Tics and Their Treatment Part 23

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 t.i.tle, "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 ent.i.ty, 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 s.e.xes 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 ma.s.se_ 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 partic.i.p.ate 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 e.j.a.c.u.l.a.t.i.o.n 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 const.i.tuents.

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 a.s.similate 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 const.i.tute 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 hemihyperaesthesia.

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 a.s.sociated with coprolalia. In addition, she suffered from echolalia, echokinesis, and _folie du doute_.

We can only repeat, of course, that each type of tic pa.s.ses 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 const.i.tute an ent.i.ty, 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 const.i.tutes a clinical ent.i.ty 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 Koster[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 ch.o.r.ea. 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 Ch.o.r.eA 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 ent.i.ty 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 ch.o.r.ea.

The form of the motor reactions in this condition warrants the application to it of the term ch.o.r.ea, but the a.n.a.logies the disease presents to tic are very close, nevertheless, and sometimes the two occur in the same individual. Patients suffering from variable ch.o.r.ea 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 ch.o.r.ea need occasion no ambiguity: the ch.o.r.ea consists in the appearance of meaningless and apparently idiopathic involuntary movements, whose repet.i.tion during rest and action alike is proof of their irrationality and incongruity; the duration of the symptoms may be limited as in ch.o.r.ea minor or Sydenham's ch.o.r.ea, or unlimited as in ch.o.r.ea major or Huntington's ch.o.r.ea.

"Variable" is the epithet we apply to the ch.o.r.ea 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 ch.o.r.ea--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 pa.s.ses for an insignificant muscular caprice of no pathological importance, while its disappearance is not a.s.sociated 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 const.i.tute 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 ch.o.r.ea; in fact, they are nought else than a form of ch.o.r.ea themselves.

The psychical peculiarities of the patient with variable ch.o.r.ea may be summed up in instability of thought and action, combined with mental infantilism. Hence the terms "polymorphous ch.o.r.ea" and "ch.o.r.ea of degenerates" are used synonymously for variable ch.o.r.ea.[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 ch.o.r.ea 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 const.i.tuted, in short, a particular kind of ch.o.r.ea, changing and changeable, and differing from intermittent ch.o.r.ea 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 ch.o.r.ea 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 const.i.tutional inferiority was superadded a ch.o.r.eiform 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 ch.o.r.ea, 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 circ.u.mstances.

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 a.n.a.lysis. 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 p.r.o.nounced 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 cla.s.sics, but evincing a perfect pa.s.sion 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 cla.s.s, 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 ch.o.r.ea. 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 cla.s.s-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 cla.s.ses.

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