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Visual Anatomy and Physiology Lab

Manual Main Version 2nd Edition


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Visual Anatomy & Physiology Lab Manual, 2e (Sarikas)
Exercise 10 Introduction to Skeletal Muscle

10.1 Pre-lab Questions

1) What is the name for embryonic muscle cells?


A) myosins
B) myoblasts
C) myofibrils
D) myofilaments
Answer: B
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

2) Which of the following proteins is the main component of thick filaments?


A) myosin
B) sarcoplasm
C) actin
D) myofibril
Answer: A
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

3) What is the cell membrane of a muscle cell called?


A) sarcolemma
B) sarcomere
C) sarcoplasmic reticulum
D) sarcoplasm
Answer: A
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

4) Which structure stores calcium ions for release in the muscle cell?
A) sarcolemma
B) sarcomere
C) sarcoplasm
D) sarcoplasmic reticulum
Answer: D
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

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5) What structure of a sarcomere contains proteins that connect and stabilize thin filaments?
A) Z line
B) H band
C) I band
D) A band
Answer: A
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

6) What is the name of the area of the sarcolemma that is associated with the neuromuscular
junction?
A) junctional folds
B) synaptic terminal
C) synaptic cleft
D) motor end plate
Answer: D
Learning Outcome: 10.2
Bloom's Taxonomy: Remembering/Understanding

7) What is the "group name" for a motor nerve fiber and all the muscle fibers it stimulates?
A) motor end plate
B) myasthenia gravis
C) synaptic terminal
D) motor unit
Answer: D
Learning Outcome: 10.2
Bloom's Taxonomy: Remembering/Understanding

8) What ion enters the muscle cell upon activation of the acetylcholine receptors?
A) sodium
B) calcium
C) potassium
D) chloride
Answer: A
Learning Outcome: 10.2
Bloom's Taxonomy: Remembering/Understanding

9) Which of the following is a globular protein that binds to calcium?


A) tropomyosin
B) troponin
C) actin
D) myosin
Answer: B
Learning Outcome: 10.3
Bloom's Taxonomy: Remembering/Understanding

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Copyright © 2018 Pearson Education, Inc.
10) Which of the following is a double-stranded protein that blocks actin-binding sites?
A) myosin
B) DNA
C) tropomyosin
D) troponin
Answer: C
Learning Outcome: 10.3
Bloom's Taxonomy: Remembering/Understanding

10.2 Post-lab Questions

1) What is the anatomical name for the tube-like structures that are open to the surface of the
sarcolemma and form a portion of a triad?
A) cisternae
B) myofibril
C) sarcoplasmic reticulum
D) T-tubules
Answer: D
Learning Outcome: 10.1
Bloom's Taxonomy: Applying/Analyzing

2) What is the anatomical name for the combination of cisternae and a transverse tubule?
A) sarcoplasmic reticulum
B) sarcolemma
C) triad
D) Z line
Answer: C
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

3) Which of the following is both a protein and a structural component of thin filaments?
A) actin
B) myosin
C) myofibrils
D) T-tubules
Answer: A
Learning Outcome: 10.1
Bloom's Taxonomy: Applying/Analyzing

4) What structure contains proteins that stabilize the thin filaments?


A) Z line
B) M line
C) A band
D) I band
Answer: A
Learning Outcome: 10.1
Bloom's Taxonomy: Applying/Analyzing
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Copyright © 2018 Pearson Education, Inc.
5) What is the name for the region of the sarcomere that contains only thick filaments?
A) Z line
B) A band
C) H band
D) I band
Answer: C
Learning Outcome: 10.1
Bloom's Taxonomy: Remembering/Understanding

6) Which neurotransmitter is released at the neuromuscular junction?


A) sodium
B) acetylcholine
C) glutamate
D) acetylcholinesterase
Answer: B
Learning Outcome: 10.2
Bloom's Taxonomy: Remembering/Understanding

7) What would most likely happen if there were a decrease in the number of acetylcholine
receptors in an NMJ?
A) decreased neurotransmitter release into the synaptic cleft
B) increased sodium ion influx at the motor endplate
C) decreased action potential generation at the motor endplate
D) increased action potential generation at the motor endplate
Answer: C
Learning Outcome: 10.2
Bloom's Taxonomy: Applying/Analyzing

8) What happens when a patient is poisoned by botulism?


A) increased respiratory rate
B) blurry, double vision
C) constricted pupils
D) increased acetylcholine release
Answer: B
Learning Outcome: 10.3
Bloom's Taxonomy: Applying/Analyzing

9) What is it called when the myosin heads pivot and pull on the actin filaments?
A) power stroke
B) tropomyosin displacement
C) action potential
D) troponin
Answer: A
Learning Outcome: 10.3
Bloom's Taxonomy: Remembering/Understanding

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10) The release of calcium ions from the sarcoplasmic reticulum in response to an action
potential is known as ________.
A) power stroke
B) sliding filament theory
C) tropomyosin
D) excitation-contraction coupling
Answer: D
Learning Outcome: 10.3
Bloom's Taxonomy: Applying/Analyzing

5
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SYMPTOMS.—An attack of chorea is usually preceded by more or less
failure of the general health and evidences of some mental
disturbance. It is quite common to be told by the parents of a child
suffering from chorea that the little patient had seemed unwell for
some time previous to the attack; that the appetite had failed, and
that the child had looked pale; that he had been irritable or excitable,
and at school the teacher had complained of restlessness or
inattention in the pupil. In a little girl who was brought to me recently
with her second attack of chorea her mother stated that for several
days before the outbreak the child had been in excessive spirits, and
that she had been singing loudly and in a peculiar manner. The
same symptoms had preceded the first attack. Sometimes nothing is
observed until it is found by the parents or teacher that there are
abnormal twitchings and movements of the limbs.

At first there is a general restlessness and fidgetiness. The child may


be punished at school for not keeping still or for dropping things.
Soon irregular movements of groups of muscles are seen. The
shoulder is shrugged or the fingers move spasmodically. At first the
patient is aware of the movements and tries to control them, but
before long the twitching and jerking are constant, and extend to
most of the voluntary muscles of the body. He is then unable to
control them for any length of time.

The sudden jerk of a limb followed by an odd grimace, the quick


protrusion of the tongue, and the rolling of the eyes or snapping of
the lids give a characteristic picture which can hardly be mistaken.

The extent of the movements varies in different cases. In some they


are slight and affect only certain muscles. Often the disorder is
confined to one lateral half of the body. In other cases the
movements involve all the limbs and the trunk, and are so violent
and constant that the patient does not seem to have a moment's
rest. The trunk may be suddenly drawn backward, then the arms are
extended or thrown up, and the legs flexed and tossed about with
great quickness. Sometimes the patient is thrown off the bed or from
the chair on which he may be to the floor.
The speech is often affected. The patient speaks in a thick or jerky
manner, as if the tongue were too large for the mouth, and saliva
usually flows in great quantities. Sometimes in bad cases there are
involuntary utterances made at frequent intervals.

The features undergo contortions continually, and when at rest


relapse into a condition of vacancy which makes the patient look
almost idiotic. The expression of a child with chorea is so peculiar
that the disease may almost be diagnosticated by this.

During sleep the movements usually cease, but generally the patient
is restless while asleep, and in some instances the irregular
movements continue even at this time.

The mental condition commonly shows some change. The child is


irritable and peevish, cries and laughs readily, or is sullen and
morose. Sometimes he is violent to those about him, but this is rare.
Intellectually the patient suffers somewhat. He is not able to study as
before, and the memory may be impaired. Sometimes there is a mild
form of dementia.

During the course of the disease there may be exacerbations, and


sometimes after convalescence has seemed established there are
relapses.

Recovery is gradual, and as the abnormal movements cease the


mental condition improves, and the patient regains his health without
any traces of the disease remaining.

We will now consider some of the symptoms separately. First, as to


disturbances of motion. As before remarked, the disorderly
movements occur soon after the general restlessness is seen. They
most commonly begin in one upper extremity. The hand is thrown
into various positions, the fingers are flexed and extended or
separated, and all of the movements occur with great rapidity. In a
day or two the whole arm is affected, and then the leg of the same
side is involved in the jerkings and twitchings. In many cases the
facial muscles are contorted, the mouth is pursed up or opened
wide, and then quickly twisted into some other shape. If the patient is
told to put out his tongue, it is protruded after a moment's hesitation,
and then suddenly retracted, the jaws coming together with a snap.
A smacking sound is made with the lips quite often, and words are
uttered involuntarily. The movements may remain confined to one
side of the body, constituting what is called hemichorea. This is quite
common, and the right side is rather more frequently involved than
the left. There is so great difference of opinion among authors on this
point that it is probable that one side is affected about as often as the
other. Of 252 cases which I have examined, 69 were right and 43 left
hemichoreas. Gerhard20 found in 80 cases of chorea that 32 were
unilateral; of these 20 were right and 12 were left. Sée, however,
found that in 97 of 154 cases the movements were either confined to
the left side or were more marked on that side. He states that in his
experience the proportion between left and right hemichorea is as 37
to 27. Pye-Smith in 33 cases of unilateral chorea found 15 on the
right and 18 on the left side. Many cases which begin as hemichorea
soon become general.
20 American Journal of Med. Sci.

The disease reaches its greatest severity in about two weeks, and if
the case is a bad one we find by this time all of the voluntary
muscles are in constant movement. At this time the French name for
chorea, folie musculaire, is most appropriate. Patients are often
unable to walk or to sit up, and sometimes they may be thrown from
the bed by violent spasmodic movements of the trunk. Strange as it
seems, patients rarely complain of fatigue, notwithstanding the
violent muscular exercise. This is probably because each set of
movements is of short duration and is constantly changing its seat.

As a rule, the movements cease completely during sleep or under an


anæsthetic. Sometimes occasional twitchings of muscles are seen in
sleep, and in rare instances we are told by the parents of a child with
chorea that the movements are as active in sleep as in the waking
hours.
The movements of chorea occur either while the limbs are at rest or
under the influence of voluntary effort. This fact has been pointed out
by Mitchell and by Gowers. In some cases the movements are most
marked when the patient is at rest. If a directed effort is made to use
the member for a time, the choreic movements are suspended. For
example, a patient may be able to carry a glass of water to the
mouth without spilling a drop, while a moment before the hand may
have been performing a continual dance. I have often observed that
while the limb to which the whole attention has been directed in
performing some movement has been steadied, the other limbs
become violently agitated.

In another class of cases the movements are comparatively slight


when the part is at rest, but when a motion is attempted the disorder
of the muscles is so much increased that it is almost impossible for
the act to be completed. The patient is told to pick up some small
object: he throws the hand out toward it, and it is jerked away before
he can grasp it. He again puts the hand forward, reaches the object,
and the fingers open and shut and sprawl over the article before it is
taken up. Sometimes it cannot be grasped at all. This has been
called choreic ataxia, but it is only one type of the cases commonly
seen.

This brings us to the influence of the will on the movements in


chorea. There are some cases, as mentioned above, in which the
movements may be controlled by the will for a brief period, but they
will sooner or later return. In other cases it is quite impossible for the
patient to check the movements at all, and one frequently sees in a
case of hemichorea the sound hand used to grasp the other, so as to
control the movements. We have referred to this because of
Niemeyer's opinion that corporeal punishment would shorten an
attack of chorea.

Chorea is sometimes confined to a single muscle or group of


muscles. When limited in this way it is generally in the head, face, or
perhaps in the shoulder. These cases of localized chorea have been
spoken of by Mitchell as habit chorea.21 They are often very
obstinate in resisting treatment, and sometimes last during life.
21 Lectures on Nervous Diseases, p. 146.

PARALYSIS.—Not infrequently in chorea there is paralysis to a greater


or less extent. It is generally one-sided, and most often involves the
upper extremity. The limb affected is the one in which the
movements were most violent. The arm may hang entirely powerless
or it may be only enfeebled, and feeling to the patient like a dead
weight. The paralysis always recovers with the chorea or soon after.

POST-PARALYTIC CHOREA.—Under this term Mitchell and Charcot have


described a variety of chorea which is seen in patients after an
attack of hemiplegia. The movements are chiefly on voluntary effort,
and are those of inco-ordination. They come on from one to several
months after an attack of unilateral paralysis, and are sometimes
seen in cases in which almost complete recovery has taken place.
Mitchell has reported22 a case which was under my care for several
years, and which he saw in consultation with me. This patient had
two attacks of left hemiplegia, the last being fatal. After the first
attack there was great gain of power to use the arm and leg, but the
movements were performed awkwardly and with an irregular jerking
movement. A post-mortem examination revealed a spot of softening
the size of a filbert in the left corpus striatum, which was apparently
recent, and a point of red degeneration in the right crus cerebri. The
vessels at the base of the brain were extensively atheromatous.
22 American Journal of the Med. Sci.

Of the electrical condition of the muscles in chorea but little is known.


Rosenthal23 found increase of faradic contractility in three cases of
hemichorea, and the galvanic test showed a high degree of
excitability, demonstrated by the fact that weak currents gave
contractions at cathodic closure, or even tetanic contractions, and
also contractions were produced at cathodic opening.
23 Ziemssen's Cyclopædia, loc. cit., p. 434.
The affection of speech which is so common in chorea is due to
disordered action of the laryngeal muscles, or it may be from choreic
action of the abdominal muscles. Sometimes it is chiefly from the
awkwardness of the tongue. The usual form of trouble is that the
patient speaks in a staccato manner and the syllables seem as if
they were driven out. When the chorea is in the laryngeal muscles,
the tone and pitch of the voice are altered.

Chorea of the heart is sometimes spoken of, but it has never been
satisfactorily demonstrated that there is any real disorder of cardiac
rhythm in chorea. It is not unusual in chorea to meet with over-action
or palpitation of the heart, but these conditions do not necessarily
depend on the disease.

Valvular murmurs are often met with from the beginning of an attack.
In some instances they are the result of an endocarditis, but
frequently they are functional or anæmic. They are usually heard at
the apex. Sometimes there is a reduplication of the first sound,
giving the idea of a want of synchronism in action of the two sides of
the heart; but this is probably not the result of chorea of the heart. I
recall one patient, a child of seven or eight years, in whom the
reduplication of the first sound was very distinct during an attack of
St. Vitus's dance. She was brought to me at the beginning of a
second attack a year later, and the reduplication of the cardiac
sounds was heard again, so it is likely that it had continued during
the interval, and was probably a congenital condition.

The pupils are commonly dilated in chorea and respond sluggishly to


light.

REFLEXES.—I have examined the condition of the patellar reflex in 50


cases. In 26 of these it was present in normal degree, in 15 it was
diminished, and in 9 it could not be excited. In one patient it was
absent during the height of the choreic movements, but could be
readily produced after the patient had recovered. The condition of
the reflexes has also been examined by Joffroy and Saric,24 and they
found that of 16 cases of chorea the reflexes were abolished or
diminished in 12.
24 L'Union médicale, Sept. 22, 1885.

SENSIBILITY.—Authors state that disorders of sensation are met with


in chorea, such as localized anæsthesia or a general hyperæsthesia:
I have never met with any such instances. Patients often complain of
pain in the joints or in the limbs, and this may be unaccompanied
with swelling or tenderness on pressure. Tenderness on pressure
over the vertebræ is rare in my experience, although others speak of
its being of frequent occurrence. Mental disorders are generally
present, but only to a slight extent. There is almost always irritability
of temper and peevishness. The most sweet-tempered children
become cross and perverse, laugh immoderately at trifling things, or
cry as readily if they are annoyed. There is generally failure of
memory and incapacity for study or thought. In most cases, however,
this exists to so slight an extent as not to be noticed except on very
close observation. Sometimes there is marked mental disorder
amounting almost to imbecility, and occasionally the mental
weakness remains for some time after the motor disorders have
recovered.

The condition of the pulse is generally unchanged, but sometimes it


is abnormally frequent. The temperature, according to Von
Ziemssen, is unchanged.

The nutrition generally suffers. The patient rapidly loses flesh, and
becomes anæmic; the skin grows dry, and the hair gets harsh. The
digestion is apt to be disordered. The tongue is large, pallid, and
coated thickly, and there is sometimes nausea or vomiting. The
appetite is not good. The bowels are often constipated. The urine
has been examined by several observers. Bence Jones found an
excess of urea at the height of the disease. Albumen is not present
except accidentally, but there is usually an excess of phosphates. In
several cases in which we have examined the urine at the Infirmary
for Nervous Diseases we found that the specific gravity was high
while the chorea was at its height, but fell to normal as the patient
recovered.
Chorea is spoken of as acute and chronic, but all cases are more or
less chronic. Those cases which last eight or ten weeks may be
considered acute, while those running on for months or years are
properly called chronic.

DURATION.—Considerable difference of opinion exists as to the


duration of chorea. Some writers speak of three or four weeks as an
average attack. Gray and Tuckwell, in a series of cases treated by
the expectant plan,25 found an average duration of ten weeks.
Occasionally a patient is seen with an attack of chorea which lasts
only a few days. The parents of a little patient whom I saw a few
days ago assured me that her second attack lasted only a week.
They are educated and intelligent persons whose statement can be
relied upon.
25 Lancet, Nov. 28, 1876.

The course is not always regular. In some cases the disease


gradually reaches a crisis, remains stationary for a few days, and
then by degrees declines; in others there are exacerbations. The
patient will seem to be almost well, and then become very much
worse for a time. Relapses are not infrequent, and are generally
caused by fright or excitement.

The recurrence of attacks of chorea is well known. A child who has


had the disease one year may have it a second or third year. It is
most likely to recur in the spring. Some cases have as many as five
attacks, but as puberty approaches the attacks are lighter, and finally
cease. Of 282 cases to which I have referred, 198 were first attacks,
47 had had chorea twice; 23 were in their third attack, 8 in the fourth,
and 3 in the fifth attack.

TERMINATION.—The disease in most instances terminates in complete


cure, but sometimes there is nervousness or want of co-ordination
remaining for a time. Rarely the inco-ordination or a certain
quickness in movement becomes permanent.
Death is a rare termination of chorea except in pregnancy. If it does
occur, it is usually from some complication. In pregnancy the
mortality is great. Of 64 cases collected by Wenzel, 18 died. In
Philadelphia, in seventy-four years from 1807 to 1881, there have
been but 64 deaths from chorea; of these, 38 were under twenty
years, and 26 over that age.

Hutchinson reports a fatal case in a boy of twelve years.26 After


complaining of headache and rheumatic pains for several days,
choreic movements began. They soon became general and very
violent. At the end of two weeks he was admitted to the
Pennsylvania Hospital. At this time the patient was so extremely
convulsed that it was impossible to keep him in bed without tying
him. The movements continued but little abated, and the child died in
two days.
26 Philadelphia Med. Times, vol. vi. p. 535.

Another case of unusual interest is reported by Hunt.27 The patient, a


man of twenty-nine years, had suffered from chorea of the face and
arms for years. In consequence of a fall on the pavement he
fractured the left humerus. The movements were immediately
exaggerated, and in spite of a carefully adjusted splint it was
impossible to keep the arm at rest. The fragments were in a state of
constant movement, and the points of bone threatened to penetrate
the skin. The skin was so much excoriated that it was determined to
dispense with the splint and attempt to keep the limb at rest by the
administration of morphia hypodermically in half-grain doses three
times daily. This failed to keep the arm quiet, and the seat of fracture
became greatly inflamed. No form of appliance or medication
succeeded in keeping the arm at rest, and the patient finally sank
and died from exhaustion on the tenth day after admission to the
hospital. The post-mortem examination revealed no gross lesion of
the brain or cord. No microscopic examination was made of the
brain.
27 Pennsylvania Hospital Reports, vol. ii.
MORBID ANATOMY AND PATHOLOGY.—In a disease so seldom fatal as
chorea it is not surprising that there have been but few post-mortem
examinations made. In the earlier autopsies, before the microscope
was extensively used, but little of value was recorded. Sée, who
collected 84 cases in which post-mortem examinations were made,
reported that in 16 no changes were found in the nervous system. In
32 there were lesions in the brain and nervous centres, usually
softening and tuberculosis, and in the remainder inflammatory
changes in the serous membranes. In 29 there were evidences of
heart disease. Sée considered that but few cases of death in chorea
were caused by inflammatory diseases of the heart, but that the
majority should be referred to nervous excitement and anæmia.

Ogle28 in a report of 96 cases of chorea mentions 16 which were


fatal. Post-mortem examinations were made in all of these. Cardiac
lesions were found in 13. In 10 of these deposits were found upon
the valves, and in 3 there was some change in the pericardium. He
speaks of having noted congestion of the nervous centres six times,
and softening of the cord once.
28 Brit. and For. Med.-Chir. Review, Jan., 1868.

In all of 11 autopsies reported by Pye-Smith29 there were cardiac


lesions found. In every case old or recent deposits were observed
upon the valves. In two instances the heart was hypertrophied, and
in one there was pericarditis. Changes in the nervous system were
less often found by this writer. In 1 case there was hyperæmia of the
cord, and in 3 cerebral hyperæmia.
29 Guy's Hospital Reports, 1874.

Dickinson found in 22 fatal cases of chorea 17 in which the heart


was diseased. “In every instance making up the large tale of cardiac
disease there were recent vegetations on the mitral valve, and often
also elsewhere.”

In the fatal case of Hutchinson referred to above the heart was found
diseased, the aortic valves were incompetent, the leaflets being
swollen and softened, and the aorta was atheromatous above the
sinus of Valsalva.

Of late years a number of careful autopsies have been made in


cases of chorea. The brain and spinal cord have been closely
examined, and in almost every instance some lesion has been found
in both of these organs.

Steiner reported in 1868 the results of post-mortem examinations in


3 fatal cases of chorea. In 1 case he found cerebro-spinal anæmia,
serous effusion into the spinal canal, and proliferation of the
connective tissue in the upper part of the cord; and in another
hyperæmia of the brain and cord.

Elischer,30 who reports a fatal case in a parturient woman who had


an attack of chorea in her eighth year, two in her sixteenth year, and
another in a previous pregnancy, found at the autopsy hyperæmia
and œdema of the brain and gray substance of the cord.
Microscopically, the brain showed fatty, amyloid, and pigmentary
changes in the nerve-elements and vessels of the large central
ganglia, small secondary extravasations of blood in the connective
tissue, and numerous emboli in the smallest vessels, especially in
the cortex. In the spinal cord there was seen abundant proliferation
of nuclei in the adventitia of the vessels. In the central canal serum
was found, and the surrounding connective tissue was harder than
usual.
30 Cyclopædia of the Practice of Medicine, Von Ziemssen, vol. xiv. p. 450.

Dickinson has contributed an excellent paper on the pathology of


chorea.31 He relates the particulars of the autopsies in 7 fatal cases
in which he personally made microscopical examination of the brain
and spinal cord. He also adds the results of post-mortem
examinations in 17 other cases at St. George's Hospital and at the
Hospital for Sick Children. In all of the 7 cases in which microscopic
examinations of the brain and cord were made there were found
hyperæmia of both of these structures, in many instances
hemorrhages into the substance of the nervous tissues, dilatation of
the smaller vessels, and in chronic cases sclerotic changes in the
course of the vessels. “The first visible change,” he remarks, “would
seem to be the injection or distension of the arteries, succeeded by
extrusion of their contents, to the irritation and injury of the
surrounding tissue.” The changes seemed to affect both brain and
cord in all cases. The parts of the brain most constantly affected lay
between the base and the floor of the lateral ventricles in the track of
the middle cerebral arteries, the substantia perforata, the corpora
striata, and the beginning of the Sylvian fissures. “Of the cord no
region was exempt, but perhaps the cervical and dorsal regions were
usually more affected than the lumbar. With regard to the vertical or
physiological divisions of the cord, these all, whether white or gray,
shared in the vascular destruction; this condition, however, was
usually most marked in the vessels belonging to or in connection
with the lateral part of the gray matter about the root of each
posterior horn. And it is to be observed that this was also the chosen
situation of the more definite and special changes, whether
hemorrhagic (as in two instances), sclerose, or exudatory. Speaking
generally, the chosen seats of the choreic changes are the parts of
the brain which lie between the beginning of the middle cerebral
arteries and the corpora striata—the parta perforata; and in the cord
the central portion of each lateral mass of gray matter comprising the
root of each posterior horn.”
31 Medico-Chirurgical Transactions, vol. xli. p. 1, 1876.

The embolic theory of chorea has been held by several investigators,


among them Hughlings-Jackson. It is undoubtedly an attractive and
reasonable view, especially when we consider the large proportion of
cases in which there is valvular disease of the heart. Dickinson,
however, does not consider this hypothesis tenable. In none of the
cases in which he made post-mortem examinations did he find
evidences of embolism. “In none of the instances described were
decolorized fibrin, detached clots, or signs of impaction detected,
and the erraticism of embolic accident was wanting: the constancy
indeed with which the changes repeated themselves in certain
positions, and the equality with which they affected both sides of the
body, are conclusive objections to this hypothesis. The corpora
striata, for example, were affected with almost absolute symmetry,
notwithstanding that these bodies receive their blood respectively
from the right and left carotids and different parts of the aortic arch.”

Rheumatism is associated with or precedes chorea in a large


proportion of cases, and this was pointed out by Kirkes in 1850 and
again in 1863. This connection between rheumatism and chorea,
and the frequent occurrence of endocarditis in chorea, has led some
authors to believe that the endocarditis is always rheumatic, and that
the chorea is the result of the endocarditis. Dickinson, however,
points out that in cases in which there is a distinct history of the
chorea beginning suddenly from fright there are often well-marked
cardiac murmurs heard. He believes that in all cases of chorea in
which there are cardiac murmurs they are due to endocarditis, and
suggests that in these cases from fright the endocarditis is due to
irregularity of cardiac action. This, of course, is mere hypothesis, and
we must bear in mind that in all cases of chorea there is anæmia,
and that the murmur may be purely functional.

H. C. Wood, in a communication read before the College of


Physicians of Philadelphia,32 gives his views of the pathology of
chorea, based upon the results of post-mortem examinations made
in a number of dogs who had the disease. He believes the history of
chorea to be this: “Owing to emotional disturbance, sometimes
stopping of various vessels of the brain, or sometimes the presence
of organic disease, there is an altered condition of the ganglionic
cells throughout the nerve-centres. If the cause is removed and the
altered condition of the nerve-cells goes only so far, it remains what
we call a functional disease. If it goes so far that the cells show
alteration, we have an organic disease of the nervous system.”
32 Philada. Med. News, May 30, 1885.

In two dogs which were choreic the movements continued after


section of the cord. This shows that in dogs, at any rate, the
movements originate in the cord. In four instances of canine chorea
in which Wool made autopsies there were found in the cords of three
mild grades of infiltration of leucocytes in the gray matter. In the
fourth, in which the dog had died of the disease, the ganglion-cells
were degenerated, and in some places had disappeared. He
concludes, therefore, that choreic movements may depend upon a
diseased condition of the motor cells of the cord.

Although there are several recorded cases of human chorea in which


lesions of the spinal ganglionic cells have been found, we cannot
believe that this can be a constant lesion in chorea. The disease is
too transient in many cases, and presents too many variations and
anomalies, for the cord to be always the seat of the diseased
condition.

In an interesting paper read by Angel Money before the London


Medical and Chirurgical Society in 1885 he detailed some
experiments in which, by injecting a fluid containing arrowroot,
starch-granules, or carmine into the carotids of animals, he produced
movements closely resembling chorea; and this was found to be
associated always with embolism of the capillaries of the cord. In the
discussion which followed Broadbent and Sturges expressed their
disbelief in the embolic origin of chorea in man. Hughlings-Jackson
said that he held the view of the cerebral origin of chorea, one of his
reasons being the frequency with which the face-muscles are
affected in this disease.

The probabilities are that in chorea there is a disordered condition of


the brain and cord more or less general. The lesions are no doubt
slight in mild cases of short duration, but in severe cases of long
standing there occur well-marked changes in portions of both brain
and cord. We cannot do better than to sum up the pathology of
chorea in the words of Dickinson: “A widely-distributed hyperæmia of
the nervous centres, not due to any mechanical mischance, but
produced by causes mainly of two kinds—one a morbid, probably a
humoral, influence which may affect the nervous centres as it affects
other organs and tissues; the other, irritation in some mode, usually
mental, but sometimes what is called reflex, which especially
belongs to and disturbs the nervous system, and affects persons
differently according to the inherent mobility of their nature.”

DIAGNOSIS.—The only diseases for which chorea may be mistaken


are paralysis agitans and disseminated sclerosis. The former occurs
only in adult life, and the tremor is of a regular rhythmical character.
In the latter the tremor occurs only on voluntary effort, and is also
more regular than the movements of chorea. There are forms of
congenital sclerosis seen in children which closely resemble chorea.
Here the duration of the disease and the association of contractures
with it distinguish it from chorea.

Hysterical subjects have a form of chorea which can only be


differentiated from the true disorder by noting the general hysterical
character of the case and the result of treatment, which strongly
influences the will-power of the patient.

PROGNOSIS.—In the great majority of cases this is favorable. If the


disease occurs in childhood and is without complications, recovery
generally takes place spontaneously after a few weeks. Should the
movements be violent and continuous, so as to interfere with sleep
and the taking of food, or should there be any complication, such as
acute rheumatism or cardiac disorder, then the prospect of recovery
is not so good.

The prognosis as to relapses should be given with caution. If in a


child, it is possible that there will be a return of the disease after a
longer or shorter interval. It is not likely to recur until after several
months, usually at about the same season the following year. As the
child grows older the intervals become longer, and it may safely be
asserted that after puberty is passed and bodily development
completed there will be no more returns of the affection.

The cure is usually complete. It must be remembered, however, that


for some time after an apparently complete cure there may be slight
inco-ordination of movements, particularly in the arms and the face.
These are shown in the unnecessary haste in making uncertain
motions or in slight grimaces, or if excited an awkwardness in the
use of the fingers.

Death is a rare termination in uncomplicated cases, especially in


children. The fatal cases are generally when acute rheumatism has
been associated with the chorea or when there has been a fracture
or an injury as a complication.

In Sée's statistics there is a mortality of 5.7 per cent. in 158 cases in


the Children's Hospital. In adults, and more particularly in pregnant
women, death is more common. Wenzel's cases referred to above
gave a mortality of 27.3 per cent.

The cause of death in chorea may be from the intensity of the


disease, and in this case the symptoms are generally violent from
the outset, increase to an extreme extent, and then collapse and
coma come on. The movements may cease when the collapse
occurs, but they may continue to the last, growing gradually less until
death.

TREATMENT.—A vast number of remedies have been popular in this


disease from all ages. The medicine which is most generally
depended upon at the present day is arsenic. It is advised by most
writers, and in my own experience is decidedly the most reliable
remedy for chorea which we know. The best way to administer it is in
the form of Fowler's solution, and it should be given in large doses. I
have given the bromide of arsenic, but did not find it superior to
Fowler's solution. The amount of arsenic which can be safely borne
by children with chorea is surprising to those who have not had
experience in its administration. The medicine should be given in
gradually increasing doses until the toxic effects are well marked or
until the patient is convalescing. In a child of six years three drops
may be given to begin with, three times a day. One drop additional
should be added to the dose each day, and the child soon acquires a
remarkable tolerance of the drug. As much as twelve or fifteen drops
at a dose is borne by a child of eight years. If vomiting or much
œdema of the face occurs, the medicine should be stopped for a day
or two, and then the original dose should be taken, to be again
increased as before.

Seguin recommends that the patient should begin again with the
dose at which tolerance ceased. For instance, if vomiting occurred
after a dose of nine drops, he stops the medicine for a day, and
begins again with eight drops. I have found that sometimes this
causes vomiting again, and I think it preferable to resume the
medicine with a small dose.

It is often seen that a patient becomes worse during the first few
days that the arsenic is taken, but improvement generally begins
after a week of the arsenical treatment, and is well marked after two
weeks.

In obstinate cases it is of marked advantage to give the arsenic


hypodermically. Cases which do not yield to the drug when given by
the mouth often improve at once when it is given hypodermically.
Chronic cases which have resisted all forms of medication
sometimes are cured by hypodermic injections of arsenic. For giving
arsenic in this way it is best to use Fowler's solution, made without
the compound spirit of lavender. It is less likely to cause abscess to
form at the point of puncture.

Other remedies enjoy a reputation in the treatment of chorea.


Sulphate of zinc is relied upon by many, and it is the means which
Ross recommends. It should be given in increasing doses like
arsenic, and very large doses may often be taken without disturbing
the stomach. Trousseau, Hammond, and Hamilton favor strychnia,
but I have had no experience in its use.

Cimicifuga and conium are both often beneficial in their effects. I


have seen the former do good when arsenic had failed. Conium to
be efficacious must be given in large doses. Eserine and
hyoscyamine have both been successfully employed, the former by
Bouchut, and the latter by Oulmont and Laurent. Recently,
DaCosta33 has reported, in a clinical lecture at the Pennsylvania
Hospital, a case of very severe chorea successfully treated with
hyoscyamine. The patient was a boy of eleven years, and the
disorder had followed an attack of acute rheumatism. He was given
1/100 gr. of hyoscyamine three times a day.

33 Philada. Med. Times, Jan. 23, 1886.

Ziegler34 has recorded several cases which recovered under the use
of nitrite of amyl. The bromides and chloral are useful adjuncts to
treatment in case of sleeplessness or mental irritability. Cases of
cure by the use of chloral alone have been reported. Bouchut gave a
girl of fourteen and a half years, with chorea and dementia, 45 grains
of chloral a day for twenty-seven days. She slept most of the time,
but improvement was seen on the fifth day, and cure was completed
on the twenty-eighth day of the use of the chloral. Electricity has
been efficient in the hands of many writers. I have found
galvanization of the spine to produce a quieting effect in some
cases.
34 Ibid., vol. vi. p. 486.

Iron is always of use in chorea; it may be given during the course of


the disease, and is generally necessary in convalescence. Cod-liver
oil or malt extract should be given in feeble persons.

It is scarcely necessary to mention the other remedies which have


been recommended. DaCosta has used the bromide of iron. H. C.
Wood has used a preparation of skunk cabbage, and there are a
great number of other remedies which have been found of value.

Next to the internal means come external applications. Baths and


frictions are useful in their effect on the general health. The ether
spray to the spine or the application of an ice-bag for ten minutes
once or twice daily is sometimes found to assist the other means.
Cold douches have been advised by some, but they may do harm.
The care of the general health of the patient is of first importance,
and his surroundings should be as quiet as possible.
It is of the greatest value in bad cases to place the patient in bed and
keep him there until the symptoms improve. John Van Bibber of
Baltimore has treated a number of cases of chorea successfully by
keeping them secluded in a darkened room. Such an extreme
degree of isolation is not often necessary, and it might make a child
more nervous.

In children the patient should always be taken from school and kept
from exciting play. Plenty of fresh air and wholesome food should be
insisted upon. Change of air to the mountains or to the seashore
often effects a cure in a short time.

Some cases do not appear to be benefited by any treatment. These


are the hereditary form of chorea and some of the localized choreas.
The latter are often helped or cured by the hypodermic use of
arsenic even in long-standing cases.

ATHETOSIS.
BY WHARTON SINKLER, M.D.

This disease was first described by Hammond in his work on Diseases of the Nervous System in 1871,
and cases have since been reported by many observers, among them Clifford Allbutt, Claye Shaw,
Eulenburg, Oulmont, and Gowers. The disease is named by Hammond from the word ἀθετος, without
fixed position.1 The principal features are an inability to retain the fingers and toes in any position in
which they may be placed, and the continual movements which persist in the parts—a condition called
by Gowers mobile spasm.
1 Diseases of the Nervous System, p. 722.

Athetosis is often connected with impaired mental powers; many of Shaw's cases were in imbecile
children.

The movements of athetosis are not confined to the hand in all cases, but they are sometimes met with
in the foot, and even in the muscles of the face and back.

The following is Hammond's original case:2 “J. P. R——, aged thirty-three, a native of Holland, consulted
Hammond Sept. 13, 1869. His occupation was bookbinding, and he had the reputation, previous to his
present illness, of being a first-class workman. He was of intemperate habits. In 1860 he had an
epileptic paroxysm, and since that time, to the date of his first visit to me, had a fit about once in six
weeks. In 1865 he had an attack of delirium tremens, and for six weeks thereafter was unconscious,
being more or less delirious during the whole period. Soon after recovering his intelligence he noticed a
slight sensation of numbness in the whole of the right upper extremity and in the toes of the same side.
At the same time severe pain appeared in these parts, and complex involuntary movements ensued in
the fingers and toes of the same side.

“At first the movements of the fingers were to some extent under the control of his will, especially when
this was strongly exerted and assisted by his eyesight, and he could, by placing his hand behind him,
restrain them to a still greater degree. He soon, however, found that his labor was very much impeded,
and he had gradually been reduced from time to time to work requiring less care than the finishing, at
which he had been very expert.

“The right forearm, from the continual action of the muscles, was much larger than the other, and the
muscles were hard and developed like those of a gymnast. When told to close his hand he held it out at
arm's length, clasped the wrist with the other hand, and then, exerting all his power, succeeded, after at
least half a minute, in flexing the fingers, but instantaneously they opened again and resumed their
movements.

“In this patient there was impairment of intellect, his memory was enfeebled, and his ideas were dull.
There was no paralysis of any part of the body, but there was slight tremor of both upper extremities.
The involuntary movements were of the right arm, and continued during sleep. Sensation was normal.
The spasm of the muscles causes severe pain in the arm, and keeps him from sleeping at night.”
Hammond used various remedies without relief, and had the patient under his charge for many years.
Finally, he showed the patient to the American Neurological Society at the annual meeting in 1883, with
almost complete relief to the movements as a result of nerve-stretching.
2 Ibid.

Athetosis is found in two forms—the hemiplegic and the bilateral varieties. In the former there has
usually been an attack of hemiplegia more or less marked, or there has been an epileptic fit or
unconsciousness from alcohol, as in Case I. There is often hemianæsthesia or some disorder of

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