CHAPTER XI THE EVOLUTION OF TIC

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TIC is, from its nature, highly variable in its evolution; each tic has a development peculiar to itself. Mental differences among individuals have their counterpart in physical differences, in health as well as in disease, and a comprehensive sketch of the evolution of tic is therefore impracticable. We shall restrict ourselves accordingly to a few general remarks.

In the great proportion of cases of tic the onset is an insidious one. We have already made a sufficiently detailed examination into the pathogenic mechanism to obviate any repetition in this place, but we may note how unsettled the earliest manifestations are, how a tic may pass from one muscle or group of muscles to another, and even when its exciting cause is patent an apprentice stage always precedes its final establishment. Of the truth of this the history of J. provides an excellent instance. Another one is from Pitres:

A nine-year-old boy received a severe shock one day through being pounced on by some companions who were in hiding behind a wood pile, and though the emotion was of short duration, he commenced a few days later to exhibit involuntary muscular twitches of the upper part of his body, and to utter suppressed cries. The phenomena increased in violence and in frequency, and, in spite of treatment, a year later he was not freed of them entirely. For an unknown reason the tics renewed their activity when he was seventeen and continued so for the next three years, until a spell of Pitres' respiratory exercises effected a complete cure.

An evolution such as the above may be considered more or less typical of the great majority of tics.

We have seen that the tic may be localised indefinitely in one and the same muscle or muscular group, but its site may also vary from day to day. Two tics may co-exist and coincide, or a third may appear with the disappearance of the others. Unexpected resurrections may succeed periods of complete repose.

Tic always shows a tendency to invade; regarded as a functional act, it moves in the direction of greater complexity.

After involving the orbicularis, for instance, a tic will spread to neighbouring groups, in particular to those muscles whose synergic contractions form a special expression of countenance. That is why tics of the eyelids are associated with movements of the pyramidales, frontales, and corrugators. Tics of the lips or of the alae nasi very commonly extend to the corresponding elevators. It is not surprising that muscular groups accustomed to act in physiological unison should also be affected together (Noir).

Moreover, the fecund imagination of the victim to tic is calculated to facilitate the invention of all sorts of modifications, complications, parodies, and caricatures of the functional acts on which his tics are grafted.

Tics are constantly varying in amplitude, degree, and frequency; as O. remarked spontaneously, "We have our good days, and we have our mauvais quarts d'heure." The sedative effect of rest, solitude, silence, and obscurity may be contrasted with the detrimental results of fatigue, noise, fear of ridicule, etc.

However incapable S. is of rotating his head to the right when requested to do so, the movement is executed with the utmost readiness should his attention be drawn in that direction. But if he hesitates, even momentarily, before looking round, he cannot then do so without the preliminary performance of all sorts of contortions, ending in a twist of his body through a half circle to the right. Sometimes he actually turns round two or three times, after the fashion of a dog chasing its tail. Let him have a pleasant visit, on the other hand, let him engage in a discussion, or be engrossed in a play, let him administer a rebuke to some one, and immediately his trouble is forgotten, his speech is accompanied with animated gestures, the vicious position of his head vanishes—in short, he becomes normal.

An intercurrent affection may act either as a deterrent or as a stimulus; with convalescence, however, there is usually a re-establishment of the mischief. The most potent influence over the phenomena of tic is wielded by a sense of well-being, to employ Janet's discriminating expression. Well-being is a panacea for the tiqueur no less than for the hysteric. The tic of the worried financier disappears, as we have had occasion to note, under the magic of a rise in stocks or a knowledge of solvency. The child's happiness is bound up in his freedom, which explains the cessation, in TissiÉ's little patient, of all convulsive movements during the holidays.

Much evidence is forthcoming to support these points, but we must admit that the why and wherefore of a tic's amelioration or aggravation often escape us, nor must we forget that both in the child and the adult spontaneous cure is not unknown.

As has been remarked, the evolution of tic does not lend itself to systematic description, but there are cases that form an exception, their course being regularly progressive. Strictly speaking, they are instances of Gilles de la Tourette's disease.

GILLES DE LA TOURETTE'S DISEASE

Under the title, "Study of a nervous affection characterised by motor inco-ordination, and accompanied with echolalia and coprolalia," Tourette[131] grouped together, in 1885, a certain number of cases presenting features in common and so enabling him to describe a morbid entity, specially remarkable for its progressive evolution. He was followed in the same line by Guinon, who supplied an account in nosographical form, and since then the disease has figured in all the text-books.

To obtain a schematic picture of the condition we shall borrow from Tourette's[132] last communication on the subject:

About the age of seven or eight a little boy or girl—for the sexes are affected equally—commonly with a wretched family history, begins to exhibit a series of tics. The attention of the parents is soon drawn to the fact, but they seldom give much heed at first, since the twitches are limited preferably to the facial musculature. At this stage, too, expiratory laryngeal noises are occasionally superadded.

The movements may be confined for a long time to the face, but under the influence of causes very difficult to determine they gradually invade the shoulders and the arms. First one shoulder is shrugged and then the other, then the trunk is inclined en masse to right or left; then the patient waves his hands or his arms, or bends backwards and forwards, or jumps up and down, flexing the knees alternately and tapping with his feet. The muscles of the larynx sometimes participate in the abnormal functioning, whence it is that many sufferers from tic give vent to quick expiratory "hems" and "ahs," which coincide often with the twitches of trunk and limbs.

The disease may be limited to this stage, but it is not uncommon to find, a few months or years after the beginning of the facial movements, that the inarticulate laryngeal sound becomes organised and develops in a particular direction, thus, in a sense, showing a pathognomonic value. Under the influence of causes whose action we are, in the majority of cases, powerless to appreciate, the patient gives vent one day to a word or short phrase of a quite special character, inasmuch as its meaning is always obscene. These words and phrases are exclaimed in a loud voice, without any attempt at restraint. There must be a complete absence of the moral sense where there is coprolalia such as this; at the moment of the ejaculation some irresistible psychical impulse must drive the patient to utter filthy words unreservedly and with no consideration for other people.

Another psychical stigma—echolalia—is occasionally, though less frequently, observed in these cases.

Such, then, is Gilles de la Tourette's disease, a clinical type of which many examples have been recorded. We do not think, however, that all tics can be brought under the same category; we lose sight of its distinguishing features if we make the attempt. Of course fruste and atypical cases are encountered, but even in them it is rare not to find a certain degree of mental instability in dependence on which echolalia and coprolalia rest, so completing the morbid syndrome, and it is important to recognise the successive development of these various constituents.

It is, indeed, this evolution of symptoms which is so characteristic of Gilles de la Tourette's disease. A careful scrutiny of recorded cases of tic, however, makes it abundantly clear that they do not all belong to the disease of convulsive tics; their localisation, form, and progress are so different that the effort to assimilate them to Tourette's disease would abolish the nosographical value of the latter. One patient may have an ocular tic all his life, and nothing else; the affection of another may be limited to a tic of the shoulder and arm; a third blinks and makes a facial grimace; a fourth is a coprolalic who has never suffered from tic. Are they all to be considered incomplete cases of the disease of convulsive tics? To answer in the affirmative is equivalent to a failure to appreciate the distinctive characters of a judiciously isolated syndrome, and a refusal to describe tics as they are met with in everyday life. One questions, in fact, whether some of the cases allotted to Tourette's disease really conform to it. Take an instance from Chabbert[133]:

A woman, aged forty-two, had had an injury to the left side of her face at the age of nine, as a result of which appeared a convulsive facial tic, accompanied at times by hysterical attacks which continued for eight years. The tic itself, an abrupt contraction of the inferior portion of the left orbicularis palpebrarum, underwent no subsequent change, in degree or extent. At a later stage a fairly definite tendency to coprolalia became manifest.

An unvarying post-traumatic palpebral tic in an hysterical subject cannot be said to constitute the syndrome of Gilles de la Tourette, in spite of the coprolalia.

In another of his cases the diagnosis is no less open to doubt:

The son of the previous patient was a youth of nineteen, with a bad heredity on the father's side. In boyhood he had been a somnambulist. Some months previously to his coming under observation he developed a convulsive tic limited to the frontalis. Stigmata of hysteria were present in dyschromatopsia, restriction of the visual fields, and left hemihyperÆsthesia.

A third case reported by the same author does probably belong to the disease of convulsive tics:

A woman aged forty-four, of a strumous diathesis, exhibited tics of face and limbs, occurring in the form of attacks sufficiently violent to cause bruises, attacks which were invariably associated with coprolalia. In addition, she suffered from echolalia, echokinesis, and folie du doute.

We can only repeat, of course, that each type of tic passes by insensible gradations into others that precede it or succeed it in the hierarchy of tics; but we must, provisionally at least, neglect the links that unite neighbouring groups if we are to avoid losing sight of admittedly distinctive characters in too comprehensive summaries. It is desirable to retain the term "disease of convulsive tics" for those cases whose progressive evolution ends in the generalisation of the convulsive movements, to the accompaniment of coprolalia and sometimes of echolalia. This clinical form represents the most advanced degree attained by the disease; it might be called the tic's apogee. From its psychical aspect, moreover, the development it undergoes may culminate in actual insanity.

According to the teaching of Magnan, the disease of convulsive tics does not constitute an entity, since each and all of its symptoms may occur separately as episodic syndromes of degeneration. The general considerations with which we introduced our study are applicable in this connection, and we shall be content to say with Noir:

We cannot deny the validity of the objections raised by Magnan and his school; but the fact that these various symptoms may and do most frequently occur singly is no reason for expunging the disease of Gilles de la Tourette from the text-books. The combination of these symptoms constitutes a clinical entity which has a specific evolution, and while its subjects are degenerates in the sense of Magnan and of Charcot, they may be ranged by themselves in a very definite group.

In some cases which apparently come under this category, psychical disturbance has not been a prominent feature.

Sciamanna[134] is the reporter of a case where a young man with neuropathic antecedents was afflicted with tics involving various muscular groups; his intellect, however, was normal, and the only psychical change was an insignificant disorder of affectivity.

In such a case it would be instructive to know the mental condition after the lapse of some years.

Two typical examples of Tourette's disease have been described by KÖster[135] as "disease of impulsive tics"; a third case—in which widespread muscular twitches, the muscles of respiration and the cremasters included, were coupled with sometimes a monotonous intonation and sometimes a jerky speech, though psychical functions were unimpaired—is considered by Kopczynski[136] to be a case of convulsive tic, which he distinguishes from the "disease of convulsive tics."

A last instance, published by Innfeld[137] as a case of "chronic progressive muscular spasm," is an unmistakable example of tic, in spite of the author's declaration that it does not correspond to any known morbid type and his attempt to liken it to chronic chorea. A boy of fifteen exhibited convulsive movements which had begun in the facial musculature and thence spread to the head, shoulders, and hands, and were accompanied with respiratory noises and involuntary exclamations. There was no alteration in sensation or in reflectivity, or in electrical excitability. Sleep banished while emotion aggravated the movements.

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 Tourette[139] 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. FÉrÉ[140] 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.

                                                                                                                                                                                                                                                                                                           

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