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Flashcards in Movement Disorders Deck (25):
1

241. A patient gives a history of chorea gravidarum. This condition only
occurs in
a. Newborns
b. Depressed men
c. Pregnant women
d. Pubescent girls
e. Pubescent boys

c. Pregnant women
( Victor, p 78.) Chorea gravidarum designates an
involuntary movement disorder that occurs during pregnancy and involves
relatively rapid and fluid, but not rhythmic, limb and trunk movements.
This type of movement disorder may also appear with estrogen use, but the
fundamental problem is a dramatic change in the hormonal environment
of the brain. At the end of pregnancy or with the withdrawal of the offending

estrogen, the movements abate. The movements that develop with
chorea gravidarum may be quite asymmetric and forceful.

2

242. The influenza epidemic of 1918 to 1926 was associated with von
Economo’s encephalitis and left many persons with a syndrome indistinguishable
from
which of the following?
a. Sydenham’s chorea
b. Alzheimer’s disease
c. Multiple sclerosis
d. Amyotrophic lateral sclerosis
e. Parkinson’s disease

e. Parkinson’s disease
( Victor, p 813.) A variety of agents can induce signs
and symptoms of parkinsonism on a temporary basis, but few will evoke a
persistent Parkinsonian syndrome. After the epidemic of encephalitis
lethargica of 1918 to 1926, there were many cases of postencephalitic
parkinsonism. The causative agent was believed to be an influenza virus,
but it could not be isolated with the techniques available at the time of the
epidemic. Postinfluenzal parkinsonism still develops, but the incidence is
too rare to establish that this virus is the only virus capable of producing
parkinsonism. Early in the infection, patients may exhibit a transient
chorea. As the chorea abates, the parkinsonism appears and persists.

3

Items 243–245
243. A 43-year-old man has a father who died from Huntington’s disease.
The son was tested and found out that he does have the gene for Huntington’s
disease.Whichofthe following is true regarding the offspring of those with Huntington’s disease?
a. Half the offspring are at risk only if the affected parent is male
b. Half the offspring are at risk only if the affected parent is female
c. Half the offspring are at risk if either parent is symptomatic for the disease
before the age of 30
d. Half the offspring are at risk for the disease
e. One out of four children is at risk for the disease

d. Half the offspring are at risk for the disease
( Kandel, p 865.) Huntington’s disease is transmitted

in an autosomal dominant fashion. The age at which the patient
becomes symptomatic is variable and has no effect on the probability of
transmitting the disease. The defect underlying this degenerative disease is
an abnormal expansion of a region of chromosome 4 containing a triplicate
repeat (CAG) sequence. Normal individuals have between 6 and 34 copies
of this CAG section; patients with Huntington’s disease may have from 37
to over 100 repeats. Once expanded beyond 40 copies, the repeats are
unstable and may further increase as they are passed on from one generation
tothenext.An increased number of repeats leads to a phenomenon known as anticipation, by which successive generations have earlier disease onset.

4

244. Atrophy in the head of the caudate nucleus in patients with Huntington’s
disease affects
the shape of the
a. Cerebellum
b. Lateral ventricle
c. Third ventricle
d. Lenticular nuclei
e. Temporal lobe

b. Lateral ventricle
(Greenberg, 2/e, pp 14–16.) As the caudate atrophies,

the frontal tip of the lateral ventricle becomes increasingly rhomboidal
in

shape. The head of the caudate is usually atrophic early in the
course of Huntington’s disease, and this will usually be evident by the time
the patient is symptomatic, if not sooner. On MRI or CT scanning, the head
of the caudate gives the frontal and parietal components of the lateral ventricle
its typical comma, or boomerang, appearance.

5

245. If this patient were to be exposed to L-dopa, which of the following
would most likely be evoked?
a. Generalized seizures
b. Partial seizures
c. Intention tremor
d. Scanning speech
e. Writhing and jerking movements of the limbs

e. Writhing and jerking movements of the limbs
( Victor, p 1125.) Writhing and jerking movements
of the limbs are part of the chorea that typically develops with Huntington’s

disease. Dopaminergic drugs, such as
L-dopa, bromocriptine, and
lisuride, may unmask chorea. This is inadvisable as a diagnostic technique

because it may contribute to the premature symptom of chorea.
Dopamine antagonists, such as haloperidol, may be used to suppress
chorea, but also carry the risk of provoking tardive dyskinesia. Huntington’s

disease is characterized pathologically by loss of several neuronal
types in the striatum (caudate and putamen). It has been hypothesized
that the occurrence of dopaminergic-induced chorea in Huntington’s disease

is related to increased sensitivity of the dopamine receptors in the
remaining striatal neurons, although there are abnormalities in several
other neurotransmitters as well. Choreiform movements develop in a
variety of other conditions; the one most similar to Huntington’s disease
is hereditary acanthocytosis.

6

246. A 26-year-old heroin addict has been using a street version of
artificial heroin. The drug actually contains 1-methyl-4-phenyl-1,2,3,6tetrahydropyridine
(MPTP).
The neurological syndrome for which he is at risk is clinically indistinguishable from
which of the following?
a. Huntington’s disease
b. Friedreich’s disease
c. Sydenham’s chorea
d. Parkinson’s disease
e. Amyotrophic lateral sclerosis

d. Parkinson’s disease
( Victor, p 73.) Young adults who have selfadministered MPTP
in an effort to achieve an opiate high
have developed progressive damage to the substantia nigra. The neurologic syndrome
that results from this damage is indistinguishable from Parkinson’s disease,
except that it evolves over weeks or months rather than years. Affected persons exhibit
rigidity, tremor, and bradykinesia. That a toxin can produce a syndrome indistinguishable from Parkinson’s disease
has increased speculation that some—perhaps many—persons with Parkinson’s disease have had environmental exposure to a toxin that produced degeneration of the
substantia nigra.

7

Items 247–250
A 61-year-old right-handed man presents with involuntary twitches of
his left hand. He first noticed between 6 months and 1 year ago that when
he is at rest, his left hand shakes. He can stop the shaking by looking at his
hand and concentrating. The shaking does not impair his activities in any
way. He has no trouble holding a glass of water. There is no tremor in his
right hand, and his lower extremities are not affected. He has had no trouble walking, and there have been no falls.
There have been no behavioral or language changes. On examination, a left hand tremor is evident when the man is
distracted. His handwriting is mildly tremulous. He has bilateral cogwheel rigidity with contralateral activation, which is worse on the left. His rapid alternating movements are bradykinetic on the left.
247. The most likely diagnosis in this case is which of the following?
a. Epilepsy
b. Guillain-Barré syndrome
c. Multiple sclerosis
d. Parkinson’s disease
e. Stroke

d. Parkinson’s disease
( Rowland, pp 679–682.) The tremor is of a Parkinsonian type.
The patient also has the classic findings of
Parkinson’s disease: asymmetric tremor, rigidity, and bradykinesia.
Epilepsy is characterized by repeated unprovoked seizures. Hand shaking can be the result of a focal motor seizure, but the presentation overall makes epilepsy an unlikely diagnosis. Guillain-Barré syndrome is a peripheral demyelinating disease
that usually presents as an ascending motor deficit. Multiple sclerosis is a
central nervous system (CNS) demyelinating disease. It presents with individual episodes of CNS deficits, which usually recover to some extent.
Stroke is characterized by the acute onset of a neurological deficit due to
nerve infarction. Tremor would be an exceedingly rare presentation for
stroke, and it would not evolve over 6 to 12 months.

8

248. Which brain structures are currently targets for deep brain stimulation
in patients with this disease?
a. Globus pallidus, medulla, and parietal lobe
b. Globus pallidus, subthalamic nucleus, and thalamus
c. Hippocampus, medulla, and thalamus
d. Medulla, occipital lobe, and subthalamic nucleus
e. Parietal lobe, temporal lobe, and thalamus

b. Globus pallidus, subthalamic nucleus, and thalamus
( Rowland, pp 686–687.) Current theory of Parkinson’s
disease
pathology
is
based
on
the
premise
that
the
substantia
nigra
pars
compacta

has decreased dopamine production, which eventually leads to
overinhibition of thalamocortical pathways. The thalamus may be directly
intervened on to decrease this overinhibition. Alternatively, the globus pallidus
interna
may
be
lesioned
or
stimulated,
because
it
directly
inhibits
the
thalamus.
A
third
approach
is
to
lesion
or
stimulate
the
subthalamic
nucleus,
which
has
an
excitatory
connection
on
the
globus
pallidus
interna
and
substantia
nigra pars reticulata.
The medulla, hippocampus, temporal lobe, and
occipital
lobe are
not involved in this pathway.

9

249. Which of the following would you use to treat this person?
a. Alteplase
b. Carbidopa-levodopa
c. Glatiramer
d. Interferon β-1A
e. Sertraline

b. Carbidopa-levodopa
( Rowland, pp 686–687.) Parkinson’s disease symptoms
are
due
in
large
part
to
dopamine
depletion.
Carbidopa-levodopa
can
replete

dopamine and alleviate symptoms. Alteplase is used to dissolve
blood clots during acute strokes or heart attacks. Glatiramer and interferon
β-1A are used to treat multiple sclerosis, and have been shown to decrease
attacks. Both are thought to work through immunomodulation. Sertraline
is a selective serotonin reuptake inhibitor. By increasing serotonin concentrations,
it is effective
for the treatment
of depression.

10

250. Neurons remaining in the substantia nigra of the patient with this
disease may exhibit
a. Intranuclear inclusion bodies
b. Intranuclear and intracytoplasmic inclusion bodies
c. Intracytoplasmic inclusion bodies
d. Neurofibrillary tangles
e. Amyloid plaques

c. Intracytoplasmic inclusion bodies
( Rowland, p 681.) The intracytoplasmic inclusion
bodies commonly seen in patients with idiopathic Parkinson’s disease are
called Lewy bodies. They are eosinophilic inclusions with poorly staining
halos surrounding them. They may be round or oblong in shape and are
most common in the substantia nigra, locus coeruleus, and substantia
innominata. They appear to consist of aggregated neurofilaments. Degenerative
changes
may
be
remarkably
asymmetric
in
patients
with
Parkinson’s
disease.

11

251. A 48-year-old female psychiatric patient has parkinsonism secondary
to long-term neuroleptic use. Which of the following medications might
minimize her parkinsonism?
a. Trihexyphenidyl (Artane)
b. Haloperidol (Haldol)
c. Methamphetamine
d. Thioridazine (Mellaril)
e.L-dopa

a. Trihexyphenidyl (Artane)
( Rowland, p 683.) Trihexyphenidyl (Artane) is an
anticholinergic drug. It is presumed to decrease signs of parkinsonism
caused by drugs that interfere with dopamine neurotransmission by creating
a
relative
deficiency
of
acetylcholine
neurotransmission.
In
a
very
simplistic

view of the CNS, the cholinergic and dopaminergic systems have
antagonistic actions.

12

Items 252–253
252. A 70-year-old woman has 1 year of worsening gait, right hand tremor,
and rigidity. She is diagnosed with Parkinson’s disease and improves dramatically
with
treatment.
If
her
disease
progresses,
the
decrement
in
speech
which
would be expected would result
in which of the following?
a. Progressively inaudible speech
b. Receptive aphasia
c. Expressive aphasia
d. Word salad
e. Neologisms

a. Progressively inaudible speech
( Victor, p 1130.) Language is not disturbed in
Parkinson’s disease, as it is with aphasias. It is the clarity and volume of
speech that suffers. Handwriting is similarly disturbed. The patient has
increasingly smaller and less legible penmanship as he or she continues to
write. This is referred to as micrographia.

13

253. Even though the physiologic deficiency in Parkinson’s disease is of
dopamine,
L-dopa rather than dopamine is given to patients because
a. L-dopa induces less nausea and vomiting than dopamine
b. Dopamine is readily metabolized in the gastrointestinal tract to ineffective com-
pounds
c.
L-dopa is more readily absorbed in the gastrointestinal tract than is dopamine
d. Dopamine cannot cross the blood-brain barrier and therefore has no therapeu-
tic effect in the CNS
e.
L-dopa is more effective at dopamine receptors than is dopamine itself

d. Dopamine cannot cross the blood-brain barrier and therefore has no therapeu-
tic effect in the CNS
( Victor, pp 1133–1134.)
L-dopa crosses the bloodbrain
barrier
easily
and
is
subsequently
converted
to
dopamine
in
the
CNS.
Conversion
of

L-dopa
to
dopamine
occurs
outside
the
CNS
in
a
wide
variety
of
tissues,
but
once
converted
to
dopamine
in
the
periphery,
the
drug
becomes

inaccessible to the brain. Peripheral conversion of
L-dopa to
dopamine is routinely inhibited by adding a dopa decarboxylase inhibitor
to the therapeutic regimen. Carbidopa, the inhibitor most widely used,
does not penetrate the blood-brain barrier substantially. Because it is
largely excluded from the CNS, carbidopa cannot inhibit the conversion of
L-dopa to dopamine in the brain

14

254. A 25-year-old man has had motor tics since age 13. They seem to be getting
worse, and now he also has involuntary obscene vocalizations. He may
have largely normal behavior while being treated with which of the following?
a. L-dopa
b. Trihexyphenidyl (Artane)
c. Phenytoin (Dilantin)
d. Carbamazepine (Tegretol)
e. Haloperidol (Haldol)

e. Haloperidol (Haldol)
( Rowland, pp 668–669.) The scenario described is
that associated with Tourette syndrome. The affected person is usually over
21 years of age and cannot control the obscene and scatological remarks.
With Tourette syndrome there appears to be an autosomal dominant pattern

of inheritance with variable penetrance. Most affected persons are
men. A variety of drugs may help suppress the tics that are characteristic of
this syndrome. These include haloperidol, pimozide, trifluoperazine, and
fluphenazine. Antiepileptics, such as carbamazepine and phenytoin, are
not useful. Trihexyphenidyl and benztropine are useful in suppressing the
parkinsonism that may develop with haloperidol administration, but are
not useful in the management of Tourette syndrome.

15

255. A 72-year-old man was diagnosed with Parkinson’s disease after pre-
senting 2 years ago with asymmetric rigidity, bradykinesia, and tremor. He
is being treated with carbidopa and
L-dopa. Carbidopa is used in combination
with
L-dopa
because it
a. Has anticholinergic activity
b. Has dopaminergic activity
c. Is an antihistaminic
d. Is an antiemetic
e. Is a dopa decarboxylase inhibitor

e. Is a dopa decarboxylase inhibitor
( Rowland, pp 686–687.) Dopa decarboxylase converts

L-dopa
to
dopamine.
Carbidopa
crosses
the
blood-brain
barrier
poorly, and so its inhibition of this enzyme is restricted to activity outside the CNS.
Conversion of
L-dopa to dopamine continues to occur in the CNS when the
patient takes Sinemet, a combination of
L-dopa and carbidopa.

16

256. After several years of successful anti-Parkinsonian treatment, a patient
abruptly develops acute episodes of profound bradykinesia and rigidity.
Remission of these signs occurs as abruptly as the onset. This patient probably
suffers
from
a. Acute dystonia
b. Absence attacks
c. On-off phenomenon
d. Complex partial seizures
e. Drug toxicity

c. On-off phenomenon
( Victor, p 1135.) The on-off effect is commonly seen
in persons who have had Parkinson’s disease for several years. Maintaining
more stable levels of anti-Parkinsonian medication in the blood does not
eliminate this phenomenon of abruptly worsening and remitting symptoms.
Variability
in
the
responsiveness
of
the
CNS
to
the
medication,
rather
than
in the medication levels, underlies the phenomenon.

17

DIRECTIONS: Each group of questions below consists of lettered
options followed by a set of numbered items. For each numbered item,
select the one lettered option with which it is most closely associated. Each
lettered option may be used once, more than once, or not at all.
Items 257–260
For each clinical scenario, select the most likely condition.
a. Meigs’ syndrome
b. Dopa-responsive dystonia
c. Parkinson’s disease
d. Olivopontocerebellar atrophy
e. Tardive dyskinesia
f. Spasmodic torticollis
g. Whipple’s disease
h. Hemifacial spasm
i. Essential tremor
257. A 53-year-old woman is unable to stop blinking forcefully, and has
frequent grimacing movements of the face. At times she protrudes her
tongue against her will. She has never taken any medications. (SELECT 1
CONDITION)
258. A 42-year-old woman has a long history of twisting movements of

a. Meigs’ syndrome
( Victor, p 115.) Meigs’ syndrome is a form of focal
dystonia characterized by blepharospasm, forceful jaw opening, lip retraction,
neck
contractions,
and
tongue
thrusting.
Sometimes
these
features
are
produced

by phenothiazine or butyrophenone use, but they may also
occur idiopathically, more often in women than men, with onset in the
sixth decade. Botulinum toxin injection has been more effective in treatment
than any oral medication.

18

258. A 42-year-old woman has a long history of twisting movements of
her head to the left. These are painful, and have resulted over the years in
muscular hypertrophy affecting the sternocleidomastoid and trapezius
muscles. There is no family history. The remainder of her examination is
normal. (SELECT 1 CONDITION)
a. Meigs’ syndrome
b. Dopa-responsive dystonia
c. Parkinson’s disease
d. Olivopontocerebellar atrophy
e. Tardive dyskinesia
f. Spasmodic torticollis
g. Whipple’s disease
h. Hemifacial spasm
i. Essential tremor

f. Spasmodic torticollis
( Victor, pp 113–114.) Spasmodic torticollis is
another very common form of focal dystonia. It usually begins in early
adult life. The contractions of the neck muscles may be painful and also
produce hypertrophy. Standing and walking worsen the contractions, and
typically a trick, or geste, such as touching the chin or resting the head
against a pillow, may reduce the spasms. Spontaneous remissions may
occur. Trihexiphenidyl (Artane) and a number of other medications may be
used, generally without much success; effective improvement generally
does not occur until botulinum toxin injections are given.

19

259. A 40-year-old literary agent has had worsening tremor of the hands.
This has been present for 2 years, but has increasingly impaired her work
ability because she is frequently required to take her clients to lunch, and
she is embarrassed by her inability to eat and drink normally. A glass of
wine with the meal typically helps somewhat. On exam, there is a mild
head tremor, but no rest tremor of the hands. When she holds a pen by the
tip at arm’s length, however, a coarse tremor is readily apparent. Exam is
otherwise normal. (SELECT 1 CONDITION)
a. Meigs’ syndrome
b. Dopa-responsive dystonia
c. Parkinson’s disease
d. Olivopontocerebellar atrophy
e. Tardive dyskinesia
f. Spasmodic torticollis
g. Whipple’s disease
h. Hemifacial spasm
i. Essential tremor

i. Essential tremor
( Victor, pp 100–103.) Essential tremor comes on during
action
and
remits
when
the
limb
is
relaxed,
unlike
the
tremor
of
Parkin-
son’s disease. It often affects the head as well as the arms, also unlike
Parkinson’s disease. Patients are often very disturbed by the tremor, particularly
as
it
leads
to
a
great
deal
of
social
embarrassment.
There
is
no
associated
slowness
of
activity
(bradykinesia),
rigidity,
or
cognitive
disturbance.
Patients
frequently
report
improvement
with
alcohol,
to
the
extent
that
some
patients
may
resort
to
use
of
alcohol
on
a
chronic
basis
to
reduce
their
symptoms.
Although
it
is
often
referred
to
as
familial
tremor,
there
is
some
disagreement on this point because it may simply be the case that patients with the condition
are
more
likely
to
refer
relatives
for
evaluation.
Beta
blockers
and
primidone
may be used to treat
this condition.

20

260. A 64-year-old man has noticed dragging of the right leg and tremor
and stiffness of the right hand. On exam, he has a tremor of the right hand,
which disappears when he reaches to grab a pen. Movements are slower on
the right than the left. He has cogwheel rigidity of the right arm. (SELECT
1 CONDITION)
a. Meigs’ syndrome
b. Dopa-responsive dystonia
c. Parkinson’s disease
d. Olivopontocerebellar atrophy
e. Tardive dyskinesia
f. Spasmodic torticollis
g. Whipple’s disease
h. Hemifacial spasm
i. Essential tremor

c. Parkinson’s disease
( Victor, pp 1128–1137.) Idiopathic Parkinson’s disease
is
characterized
by
the
classic
combination
of
tremor,
rigidity,
bradykinesia,

and postural instability. The typical tremor is a 4-Hz pill-rolling
tremor, affecting one side more than the other. Action tremor may also
occur. The classic pathologic hallmarks of the disease are a loss of pigmented
cells
in
the
substantia
nigra
and
other
nuclei,
and
the
finding
of
the
Lewy
body,
which
is
an
eosinophilic
cytoplasmic
inclusion
in
the
remaining
cells of the substantia nigra.

21

Items 261–265
For each clinical scenario, pick the most likely diagnosis.
a. Hepatolenticular degeneration
b. Hyperparathyroidism
c. Central pontine myelinolysis
d. Akinetic mutism
e. MPTP poisoning
f. Locked-in syndrome
g. Postencephalitic parkinsonism
h. Neuroleptic effect
i. Essential tremor
j. Vegetative state
k. Hypermagnesemia
l. Rhombencephalitis
261. A 34-year-old man develops progressive depression and memory
impairment over the course of 6 months. His initial neurologic evaluation
reveals a metabolic acidosis associated with his dementia. His liver is firm
and his spleen appears to be slightly enlarged. He has tremor and rigidity
in his arms and walks with relatively little swing in his arms. His blink is
substantially reduced, which gives him the appearance of staring. An MRI
of the brain reveals some atrophy of the putamen and globus pallidus. His
CSF is normal. His EEG is unremarkable. (SELECT 1 DIAGNOSIS)

a. Hepatolenticular degeneration
( Bradley, p 1622.) Hepatolenticular degeneration
(Wilson’s disease) often becomes symptomatic in the second or third
decade of life, but its initial presentation may be delayed until the fourth or
fifth decade. Renal tubular acidosis develops along with hepatic fibrosis.
Systemic problems include heart and lung damage, but most patients
become most symptomatic from their brain and liver disease. Dementia is
progressive if the patient is not treated. Hepatic disease will progress to
hepatic failure if the patient is left untreated. Appropriate treatment
includes the chelating agent penicillamine, which depletes the body of
copper.

22

262. A 19-year-old woman develops auditory hallucinations and persecu-
tory delusions over the course of 3 days. She is hospitalized and started on
haloperidol (Haldol), 2 mg three times daily. Within 1 week of treatment,
she develops stooped posture and a shuffling gait. Her head is slightly
tremulous and her movements are generally slowed. Her medication is
changed to thioridazine (Mellaril), and trihexyphenidyl (Artane) is added.
Over the next 2 weeks, she became much more animated and reports no
recurrence of her hallucinations. (SELECT 1 DIAGNOSIS)
a. Hepatolenticular degeneration
b. Hyperparathyroidism
c. Central pontine myelinolysis
d. Akinetic mutism
e. MPTP poisoning
f. Locked-in syndrome
g. Postencephalitic parkinsonism
h. Neuroleptic effect
i. Essential tremor
j. Vegetative state
k. Hypermagnesemia
l. Rhombencephalitis

h. Neuroleptic effect
( Bradley, p 1905.) Butyrophenones, the most commonly
prescribed
of
which
is
haloperidol,
routinely
produce
some
signs
of
parkinsonism
if they are
used at high doses for more
than a few days. This
psychotic

young woman proved to be less sensitive to the Parkinsonian
effects of the phenothiazine thioridazine than she was to haloperidol.
Adding the anticholinergic trihexyphenidyl may also have helped to reduce
the patient’s parkinsonism. Another commonly used medication that can
cause parkinsonism, in addition to tardive dyskinesia, is metoclopramide
hydrochloride (Reglan).

23

263. A 65-year-old man develops slurred speech, difficulty swallowing,
and labored breathing over the course of 30 min. When he arrives at the
emergency room, he requires ventilatory assistance. His arms and legs are
flaccid, and he exhibits no voluntary movements in any of his limbs. He is
able to blink his eyes when instructed and appears to have completely
intact comprehension of spoken and written language. An MRI reveals
extensive infarction of the ventral pons. The basilar artery is not visible on
MRA. (SELECT 1 DIAGNOSIS)
a. Hepatolenticular degeneration
b. Hyperparathyroidism
c. Central pontine myelinolysis
d. Akinetic mutism
e. MPTP poisoning
f. Locked-in syndrome
g. Postencephalitic parkinsonism
h. Neuroleptic effect
i. Essential tremor
j. Vegetative state
k. Hypermagnesemia
l. Rhombencephalitis

f. Locked-in syndrome
( Bradley, pp 1133–1134.) Consciousness is preserved

in the locked-in syndrome, but the patient is paralyzed from the
eyes down. Survival is usually limited to days or weeks in patients with this
clinical syndrome. In most cases, the locked-in syndrome develops because of ischemic or hemorrhagic damage to the pons, such as that occurring
with basilar artery occlusion.

24

264. A 72-year-old man requires bypass surgery to alleviate myocardial
ischemia. During surgery, he has a massive myocardial infarct and protracted
asystole. Resuscitative measures
succeed in reestablishing
a normal
sinus
rhythm,
but
postoperatively
the
patient
remains
unconscious
after
48
h.
Over
the
ensuing
weeks,
the
patient’s

level of consciousness improves
slightly. He appears awake at times, but does not interact in meaningful
ways with visitors. He breathes independently and even swallows food
when it is placed in his mouth, but he remains mute. With painful stimuli,
he exhibits semipurposeful withdrawal of his limbs. His clinical status
remains unchanged for several more months. (SELECT 1 DIAGNOSIS)
a. Hepatolenticular degeneration
b. Hyperparathyroidism
c. Central pontine myelinolysis
d. Akinetic mutism
e. MPTP poisoning
f. Locked-in syndrome
g. Postencephalitic parkinsonism
h. Neuroleptic effect
i. Essential tremor
j. Vegetative state
k. Hypermagnesemia
l. Rhombencephalitis

j. Vegetative state
( Bradley, p 1469.) The vegetative state is a clinical
condition in which autonomic activity is sustained with little evidence of
cognitive function. With protracted asystole, the patient may sustain extensive
damage
to
the
cerebral
cortex
with
little
damage
to
the
brainstem.
The
ischemic
damage
to
the
cerebrum
should
be
evident
on
MRI
soon
after
the
injury.
This
type
of
damage
is
usually
responsible
for
the
appearance
of
the
vegetative
state.
It
also
may
develop
with
drowning
or
other
causes
of
protracted
hypoxia.

25

265. A 62-year-old man exhibits excessive sleepiness, slowing of movements,
mild
depression,
and
proximal
muscle
weakness.
His
proximal
limb
muscles
are
obviously
atrophied.
Although
his
blood
count
is
normal,
routine
screening
of
serum
chemistries
reveals
an
elevated
calcium
level.
He
also

has an elevated serum creatinine with reduced creatinine clearance.
The patient has had abdominal discomfort intermittently for several
months and has been told that his episodes of joint swelling were due to
pseudogout. (SELECT 1 DIAGNOSIS)
a. Hepatolenticular degeneration
b. Hyperparathyroidism
c. Central pontine myelinolysis
d. Akinetic mutism
e. MPTP poisoning
f. Locked-in syndrome
g. Postencephalitic parkinsonism
h. Neuroleptic effect
i. Essential tremor
j. Vegetative state
k. Hypermagnesemia
l. Rhombencephalitis

b. Hyperparathyroidism
( Bradley, p 1033.) Primary hyperparathyroidism
develops in the elderly and may be overlooked or misdiagnosed. The elevated
calcium (over 11.5 mg/dL) that is characteristic of the disturbance is
dismissed
as an immobilization phenomenon or misconstrued as evidence
of

an occult neoplasm. The appearance of pseudogout should raise the
probability of hyperparathyroidism substantially. The calcium level may in
fact be normal when it is checked, but the parathyroid hormone levels will
be elevated.