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contrary, they are often " superior degenerates," bright and
lively, but mentally immature, capricioas, emotional, psychas-
thenic, and frequently the subjects of obsessions and various
forms of " phobia." The greater
the psychical abnormality, the
more inveterate is the tic.
" Tiqueurs " often have other
evidences of the psychasthenic
constitution, such as explosive
articulation, " word-swallowing,"
sudden stoppage of speech, dis-
ordered respiration, echolalia (re-
petition of a particular word or
phrase), or coprolalia (repetition of
a blasphemous or obscene word).

Spasmodic torticollis is one of
the commonest and most severe
varieties of tic. Though the result
of the movement is to jerk the
head to one side, commonly to
the left (see Fig. 47), the spasm is
really a bilateral affair, since muscles on both sides of the neck,
protagonists and antagonists, are employed to produce the move-
ment. It is sometimes combined with a backward jerk, a so-
called retro-collic spasm. The movement may be tonic, clonic, or
a combination of the two — tonico-clonic. The patient can often
curb the movement by means of some antagonistic gesture of his
own invention, e.g. by light pressure on the chin with his finger.
Severe torticolHs usually begins after middle life. At first occurring
in paroxysms, it ultimately becomes continuous during waking hours,
and the affected muscles become hypertrophied from over-use.

Besides idiopathic spasmodic torticollis, which comes on ap-
parently spontaneously, certain cases have a definite exciting
cause and should be classed, not with the tics, but with the reflex
spasms which we shall consider presently. Thus, for example,
we may have neuralgic torticollis,^ in which an occipital neuralgia

1 Cruchet, Traite des Torlicolis Spasmodiques, Paris, 1907.



Fig. 47. — Spasmodic torticollis, with
secondary hypertrophy of right
sterno-mastoid.



102 NERVOUS DISEASES

or a painful tooth is followed by spasmodic torticollis, usually tonic
in form. When the neuralgia passes off, the muscular spasm ceases
also. Again, we may have labyrinthine torticollis,^ due to chronic
irritation of one of the semicircular canals. In such cases the
torticollic spasm has the object of mitigating vertigo, which would
occur if the head were not kept leaning over to the opposite side.
A torticollis thus initiated may become inveterate, but some cases
are cured by the administration of quinine. A small proportion
of cases are examples of professional torticollis, occurring only at
the moment of performing a special act, especially in cobblers and
tailors, who have to turn the head and eyes to follow the needle.
Such cases are more properly to be classed with the occupation-
neuroses (see p. 265). Spasmus nutans, head-rolling, and other
rhythmic movements (see p. 94), must not be confounded with
true torticollis, in which the spasms are either tonic or irregularly
clonic.

There is another group of movements which, originally excited
by some peripheral irritation, are classed as reflex spasms. A
spasm, unlike a tic, begins locally, perhaps in a single muscle,
and spreads to adjacent muscles. When the exciting stimulus
is unilateral, the reflex spasm is usually unilateral also, but
not invariably so, for bilateral reflex spasms also occur, as in
tonic and clonic contraction of the orbiculares oculorum (ble-
pharospasm), the result of corneal or conjunctival inflammation,
or as in the case where a vaginal caruncle or anal fissure
produces vaginismus with bilateral adductor spasm. Of the
unilateral reflex spasms one of the most striking is the intense
facial spasm which occurs in severe cases of trigeminal neuralgia or
tic douloureux, which is not a true tic but a reflex spasm. In
this disease the patient has paroxysms of agonising pain in one or
more divisions of the trigeminal nerve. During a paroxysm, the
face on the side of the pain is thrown into strong tonic con-
traction, the eye is closed, the mouth is drawn up on the affected
side, and the patient often presses his hand desperately over
the site of pain. Only when the acute stage of the paroxysm
passes off do the facial muscles relax.

Less severe paroxysmal facial hemi-spasm, either tonic or clonic,
usually commencing with waves of flickering fibrillary movement,
especially in the orbicularis oculi, closely resembling the move-

^ Curschmann, Deutsche Zeitschrift fiir Nervenheilkunde, 1907, p. 305.



INVOLUNTARY MOVEMENTS



103



merits produced by faradic stimulation and implicating some or
all of the facial muscles, may arise from other reflex causes,
generally in the territory of the fifth nerve, such as a decayed
tooth, a non-erupted wisdom-tooth, a nasal polypus, &c. Facial
hemi-spasm, unUke tic
douloureux, is painless.
Occasionally it occurs
primarily, without ap-
parent cause, as in the
patient shown in Fig. 48.
It also occurs, less fre-
quently, in lesions of the
facial nerve itself, as, for
example, when tumours
or abscesses compress the
nerve. Sometimes it fol-
lows an attack of ordinary
facial palsy, less com-
monly it may precede its
onset, so that in every case
of facial hemi-spasm we
should search for local
lesions in the territory
not only of the trigeminal
but also of the facial nerve.

Sometimes a reflex spasm may persist as a habit-spasm, long
after the original exciting cause has passed away. Such cases
can usually be diagnosed by their history. For example, a lad
lost his left arm by avulsion in a machinery accident. The stump
was amputated at the shoulder- joint, but clonic spasms appeared
in the trapezius and scapular muscles, and these persisted after
all the posterior nerve-roots in that region were divided by
operation. Certain well-marked types of blepharospasm sometimes
follow shell-explosions in war. In one variety the retinae, owing
to the intense flash of the bursting shell, are over-stimulated and
become hypersesthetic. The patient endeavours to protect them
from light by a vigorous blepharospasm, which may be bilateral,
or, more commonly, unilateral. In other cases, again, particles of
dust are driven into the conjunctivae by a missile landing close in
front, and here also a reflex blepharospasm results, as in the patient
shown in Fig. 48a, who was struck by a fragment of bomb in the




Fig. 48. — Left-sided facial hemi-spasm.



104



NERVOUS DISEASES




right frontal region, producing only a superficial abrasion. The
right eye, however, was bHnd for five days, and subsequently re-
mained closed for over a month by spasm of the orbicularis, the
right eyebrow standing at a lower level than the left. On
forcibly opening the lids, there was acute photophobia, accom-
panied by spasmodic contrac-
tion of the orbicularis. The
pupil was slightly larger than
on the left side, but reacted
normally to light, whilst the
external ocular movements were
normal.

In any variety of blepharo-
spasm, if we forcibly open the
lids, the vision is usually found
to be little affected, but photo-
phobia is usually intense. The
blepharospasm may persist for
periods varying from a few
hours to several months.
Fig. 49 shows a case of ulnar
spasm in a blacksmith who
sustained a severe jar of the left elbow when holding an iron
bar which was being hammered by a fellow workman. The
muscles of the left hand at once became weak. Five months
later, tonic spasm gradually set in, limited to the intrinsic
muscles of the hand suppHed by the ulnar nerve. From con-
traction of the interossei the fingers were tightly adducted,
whilst the hypothenar muscles produced opposition of the Little
finger. When examined a year after the accident, this tonic
spasm still persisted, and there was blunting of sensation to
cotton-wool and pin-pricks in the ulnar territory of the hand,
with loss of vibration-sense in the two ulnar fingers. Prolonged
massage and electrical treatment to the limb having failed to
produce improvement, the ulnar nerve at the elbow was injected
with normal saline solution containing a little cocaine. The
spasm was promptly relieved, and a week later no sensory or
motor abnormality could be detected.

But other cases occur, even of unilateral spasm, without any
reflex exciting cause or the history of one, and they are difficult
to classify. Thus a lady whose menopause occurred at the age of



Fig. -iHA.



INVOLUNTARY MOVEMENTS 105

forty-five, at the same time also lost most of her property through
the failure of a bank. She gradually developed clonic spasm of
the left facial muscles. At first, this consisted merely in a sUght
flickering of -the lower lid for a second or two, every few days,
but the condition gradually increased in severity until, when she
came under observation thirteen years later, the spasms affected
all the facial muscles on one side, beginning as a flickering move-
ment, and then becoming tonic and lasting from twenty to thirty
seconds at a time, the eye being closed, the eyebrow elevated, the
angle of the mouth drawn outwards, and the platysma thrown
into strong contraction. In the intervals between attacks the face




Fig. 49. — Ulnar spasm.

was symmetrical. Under treatment by bromides and galvanism
this patient rapidly became better.

Finally, there are numberless varieties of hysterical spasms
apart from the hysterical " fits," which have already been dis-
cussed. We can only refer to some of the commoner types.
Thus saltatory spasm consists of a series of jumping or skipping
movements, which occur whenever the patient assumes the erect
posture. A similar spasm, less severe in degree, may produce
paroxysms of trembling in the legs, as in a hysterical girl of nineteen
with many other stigmata of hysteria, in whom the spasms ceased
at once when she lay down. All sorts of movement, however,
may occur in hysteria, simulating almost any kind of tremor. For
instance, a hysterical woman of twenty-one had constant movements
of the face, left arm, and both legs, resembling those of athetosis
but very much faster. In her case typical hysterical contractures
and segmental anaesthesia of the hysterical type, together with the
normal state of the reflexes, aided in the diagnosis of hysteria,
which disease will be further considered in a subsequent chapter.



CHAPTER VII

APHASIA

We exchange ideas with our fellow-men chiefly by means of speech.
Speech is an arbitrary code of signals, vocal or written. These
signals are perceived by our auditory or visual centres. Every
country has its own particular code or language, which is learned
by each of its inhabitants. Gestures and mimic movements, as
a means of communication, although international, have a very
limited field of usefulness as compared with speech. Two indi-
viduals, each ignorant of the other's language, can certainly com-
municate with each other after a fashion by means of gestures
alone, yet they cannot express many ideas in this way, but
only simple primitive emotions such as pleasure, anger, surprise,
and so on, or pantomimic imitations of certain acts.

There are three chief classes of cases in which the functions
of articulate speech may be lost. Firstly, there are conditions in
which the patient's higher intellectual functions are in abeyance,
either congenitally as in idiots, or from disease as in acquired
dementia, coma, stupor, or in certain cases of hysteria. Such
patients are speechless, but they are not aphasics. Secondly, there
are the cases where the higher intellectual centres are capable
of function, but the cortical speech-centres which control the
motor acts of speaking and writing, or the sensory processes of
recognising spoken or written words, are diseased, and yet the
patient has not necessarily any paralysis of the peripheral organs of
speech, nor is he necessarily deaf or blind. To this group the term
" aphasia " is applied. Lastly, there are the cases where, with
intact intellectual functions and normal cortical speech-centres,
there are defects in the peripheral organs of articulation, so that
tha patient is unable to articulate distinctly — for example, cases
of cleft palate, post-diphtheritic palsy of the palate, facial or hypo-
glossal paralysis, bulbar paralysis, and so on. These are afiections,
not of speech proper, but of articulation.

Aphasia may be defined as impairment or loss of speech due

106



APHASIA



107



to the loss of memory for those signs, vocal or written, by means
of which we exchange ideas with our fellow-men. An aphasic,
unless his higher intellectual centres are impaired, usually pre-
serves his powers of gesture and of pantomime. Aphasia is due
to disease, organic or functional, of certain well-defined special
centres in or near the cortex of the brain. These cortical centres
exist on both sides of the brain, but ordinarily in right-handed
people the speech-centres on the left side of the brain are pre-
dominant.

Let us consider the speech-centres somewhat more in detail.
For the interchange of ideas two distinct processes are required —




Fig. 50. — Diagram of left cerebral hemisphere, showing approximate
positions of the centres concerned in speech.
A. Auditory word-centre. ) <,„,,.„.„
V. Visual word-centre. ^-seusory.

Sp. Vocal word-centre. ) \rnfnr

W. Motor centre for writing, j"^"''"'-

one sensory, the other motor. The sensory process includes the
hearing and understanding of spoken words, and also the seeing and
understanding of written or printed letters. The memories of
words heard and seen are stored up in specialised parts of the
auditory and Adsual centres, named respectively the auditory
word-centre and the visual word-centre (Fig. 50). The auditory
word-centre is at the upper or Sylvian surface of the temporal
lobe (anterior transverse gyrus of Heschl, Flechsig's " auditory
gyrus ") and in the adjacent posterior end of the first temporal
convolution ; the visual word-centre (in individuals who have
learned to read) is in the angular gyrus. Either centre may
be diseased ; so that we have two varieties of sensory aphasia,
viz. — auditory aphasia and visual aphasia. Then there is in



108 NERVOUS DISEASES

speech the motor element, consisting of the motor act of expressing
ourselves in words, either vocally or by means of writing. The
memories of these motor acts of vocal speech are usually sup-
posed to be stored up at the posterior end of the inferior frontal
(Broca's) convolution, and in the adjacent part of the pre-central
convolution and of the insula. If this centre be destroyed, motor
aphasia or aphemia results, the patient being unable to utter words
of which his motor memories have been destroyed.

Marie, however, has recorded cases of destruction of Broca's
convolution without any speech defect, and denies that it has any .
special importance in the mechanism of speech. He considers that
cases of so-called motor aphasia are really examples of ordinary
sensory aphasia combined with articulative difficulty (anarthria or
dysarthria) due to a lesion of the lenticular nucleus and its surrounding-
white matter ; and maintains that isolated lesions of Broca's convolu-
tion are accidental and of minor significance.

Earlier writers used also to describe a separate centre for
writing (independent of the vocal word-centre), a lesion of which
would produce loss of the faculty of writing — -agraphia. But no
case has been verified pathologically in which a focal lesion has
produced pure agraphia without affection of vocal speech, so that
the writing-centre, although it may be represented diagram-
matically in a theoretical scheme of cortical speech-centres, is
probably merely a part of the ordinary psycho-motor centre for
the upper extremity.

Fig. 51 is a scheme of the connection of the various centres
concerned in speech. Let us first notice that the motor vocal
word-centre is subservient to the auditory word-centre, and that
the writing-centre is similarly subservient to the visual word-
centre. A child first learns to speak by hearing spoken words
and then imitating them. Therefore speech is at first entirely
auditory in origin. Later, in learning to read, the meaning of each
word is learned by associating the letters seen with words heard
spoken, so that the auditory word-centre acts as the instructor of
the visual word-centre.

In most people, during the process of silent thought, words
are revived primarily in the auditory word-centre, and there is
usually a simultaneous revival of the same words in the visual
word-centre. But in other people the revival in the visual word-
centre comes to be of greater importance. Accordingly we may
classify people into " auditives " and " visuals " according to their



APHASIA



109



mode of revival of words in thought. Most of us are " auditives."
Simultaneous revival of word-images in several speech-centres
makes our comprehension of the idea more perfect. Thus a
difficult concept is better understood if we read it aloud, because
this involves the activity of the visual, auditory and vocal
word-centres.

Aphasia commonly results from organic disease of one or more
of the cortical speech-centres, or of the sub-cortical fibres connect-
ing them. The most frequent organic causes are embolism, hsemor-
rhage, or thrombosis, cerebral abscesses and cerebral tumours. The




FlG. 51. — Diagram of Speech-Centres (after Bramwell).

A. Auditory word-centre. V. Visual word-centre.

Sp. Motor vocal word-centre. W. Motor centre for writing.
HV. Half-vision centre.

The interrupted lines indicate possible but less habitual routes for transmission of impulses.

differential diagnosis between these various conditions depends
largely on the history ; embolism producing the symptoms suddenly,
haemorrhage taking several minutes, thrombosis taking perhaps
hours, abscesses being more gradual in onset and tumours still
more so. But we also meet with cases of temporary or functional
aphasia, sometimes from mere debility or exhaustion, sometimes
from localised vascular spasm, sometimes following a '' congestive
attack " in general paralysis or an epileptic fit, or accompanying
a paroxysm of migraine, or an attack of uraemia.

In investigating a case of aphasia we should first note whether
the patient has other signs of gross cerebral lesion, such as
hemianopia, or hemiplegia, and should inquire whether he is
naturally right- or left-handed. Most children are taught to write



110 NERVOUS DISEASES

witli the right hand, whether they are right-handed or not, and
therefore in determining this point we inquire with which hand
a man draws a cork, throws a stone, &c. ; or if a woman, with
which hand she combs her hair or threads a needle ; or, in either
sex, which hand is used in cutting bread.

The following series of inquiries (based on Beevor's scheme)
should then be made. The capital letters in parenthesis indicate
the parts of the brain involved in each case.

1. Can the patient spontaneously utter intelligible words ?
(Sp.) Note the extent of his vocabulary. Can he pronounce all
words or only a few ? Get the patient to talk spontaneously, and
observe whether he talks fluently or misplaces words or syllables,
whether he talks in disjointed phrases (" telegraphic " type of
speech), or whether he talks unintelligible jargon.

2. Can he understand words which he hears ? (A.) Ask him
to touch his nose, ear, eye, chin, &c., in turn, thus testing his
interpretation of nouns. Then ask him to smile, whistle, shut
his eyes, &c., thus testing his comprehension of verbs. Some-
times we find that the patient executes the first command correctly,
but continues to repeat the same act in response to different
commands. A patient can sometimes sing the words and air of
a song, when he is unabb to repeat the words in a speaking voice.

3. Can he understand written questions or commands which
he sees ? (V.) Write down and show him simple sentences, such
as " How old are you ? " " Put out your tongue." " Give me
your left hand."

4. Can he write spontaneously ? (W-) If his right hand is
paralysed, let him try with the left. Observe whether he writes
intelligibly, whether he misplaces words or syllables, or whether he
scribbles meaningless signs.

5. Can he copy from printed to written letters ? (V^W.)
Print some word such as " Hospital " or " Monday," and get him
to copy this.

6. Can he write to dictation words which he hears ? (A^V-^W.)

7. Can he pick out objects of which he hears the name ?
(A-*V.) Place in front of him a heap of objects, such as a key,
a shilling, a match, a pencil, and ask him to pick out each in turn.

8. Can he repeat words heard ? (A-*Sp.) Try him first with
simple words and phrases ; e.g. " cat," " dog," " nurse," " good-
morning," &c.



APHASIA 11]

9. Can he name objects seen, and can he read aloud from words
shown to him ? (V^A-^Sp) Point to different objects and ask
him what they are.

10. Does he understand gestures and pantomimic movements ?
Without speaking to him, get him to imitate you when touching
the nose, spreading out the fingers, protruding the tongue, &c.

Auditory Aphasia, or Word-Deafness. — The patient in this
case is not deaf, but simply word-deaf. He hears ordinary sounds
and noises, but spoken words are not understood ; they sound to
him like an unknown tongue. The character of the symptoms
varies according as the lesion is subcortical or cortical in position,

(a) Subcortical, or Pure Auditory Aphasia.— This is
extremely rare (Marie, in fact, denies its existence). Here the
lesion simply blocks the way-in for spoken words. The patient
therefore has word-deafness — i.e. he is unable to understand what
is said to him ; he is also unable to repeat spoken words or to write
from dictation. But the auditory word-centre being still intact, he
possesses all his memories of auditory speech, and therefore
spontaneous speech is perfect. Moreover, the visual word-centre
being in normal working order, he is still able to read, and, as
a matter of fact, reading is his only means of receiving messages
from other people.

(6) Cortical Word-Deafness. — This is much commoner than
the other variety. Here the lesion involves the cortical centre itself,
and the auditory memories of spoken words are obhterated. And
therefore, in addition to the previous defects of word-deafness with
inability to repeat spoken words or to write from dictation, there
are other symptoms due to the fact that the motor speech-centre is
no longer controlled by the auditory word-centre. Internal speech
and thought are impaired, and so the patient makes mistakes
whether in speaking spontaneously or in reading aloud. He also
makes mistakes in writing, especially in spelling. He talks fluently
enough, it is true, but he tends to mix up "his words or syllables,
and in a severe case may jabber unintelligible jargon. Word-
deafness renders the patient unaware of his own errors. This, as
we shall see, is in marked contrast with motor aphasia, where the
patient recognises his own mistakes as soon as he has uttered
them. If the lesion of the auditory word-centre be incomplete,
the word-deafness and resulting errors of speech are also partial.



112 NEEVOUS DISEASES

These latter may, in a slight case, be confined to inability to name
objects, i.e. nouns, the patient being still able to express abstract
ideas. Thus a partially word-deaf patient, who is unable to name
a knife shown him, may say, " It is for cutting." Or again, partial
word-deafness may produce simply confusion of words ; the patient
may say one word when he means another (par -aphasia). It rarely
happens that word-deafness remains permanent and complete ; the
auditory word-centre in the opposite hemisphere generally com-
pensates, to some extent, as time goes on.

The extent of mental disturbance in word-deafness varies accord-
ing to whether the patient be a strong " auditive " or a strong
"visual. ' In the latter case the mental impairment is much less than
in the former, and the disturbances of motor speech are but shght.

The auditory and visual word-centres are fairly close together,
and more than this, they are supplied by the same branch of the
middle cerebral artery (see Fig. 32, p. 50) ; so that it is not un-
common for a single arterial lesion, e.g. a thrombosis, to affect both
centres together and to produce a combination of word-deafness
and word-blindness.

Visual Aphasia, or Word-Blindness (Alexia). — In word-blind-
ness the patient can see, but cannot understand, printed or written
characters. They appear to him hke strange hieroglyphics. He sees



Online LibraryPurves StewartThe diagnosis of nervous diseases → online text (page 9 of 48)