DIAGNOSIS
TICS AND STEREOTYPED ACTS
We have already, on more than one occasion, drawn attention to the
phenomena known as stereotyped acts, demonstrating their intimate
kinship with the tics and the frequent difficulty of establishing a
differential diagnosis. To ensure precision of ideas and of terminology,
we must restrict the expression to motor disturbances in which the
characters of the muscular contraction are identical with those of
normal acts. On this view many motor reactions are really classifiable
as stereotyped acts, and among them are those denominated by Letulle
"habit tics."
Stereotyped acts occur in normal individuals, and it may fairly be said
there is no one but has his habitual gesture, his movement of
predilection. As a matter of fact, a certain number of what Letulle
calls co-ordinated tics belong to the group under consideration; others,
no doubt, are genuine tics, and between the two may be found innumerable
intermediate varieties.
From the diagnostic standpoint the stereotyped acts that occur in the
course of mental disease, of which a conscientious study has recently
been made by Cahen,[168] are highly instructive. He defines them as
non-convulsive, co-ordinated attitudes or movements, resembling
intentional or professional acts, repeated at frequent intervals and
always in the same fashion, till their conscious and voluntary
performance is replaced by a degree of subconscious automatism. In the
case of the insane they are secondary to some delusion, and persist
though the latter may disappear. Hence the patient may be incapable of
explaining his movements and attitudes, however much he may persevere in
their automatic execution--an evolutionary process akin to that of the
tics.
A typical instance may be quoted from Séglas:
B. passed under observation in 1891, suffering from delusions of
persecution, and not long afterwards it was noticed that from time
to time he used to come to a halt in the courtyard, gaze at the
sun, and rotate his hands round an imaginary axis. The reply he
vouchsafed to interrogation on this point was that he was effecting
the sun's revolution. At present, however, he has sunk into a state
of dementia, and while the gesture continues he is unable to
furnish any explanation of it.
Of course it is inadmissible to apply the term to co-ordinated acts that
are neither conscious nor voluntary, such as the teeth grinding of the
general paralytic, or the body oscillation of the idiot. Similarly one
must differentiate them from impulsive seizures, abrupt irresistible
motor explosions neither frequent nor prolonged.
A distinction has been drawn between _akinetic_ (attitude) stereotyped
acts and _parakinetic_ (movement) stereotyped acts. As instances of the
former we may give the following:
A woman reclines continuously in bed because she believes she has
an infernal machine in her abdomen.
Another patient sits on the ground all day long, buttoning and
unbuttoning his clothes.
An old gymnast maintains while he stands a professional attitude
in which his head is raised, his right fist closed on his hip, his
right leg crossed in front of the left, and his right foot elevated
vertically.
Conditions such as these present the most intimate analogies to our
attitude tics, though in the case of the latter there is always a more
or less pronounced exaggeration of muscular contraction, a certain
degree of tonic convulsion.
Parakinetic stereotyped acts are of common occurrence, and embrace every
variety of movement or gesture.
A former acrobat leaps staircases, climbs railings, exercises his
arms rhythmically and regularly, etc.
A patient promenades untiringly in the same corner and at the same
pace.
An old engraver, now a dement, passes the day in reproducing in a
more or less modified form certain actions associated with his
former profession.
Alike in tics and in stereotyped acts, a time comes when the motor habit
establishes itself, for no apparent reason or purpose; hence the
co-existence of the two classes in chronic delusional insanity, in
dementia precox, in catatonic states, in systematised mental disease of
other forms, and in general paralysis.
Stereotyped acts may be the embodiment of ideas of persecution and of
grandeur, or the outcome of mystical, hypochondriacal, and other states.
A patient with delusions of persecution writhes because he is being
"electrified." A hypochondriac rests motionless because he believes
himself made of glass. A mystic maintains an attitude of genuflexion for
hours at a time.
Obsessions also play a part in the genesis of the acts we have under
consideration, but of all delusional ideas those of defence are the most
fertile in this respect.
A patient under the care of A. Marie used to carry a fragment of glass
between his teeth and other pieces beneath the soles of his feet, the
idea being that they formed insulating cushions whereby to protect
himself from the electricity of his enemies.
The suggestion was thrown out by Bresler that the movements of tic are
often of a defensive character--that the disease, in fact, is a sort of
"defence neurosis" linked to hyperexcitability of psychomotor centres.
This theory is not unlike the view of hysteria taken by Brener and
Freud, and as the movements themselves are usually of the nature of
mimicry, Bresler has proposed the term _mimische Krampfneurose_.
In some cases of mental torticollis, the attitude assumed may be
considered as a stereotyped act. Martin has recorded an example of
torticollis in relation to melancholia. Another of his patients suffered
from rotation of the head to the left, a position which could easily be
rectified by asking the man to make the sign of the cross. The moment he
put his finger on his forehead the displacement of the head was
corrected. If, however, he were requested to look straight in front of
him, he remained incapable of altering the vicious attitude, the reason
he advanced being that he could no longer see the sun.
One cannot but be struck with the remarkable analogies to the cases
given by Cohen. And it is worth remembering further, that sometimes
mental torticollis degenerates into actual dementia.
TICS AND SPASMS
Nothing is more arduous, at first sight, than the differentiation of a
tic from a spasm, the similarity of their external forms being a fertile
source of confusion. Yet the establishment of a correct diagnosis is of
prime importance, since in their case prognosis and treatment alike are
diametrically opposed.
Tic is a psychical affection capable of being cured, if one can will to
cure it: at the worst we may fail, but there is no idea that it is
indicative of a grave organic lesion prejudicial to life. A spasm, on
the contrary, though it appear in almost identical garb, is excited by a
material lesion on which depends the degree of its gravity. The focus of
disease may disappear, no doubt, but it is only too likely to persist
and to occasion other disorders. Hence the desirability of making sure
of one's diagnosis--a proceeding not necessarily of insuperable
difficulty. If we apply the principles of diagnosis enunciated by
Brissaud, to which our attention has already been directed, we shall not
find the task beyond our powers.
Let us take a concrete instance.
Here is a cabman, forty-nine years of age, the left half of whose
face is the seat of convulsive twitches. These commenced eighteen
months ago by brief insignificant contractions of the left
orbicularis palpebrarum, which have gradually spread to the whole
of the muscular domain supplied by the left facial nerve. Their
momentariness and rapidity, their apparent independence of
extraneous stimuli, their indifference to treatment and resemblance
to the twitches produced by electrical excitation, their occurrence
in sleep, the fact of voluntary effort, of attention or
distraction, serving so little to modify their range and
intensity--all make clear the spasmodic nature of the condition.
The motor manifestation is the consequence of irritation at some
point on a bulbo-spinal reflex arc; its abruptness and
instantaneousness negative the possibility of recognising in it any
sign of functional systematisation. It is not a co-ordinated act of
a purposive nature, but a simple, unvarying, constant motor
reaction to a particular stimulus. That its intensity should be in
direct proportion to the intensity of the latter, changing from
feeble contractions to a state of transient tetanus, is further
proof of its spasmodic origin. When the excitation is at its
maximum, there is sometimes involvement of the opposite side of the
face, by virtue of the law of the generalisation of reflexes.
It is true there is no association of pain with his attacks, as in
so-called tic douloureux, but the spasm is heralded by a tingling
sensation below and to the inner side of the outer corner of the
eye. This sensation, "like an electric battery," persists during
the spasm and disappears in the intervals. Its occurrence suggests
that the ascending branch of the infraorbital nerve, springing
from the trigeminal, is affected, and indeed pressure over its
point of emergence evokes a certain amount of pain. Moreover, there
is occasionally a flow of tears when the spasm is at its height. It
may be difficult to decide whether this is the result of mechanical
compression of the lachrymal gland or an exaggerated secretion of
tears under the influence of stimulation of the lachrymo-palpebral
twig of the orbital nerve. In any case the pathogeny of this facial
spasm is entirely comparable to that of tic douloureux of the face,
and it is quite within the bounds of possibility that a minute
hæmorrhage--for the patient is of a very florid type--somewhere on
the centrifugal path of the trigemino-facial reflex arc, may be
giving rise to the phenomena.
What we wish to insist on, however, is the dissimilarity between
this facial spasm and tic. In the movements we have been describing
we fail to distinguish any purposive element, any co-ordination for
the fulfilment of a particular function: they are not imitative in
character, nor do they express any sentiment; no impulse precedes
their execution, no satisfaction follows.
The patient's mental state presents no peculiarities, as far as we
have been able to discover. There is no volitional debility or
instability; if he cannot control the convulsions, it is to be
remarked that he cannot control them even for a moment, whereas all
sufferers from tic are capable of inhibiting it for a longer or
shorter period by an effort of the will, by concentrating their
attention on it.[169]
The following remarks on this case are due to Professor Joffroy:
If the patient be asked to open his mouth, the spasm of the left
cheek remains in abeyance at long as it is open, but the platysma
of the same side then begins to twitch spasmodically. Or if he be
requested to shut his eyes, so long as they continue closed the
cheek is quiescent; but, on the other hand, both orbiculares
palpebrarum, as well as the pyramidal muscles and the adjacent
fibres of the frontalis, are seen to contract irregularly. There is
a sort of transference of spasm, and this is of peculiar interest,
inasmuch at it affords evidence that the lesion is not so
restricted as one might suppose.
The explanation no doubt is to be sought in the law of the
diffusion of reflexes, confirming the diagnosis of an irritative
lesion at some point on the trigemino-facial reflex arc.
In the differential diagnosis of spasm assistance may be obtained by a
consideration of the following points:
The extreme abruptness of the movement recalls the contractions produced
by electrical stimulation.
There is no purposive or co-ordinated feature in the spasm, which is
confined to some nerve area anatomically limited.
Volition, attention, distraction, emotion, all fail to effect any
modification of the phenomena.
No irresistible impulse precedes their manifestation, nor is it
succeeded by a feeling of satisfaction. Sometimes the spasm is
accompanied by severe pain.
As a general rule the patient's mental state does not present the
anomalies met with so frequently among those who tic.
Important information may be gleaned from a scrutiny of the condition
during sleep. Should the convulsive movement persist, it may be said
with confidence to be a spasm; whereas if it completely disappear, it is
probably a tic. Whether a spasm may vanish in sleep, however, is another
question, which clinical observation has not yet satisfactorily
answered, and if no other indication of organic disease be forthcoming,
the problem must in the present state of our knowledge be left unsolved.
A. Tic or Spasm of the Face
In cases where the face is the seat of the convulsive movements this
problem of diagnosis becomes one of the utmost nicety. That a
distinction may be drawn, however, is universally admitted.
Hallion,[170] for instance, specifically separates clonic spasms due to
structural changes from the "nervous movements" of neuroses such as
chorea or tic. Facial spasm is rigorously limited to the distribution of
the nerve, and is commonly the result of some alteration in it effected
by causes similar to those that occasion facial paralysis.
Clonic spasms of the face are occasionally a sequel to local
traumatism--that is to say, they are the result not of direct but of
reflex excitation of the facial nerve. Tic douloureux belongs to this
class. Tic non-douloureux also is sometimes merely a simple reflex
spasm.
One of the most pregnant of Brissaud's lessons is devoted to the
elucidation of this part of our subject, and we have already made
several quotations from it. In many cases he is forced to say, "I
decline to hazard a diagnosis when etiology is silent." We too have been
face to face with this diagnostic difficulty on several occasions, and
it may be instructive to give the details of one or two cases where no
definite conclusion could be arrived at.
A man thirty-seven years of age had been suddenly seized with
facial paralysis on the left side thirteen years before,
accompanied after an interval of eight days by bilateral
fronto-temporal cephalalgia, nausea, vomiting, and disturbances of
vision. These attacks recurred irregularly during the next four
years, since when they have ceased, although the palsy persists.
Recently the patient woke up abruptly in the middle of the night to
find that the left side of the face was in a state of spasmodic
contraction, a condition which has continued absolutely without
intermission. There is no pain in relation to the spasm, merely a
peculiar sensation at the site of the muscular twitches. Of what
nature are they?
If we analyse the muscular play somewhat more closely, we observe
that with the exception of the frontalis all the muscles of the
left face, including the platysma, contribute. On a background of
more or less permanent contraction are outlined short, incomplete,
greatly varying twitches, affecting one muscle after another, and
sometimes only a few fibres, in a highly erratic way. The march of
the movements obeys no law, either of space or time, nor is there
any co-ordination in their activity. That the condition is one of
tic, therefore, is scarcely conceivable. No purposive element is
discoverable in the phenomena, no systematisation, no expression
of emotional excess. All is disorder, confusion, contradiction.
We should, accordingly, be content to make a diagnosis of spasm,
but an examination of the patient's mental condition must not be
neglected, and in this particular case it is very instructive.
It appears that his imagination has always been singularly fertile,
amounting indeed to eccentricity. The picturesque description he
furnished of the unusual sensations in face and neck lent support
to the view that his muscular activity was intended, consciously or
unconsciously, to free himself from their insistence, so that his
grimacing may have been but a gesture of defence.
But however much his lack of psychical equilibrium may favour the
relegation of his affection to the category of tic, certain
considerations make one question the validity of the hypothesis.
In the first place, it is rather an uncommon functional adaptation
of the facial muscles to utilise them in an attempt to disembarrass
oneself of disagreeable sensations; and in the second it is no less
uncommon for the sufferer from tic to be unable to restrain his
muscles even momentarily, as our patient appears to be. The actual
time of onset of the movements is significant enough, but of
supreme importance is the fact of their supervention in an area
previously the seat of paralysis. To our mind this is more than a
coincidence; from the history supplied by the patient it is plain
that the paralysis was peripheral and that the lesion involved the
facial trunk somewhere in its intracranial course after its
emergence from the side of the pons. Thirteen years later,
convulsive movements appear in the same domain. Taking all the
circumstances into consideration, we think the hypothesis tenable
that the trigeminal is implicated in the pathogeny of the spasm,
although the condition is not strictly comparable to the classic
tic douloureux.
The exact nature of the lesion is more difficult to determine. A
review of the details of the facial palsy suggests its vascular
origin, to which theory the headache, nausea, and photophobia of
succeeding days and months--indicating, as they do, a circulatory
disturbance in the basilar region--lend support. With the gradual
restoration of vascular equilibrium the migrainous attacks lessened
in frequency and severity, though the facial trunk remained
compressed, till the spasm appeared, no less suddenly than had the
paralysis. It is feasible that the former, too, is the derivative
of a minute hæmorrhage irritating either the centrifugal or the
centripetal arm of the facial reflex arc, probably the latter,
which would explain the paræsthesiæ.
The possibility of this explanation being accurate is confirmed by
a case reported by Schültz, where facial spasm of ten years'
duration was shown at the autopsy to have been caused by an
aneurism of the left vertebral artery impinging on the facial nerve
in the neighbourhood of the basilar trunk.
The arguments, therefore, which plead in favour of the spasmodic
nature of the condition seem to us so cogent that the hypothesis of
tic must be rejected. We ought not to forget, on the other hand,
that a spasm, of whatsoever origin, may be transformed into a tic
by the perpetuation of a morbid habit.
Let us take a second case, no less instructive than the preceding.
Madame L. was sent to one of us by Professor Pierre Marie. She had
always been nervous, impressionable, and high-spirited, but had
never suffered from fits. At the age of eight years, during
convalescence from one of the exanthemata, she got a chill, and the
very next day developed an acutely painful torticollis, the head
resting on the right shoulder and the chin touching the left
clavicle. A complete cure ensued, but from that time a certain
degree of facial asymmetry was remarked. At the age of eight and a
half menstruation commenced, and it still continues, at the age of
fifty-nine.
From youth she had at intervals been stricken with pains in the
limbs, and with recurrent bilious attacks. Two years ago the death
of her husband was the occasion of great mental strain and
distress. Sixteen months ago she noticed a curious sensation in the
right eye, not painful, accompanied from time to time by blinking
of the lids. Very gradually the convulsive movements spread over
the whole of the right face, and for the last month their frequency
and intensity have been such that rest is an impossibility.
When she came under observation what impressed the mind first was
the remarkable asymmetry of her figure: the right side of the face
was smaller than the left, the right eye appeared to be at a lower
level than the other, while the mouth was strongly deviated to the
right and the chin twisted in the same direction. For a minute or
two the facial contortion held sway, disappearing only to reappear
quickly.
Not solely to the old torticollis was the facial asymmetry
attributable, but also to the convulsive movements of the right
half of the face. The effect of these was to close the right eye,
deflect the nose to the same side, drag the mouth in a similar
fashion, and wrinkle the skin of the chin and neck. Hence was
evolved a unilateral grimace quite unlike any ordinary expression,
resembling rather the facies in contracture secondary to facial
paralysis.
During the next few months there was a gradual change from this
tonic to a clonic stage, in which the movements were of less
frequent occurrence, but more rapid. In repose there was no further
indication of the old facial palsy than the flattening of the
facial lines on the right. Under the influence of any emotion, or
any passing contrariety, or in the course of an animated
conversation, or if circumstances call for their repression, the
spasms increase in number and degree, whereas solitude and
tranquillity favour their subsidence.
A recent development has been the discovery of a means of checking
the spasm--viz. by compressing the larynx with the fingers of the
two hands. Madame L. admits the illogical nature of the
manOEuvre, but extols its efficacy. As a matter of fact, it
sometimes fails of its object.
How, then, is this localised convulsive movement to be designated?
Is it a tic or is it a spasm?
The march of the disease, its painlessness, the absence of any
reaction in sleep, the success of the little laryngeal trick, the
inhibitory effect of the will, the definite influence of attention,
distraction, in short of the psychical condition of the moment--all
plead in favour of its classification in the former category. On
the other hand, we cannot shut our eyes to the fact of the
pre-existence of specific organic disease, and, moreover, the spasm
is strictly confined to the anatomical distribution of the facial
nerve. Even in periods of repose there is a certain amount of
fibrillation on that side. On these counts are we to hazard the
diagnosis of facial trophoneurosis?
A subsequent opportunity of examining the same patient served to
confirm the diagnosis of spasm secondary to facial dystrophy, and
treatment failed to make any impression on the condition.
Our object in giving these cases has been to point out the difficulties
in the way of diagnosis, especially where spasm is superadded to a
mental state that itself predisposes to tic. The wisest plan in many
instances is to confine oneself to a description of the symptoms and to
tabulate the arguments for and against a particular view, without
perpetrating the error of committing oneself.
Many cases labelled convulsive tic might be quoted where the expression
of so definite an opinion ought to have been reserved, as in one
reported by Mayer[171] under the title of convulsive tic consecutive to
infraorbital neuralgia:
A man, thirty-two years of age, had suffered from a severe
infraorbital neuralgia of some weeks' duration, apparently
attributable to a chill. The pains recurred at intervals till their
substitution five years later for slight spasmodic twitches of the
left eyelid, which gradually developed into violent convulsions of
the whole of the left half of the face. These spasms were preceded
by a sensation of numbness in the left ear, while during repose no
modification of facial expression was to be remarked.
Further, there was a history of exactly similar neuralgia and spasm
in the mother of the patient, although in her case the latter had
been the first to appear, and had been replaced after a six years'
interval by left facial neuralgia, which resection of the nerve
failed to relieve.
In these cases the condition is undoubtedly one of painful facial spasm,
inaccurately and unfortunately styled "tic douloureux."
Bruandet[172] has recorded a typical example of right facial hemispasm
consequent on facial neuralgia, in which, however, no certain
macroscopical or microscopical lesion was detected, in either cortex or
bulb. But the mere fact that no structural alteration was discovered
post-mortem cannot invalidate the diagnosis; the imperfection of our
methods of investigation suffices to explain the negative results of
such researches.
B. Tic or Spasm of the Neck--Torticollis Tic and Torticollis Spasm
To make a diagnosis of torticollis, it is essential to satisfy oneself
of the integrity of the bones, muscles, and articulations of the
cervico-scapular region, previous to directing attention to the
psychical state of the patient. In regard to the latter point, the
question of heredity must not be neglected. If personal and hereditary
defects are prominent, the presumption is in favour of mental
torticollis; and if the convulsive movements present the characters of
tic, the diagnosis is practically certain.
In three cases under the observation of Fornaca,[173] for instance,
there is no room for doubt. Not merely was there no sign of irritation
from peripheral sources, but also no one of the three was psychically
normal.
Nevertheless we frequently find ourselves confronted by the question: is
the movement a tic, or is it a spasm? For, strictly speaking, there are
both a torticollis tic and a torticollis spasm, and their separation one
from the other is often a matter of the greatest perplexity.
We must refer the reader to the chapter devoted to mental torticollis
for a consideration of the features of that condition, and we need not
dwell on those cases of spasmodic torticollis that are obviously
occasioned by irritative lesions of nervous centres or conductors. In
this latter category may be placed the case put on record by Oppenheim,
where torticollic spasms were produced by pressure of a cerebellar
tumour on the cranial nerves.
But in the affection known as hyperkinesis of the accessory of Willis we
have little doubt both tics and spasms have been included. Apart from
the cases of spasmodic torticollis, so called, which Babinski has
published and to which reference has already been made, we may be
allowed to cite one or two more, in order to exemplify the differences
of interpretation to which they are liable.
At the Congress of Toulouse two patients were shown by Desterac,[174]
both of whom had suffered since the age of eight from a disease akin
either to Friedreich's disease or to hereditary cerebellar ataxia.
They presented the spastic gait of the former with the involuntary
movements of the latter, in addition to spasm of the hand in
writing, spasmodic movements of the trunk, and spasmodic
torticollis. Both had club foot and scoliosis, and one was
afflicted with spasm of the face and left arm. In his case,
further, there was nystagmus, together with loss of reflexes and
difficulty in articulation, while fibrillary contractions were to
be observed in his muscles. The other patient's reflexes were
exaggerated, and he showed a double extensor response.
In Desterac's opinion their spasmodic torticollis was dependent on this
congenital constitutional affection, which might be regarded as a
_fruste_ form of one of the diseases above mentioned.
Through the kindness of M. Desterac the opportunity has been granted one
of us of examining the two patients, and we should like to point out why
we think his interpretation of their symptoms must be considered with
reserve.
Speaking generally, we thought the cases closely resembled those in
which a long-standing mental torticollis is accompanied with convulsive
movements of the limbs. The scoliosis was not permanent, the deformation
of the foot could be overcome, and at the same time we failed to
convince ourselves of the presence of nystagmus and the absence of the
knee-jerks. Moreover, we happened to observe one of the patients in the
street unawares, and remarked how between two phases of bizarre
contortions his vicious attitudes and convulsive gestures almost
entirely vanished. In fact, the clinical picture seemed to us to be
quite other than that associated with organic disease such as
Friedreich's disease or hereditary cerebellar ataxia.
Another case recently brought before the Neurological Society of Paris
by Marie and Guillain[175] serves even better to illustrate the
intricacies of diagnosis.
The patient was a man of fifty-eight, who for years had exhibited
certain movements apparently of an athetoid nature. His head was
extended and rotated to the right synchronously with elevation and
eversion of the left shoulder, then it passed into flexion. Except
for a few odd movements of the tongue, the face conserved
immobility. In the arms the localisation of the contractions was
mostly proximal, though there were alternating flexion and
extension movements of the fingers which suggested athetosis.
Flexion, inversion, and adduction of the thighs also occurred. The
recti abdominis were similarly involved.
Under the influence of emotion the movements were increased, but
they could not be inhibited by an effort of attention. Their rate
was too slow for chorea. Ordinary voluntary movements were
performed without apparent trouble; the patient was able to dress
himself, and to drink without spilling the liquid. Diminution of
the knee-jerks was noticed, with what seemed to be an extensor
response. Slight scoliosis of the vertebral column and a misshapen
right foot recalled Friedreich's ataxia. There was nothing to
justify a diagnosis of hysteria.
This curious condition dated from the year 1874, when the patient
had a febrile attack, in the course of which pain and tingling
appeared in the toes of the right foot, followed by involuntary
movements of the same member. Analogous symptoms were not long in
appearing in the left arm. Two months later the condition had
become general, but from that time no special modification took
place.
In the subsequent discussion it was remarked by Souques that the case
resembled one recorded by Chauffard[176] as Friedreich's disease with
athetotic attitudes, where the patient was a child with club foot,
diminution of the knee-jerks, and generalised athetotic movements.
Notwithstanding our inability to assign a definite nosographical
position to examples of this kind, we think it desirable to make some
reference to them, in the hope that further observations will aid in
their diagnosis. They at least remind us that convulsions occurring in
the course of organic disease may be simulated by the manifestations of
certain motor neuroses.
TICS AND CHOREAS
A. Sydenham's Chorea
It would be difficult to find a better description of chorea minor than
that given originally by Sydenham himself:
The dance of Saint Guy, chorea Sancti Viti in Latin, is a sort of
convulsion whose incidence is greatest, in both sexes, between the
age of ten and puberty. Its onset is characterised by weakness of
one limb, which the patient drags behind him, and soon the arm of
the same side is affected in the same way. He finds it impossible
to maintain the same position of the arm for two consecutive
moments, however great be his efforts to attain this object. Before
he can bring a full glass to his lips he makes innumerable gestures
and antics, as the convulsive moments of the limb deviate it from
one side to the other, until at length he has piloted the glass
opposite his mouth, when he empties it at a gulp.
If we were to confine ourselves to this description by Sydenham, which
so far as typical cases of the disease are concerned is perfectly
accurate, differentiation between tic and chorea would not be a matter
of any complexity. Unfortunately, however, the varieties of this form of
chorea are legion, and in practice one constantly meets with conditions
suggesting alike the gesticulations of chorea and the convulsive
reactions of tic. Moreover, it has been pointed out by Oddo[177] that
the fact of the habitual exaggeration of tic during the very years when
chorea is liable to appear is calculated to confuse the issue.
He has attempted, however, to specify certain factors in the
differential diagnosis. In the first instance, the form of the movements
is of significance: there is no co-ordination in the muscular play of
the choreic; it is amorphous, indefinable, and erratic, whereas the
gestures of tic are purposive, and may be said to have a shape. One
never sees in chorea a succession of similar movements, but though a
patient be suffering from several tics, each of them is reproduced
always in the same fashion. Unilaterality of distribution is more common
in chorea than in tic; in other words, chorea, more or less, follows
anatomical lines in the regions it affects, whereas the incidence of tic
is physiological.
Both are arhythmic in their manifestation; nevertheless the repetition
of tic is noteworthy for its regularity as compared with the changing
mode and rate of the other. Noir emphasises the diagnostic value of its
frequency, abruptness, and reiteration of identical movements. In a
majority of cases the interference of the will is futile as far as
chorea is concerned, while the victim to tic is usually capable of
restraining his muscular activity at least for a space. The choreic
exhibits his movements in public, but the _tiqueur_ seeks the seclusion
of his own room. The association of tic with obsessional ideas is
frequently encountered, but there is no similar connection between
obsessions and chorea. In addition, the myasthenia, pains, and
alterations in the reflexes that often characterise chorea are awanting
in the other affection.
It cannot be gainsaid, however, that the frequency with which atypical
varieties of chorea occur is inimical to a ready diagnosis, and the
onerous nature of the task is not lessened by the circumstance that many
choreics are the offspring of neuropathic parents and reveal psychical
anomalies comparable to those of the subjects of tic.
In a disease such as variable chorea, which has features in common both
with tic and with chorea properly so called, the problem of diagnosis is
still more complicated, though excellent hints for its solution have
been furnished by Brissaud.[178]
However frequently and warmly the theory of the origin of chorea in
a neuropathic predisposition was advocated by Charcot, the fact of
its usual evolution consecutive to some toxic or infective process
is no less certain. Its incidence is greatest in children and the
adolescent; it runs a regular course of increase and decrease; and
the circumstances which cause the symptoms to vary during this
cycle are never sufficiently potent to bring about even transitory
suppression of them.
It is true that changes in the intensity of the symptoms seem to
confer a remittent character on the affection, but there is nothing
at all comparable to the sudden and unexpected waxing and waning of
the form of chorea at present under consideration. None of the
pathological attributes just mentioned concerns variable chorea,
which, in addition, differs from Sydenham's chorea in two
points--the multiplicity of the types of movement, and the fact
that the patient can voluntarily check his involuntary actions. For
these reasons, assimilation of the two clinical varieties is
impossible, and the confusion of the two in practice need never
occur.
A form of chorea entitled "habit spasm" by Gowers, and "habit chorea" by
Weir Mitchell, has been the subject of further study by Sinkler,[179]
but in all probability the cases of this description reported are
instances of the variable chorea of Brissaud.
B. Huntington's Chorea
In spite of the preponderating etiological significance of heredity and
the constancy of psychical imperfections in the chronic chorea of
Huntington, its confusion with tic is not at all likely to occur.
Difficulties might arise in distinguishing chorea major from variable
chorea, however, and here we have the views of Brissaud to help us.
True chronic chorea is an incurable neurosis, of life-long
duration. We have no trouble in pronouncing a diagnosis of chronic
chorea 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 chorea as yet only a few weeks or months
old.
The involuntary movements of chronic chorea, like those of
Sydenham's chorea, are illogical, but they are combined in a
co-ordinate manner--that is to say, certain functionally associated
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
participation 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 chorea.
C. Hysterical Chorea
The conditions to which the name of hysterical chorea is applied may
assume two forms, the commoner being known as rhythmical chorea, the
other as arhythmical chorea. 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 chorea), or
of swimming (natatory chorea), or such professional movements as those
of the blacksmith (_chorée malléatoire_). 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 title 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 chorea," and
protests that the systematisation and co-ordination of the movements are
very different from the clinical picture of Sydenham's chorea, while
their rhythmical nature does not allow of their being classified as tic.
Sometimes hysterical chorea is arhythmical--that is to say, the
movements are irregular and contradictory, as in ordinary chorea. True
chorea in cases of hysteria comes under this heading, as well as those
cases where hysterical patients imitate the movements of chorea. The
presence of the distinctive characters of hysteria makes a diagnosis of
tic improbable.
The separation of hysterical from variable chorea 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 chorea.
The condition described as chorea gravidarum may be placed at one time
in the category of hysterical chorea, at another in that of ordinary
chorea. In it there is intense motor restlessness, and accompanying
mental symptoms are not awanting in a majority of instances.
D. Electric Chorea, Bergeron's Chorea, Dubini's Chorea, Fibrillary
Chorea of Morvan
To render the study complete, we may remind ourselves of those still
imperfectly differentiated forms known as electric chorea
(Hénoch-Bergeron) and Dubini's chorea.
Bergeron's chorea 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 chorea 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 chorea, and Ricklin
is inclined to consider the two identical, but further study of the
question is desirable.
Dubini's chorea 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 chorea, or with
the fibrillary chorea of Morvan, which is a disease of adolescence,
characterised by fibrillary contractions in the calves and thighs,
passing 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 classed as myoclonus, and
that for two reasons: in the first place, we cannot admit that the
latter form a distinct clinical or nosographical entity, 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 associated with tremor and choreic 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 chorea, 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
uræmia, 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 classification 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 diminishing 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 title 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 entitled, 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
chorea; the latter, however, are usually more abrupt and pass less
readily one into the other. The gesticulations of athetosis are
undulatory, so to speak, and their excess leads to deformities
principally 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 alæ 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
chorea 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 classification can be attempted.
The following case has recently been published by Marina[189]:
A blacksmith, aged seventeen years, already treated three times for
recurrent chorea, 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 chorea 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, toxæmia, hysteria, ex-ophthalmic
goitre, etc., are not liable to be mistaken for tic.
It is true, of course, that tremor is sometimes combined with choreiform
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 passing 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.
A sudden twitch of the whole body Letulle particularises as a "tic of
starting," and Noir too thinks that a start of this nature may
constitute a tic, but we are inclined to consider it a generalised
reflex.
TICS AND PROFESSIONAL CRAMPS
We have already had occasion to enlarge on the distinguishing features
of professional or occupation cramps, spasms, or neuroses. Writers,
pianists, violinists, flutists, dressmakers, telegraphists, watchmakers,
milkers, knackers, blacksmiths, shoemakers, tailors, dancers,
embroiderers, barbers, etc., etc., are all liable to suffer from
occupation cramps. In every case the condition is one of inability to
perform the professional movement, and that alone.
Grasset proposes to separate intra-professional from post-professional
spasm, the former consisting in the impossibility of making the
necessary professional movements, the latter in the involuntary
over-reproduction of the familiar act. Properly speaking, the
post-professional spasm is a tic.
We need not do more than remind the reader of the close affinities we
have already seen to exist between tics and professional cramps, and of
the mental instability which both classes of patient present.
L. supplies an instance of variable hemichorea followed by writers'
cramp and later by mental torticollis.
When L. was eight years old choreiform movements of the right arm
began to appear, and soon rendered writing an impossibility. The
disease continued for so long a time that one might not
unreasonably expect to find considerable actual impairment of her
caligraphy. As a matter of fact, it is scarcely affected: the
patient can make her letters correctly, but after each letter she
lifts her pen to allow her fingers to perform an abrupt movement,
then she proceeds.
It cannot therefore be considered a true writers' cramp, but when
she had learnt to write with the other hand it was not long ere
that became the seat of a genuine cramp. The moment she attempted
to make the pen move over the paper her grasp of it tightened and
her fingers stiffened; her wrist would no longer answer her. To
obviate the trouble she used a pencil, at first with complete
success; but the cramp occurred afresh, and she gave up writing
altogether. Prolonged holidaying, however, and respite from the
exercise, had a salutary effect, and to-day there is no trace of
former mischief.