grown for age. The nutrition of the affected side is good, but the
left side of the face is markedly smaller than the right, although
there is no paralysis. The position of the arm attracts immediate
attention. The arm, hand, and fingers are in extreme extension, as
shown in the cut, which is made from a photograph. The arm-muscles are
tense and rigid. The fingers are continually in motion, sometimes
flexed, sometimes extremely extended; then in a few moments they will
be widely separated or distorted in some other way. The favorite {459}
position, however, seems to be with the fingers extended until bent
backward, separated from each other, and the thumb adducted slightly.
The patient is unable to bring the arm down by a voluntary effort, but
when asked to put it by his side he pulls it down with the right hand,
and keeps it down by sitting upon the hand. The muscles of the arm are
hypertrophied, especially those of the upper arm and shoulder. The
circumference of the left arm around the biceps is almost an inch
greater than that of the right. The leg is stiff and the foot is
usually inverted. Here also the position changes at different times.
When he walks the stiffness increases and the foot is dragged. The
speech is clear and distinct, and the intellect perfect. He goes to
school, and is fully equal to or more advanced than boys of his age.
There is no evidence of cardiac disease.
[Illustration: FIG. 26. Case of Athetosis.]
The patient's condition in Oct., 1885, had changed somewhat. The arm
is in a different attitude. It is still extended, but is held down by
the side or away from the body. At times the forearm is strongly
supinated. There are still constant but slowly-changing movements of
the fingers. One plan which the patient has of keeping the arm flexed
is to put the forearm behind the back with the right hand, and it
becomes locked there by the action of the extensors. By a strong
effort of will he can slowly and with great difficulty open and shut
the fingers, and can flex the forearm. The leg is in the same
condition as before.
The resemblance between athetosis and post-paralytic chorea is very
close. Most of the cases of the former disorder which have been
reported have been of the hemiplegic type. In Case I. the disease came
on after an attack of delirium tremens, which was followed by six
weeks of unconsciousness. The first symptom the patient was capable of
noticing was numbness of the right arm and leg. The involuntary
movements came on later. This would look as if there had been some
lesion involving the left hemisphere of the brain. It is stated that
no paralysis existed when the case was examined by Hammond, but there
may have been a slight hemiplegia which had passed away. In another
case reported by the same author the peculiar movements were preceded
by hemiplegia and aphasia.
Oulmont has written a complete essay on athetosis, and has collected
therein all the literature of the subject.[3] He sustains the view of
the close connection between athetosis and post-paralytic chorea, but
he {460} considers that, although nearly allied, a difference does
exist between the two affections. He believes this is most marked in
the bilateral form of athetosis, for here the disorder is usually not
preceded by paralysis, and it is not accompanied by disturbances of
sensation.
[Footnote 3: _Étude clinique sur l'Athétose_, Paris, 1878.]
Gowers[4] has also collected a number of cases, some of which came
under his own observation, and has written a valuable treatise on the
subject.
[Footnote 4: _Medico-Chirurgical Transactions_, 2d Series, vol. xli.]
PATHOLOGY.—The symptoms point to a brain lesion, probably in the gray
matter, and a perverted condition of the nerve-cells which leads to
over-action, either spontaneously or under the influence of a motor
impulse. In most of the cases reported by Claye Shaw there was
imbecility. Charcot found in three post-mortem examinations lesions in
each instance in the posterior portion of the optic thalamus, the most
posterior part of the caudate nucleus, and the most posterior part of
the corona radiata. Gowers has made an autopsy in one case in which
there was post-hemiplegic inco-ordination affecting the arm only,
without the spasmodic fixation of the limb. In the brain was found but
one lesion, and that was a cicatricial induration of the optic
thalamus, extending across its centre beneath its upper surface, and
approaching at its outer part, but not involving, the ascending white
fibres of the crus. No secondary degeneration was found in the cord.
PROGNOSIS.—This is almost always unfavorable. In some cases the
inco-ordination decreases and the spasm becomes less violent, but it
seldom disappears altogether. One of Gowers's cases was benefited by
treatment, and two of those related by Hammond were relieved.
TREATMENT.—Many remedies have been given without marked benefit. In
Gowers's case there is no doubt that the decided improvement which
took place was due to galvanism. The treatment lasted three months,
and a descending galvanic current was used. The positive pole was put
upon the nucha, and the negative on the over-acting muscles and on the
hand and foot. One of Hammond's cases, as related above, was
apparently cured by stretching the median nerve. A prolonged course of
galvanism and some alterative, like mercury or chloride of gold and
sodium, would seem to offer the best prospect of benefit.
{461}
LOCAL CONVULSIVE DISORDERS.
BY ALLAN MCLANE HAMILTON, M.D.
Thomsen's Disease.
SYNONYMS.—Rigidité musculaire avec impuissance de la volonté; Rigidité
et hypertrophie musculaire; Myotomé congenitale.
In the year 1876, Thomsen,[1] the medical officer of Kappeln,
described a curious form of nervous disease which affected several
members of his own family, himself included. It consisted of a
tendency to cramp and limited spasm when a voluntary act was
attempted, and a seeming and sudden loss of power. This condition
disappeared after the performance of the act and its repetition. An
attempt at walking would be attended by spastic rigidity of the flexor
muscles of the lower extremities, by flexion of the thighs and legs at
an angle of 120°, so that the patient often fell forward upon his
knees. An attempt to arise from the chair was attended by the same
difficulty, the person becoming utterly helpless. The arms and legs
were most frequently affected, the trunk-muscles being usually exempt.
In some cases the muscles supplied by cranial nerves were the seat of
spasm, so that the patient could not close his mouth nor shut his
eyes. Westphal, Erb, Peters, Schönfeld, Engel, and others have
reported about eight cases in all, and in addition to the symptoms
above referred to it was found that mental excitement and cold chiefly
precipitated the seizure. The sphincters were never affected, and the
general nervous functions were not impaired. The patients talked
stiffly, and this was probably due to a spasmodic affection of the
lips, tongue, and other articulating organs. The tendinous reflexes
were normal, but irritation of the soles produced a peculiar cramp of
the leg-flexors and bending of the knees. There is a species of
muscular increase which resembles pseudo-hypertrophic paralysis.
[Footnote 1: _Berliner klin. Woch._, Mar. 12, 1883.]
The malady seems to be of an hereditary nature, if we are to judge by
Thomsen's cases. It begins early in life, and does not materially
shorten the same. I have seen one case, a young child, which presented
the main symptoms of, and was first mistaken for, pseudo-hypertrophic
paralysis.
Westphal regards the affection as an “anomaly of muscular tonus;”
others have shed no light upon its pathology.
I believe the condition to be an occasional feature of certain organic
disorders, notably posterior spinal sclerosis, and there is a variety
of paralysis agitans where there is no tremor which presents all the
symptoms. The very rare nature of the malady and its peculiar {462}
expression render diagnosis easy. It possibly may be mistaken for
pseudo-hypertrophic paralysis in young subjects, but in this latter
disease we find electrical change in the muscles, and an absence of
the tendon reflex. An examination of the enlarged muscles will not
reveal fatty increase.
Painless Facial Spasm.
There is a form of spasm of the muscles supplied by the facial nerves
which differs from ordinary tic douloureux by the fact that there is
no pain in the former. The trouble may be one of a very limited
nature, consisting of the involvement of a few fibres, or of limited
groups of muscles, or of all the muscles of the side of the face. I
never have seen a case of double spasm, though such undoubtedly exist.
The nature of the paroxysm is cumulative, and, as a rule, the attack
increases until it reaches an acme of intensity, then quite suddenly
ceases. It often begins by a slight drawing of the corner of the
mouth, the levator anguli oris and other muscles in the neighborhood
being the seat of chronic spasms. This limited spasmodic action is
followed by further facial contractions. The orbicularis palpebrarum
is often the sole seat of the convulsive movement, and this is common
in excessive smokers.
As a rule, the trouble is peripheral and due to some reflex cause,
such as cold, injury, bad teeth, which produces reflected irritation
through the branches of the fifth nerve, or in rare cases it may be
central or due to some bony or other pressure upon the nerve in its
passage through the skull. In a recent case the trouble was of evident
central origin, and I obtained a history of lightning pains in the
lower extremities, some inco-ordination of muscles in both upper and
lower extremities, and some paresis of the facial muscles. In this
case the spasms involved all the muscles of the right side of the
face, and recurred every few minutes. They had first appeared five or
six years before I saw the patient, and had gained in frequency from
two or three daily until within the past four months they occurred, as
I have said, every few minutes. The other symptoms were of
insignificant character compared to the spasms.
Of the large number of cases I have seen, many impressed me as being
the result of a simple bad habit, yet moral or other measures did not
avail much.
It is important to diagnose certain unusual forms of facial spasm from
the petit mal of epilepsy, and careful observation will detect a
transitory loss of consciousness in the latter. It is equally
important to find a cause if any exists, and a careful examination of
the state of the teeth, the integrity of vision, and the possible
existence of aural disease should be made in all cases. In some cases
over-use of the eyes, which may be defective in their power of
accommodation, may precipitate blepharospasm. In other cases the act
of masticating hard substances or taking very hot or cold fluids into
the mouth may give rise to the spasm.
Hyoscyamine in repeated doses of from 1/200 to 1/25 grain of Merck's
crystals does more in the way of relieving the spasms than any other
drug of which I know. If this does no good, gelsemium cautiously used
is often of great service. Local galvanic applications with currents
of great intensity will form a valuable adjuvant. So far, I have never
tried {463} nerve-section or stretching in these cases, but recommend
them as a dernier ressort.
Torticollis.
SYNONYMS.—Wry neck, Rheumatismus colli, Obstipite, Cephaloxia.
Wry neck or torticollis consists essentially of a spasm of the
sterno-cleido-mastoideus, though other muscles are nearly always
involved: the result is a peculiar and striking distortion which is
quite familiar. It consists in the drawing downward and backward of
the head on the affected side, while the chin is pointed forward and
upward to the other. The disease is presented in several forms. It may
be a temporary disorder as the result of a rheumatic condition,
disappearing rapidly, or occurs as an hysterical affection, or it may
be a chronic and intractable nervous disease. We must also consider it
from the standpoint of the form of distorted motility. In some cases
there is simple tonic contraction, which may eventually result in
tense contracture, shrinking, and tendinous hardness; and in others
the attention of the physician is attracted by a species of tremor and
agitation. Unlike the tremor of sclerosis, this is uninfluenced by the
attempts of control upon the part of the patient, but is aggravated by
fatigue and excitement. It rarely happens that both muscles are
affected so that the head is drawn backward. Most of the cases are
single, chronic, and progressive, and, though very slow in the onward
march, are usually beyond the reach of remedies. The patient becomes
greatly annoyed by his infirmity, and seeks every measure to overcome
his unfortunate deformity. He commonly tries to hold his head or chin,
pressing the latter downward, or, holding his cane against his head
upon the dependent side, strives to keep it up. As a result, there is
a sagging or drooping of the affected side of the body, so that one
shoulder is lower than the other.
The disease, as a rule, begins in adult life, yet there are many young
cases.[2] Wilks calls attention to the fact that in these latter there
is apt to be some facial asymmetry on the contracted side. One side of
the head is smaller than the other, and one eye seems to be lower.
[Footnote 2: _Diseases of the Nervous System_, p. 454.]
Most of the cases I have seen have been men, though I have met with
many hysterical examples in young girls. The double torticollis
(Newnham's salaam convulsion) usually affects children, and it is the
rule to find associated strabismus and intellectual disturbance. In
the adult cases there has usually been a history of hereditary
neurotic influence and overwork.
Electrically, we find a susceptibility to both currents, and the
reaction of degeneration may be detected in the affected muscles in
old cases. The opposing muscles are usually the seat of diminished
electrical excitability.
Torticollis may be due to peripheral or deep causes. Cold, reflex
irritation, forced and uncomfortable positions, are to be mentioned
among the former, and intracranial or vertebral disease as examples of
the latter. The pathological explanation lies in a disorder of the
motor fibres of the spinal accessory nerve. Any affection of the
external branch will result in the condition above described.
{464} In young cases the PROGNOSIS is good as a rule, though this is
by no means invariably so, for sometimes the special symptoms are but
forerunners of others of a more grave character. Hysterical
torticollis is often instantly, or at least very readily, cured by
electro-therapeutics and cauterization. A well-established adult case
is almost hopeless and resists all ordinary treatment.
Many forms of TREATMENT have been recommended from time to time, and
such drugs as the bromides, chloroform, conium, and hyoscyamine are
suggested. The latter I believe to be the most serviceable remedy.
Electricity has done good in either form, and vigorous faradization
with the electric brush is earnestly recommended in hysterical,
rheumatic, or functional cases. Some years ago I devised a method
which in a number of cases has been of great service. I allude to the
combined and simultaneous use of the galvanic and induced currents. A
double electrode is applied to the back of the neck. This contains the
anodal pole of the galvanic current and the cathodal of the faradic.
The two other poles are placed—one, the galvanic cathode, over the
origin of the affected muscle, the induced anodal pole over the
weakened muscle which is not in spasm. I suppose in old cases the most
valuable treatment is that of a surgical nature. I have twice seen the
spinal accessory exsected with the result of a complete cure; and I
think this is the only sure measure. H. B. Sands of this city has
performed the operation quite successfully in other instances. Care
should be taken not to operate in cases presenting other symptoms
which suggest the remotest suspicion of organic cerebral disease, and
the existence of unilaterally increased tendinous reflexes or tremor
should be carefully looked for as contraindications.
Braces and apparatus are often worse than useless, increasing not only
the patient's discomfort, but aggravating the malady.
Eclampsia.
(_Εχλαμψις_ and _λαμβω_, “I shine,” brilliancy, flashes of light from
the eyes.—Dunglison.)
DEFINITION.—A term used to express certain irregular convulsive
attacks, as a rule due to eccentric irritation, uræmic poisoning, or
like causes, and chiefly used in connection with the convulsions of
infants.
This term has gradually been dropped in scientific medical literature,
and many authors think it useless, believing the condition to be
usually epileptoid. Nothnagel,[3] who is more liberal than many other
clinicians, says: “What is there now remaining of what was formerly
eclampsia? Are we altogether justified in still retaining the name? We
believe so, and are of the opinion that the title eclampsia should be
reserved as the name of an independent affection, which, it is true,
can at present be defined only by its clinical symptoms. We propose
that the designation eclampsia should be made use of for those cases
of epileptiform spasms which, independent of positive organic
diseases, present themselves as an independent acute malady, and in
which, so far as our present knowledge allows us to judge, the same
processes arise generally in the way of reflex {465} excitements, and
the same mechanism in the establishment of the paroxysms comes into
play, as in the epileptic seizure itself.”
[Footnote 3: _Cyclopædia of the Practice of Med._, Von Ziemssen, Am.
ed., vol. xiv. pp. 301, 302.]
If any distinction at all be made, it should be one founded upon the
fact that epilepsy itself is usually an organic disease, or, more
precisely, a disease which when established is dependent upon some
disorganization, while eclampsia is used to express those seizures of
a purely functional nature.
By far the greater number of eclamptic seizures are found among young
children. There is no uniformity in their expression or return. Any
eccentric irritation is apt to precipitate one or more attacks, and
those forms of irritation dependent upon sensory disturbances of the
mucous membrane are commonly present. The convulsions of teething
belong to this class, as well as those in which gastric disorder plays
a part; and in the first instance the cutting of the large teeth, and
in the latter the presence of indigestion with diarrhœa, are quite
commonly associated with the convulsion.
The high degree of irritability of the nervous system of children
renders them peculiarly susceptible to causes which in after years
would effect little or no disturbance; and this is especially true
before the fifth year. West and Reynolds are of the opinion that
convulsions occur in children as delirium in adults; in other words,
they are the most common expression of neurotic instability.
Infantile convulsions are usually general, and neuro-spasms are not
common, except when they are dependent upon neoplasm, ventricular
dilatation, or local meningitis. It evidently takes very limited
peripheral excitement to precipitate a general convulsion in the
child, and a familiar example is the disturbance which may involve the
peripheral branches of the fifth nerve in difficult dentition.
Eclamptic seizures occur at any time, and may be very slight: a
trifling twitching may be all, or, on the other hand, the attack may
consist of violent opisthotonos. There is great difficulty, of course,
in getting anything from a young child as to its feelings before an
attack, and I hardly think we are authorized in saying that there is
any aura or ascertainable precursor. The history of a previous nervous
state is, however, usually ascertainable, which is expressed by crying
fits, peevishness, and great restlessness. Sometimes there is a
disposition to sleep which almost amounts to stupor. The behavior of a
child is often likened to that which marks the commencement of acute
tubercular meningitis—night-terrors, grinding of the teeth, and
flushing of the face. The first convulsions may be only partial, but
in a short time their character becomes general, and they become
exceedingly violent, and are sometimes fatal. The clinical features of
an eclamptic seizure may in every respect resemble one of epilepsy,
making allowance for the age of our patient.
The stage of pallor is perhaps more extended in the child.
Handfield Jones speaks of a form of eclampsia of hyperæmic causation
in association with certain exanthemata. In children convalescing from
scarlatina he observed the development of attacks which were relieved
by carotid pressure and bleeding in subjects who presented anasarca
and other indications of renal disease.
We are familiar enough with the convulsions of puerperal women, which,
as a rule, though not always, are presented by subjects whose {466}
urine is albuminous. It can sometimes distinctly be traced to what
must be regarded as uterine excitement. Just as chorea is often a
feature of the pregnant state, so may convulsive seizures arise. The
peculiarities of the epilepsy of pregnancy will, however, be elsewhere
considered.
I shall purposely refrain from the consideration of those forms of
symptomatic infantile convulsions which mark the occurrence of
cerebral accidents or grave disease.
{467}
EPILEPSY.
BY ALLAN MCLANE HAMILTON, M.D.
SYNONYMS.—Epilepsia; l'Épilepsie (_Fr._); Fallsücht (_Ger._);
Epilessia (_Ital._); Epilepsin fallendsot (_Scand._); Falling
sickness; Fainting sickness.
DEFINITION.—According to the most recent authorities, an epileptic
attack is nothing more nor less than a discharge of nervous energy
from an overexcited, or what may be called a dynamo-pregnant
nerve-centre, or collection of centres, and the predominance of motor
or sensory phenomena determines the extent and order of the parts
involved. As a rule, an epileptic paroxysm is but a symptomatic
expression of a complex derangement, and it is best to formulate our
nomenclature with the idea, in the first place, of location; in the
second, with reference to the prominence of motor or sensory
expressions; and, finally, with regard to etiology.