Part 23 (1/2)

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 distinguis.h.i.+ng 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.”