Tics and Their Treatment Part 30

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.

In a patient, aged twenty-six, suffering from general paralysis, Hermann[183] noted the presence of abrupt, irregular, myoclonic twitches in the sternomastoids, recti abdominis, adductors, and in some of the toes and fingers, first on one side and then on the other, also in both legs, and subsequently in both arms, the face being unaffected.

Jancowicz considers diagnosis possible only in typical cases, and expresses the opinion that paramyoclonus is a syndrome common to many affections. Further, Schupfer makes the perfectly justifiable remark that under this denomination have been included cases of ch.o.r.ea, tic, hysteria, and rhythmic spasm; others have been secondary to organic disease of the cerebro-spinal axis, such as rolandic lesions, spinal muscular atrophy, chronic poliomyelitis, syringomyelia. Others, again, depend on one or other of the psychoses, others on infective conditions such as malaria, diphtheria, typhoid, or on intoxications such as uraemia, mercurialism, or lead poisoning. Only a few recorded cases cannot be attributed to any of the conditions enumerated above, hence Schupfer's objection to the promiscuous cla.s.sification of them all as paramyoclonus multiplex is quite warranted, in the absence of a uniform etiology and symptomatology.

Schultze[184] has suggested the term monoclonus for the tics, and he distinguishes monoclonus, polyclonus, and paraclonus. Embraced in the last of these is the paramyoclonus of Friedreich, which, according to Schultze, is usually unilateral, voluntary action diminis.h.i.+ng the intensity of the involuntary movements, whereas the converse is the case in tic or monoclonus. Mixed forms are met with, however, and Schultze himself mentions one in which the movements were bilateral and increased with voluntary activity.

Heldenberg[185] applies the term intermittent functional myoclonus to twitches occurring from time to time in antagonistic muscles during voluntary movement, twitches exaggerated by excitement and diminished with rest. They occur in combination with well-marked vasomotor phenomena.

The myokymia of Kny and Schultze is characterised by fibrillation, pain, hyperidrosis, and changes in electrical excitability.

A case which seemed to be a combination of paramyoclonus with Thomsen's disease has been reported by Hajos[186] under the t.i.tle myospasmia spinalis.

There cannot possibly be any hesitation in arriving at a diagnosis between tonic tic and Thomsen's disease, a condition consisting in slowness of relaxation of a strongly contracted muscle, and conceivably due to defective metabolism or organic change in muscular tissue.[187]

Examples such as the above, culled at random from an abundant medical literature, and variously ent.i.tled, will serve to demonstrate the protean nature of what the medical world is content to call myoclonus, and if from this collection of motor disorders we may hope to extricate the tics, there will remain still no inconsiderable labour of differentiation for the student.

TIC AND ATHETOSIS

The athetotic movements that may accompany hemiplegia are scarcely likely to be confused with those of tic, but difficulties may arise where the athetosis is double.

It has been universally remarked that athetotic movements of the face reproduce the expression of emotions, such as admiration, astonishment, sorrow, gaiety, etc. Of course the same may be said of the grimaces of ch.o.r.ea; the latter, however, are usually more abrupt and pa.s.s less readily one into the other. The gesticulations of athetosis are undulatory, so to speak, and their excess leads to deformities princ.i.p.ally in the direction of forced extension. The musculature is often rigid, and the reflexes are increased in activity. Sometimes there is a considerable degree of mental disturbance.

Now, it is precisely in cases where mental deterioration is a prominent feature that "nervous movements" have been described resembling those of athetosis, for which the term pseudo-athetosis has been coined. Two examples may be quoted from Noir.

E. is a girl of eleven years. Her expression is grimacing; her tongue is often protruded, but never bitten; her head is regularly flexed or extended, or rotated rhythmically to left or right. The arms are moved spasmodically at shoulder and elbow, while the hands are the seat of athetotic movements. She walks curiously, throwing her feet out in advance without bending her knees. She has a silly smile, and her mouth almost invariably hangs open. On request she can keep her hands quite steady, but one observes at once the effort this entails in the sudden seriousness of her expression.

The ordinary acts of every-day life are performed satisfactorily enough: she can dress and undress, use a knife and fork, thread a needle, sew, etc.

J. is eleven years old also. She puckers her lips, contracts her eyebrows, elevates her alae nasi; at the same time she exhibits pseudo-athetotic movements of her fingers which are entirely under voluntary control.

The question may indeed be asked whether pseudo-athetosis and variable ch.o.r.ea are not really identical. Further, all sorts of combinations of athetosis and myotonia have been noted,[188] but more light must be shed on the subject before any further cla.s.sification can be attempted.

The following case has recently been published by Marina[189]:

A blacksmith, aged seventeen years, already treated three times for recurrent ch.o.r.ea, suffered from slow contractions of the shoulder muscles, involving the elevators and internal and external rotators successively, and accompanied by movements of the head and arm, and by twitches of the quadriceps. Nothing seemed to have any influence over these movements except sleep. The faradic excitability of the shoulder muscles was augmented, the galvanic excitability diminished. Application of the constant current to the head and back sufficed to effect a cure in three weeks.

Marina proposes the term athetotic myospasm for these incessant slow alternating contractions, impulsive myospasm being employed to signify convulsive movements of more than one muscular group, purposive yet irresistible, as in tic and ch.o.r.ea major. Simple myospasm consists of single twitches in individual muscles, recalling those produced by electrical excitation. If several muscles are implicated, the condition is one of multiple myospasm or myoclonus.

TICS AND TREMORS

All tremors, whether they occur during muscular repose or muscular activity, are distinguished by the relative restriction of their range and the regularity of their time. The tremors of paralysis agitans, disseminated sclerosis, senility, toxaemia, hysteria, ex-ophthalmic goitre, etc., are not liable to be mistaken for tic.

It is true, of course, that tremor is sometimes combined with ch.o.r.eiform or athetotic movements in patients with psychical stigmata.[190] A proposal, too, has been made to unite hereditary and functional tremor and to describe them as a tremor neurosis.[191]

However simple be the diagnosis between tremor and tic, it is worth while to note in pa.s.sing the etiology they may have in common. In a case recorded by van Gehuchten an intention tremor of the right arm co-existed with a tic of the right sternomastoid.