all his actions. Anything he put his hands to he thought might be better accomplished if he had a system for the purpose; he had, for instance, all sorts of plans for improving his caligraphy, for holding the pen, interminable "tips" for correct punctuation, for learning, for reciting. To such an extent was he embarrassed by these procedures that he could not write two letters consecutively. Purposeless voyages to Africa ended in his contracting conjunctivitis, malaria, and dysentery, and he returned to France worn out and more eccentric than ever. Thereafter the state of his health, and above all his functions of respiration and digestion, became matters of absorbing attention. A system had to be thought out for breathing better and for avoiding possible suffocation. He next devoted himself to the question of alimentation, and conceived the idea of moistening each mouthful of food with water, soon finding it desirable to wet his lips, apart from meal time, in order to breathe better. One day during a journey by train he suffered agonies from want of his drop of water.

Examples such as these serve to illustrate how the misplaced ingenuity of the sufferer from tic complicates his misfortunes instead of banishing them, and indicate to what extremes his eagerness to obtain respite may lead him.

All these gestures and stratagems may be considered as manifestations of ideas of defence, comparable to what obtains among those afflicted with obsessions and delusions of persecution.