Part 30 (1/2)

True chronic ch.o.r.ea is an incurable neurosis, of life-long duration. We have no trouble in p.r.o.nouncing a diagnosis of chronic ch.o.r.ea 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 ch.o.r.ea as yet only a few weeks or months old.

The involuntary movements of chronic ch.o.r.ea, like those of Sydenham's ch.o.r.ea, are illogical, but they are combined in a co-ordinate manner--that is to say, certain functionally a.s.sociated 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 partic.i.p.ation 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 ch.o.r.ea.

C. Hysterical Ch.o.r.ea

The conditions to which the name of hysterical ch.o.r.ea is applied may a.s.sume two forms, the commoner being known as rhythmical ch.o.r.ea, the other as arhythmical ch.o.r.ea. 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 ch.o.r.ea), or of swimming (natatory ch.o.r.ea), or such professional movements as those of the blacksmith (_ch.o.r.ee malleatoire_). 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 t.i.tle 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 ch.o.r.ea,” and protests that the systematisation and co-ordination of the movements are very different from the clinical picture of Sydenham's ch.o.r.ea, while their rhythmical nature does not allow of their being cla.s.sified as tic.

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

The separation of hysterical from variable ch.o.r.ea 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 ch.o.r.ea.

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

D. Electric Ch.o.r.ea, Bergeron's Ch.o.r.ea, Dubini's Ch.o.r.ea, Fibrillary Ch.o.r.ea of Morvan

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

Bergeron's ch.o.r.ea 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 ch.o.r.ea 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 ch.o.r.ea, and Ricklin is inclined to consider the two identical, but further study of the question is desirable.

Dubini's ch.o.r.ea 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 ch.o.r.ea, or with the fibrillary ch.o.r.ea of Morvan, which is a disease of adolescence, characterised by fibrillary contractions in the calves and thighs, pa.s.sing 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.

TIC AND PARAMYOCLONUS MULTIPLEX--TIC AND MYOCLONUS

It is not our intention here to seek to provide a differential diagnosis between tic and the various conditions usually cla.s.sed as myoclonus, and that for two reasons: in the first place, we cannot admit that the latter form a distinct clinical or nosographical ent.i.ty, since the term myoclonus seems simply to be an abbreviation for clonic muscular convulsion, and is a symptom rather than a clinical syndrome; secondly, the fact that the tics themselves have been incorporated with myoclonus involves the investigation of all the published cases with a view to their critical sifting. This task we have pursued for our own edification, but to enter on it here would serve no useful purpose, and we shall rest content with examining succinctly several recent cases described as myoclonus, in the hope that the prosecution of further research will introduce order into what is at present chaos.

Among the various forms of myoclonus there is one which presents a certain individuality, and which was described originally by Friedreich under the name of paramyoclonus multiplex.

This disturbance of motility supervenes, in patients with a neuropathic heredity, after some psychical accident such as a sudden fright or emotion, and consists in clonic muscular convulsions affecting the body generally, with the exception of the face. The contractions appear without obvious cause in one or in several muscles, are instantaneous, involuntary, and usually bilateral, but their most important feature is their inequality and irregularity. They may or may not effect displacement of the limbs; in any case they compose neither gesture nor gesticulation. Volition occasionally seems to have some transient inhibitory influence over them; they are exaggerated by cold and by emotion, and usually disappear in sleep.

It is obvious that this account of a typical case precludes the possibility of any confusion with tic, but the published cases are not always in conformity with it.

In 1892 Lemoine[181] reported a case where the movements of paramyoclonus multiplex were accompanied with echolalia and psychical changes. Raymond quotes an instance of the disease being preceded by facial tic, and another a.s.sociated with tremor and ch.o.r.eic movements.

D'Allocco[182] has recorded twenty-four cases of differing forms of myoclonus, of which nineteen occurred as a family disease, in conjunction with stigmata of degeneration, epilepsy, and hysteria.