Neurology

Question 1

Match the following:

Horner’s syndrome and facial numbness
A. Tolosa-Hunt syndrome
B. Gradenigo’s syndrome
C. Raeder’s syndrome

Answer 1

A. Tolosa-Hunt syndrome
B. Gradenigo’s syndrome
C. Raeder’s syndrome

Raeder’s para trigeminal neuralgia is often localized adjacent to the trigeminal nerve as it courses through
the middle cranial fossa. The cause of this syndrome is often
unclear, but it is usually characterized by a partial Horner’s
syndrome and unilateral trigeminal nerve problems, including tic-like pain, numbness, and/or masseter weakness.
Gradenigo’s syndrome, also lmown as apical petrositis, often
consists of the classic triad of abducens nerve palsy, retroorbital pain, and a draining ear. Tolosa-Hunt syndrome is a
diagnosis of exclusion; this condition is believed to result
from inflammation adjacent to the superior orbital fissure. It
is characterized by painful ophthalmoplegia, cranial nerves
III, IV, and VI palsies, and recurrent attacks and remissions;
it is typically treated with intravenous steroids (Greenberg,
pp.581-582).

Question 2

Match the following:

Retro- or bital pain and sixth nerve palsy
A. Tolosa-Hunt syndrome
B. Gradenigo’s syndrome
C. Raeder’s syndrome

Answer 2

A. Tolosa-Hunt syndrome
**B. Gradenigo’s syndrome **
C. Raeder’s syndrome

Raeder’s para trigeminal neuralgia is often localized adjacent to the trigeminal nerve as it courses through
the middle cranial fossa. The cause of this syndrome is often
unclear, but it is usually characterized by a partial Horner’s
syndrome and unilateral trigeminal nerve problems, including tic-like pain, numbness, and/or masseter weakness.
Gradenigo’s syndrome, also lmown as apical petrositis, often
consists of the classic triad of abducens nerve palsy, retroorbital pain, and a draining ear. Tolosa-Hunt syndrome is a
diagnosis of exclusion; this condition is believed to result
from inflammation adjacent to the superior orbital fissure. It
is characterized by painful ophthalmoplegia, cranial nerves
III, IV, and VI palsies, and recurrent attacks and remissions;
it is typically treated with intravenous steroids (Greenberg,
pp.581-582).

Question 3

Match the following:

Painful ophthalmoplegia and third, fourth , and fifth nerve palsies
A. Tolosa-Hunt syndrome
B. Gradenigo’s syndrome
C. Raeder’s syndrome

Answer 3

**A. Tolosa-Hunt syndrome **
B. Gradenigo’s syndrome
C. Raeder’s syndrome

Raeder’s para trigeminal neuralgia is often localized adjacent to the trigeminal nerve as it courses through
the middle cranial fossa. The cause of this syndrome is often
unclear, but it is usually characterized by a partial Horner’s
syndrome and unilateral trigeminal nerve problems, including tic-like pain, numbness, and/or masseter weakness.
Gradenigo’s syndrome, also lmown as apical petrositis, often
consists of the classic triad of abducens nerve palsy, retroorbital pain, and a draining ear. Tolosa-Hunt syndrome is a
diagnosis of exclusion; this condition is believed to result
from inflammation adjacent to the superior orbital fissure. It
is characterized by painful ophthalmoplegia, cranial nerves
III, IV, and VI palsies, and recurrent attacks and remissions;
it is typically treated with intravenous steroids (Greenberg,
pp.581-582).

Question 4

A 56-year-old male with a long history of smoking and hypertension presents to a stroke neurologist with a unique vascular insult involving the cephalad portion of the nucleus ambiguus. Which of the following problems can often be avoided with such an insult?

A. Palatal paralysis
B. Pharyngeal paralysis
C. Laryngeal paralysis
D. None of the above
E. All of the above

Answer 4

A. Palatal paralysis
B. Pharyngeal paralysis
**C. Laryngeal paralysis **
D. None of the above
E. All of the above

Lesions within the nucleus ambiguus may occur as
a result of vascular insults, tumors, syringobulbia, motor
neuron disease, and inflammatory disease. Lesions in this
location often result in palatal, pharyngeal, and laryngeal
paralysis that is often associated with other adjacent cranial
nerve and brainstem abnormalities. If the cepha lad portion
of the nucleus ambiguus is injured , however, laryngeal function is often spared due to the somatotopic organization of
this motor nucleus. This is referred to as “palatopharyngeal
paralysis of Avellis” (Brazis, pp. 321- 322).

Question 5

Ipsilateral trapezius and sternocleidomastoid muscle wealmess, dysphonia and dysphagia, loss of taste over the posterior third of the tongue , and depressed sensation over the pharYlu characterizes what syndrome?

A. Collet-Sicard syndrome
B. Vernet’s syndrome
C. Schmidt’s syndrome
D. Garcin syndrome
E. Weber’s syndrome

Answer 5

A. Collet-Sicard syndrome
**B. Vernet’s syndrome **
C. Schmidt’s syndrome
D. Garcin syndrome
E. Weber’s syndrome

Refer to Table 3-SA. The spinal accessory nerve enters
the jugular foramen accompanied by cranial nerves IX and X.
Lesions of the jugular foramen including tumors, infections,
and fractures can result in Vernet’s syndrome, which is cha racterized by ipsilateral trapezius and sternocleidomastoid
muscle wealmess, dysphonia and dysphagia , loss of taste
over the po~terior third of the tongue, and depressed sensation over the pharynx (Brazis, pp. 330-331).

Question 6

A 7 -year-old boy presented to the emergency room with involuntary laughter (gelastic seizures) and precocious puberty. An MRI study of the brain may show a lesion in what location?

A. Amygdala
B. Hippocampus
C. Cingulate gyrus
D. Hypothalamus
E. Sella turcica

Answer 6

A. Amygdala
B. Hippocampus
C. Cingulate gyrus
**D. Hypothalamus **
E. Sella turcica

An [vIR} study of the brain in a patient with seizures
accompanied by involuntary laughter (gelastic seizures) that
alternates with crying or sobbing spells would likely show
a lesion in the hypothalamus. A small series of cases have
been reported in the literature to date describing this
phenomenon; in one report, 4 of 16 patients were found to
harbor a hypothalamic hemartoma. Gelastic seizures or
laughing fits have been reported to occur in up to 21% of
patients with hypothalamic hemartomas. Most patients with
this lesion present with isosexual precocious puberty by the
age of 3 years, although patients as old as 8 years have been
reported. Hypothalamic hemartomas may be associated
with midline deformities such as callosal ageneSis, optic
malformations, and hemispheric dysgenesis (Kaye and Laws,
pp. 593- 596).

Question 7

A 52-year-old construction worker noted some
weakness in his hands while at work, followed by thickening
of his speech and swallowing problems a few months later.
Although he complained of generalized fatigue and aching in his upper and lower extremities, no numbness or other
sensory abnormalities were noted on physical examination.
There were marked fasciculations and atrophy of his arms, legs, and tongue, as well hyperactive retlexes and Babinski
Signs.

The most likely diagnosis in this middle-aged man would be?
A. Cervical myelopathy
B. Multiple sclerosis
C. Myasthenia gravis
D. Guillain-Barre syndrome
E. Amyotrophic lateral sclerosis

Answer 7

A. Cervical myelopathy
B. Multiple sclerosis
C. Myasthenia gravis
D. Guillain-Barre syndrome
E. Amyotrophic lateral sclerosis

In this patient, the multiple motor deficits and
signs of anterior horn cell disease unaccompanied by any
sensory abnormalities suggest a diagnosis of amyotrophic
lateral sclerosis (ALS). j’vlost individuals with ALS die within
5 years of symptom onset, especially if there are both upper
and lower motor neuron signs. There is a very high incidence
of this disease among the Chamorro Indians of Guam, and
there is also an association between ALS and a Parkinsonlike dementia complex (Merritt, pp. 710- 712)

Question 8

A 52-year-old construction worker noted some
weakness in his hands while at work, followed by thickening of his speech and swallowing problems a few months later. Although he complained of generalized fatigue and aching in his upper and lower extremities, no numbness or other sensory abnormalities were noted on physical examination. There were marked fasciculations and atrophy of his arms, legs, and tongue, as well hyperactive retlexes and Babinski Signs.

What is the prognosis ?
A. Relatively good with improved blood sugar levels
B. Often the patient can go into long-term remission with
plasmapheresis and steroid treatment
C. Excellent with anticholinergic medications
D. Surgery can help prevent neurologic progression
E. Often fatal within 3-5 years

Answer 8

A. Relatively good with improved blood sugar levels
B. Often the patient can go into long-term remission with
plasmapheresis and steroid treatment
C. Excellent with anticholinergic medications
D. Surgery can help prevent neurologic progression
E. Often fatal within 3-5 years

In this patient, the multiple motor deficits and
signs of anterior horn cell disease unaccompanied by any
sensory abnormalities suggest a diagnosis of amyotrophic
lateral sclerosis (ALS). j’vlost individuals with ALS die within
5 years of symptom onset, especially if there are both upper
and lower motor neuron signs. There is a very high incidence
of this disease among the Chamorro Indians of Guam, and
there is also an association between ALS and a Parkinsonlike dementia complex (Merritt, pp. 710- 712)

Question 9

A 52-year-old construction worker noted some
weakness in his hands while at work, followed by thickening
of his speech and swallowing problems a few months later.
Although he complained of generalized fatigue and aching
in his upper and lower extremities, no numbness or other
sensory abnormalities were noted on physical examination.
There were marked fasciculations and atrophy of his arms,
legs, and tongue, as well hyperactive retlexes and Babinski
Signs.

This disease can be associated with what other illness or abnormality?
A. Infantile spasms
B. IGUver-Bucy syndrome
C. Parkinsonism
D. Down’s syndrome
E. Autonomic nervous system degeneration

Answer 9

A. Infantile spasms
B. IGUver-Bucy syndrome
C. Parkinsonism
D. Down’s syndrome
E. Autonomic nervous system degeneration

In this patient, the multiple motor deficits and
signs of anterior horn cell disease unaccompanied by any
sensory abnormalities suggest a diagnosis of amyotrophic
lateral sclerosis (ALS). j’vlost individuals with ALS die within
5 years of symptom onset, especially if there are both upper
and lower motor neuron signs. There is a very high incidence
of this disease among the Chamorro Indians of Guam, and
there is also an association between ALS and a Parkinsonlike dementia complex (Merritt, pp. 710- 712)

Question 10

Akinetic ‘1ll1ltislll refers to a state in which patients,
although seemingly awake, remain motionless and silent. It
has commonly been descri bed wi th lesions involving all of
the following structures EXCEPT?

A. Hypothalamus
B. Ascending dopaminergic activating system
C. Cingulate gyrus
D. Thalamus
E. Raphe nucleus

Answer 10

A. Hypothalamus
B. Ascending dopaminergic activating system
C. Cingulate gyrus
D. Thalamus
E. Raphe nucleus

Refer to Figure 3.10A. Animal studies and case reports
have disclosed two primary lesion sites that may result in
akinetic mutism: the mesencephalic-diencephalic reticular
activating system including the midbrain reticular formation, thalamus, and hypothalamus, and lesions involving the
anterior cingulate gyrus and adjacent mesial frontal lobes.
These two major lesion sites occur along the pathways that
originate in the mesencephalon and project widely to
dopamine receptors located in spinal cord, brainstem, diencephalon, corpus striatum, and mesiofrontal lobes as the
ascending dopaminergic activating system. Mutism related
to dentate nucleus damage has been shown to occur after
removal of cerebellar tumors, especially in children, but this
type of mutism is distinct from classic akinetic mutism.
(Brazis, p. 566).

Question 11

All of the following are characteristics of tuberculous Meningitis EXCEPT?
A. The mortality rate is often higher than in bacterial meningitis
B. Treatment initially includes isoniazid, rifampin, pyrazinamide, and ethambutol
C. Treatment, if started early, is effective in preventing poor outcome in the majority of patients
D. The primary focus of infection with tuberculosis is likely from a region outside the brain
E. Bacterial meningitis is marked by basal meningitis, whereas in tuberculosis the base of the brain is relatively spared of infection

Answer 11

A. The mortality rate is often higher than in bacterial meningitis
B. Treatment initially includes isoniazid, rifampin, pyrazinamide, and ethambutol
C. Treatment, if started early, is effective in preventing poor outcome in the majority of patients
D. The primary focus of infection with tuberculosis is likely from a region outside the brain
E. Bacterial meningitis is marked by basal meningitis, whereas in tuberculosis the base of the brain is relatively spared of infection

Tuberculous meningitis is always secondary to an
infection elsewhere in the body, especially the lungs. It
differs from infections caused by other common bacteria in
that the time course is often more protracted, the mortality
rate is higher, and the CSF changes may not be very helpful
or diagnostic initially. Tuberculous meningitis is also often
characterized by marked basal meningitis, as opposed to
bacterial meningitiS, which tends to produce a meningeal
reaction over the convexities of the brain. The diagnosis is
often established by isolating the organism from the CSF.
CSF findings include slightly increased pressure, moderate
pleocytosis of 25 to 500 cells/mm3 with lymphocytic predominance, increased protein content, decreased glucose values
in the range of 20 to 40 mg/dL, and the absence of growth on
routine CSF culture media. The natural course of the disease
is death within 6 to 8 weeks if left untreated. With early
diagnosis and treatment, the recovery rate approaches 90%.
Treatment is commonly started with four drugs, including
isoniazid, rifampin, pyrazinamide, and ethambutol; streptomycin is an alternative in case one of the agents cannot
be used. The drug regimen can be modified later, once sensitivities of the mycobacterium are lO1own, but are typically
administered for 18 to 24 months (Merritt, pp. 108-111)

Question 12

Gerstmann’s syndrome includes all of the following
EXCEPT?

A. Finger agnosia
B. Acalculia
C. Agraphia
D. Right and left confusion
E. Anosognosia

Answer 12

A. Finger agnosia
B. Acalculia
C. Agraphia
D. Right and left confusion
E. Anosognosia

Gerstmann’s syndrome can result from injury in the
dominant inferior parietal lobule (angular and supramarginal
gyri) and is characterized by confusion of the right and
left limbs, difficulty in distinguishing the fingers on the
hand (finger agnosia), acalculia, and agraphia. Anosognosia,
which is characterized by unawareness of the opposite side
of the body, often results from lesions in the nondominant
parietal lobe and is not a feature of Gerstmann’s syndrome
(Carpenter, p. 429)

Question 13

The biochemical defect in Refsum’s disease has been identified as defiCiency of what enzyme?

A. Arylsulfatase A
B. Phytanoyl-coenzyme A hydroxylase
C. p~Glucosidase
D. a-Galactosidase
E. Acid ceramidase

Answer 13

A. Arylsulfatase A
**B. Phytanoyl-coenzyme A hydroxylase **
C. p~Glucosidase
D. a-Galactosidase
E. Acid ceramidase

Refsum disease is an autosomal recessive disease
caused by a defiCiency of phytanoyl-coenzyme A hydroxylase and accumulation of phytanic acid in the body. This
disease is unique among the lipidoses because phytanic acid
is not syntheSized in the boely but is obtained exclusively
from the diet. Limiting phytanic acid or its precursor, phytol
(dairy products, ruminant fat, and chlorophyl-containing
foods), from the diet reduces plasma phytanic acid levels.
Plasmapheresis may further help eliminate phytanic acid
from the body in severe cases. Symptoms typically begin in
childhood but in some patients may be delayed until the fifth
decade. Night blindness typically appears first, followed by
limb wealO1ess and gait abnormalities. Some patients may
develop psychiatric symptoms, peripheral neuropathy, pigmentary retinopathy, deafness, cataracts, bone deformities,
or cardiac arrhythmias (Merritt, pp. 514-529, 539-540).

Question 14

A 56-year-old female was diagnosed with paranoid
schizophrenia and hospitalized. She was started on low-dose
risperidone (Risperdal). A few days into her hospitalization,
she complained of worsening abdominal pain. On examination, there was no abdominal rigidity, but fever, leulwcytosis,
diarrhea, hypertension, and tachycardia were noted. Imaging studies of the brain and abdomen were unremarkable

What is the most likely diagnosis?

A. Conversion disorder
B. Schizophrenia with depressive features
C. Acute intermittent porphyria
D. Appendicitis
E. Risperidone-induced infusion syndrome

Answer 14

A. Conversion disorder
B. Schizophrenia with depressive features
**C. Acute intermittent porphyria **
D. Appendicitis
E. Risperidone-induced infusion syndrome

The symptoms of acute intermittent porphyria (AlP) are most commonly gastrointestinal, psychiatric,
and neurologic. The precise etiology remains uncertain, but
large and small nerve fibers, as well as autonomic nerves,
have been shown to be affected. Abdominal pain is the most
common finding, but often occurs concomitantly with a
psychiatric or neurologiC disorder. There is usually no
abdominal rigidity, but fever, leukocytosis, diarrhea, tachycardia, and hypertension are often evident. Appendicitis or
other serious abdominal problems may be difficult to rule
out; in fact, some patients have been subjected to laparotomy for abdominal exploration. The most reliable test to
confirm AlP is the assay for porphobilinogen deaminase
activity in red blood cells, but often a good clinical history
and examination can give clues to the diagnosis. The autonomic manifestations, abdominal pain, and anxiety may be
reversed with propranolol. Hematin has also been shown to
reverse the neuropathy and abdominal pain by suppressing
aminolevulinic acid dehydratase levels. This patient’s history and multiple clinical findings are not consistent with
volume depletion, conversion disorder, appendicitis, or hysteria. Barbiturates, sulfonamides, gabapentin, and hormone
replacement therapy may cause or exacerbate AlP (Merritt,
pp. 549- 551).

Question 15

A 56-year-old female was diagnosed with paranoid
schizophrenia and hospitalized. She was started on low-dose
risperidone (Risperdal). A few days into her hospitalization,
she complained of worsening abdominal pain. On examination, there was no abdominal rigidity, but fever, leulwcytosis,
diarrhea, hypertension, and tachycardia were noted. Imaging studies of the brain and abdomen were unremarkable

The most reliable test or intervention that confirms this
diagnosis would include?

A. Aminolevulinic acid synthase
B. Assay for homocysteine synthetase
C. Assay for porphobilinogen deaminase activity
D. Exploratory celiotomy
E. Discontinuation of the antipsychotic medication with resolution of symptoms

Answer 15

A. Aminolevulinic acid synthase
B. Assay for homocysteine synthetase
**C. Assay for porphobilinogen deaminase activity **
D. Exploratory celiotomy
E. Discontinuation of the antipsychotic medication with resolution of symptoms

The symptoms of acute intermittent porphyria (AlP) are most commonly gastrointestinal, psychiatric,
and neurologic. The precise etiology remains uncertain, but
large and small nerve fibers, as well as autonomic nerves,
have been shown to be affected. Abdominal pain is the most
common finding, but often occurs concomitantly with a
psychiatric or neurologiC disorder. There is usually no
abdominal rigidity, but fever, leukocytosis, diarrhea, tachycardia, and hypertension are often evident. Appendicitis or
other serious abdominal problems may be difficult to rule
out; in fact, some patients have been subjected to laparotomy for abdominal exploration. The most reliable test to
confirm AlP is the assay for porphobilinogen deaminase
activity in red blood cells, but often a good clinical history
and examination can give clues to the diagnosis. The autonomic manifestations, abdominal pain, and anxiety may be
reversed with propranolol. Hematin has also been shown to
reverse the neuropathy and abdominal pain by suppressing
aminolevulinic acid dehydratase levels. This patient’s history and multiple clinical findings are not consistent with
volume depletion, conversion disorder, appendicitis, or hysteria. Barbiturates, sulfonamides, gabapentin, and hormone
replacement therapy may cause or exacerbate AlP (Merritt,
pp. 549- 551).

Question 15

A 56-year-old female was diagnosed with paranoid
schizophrenia and hospitalized. She was started on low-dose
risperidone (Risperdal). A few days into her hospitalization,
she complained of worsening abdominal pain. On examination, there was no abdominal rigidity, but fever, leulwcytosis,
diarrhea, hypertension, and tachycardia were noted. Imaging studies of the brain and abdomen were unremarkable

The abdominal pain may improve dramatically with
what medication?
A. Propranolol
B. Barbiturates
C. SuIfonamides
D. Gabapentin
E. Mercury

Answer 15

**A. Propranolol **
B. Barbiturates
C. SuIfonamides
D. Gabapentin
E. Mercury

The symptoms of acute intermittent porphyria (AlP) are most commonly gastrointestinal, psychiatric,
and neurologic. The precise etiology remains uncertain, but
large and small nerve fibers, as well as autonomic nerves,
have been shown to be affected. Abdominal pain is the most
common finding, but often occurs concomitantly with a
psychiatric or neurologiC disorder. There is usually no
abdominal rigidity, but fever, leukocytosis, diarrhea, tachycardia, and hypertension are often evident. Appendicitis or
other serious abdominal problems may be difficult to rule
out; in fact, some patients have been subjected to laparotomy for abdominal exploration. The most reliable test to
confirm AlP is the assay for porphobilinogen deaminase
activity in red blood cells, but often a good clinical history
and examination can give clues to the diagnosis. The autonomic manifestations, abdominal pain, and anxiety may be
reversed with propranolol. Hematin has also been shown to
reverse the neuropathy and abdominal pain by suppressing
aminolevulinic acid dehydratase levels. This patient’s history and multiple clinical findings are not consistent with
volume depletion, conversion disorder, appendicitis, or hysteria. Barbiturates, sulfonamides, gabapentin, and hormone
replacement therapy may cause or exacerbate AlP (Merritt,
pp. 549- 551).

Question 15

All of the follOWing are characteristic of Diphtheritic
neuropathy EXCEPT?

A. The organism isolated from the laryn .. : and pharYlu is frequently Corynebacterium diphtheriae
B. The organisms often release an endotoxin that can cause myocarditis or asymmetric neuropathy
C. The neuropathy often begins with impaired visual function
D. Diphtheria and the associated neuropathy can be prevented by immunization
E. It often produces a demyelinating neuropathy

Answer 15

A. The organism isolated from the laryn .. : and pharYlu is frequently Corynebacterium diphtheriae
**B. The organisms often release an endotoxin that can cause myocarditis or asymmetric neuropathy **
C. The neuropathy often begins with impaired visual function
D. Diphtheria and the associated neuropathy can be prevented by immunization
E. It often produces a demyelinating neuropathy

Diphtheria infection produces neuropathy in about
20% of infected patients. Corynebacterium diphtheriae
is often isolated from the throat and releases an exotoxin
(not endotOXin) that can cause myocarditis or symmetric
neuropathy. The neuropathy is often characterized by poor
visual accommodation, paresis of throat muscles, quadriparesis, and slow nerve conduction velocities secondary to
demyelinating neuropathy. This disease can be prevented
by immunization and often responds to antibiotics (Merritt,
pp. 620-621).

Question 16

The lesion in Korsakoff’s psychosis often involves what
brain structure?
A. Dorsomedial (DM) nucleus of the thalamus
B. Globose nucleus
C. Amygdala
D. Vermis of the cerebellum
E. Den ta te gyrus

Answer 16

**A. Dorsomedial (DM) nucleus of the thalamus **
B. Globose nucleus
C. Amygdala
D. Vermis of the cerebellum
E. Den ta te gyrus

Although Wernicke’s and Korsakoff’s syndromes are
often described together, these appear to be two distinct entities that result from thiamine deficiency. ‘Wernicke’s
syndrome consists of mental symptoms (global confusional
state), eye movement problems (nystagmus, lateral rectus
palsy, and lateral gaze palsy), and gait atmda , while Korsakoff’s
syndrome is a purely amnestic syndrome usually associated
with lesions in the DM nucleus of the thalamus and mammillary bodies. With treatment (thiamine), ocular abnormalities, nystagmus, and global confusion often improve to
varying degrees, leaving Korsakoff’s amnesia in about 80%
of patients. (Merritt, pp. 924-925).

Question 17

That is the most common clinical feature of neuroborreliosis?
A. Painful sensory radiculitis that appears about 3 weeks after erythema migrans
B. Cranialmononeuropathy
C. Limb paresis
D. Arthralgia
E. Vertigo

Answer 17

**A. Painful sensory radiculitis that appears about 3 weeks after erythema migrans **
B. Cranialmononeuropathy
C. Limb paresis
D. Arthralgia
E. Vertigo

Lyme disease is caused by the tick-borne spirochete
Borrelia. burgdmferi. The most common clinical feature
is painful sensory radiculitis, which often appears about
3 weeks after the erythema migrans. Other problems
include cranial neuropathy (61%), limb weakness (12%),
oculomotor paresis, arthralgias, and cardiomyopathy.
Peripheral nerve biopsy shows perineurial and epineurial
vasculitis and a,’(:onal degeneration. The prognosis is good
after high-dose antibiotic treatment (usually penicillin)
(Merritt, p. 625).

Question 18

Which of the following disorders is not a mitochondrial
DNA abnormality?

A. 1vIitochondriai myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)
B. Myoclonic epilepsy with ragged red fibers (MERFF)
C. Leber’s hereditary optic neuropathy (LHON)
D. Kearns-Sayre syndrome (KSS)
E. Leigh’s disease

Answer 18

A. 1vIitochondriai myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)
B. Myoclonic epilepsy with ragged red fibers (MERFF)
C. Leber’s hereditary optic neuropathy (LHON)
D. Kearns-Sayre syndrome (KSS)
E. Leigh’s disease

MELAS, MERFF, LHON, and I<SS are a group of disorders related to mitochondrial (mt) DNA abnormalities.
Although specific syndromes are often identified by a variety
of signs/symptoms, several clinical manifestations seem to
be prevalent with mtDNA abnormalities and include short
stature, hearing loss, and diabetes mellitus. Lactic acidosis
is the most common laboratory finding, while pathologic
sectioning reveals enlarged mitochondria in muscle fibers,
which forms the basis for ragged red fibers (RRF). Leigh’s
disease is a disorder of mitochondrial metabolism in which
the primary defect involves proteins encoded by nuclear
DNA instead of mitochondrial DNA (Ellison, pp. 457- 465).

Question 19

A 74-year-old male with peripheral vascular disease presents to the emergency room with wild, involuntary
left arm flinging. Magnetic resonance imaging (MRl) of the
brain reveals ventriculomegaly, diffuse cortical atrophy, and
multiple lacunar infarcts.

What structure was likely affected to produce this clinical picture?

A. Lateral globus pallid us
B. Subthalamic nucleus
C. Thalamus
D. Red nucleus
E. Caudate nucleus

Answer 19

A
B
C
D
E

‘Vild, involuntary t1inging of an extremity
may be secondary to a cerebrovascular accident affecting
the subthalamic nucleus and is commonly referred to as
hemiballismus. Normally, glutamatergic projections from
the subthalamic nucleus to the Gpi and SNr suppress the
motor nuclei of the thalamus. After damage to the subthalamic nucleus, the thalamic motor neurons are disinhibited
and provide excessive activation of the motor cortex. The
dopaminergic projections, however, remain intact, providing
a constant and unbalanced activation of motor neurons
throughout the basal ganglia. This is the basis for using
neuroleptic agents, including dopamine receptor blockers
(haloperidol and perphenazine) and presynaptic dopamine
depletors (reserpine and tetrabenazine) to treat this disease
process (Tarsy, p. 9; Pritchard, pp. 330- 331).

Question 20

A 74-year-old male with peripheral vascular disease presents to the emergency room with wild, involuntary
left arm flinging. Magnetic resonance imaging (MRl) of the
brain reveals ventriculomegaly, diffuse cortical atrophy, and
multiple lacunar infarcts.

What is the most likely neurotransmitter abnormality in this patient?
A. Dopamine
B. Glutamate
C. GABA
D. Norepinephrine
E. Acetylcholine

Answer 20

A. Dopamine
B. Glutamate
C. GABA
D. Norepinephrine
E. Acetylcholine

‘Vild, involuntary t1inging of an extremity
may be secondary to a cerebrovascular accident affecting
the subthalamic nucleus and is commonly referred to as
hemiballismus. Normally, glutamatergic projections from
the subthalamic nucleus to the Gpi and SNr suppress the
motor nuclei of the thalamus. After damage to the subthalamic nucleus, the thalamic motor neurons are disinhibited
and provide excessive activation of the motor cortex. The
dopaminergic projections, however, remain intact, providing
a constant and unbalanced activation of motor neurons
throughout the basal ganglia. This is the basis for using
neuroleptic agents, including dopamine receptor blockers
(haloperidol and perphenazine) and presynaptic dopamine
depletors (reserpine and tetrabenazine) to treat this disease
process (Tarsy, p. 9; Pritchard, pp. 330- 331).

Question 21

A 74-year-old male with peripheral vascular disease presents to the emergency room with wild, involuntary
left arm flinging. Magnetic resonance imaging (MRl) of the
brain reveals ventriculomegaly, diffuse cortical atrophy, and
multiple lacunar infarcts.

Treatment may include all of the following EXCEPT?

A. Haloperidol
B. Perphenazine
C. Reserpine
D. Tetrabenazine
E. L-DOPA

Answer 21

A. Haloperidol
B. Perphenazine
C. Reserpine
D. Tetrabenazine
E. L-DOPA

‘Vild, involuntary t1inging of an extremity
may be secondary to a cerebrovascular accident affecting
the subthalamic nucleus and is commonly referred to as
hemiballismus. Normally, glutamatergic projections from
the subthalamic nucleus to the Gpi and SNr suppress the
motor nuclei of the thalamus. After damage to the subthalamic nucleus, the thalamic motor neurons are disinhibited
and provide excessive activation of the motor cortex. The
dopaminergic projections, however, remain intact, providing
a constant and unbalanced activation of motor neurons
throughout the basal ganglia. This is the basis for using
neuroleptic agents, including dopamine receptor blockers
(haloperidol and perphenazine) and presynaptic dopamine
depletors (reserpine and tetrabenazine) to treat this disease
process (Tarsy, p. 9; Pritchard, pp. 330- 331).

Question 22

Vhich of the following about Huntington’s disease is
true?

A. It is a trinucleotide (CAG) repeat disorder that localizes to chromosome 4
B. It is an autosomal recessive condition with incomplete penetrance
C. Primarily a disease affecting the GABA/enkephalin projections from the amygdala to the striatum
D. More common in females than males
E. Has a later onset in successive generations

Answer 22

**A. It is a trinucleotide (CAG) repeat disorder that localizes to chromosome 4 **
B. It is an autosomal recessive condition with incomplete penetrance
C. Primarily a disease affecting the GABA/enkephalin projections from the amygdala to the striatum
D. More common in females than males
E. Has a later onset in successive generations

HD is an autosomal dominant condition with
complete penetrance that varies in symptom onset from
juveniles to late adulthood, with average onset between
35 and 40 years of age. Sporadic cases of I-ID are rare. HD, a trinucleotide repeat (CAG) disorder that localizes to chromosome 4, is more common in males than females and
exhibits anticipation (earlier onset in successive genera -
tions). The abnormal gene product results in protein conformational changes that lead to aggregation in the cytosol
and eventually cellular apoptosis. I-ID primarily affects
the GABl-Venkephalin projections from the striatum to the
external segment of the globus pallidus (indirect pathway),
resulting in thalamic facilitation of motor cortical areas and
hyperkinesia. Haloperidol may be effective in suppressing
abnormal movements early in the disease course, but the disease is inevitably progressive and usually results in death
within 20 years from symptom onset (Merritt, pp. 659- 664,
696- 699).

Question 23

Select one answer with which it is most closely associated: Cloverleaf-shaped skull A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 23

A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull **G. IGeeblattschadel ** H. None of the above ## Footnote Craniosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 24

Select one answer with which it is most closely associated: Tower skull A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 24

A B C D **E** F G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 25

Select one answer with which it is most closely associated: Chiari malformation A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 25

A B C D E **F** G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 26

Select one answer with which it is most closely associated: Metopic synostosis A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 26

**A** B C D E F G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 27

Select one answer with which it is most closely associated: Unilaterally tlattened head A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 27

A B **C** D E F G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 28

Select one answer with which it is most closely associated: Boat-shaped skull A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 28

A **B** C D E F G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 29

Select one answer with which it is most closely associated: Premature sagittal suture closure A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 29

A **B** C D E F G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 30

Select one answer with which it is most closely associated: Bilaterally flattened head A. Trigonocephaly B. Scaphocephaly C. Plagiocephaly D. Brachycephaly E. Oxycephaly F. Lacunar skull G. IGeeblattschadel H. None of the above

Answer 30

A B C **D** E F G H ## Footnote Cra.niosynostosis refers to the premature closure of cranial sutures. It is more common in males than females, and sagittal synostosis accounts for 50% of all cases of craniosynostosis. Sagittal synostosis is identified in a child with an oblong-shaped skull (scaphocephaly or dolichocephaly), which results in an increased AP skull diameter and a narrowed biparietal diameter. There is usually a palpable "keel" along the course of the sagittal suture. Trigonocephaly refers to premature closure of the metopic suture, and results in a wedge-shaped head. Unilateral suture closure (coronal or lambdoid) often results in a misshapen and unilaterally tlattened head (plagiocephaly), while premature bilateral coronal or lambdoid suture closure often results in a broad biparietal skull diameter or brachycephaly. Other forms of craniosynostosis can result from premature closure of all the sutures, which can result in a tower-shaped skull (oxycephaly) or a grossly abnormal-appearing skull with a cloverleaf shape (Kleeblattschiidel). Lacunar skull can be seen in patients with Chiari II malformation, and does not result from premature closure of the cranial sutures. The indications for surgery with craniosynostosis are usually for intracranial hypertension (VP shunt) and cosmetic deformity (Wilkins, pp. 3673-3679; Merritt, pp. 491- 492).

Question 31

A 34-year-old male with HIV presents to the emergency room with headaches, confusion, and a right homonymous hemianopia . Tl-weighted MRI of the brain with contrast shows a 3- by 4-cm nonenhancing left parieto-occipital lesion, which exhibits increased signal on FLAIR IvfRI images. What is the most likely etiology of this lesion ? A. A measles infection that disseminated to the brain B. A monophasic autoimmune attack by T cells against myelin basic protein C. Perivenular inflammation and demyelination D. Reactivation of a papovavirus against oligodendrocytes in the subcortical white matter E. None of the above

Answer 31

A B C **D** E ## Footnote Progressive multifocalleukoencephalopathy (PML) is a subacute demyelinating disease that results from reactivation of a papovavirus (JC virus) in immunocompromised patients. The reactivated JC virus infects oligodendrocytes, and results in multifocal areas of demyelination predominantly in the subcortical white matter. PML affects 4% of AIDS patients and has been associated with other illnesses characterized by defective cell-mediated immunity, such as lymphomas and leukemias. The lesions of PML are often parieto-occipital, nonenhancing, and exhibit increased signal on T2 and FLAIR MRI with no mass effect. Symptoms of PML depend on the location of lesions and include hemiparesis, visual field cuts, sensory changes, and eventually dementia. The diagnosis of PML is established by biopsy or PCR amplification of JC virus RNA in the CSF. The prognosis of PML is poor, and approximately 80% of patients die within 9 months of symptom onset. Choices A, B, and C are associated with subacute sclerosing panencephalitis (A) and acute disseminated encephalomyelitis (B and C) (Merritt, pp. 151- 156)

Question 32

Patients who undergo rapid correction of hyponatremia are at risk of developing which of the following problems that can lead to seizures, dysarthria, pseudobulbar palsy, coma, and/or death? A. Marchiafava-Bignami disease B. Central pontine myelinolysis C. Acute disseminated leukoencephalopathy D. Krabbe's disease E. Alexander's disease

Answer 32

A. Marchiafava-Bignami disease **B. Central pontine myelinolysis ** C. Acute disseminated leukoencephalopathy D. Krabbe's disease E. Alexander's disease ## Footnote Central pontine myelinolysis (CPM) is an acute demyelinating condition that primarily affects the pons, although 10% of cases have concomitant extrapontine myelinolysis. CPlvI most commonly occurs in alcoholic or malnourished patients who experience rapid correction of chronic hyponatremia. Neurologic symptoms usually occur 2 to 3 days after correction of the hyponatremia and vary extenSively. Symptoms include seizures, dysarthria , dysphagia , pseudobulbar palsy, behavioral abnormalities, hyperreflexia , quadriplegia, and coma. Patients may also be completely asymptomatic with CPM. In symptomatic patients, the course is usually rapid and progresses to death within days to weeks of symptom onset. The incidence of CPII'1 is extremely low if the serum sodium is corrected by no more than 12 mmollL in 24 hours. MRI, brainstem auditory evoked potentials, and CSF studies (increased MBP and protein levels) can all assist in the diagnosis of CPM. Acute disseminated leukoencephalomyelitis, Krabbe's disease, Alexander's disease, and Marchiafava-Bignami disease do not result from rapid correction of hyponatremia (Merritt, pp. 794- 796).

Question 33

A 16-year-old male is trying out for his high school soccer team and noticed severe leg cramps that were relentless in nature. Although he has experienced this problem in the past during exercise, his cramps were never this severe. His neurologic exam was unremarkable, but he was found to have an abnormally deficient level of the enzyme myophosphorylase. What is the likely diagnosis? A. Von Gierke's disease B. Pompe's disease C. McArdle's disease D. Cori's disease E. Late-onset Duchenne's dystrophy

Answer 33

A. Von Gierke's disease B. Pompe's disease **C. McArdle's disease ** D. Cori's disease E. Late-onset Duchenne's dystrophy ## Footnote Glycogen storage diseases are primarily autosomal recessive disorde rs that result from deficiencies of the enzymes il1volved with the metabolism of glucose and glycogen. There are more than 12 different types of glycogenoses; they affect different tissues depending on the expression of the defective enzyme. Type I glycogenosis (Von Gierke's disease) is characterized by deficiencies of glucose-6-phosphatase and primarily affects the liver. Patients with Von Gierke's disease present with hepatomegaly and experience severe episodes of hypoglycemia. Type II glycogenosis (Pompe's disease) results from deficiencies of the enzyme acid maltase a-glucosidase and has three different forms. The infantile type of Pompe's disease results in hypotonia, macroglossia , hepatomegaly, cardiomegaly, and death by 2 years of age secondary to ca rdiac failure. The juvenile and adulthood variants of Pompe's disease are less severe and present with myopathy. Type III glycogenosis (debranching enzyme defiCiency or Cori's disease) is rare and results in hepatomegaly, seizures, and growth retardation in children secondary to deficiencies of the debranching enzyme amylo-l,6-glucosidase. Type V glycogenosis (McArdle's disease) is a more benign condition that results in muscle cramps and occaSionally myoglobinuria during intense exercise due to deficiencies of the enzyme myophosphorylase. Type VII glycogenosis (Tauri's disease) presents with myalgias, cramps, and early fatigue with exercise (similar to McArdle's disease) in children and is secondary to

Question 34

# * A 16-year-old male is trying out for his high school soccer team and noticed severe leg cramps that were relentless in nature. Although he has experienced this problem in the past during exercise, his cramps were never this severe. His neurologic exam was unremarkable, but he was found to have an abnormally deficient level of the enzyme myophosphorylase. What is most likely to be seen on urinalysis in this patient? A. Jyoglobinuria B. Salt wasting C. Hyperglycemia D. Hypercalcemia E. Hyperphosphatemia

Answer 34

**A** B C D E ## Footnote Glycogen storage diseases are primarily autosomal recessive disorde rs that result from deficiencies of the enzymes il1volved with the metabolism of glucose and glycogen. There are more than 12 different types of glycogenoses; they affect different tissues depending on the expression of the defective enzyme. Type I glycogenosis (Von Gierke's disease) is characterized by deficiencies of glucose-6-phosphatase and primarily affects the liver. Patients with Von Gierke's disease present with hepatomegaly and experience severe episodes of hypoglycemia. Type II glycogenosis (Pompe's disease) results from deficiencies of the enzyme acid maltase a-glucosidase and has three different forms. The infantile type of Pompe's disease results in hypotonia, macroglossia , hepatomegaly, cardiomegaly, and death by 2 years of age secondary to ca rdiac failure. The juvenile and adulthood variants of Pompe's disease are less severe and present with myopathy. Type III glycogenosis (debranching enzyme defiCiency or Cori's disease) is rare and results in hepatomegaly, seizures, and growth retardation in children secondary to deficiencies of the debranching enzyme amylo-l,6-glucosidase. Type V glycogenosis (McArdle's disease) is a more benign condition that results in muscle cramps and occaSionally myoglobinuria during intense exercise due to deficiencies of the enzyme myophosphorylase. Type VII glycogenosis (Tauri's disease) presents with myalgias, cramps, and early fatigue with exercise (similar to McArdle's disease) in children and is secondary to

Question 35

A 16-year-old male is trying out for his high school soccer team and noticed severe leg cramps that were relentless in nature. Although he has experienced this problem in the past during exercise, his cramps were never this severe. His neurologic exam was unremarkable, but he was found to have an abnormally deficient level of the enzyme myophosphorylase. What is the mode of transmission of this disease process ? A. J\<{yoglobinuria B. Salt wasting C. Hyperglycemia D. Hypercalcemia E. Hyperphosphatemia

Answer 35

A **B ** C D E ## Footnote Glycogen storage diseases are primarily autosomal recessive disorde rs that result from deficiencies of the enzymes il1volved with the metabolism of glucose and glycogen. There are more than 12 different types of glycogenoses; they affect different tissues depending on the expression of the defective enzyme. Type I glycogenosis (Von Gierke's disease) is characterized by deficiencies of glucose-6-phosphatase and primarily affects the liver. Patients with Von Gierke's disease present with hepatomegaly and experience severe episodes of hypoglycemia. Type II glycogenosis (Pompe's disease) results from deficiencies of the enzyme acid maltase a-glucosidase and has three different forms. The infantile type of Pompe's disease results in hypotonia, macroglossia , hepatomegaly, cardiomegaly, and death by 2 years of age secondary to ca rdiac failure. The juvenile and adulthood variants of Pompe's disease are less severe and present with myopathy. Type III glycogenosis (debranching enzyme defiCiency or Cori's disease) is rare and results in hepatomegaly, seizures, and growth retardation in children secondary to deficiencies of the debranching enzyme amylo-l,6-glucosidase. Type V glycogenosis (McArdle's disease) is a more benign condition that results in muscle cramps and occaSionally myoglobinuria during intense exercise due to deficiencies of the enzyme myophosphorylase. Type VII glycogenosis (Tauri's disease) presents with myalgias, cramps, and early fatigue with exercise (similar to McArdle's disease) in children and is secondary to

Question 36

All of the following are features of Friedreich's ataxia EXCEPT? A. It is an autosomal recessive trinucleotide repeat (GAA) disorder involving the frataxin gene B. Onset of symptoms usually occurs by the age of 10 to 15 years C. Often results in the degeneration of the posterior columns and spinocerebellar tracts D. Cerebellar and cerebral cortical ischemic changes can often be associated with concomitant cardiomyopathy E. With effective treatment, the disease progression associated with neuronal degeneration can be halted about 60% of the time , although heart failure is typically refractory to therapy

Answer 36

A B C D **E** ## Footnote Friedreich's ataxia (FA) is an autosomal recessive trinucleotide repeat (GAA) disorder that involves repeat expansion in the frataxin gene, which encodes a mitochondrial protein and is located on chromosome 9q. The onset of symptoms with FA usually occurs by the age of 10 to IS years; it is usually fatal by the fifth to sL'-:th decades of life. FA results in degeneration of the posterior columns, spinocerebellar tracts, corticospinal tracts, and Clarke's nucleus in the spinal cord. The medulla (vestibular, cochlear, gracile, and accessory cuneate nuclei), subthalamic nucleus, and pallidum also exhibit evidence of gliosis. Symptoms of FA include ata.xic gait, dysarthria , areflexia, lower limb weakness, and loss of vibratory sense and proprioception . Some patients with 1<"1\ also develop cardiomyopathy, which can lead to cerebellar and cerebral cortical ischemic changes. FA is associated with the development of peripheral neuropathy as well, with concomitant degeneration of the dorsal root ganglia. Orthopedic deformities, such as hammertoes, kyphoscoliosis, and pes cavus are also common. There is no effective treatment for FA (Merritt, pp. 645-646).

Question 37

Lesch-Nyhan syndrome is an X-linked recessive disorder that results from a deficiency of what enzyme? A. Glucose transporter-l protein B. Phenylalanine hydroxylase C. Hypoxanthine-guanine phosphoribosyltransferase D. Cystathionine synthase E. Purine decarboxylase

Answer 37

A. Glucose transporter-l protein B. Phenylalanine hydroxylase **C. Hypoxanthine-guanine phosphoribosyltransferase ** D. Cystathionine synthase E. Purine decarboxylase ## Footnote Lesch-Nyhan syndrome (LNS) is an X-linked recessive disorder of purine metabolism that results from deficiencies of the enzyme hypoxanthine-guanine phosphoribosyltransferase (HPRT). Levels of uric acid are increased in LNS secondary to increased purine metabolism, and urate deposition can result in severe nephropathy and gout. Patients with LNS exhibit mental retardation, choreoathetosis, spasticity, self-mutilating behavior, and usually die from renal failure in the second or third decade. Glucose transporter protein syndrome (GTPS) results from deficiencies of the glucose transporter-l protein (GLUT-I), which is responsible for the facilitative transport of glucose across the blood-brain barrier. Patients with GTPS exhibit decreased CSF glucose levels (hypoglycorrhachia), seizures, developmental delay, microcephaly, at~Lxia, and hypotonia. Phenylketonuria is an autosomal recessive disorder resulting from phenylalanine hydroxylase defiCiency, and cystathionine ~-synthase deficiency results in homocystinuria (Merritt, pp. 512- 513).

Question 38

Select one answer with which it is most closely associated: May be secondary to weakness/paralysis of the pretibial and peroneal muscles A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 38

A B C **D** E F G H

Question 39

Select one answer with which it is most closely associated: Parkinson's disease A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 39

A **B** C D E F G H

Question 40

Select one answer with which it is most closely associated:Associated with circumduction of the leg A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 40

A B **C** D E F G H

Question 41

Select one answer with which it is most closely associated: Chronic or progressive Wohlfart-Kugelberg-Welander syndrome A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 41

A B C D **E** F G H

Question 42

Select one answer with which it is most closely associated: May accompany a lesion in the thoracic spine A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 42

**A** B C D E F G H

Question 43

Select one answer with which it is most closely associated: Often seen with foot drop A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 43

A B C **D** E F G H

Question 44

Select one answer with which it is most closely associated: Internal capsule infarct A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 44

A B **C** D E F G H

Question 45

Select one answer with which it is most closely associated: Anterior and lateral borders of shoe sole often worn clown A. Scissoring gait B. Festinating gait C. Hemiplegic gait D. Steppage gait E. Waddling gait F. Reeling gait G. Toppling gait H. None of the above

Answer 45

A B **C** D E F G H

Question 46

A 34-year-old female suddenly develops a left hemiparesis. She experienced a deep venous thrombosis in her left leg about 4 years earlier. The most likely cause of this patient's deficit is A. Atrial fibrillation B. Metastatic brain tumor C. Multiple sclerosis D. Lupus anticoagulant E. Atrial septal defect

Answer 46

A. B C **D** E

Question 47

A 34-year-old female suddenly develops a left hemiparesis. She experienced a deep venous thrombosis in her left leg about 4 years earlier. A nonspecific laboratory feature of this disorder may include which one of the following? A. Prolonged PT and PTT that does not reverse when the patient's plasma is mL;:ed with normal plasma B. Oligoclonal bands in the CSF C. Abnormal dystrophin gene D. Elevation of IgE antibodies in the serum E. None of the above

Answer 47

**A. Prolonged PT and PTT that does not reverse when the patient's plasma is mL;:ed with normal plasma ** B. Oligoclonal bands in the CSF C. Abnormal dystrophin gene D. Elevation of IgE antibodies in the serum E. None of the above

Question 48

A 34-year-old female suddenly develops a left hemiparesis. She experienced a deep venous thrombosis in her left leg about 4 years earlier. Which of the following can confirm the diagnosis? A. Demonstrating an inhibitor reaction within the clotting system using the thromboplastin inhibition test (TTl) and dilute Russel venom viper assay B. Confirming a defect in factor V C. Multiple lesions in the deep white matter on magnetic resonance imaging (MRl) D. Noting the presence of an atrial septal defect on echocardiography E. Excessive bleeding after shaving

Answer 48

**A. Demonstrating an inhibitor reaction within the clotting system using the thromboplastin inhibition test ** (TTl) and dilute Russel venom viper assay B. Confirming a defect in factor V C. Multiple lesions in the deep white matter on magnetic resonance imaging (MRl) D. Noting the presence of an atrial septal defect on echocardiography E. Excessive bleeding after shaving

Question 49

A 34-year-old female suddenly develops a left hemiparesis. She experienced a deep venous thrombosis in her left leg about 4 years earlier. Optimal therapy for this disease may include 1. Maintaining an international normalized ratio (INR) of at least 3.0 with warfarin 2. Corticosteroids 3. Antiplatelet agents 4. Plasmapheresis A. 1,2, and 3 are correct B. 1 and3 are correct C. 2 and4 are correct D. Only 4 is correct E. All of the above

Answer 49

**A. 1,2, and 3 are correct ** B. 1 and3 are correct C. 2 and4 are correct D. Only 4 is correct E. All of the above

Question 50

A previously healthy 72-year-old female develops auditory hallucinations and depression. The patient has difficulty providing the details but believes that her son is speaking to her. She is not cooperative during the interview, but the physical examination is unremarkable. She takes multivitamins and an aspirin each day. Which of the following is the most likely diagnosis? A. Hyperthyroidism B. Complex partial seizures C. Alzheimer's disease D. Multi-infarct dementia E. Hyperparathyroidism

Answer 50

A. Hyperthyroidism B. Complex partial seizures **C. Alzheimer's disease** D. Multi-infarct dementia E. Hyperparathyroidism

Question 51

A previously healthy 72-year-old female develops auditory hallucinations and depression. The patient has difficulty providing the details but believes that her son is speaking to her. She is not cooperative during the interview, but the physical examination is unremarkable. She takes multivitamins and an aspirin each day. The most common way to obtain this diagnosis includes A. Obtaining a thyroid-stimulating hormone (TSH) level B. Electroencephalography (EEG) C. Detailed history and physical examination D. Brain biopsy E. Obtaining an angiotensin-converting enzyme (ACE) level

Answer 51

A. Obtaining a thyroid-stimulating hormone (TSH) level B. Electroencephalography (EEG) **C. Detailed history and physical examination** D. Brain biopsy E. Obtaining an angiotensin-converting enzyme (ACE) level

Question 52

A previously healthy 72-year-old female develops auditory hallucinations and depression. The patient has difficulty providing the details but believes that her son is speaking to her. She is not cooperative during the interview, but the physical examination is unremarkable. She takes multivitamins and an aspirin each day. A treatment regimen for the auditory hallucinations could include A. Tacrine B. Donepezil C. Vitamin E D. Haloperidol E. All of the above

Answer 52

A. Tacrine B. Donepezil C. Vitamin E **D. Haloperidol ** E. All of the above

Question 53

A 45-year-old lawyer complains of difficulty holding and using his pen. He notes the development of right arm and hand spasms for the past 3 months only when writing. Physical examination is unremarkable. What is the most likely etiology for this patient's signs/symptoms? A. Early Parkinson's disease B. Athetosis C. C7 radiculopathy D. Dystonia E. Carpal tunnel syndrome

Answer 53

A. Early Parkinson's disease B. Athetosis C. C7 radiculopathy **D. Dystonia** E. Carpal tunnel syndrome ## Footnote Writer's cramp is a focal dystonia of unlmown etiology. Patients develop sustained muscle contractions that yield abnormal postures or twisting movements when attempting to write. It differs from athetosis by the persistent nature of the abnormal posture or movement. It can be focal (writer's cramp), segmental (e.g., face), or rarely multifocal (e.g., face and leg). Dystonia can also be either primary (inherited) or secondary (stroke, toxin, medication, etc.). The medical treatment is variable, often empiric, and can include local botulinum toxin injections, anticholinergics, benzodiazepines, anticonvulsants, lithium, reserpine, baclofen, and levodopa. Micrographia can be seen with Parkinson's disease but is often accompanied by signs of rigidity, tremor, and bradyltinesia. Carpal tunnel syndrome is due to pressure on the median nerve and is associated with numbness, wealmess, and pain. A cervical radiculopathy rarely if ever presents with the findings seen in this patient; instead, there is pain, weakness, numbness, and retlex changes in the distribution of the affected nerve (Merritt, pp. 669- 677).

Question 54

Select one answer with which it is most closely associated: Antibody isolated with small cell lung carcinoma; sensory neuropathy, encephalomyelitis A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 54

**A** B C D E F G H

Question 55

Select one answer with which it is most closely associated: Lambert-Eaton myasthenic syndrome A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 55

A B C D **E ** F G H

Question 56

Select one answer with which it is most closely associated: Opsoclonus-myoclonus syndrome A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 56

A **B** C D E F G H

Question 57

Select one answer with which it is most closely associated: Reacts with Purkinje cells of the cerebellum A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 57

A B C D E **F** G H

Question 58

Select one answer with which it is most closely associated: Polymyositis A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 58

A B **C** D E F G H

Question 59

Select one answer with which it is most closely associated: Hodgkin disease A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 59

A B C D E **F** G H

Question 60

Select one answer with which it is most closely associated: Antibody primarily found in patients with testicular cancer A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 60

A B C D E F **G** H

Question 61

Select one answer with which it is most closely associated: Retinal degeneration A. Anti-I-Iu antibody B. Anti-Ri antibody C. A.nti-Jo antibody D. Anti-Yo antibody E. Anti-VGCC antibody F. Anti-Tr antibody G. Anti-Ta antibody H. None of the above

Answer 61

A **B** C D E F G H

Question 62

A mother brings her 4-month-old male infant to your office after witnessing an episode characterized by sudden extensor spasms involving the head, trunk, and limbs. The EEG is most likely to show A. 3-Hz spike-wave discharges B. Periodic lateralizing epileptiform discharges (PLEDs) C. I-Iypsarrhythmia D. Generalized slowing E. 4- to 6-Hz spike waves

Answer 62

A. 3-Hz spike-wave discharges B. Periodic lateralizing epileptiform discharges (PLEDs) **C. I-Iypsarrhythmia ** D. Generalized slowing E. 4- to 6-Hz spike waves

Question 63

Increased I-I-reflex latencies in conjunction with normal F wave latencies localize lesions to what location? A. Neuromuscular junction B. Anterior horn cells of the spinal cord C. Dorsal roots D. Ventral roots E. None of the above

Answer 63

A. Neuromuscular junction B. Anterior horn cells of the spinal cord **C. Dorsal roots ** D. Ventral roots E. None of the above ## Footnote The F wave and H reflex evaluate certain aspects of nerve conduction. ,"Vhereas sensory nerve action potentials (SNAP) and compound muscle action potentials (CMAP) are best at evaluating distal nerves, the F wave and H retlex are the two most commonly used methods in evaluating the proximal portions of nerves. The F wave (F response) measures the entire length of the nerve, including the ventral root. It results from supramaximal stimulus of distal motor nerves with impulse propagation in an antidromic direction. The stimulus travels proximally up the motor nerve and stimulates the anterior horn 1110tor neurons. The variable backfiring of nonrefractory anterior horn cells then results in impulse propagation back down the ventral root and motor nerve to the distal electrode. F-wave latencies are most sensitive for disorders causing generalized or multifocal demyelination (GBS or extensive plexopathies). This is because any focal conduction slowing is diluted by the normal conduction velocity over most of the F-wave pathway. The H retlex is the electrical equivalent of the stretch retlex and is obtained with submaximal stimulation of the median and tibial nerves. The H retlex therefore involves an afferent (sensory) limb and an efferent (motor) limb, similar to monosynaptic retlex arcs. The I-I retlex exhibits increased latency in proximal neuropathies and radiculopathies (Le., C6, C7, or Sl root lesions). Increased I-I-reflex latencies in conjunction with normal F-wave latencies localize lesions to the d

Question 64

All of the following are characteristic of demyelinating neuropathies on motor nerve conduction studies EXCEPT? A. Prolonged distal latency B. Decreased segmental velocity C. Normal or slightly decreased evoked response amplitude D. Decreased F wave E. Temporal dispersion

Answer 64

A. Prolonged distal latency B. Decreased segmental velocity C. Normal or slightly decreased evoked response amplitude **D. Decreased F wave** E. Temporal dispersion ## Footnote Nerve conduction studies (NCSs) vary in conditions that result in either demyelination or a;wnal degeneration. Conduction velocity, amplitude of evoked response, latency (latency from the stimulus to recording electrodes), and duration of response all provide information about the integrity of 1110tor and sensory nerves. NCSs of the sensory nerves generate a sensory nerve action potential (SNAP), while NCSs of 1110tor nerves generate a compound muscle action potential (CMAP). Refer to Table 3.66A for summary of electrophysiologic findings with axonal degeneration and demyelinating neuropathies (Merritt, pp. 73- 75; Geyer, pp. 226-227; Youmans, pp. 3851- 3855).

Question 65

All of the following are characteristics of normal single-motor-unit potentials on electromyography (EMG) EXCEPT? A. Duration of 5- 15 ms B. Two to four phases C. 0.5-3 mV D. Fibrillation potentials E. Insertional activity

Answer 65

A. Duration of 5- 15 ms B. Two to four phases C. 0.5-3 mV **D. Fibrillation potentials** E. Insertional activity ## Footnote Normal muscle potentials appear as waveforms with a duration of 5 to 15 ms, 2 to 4 phases, and an amplitude of 0.5 to 3 mV. Fibrillation potentials are abnormal, involuntary contractions of single muscle fibers that cannot be seen through the sltin . They indicate reinnervation of muscle fibers from a variety of causes. Positive sharp waves are similar to fibrillation potentials and appear on EMG as a downward wave after needle insertion, which is indicative of needle irritation of denervated muscle fibers. Insertional activity is the discharge of a single muscle fiber during insertion of the EMG needle and does not necessarily indicate abnormality unless significantly increased activity is seen. Increased insertional activity can also indicate irritable muscle fibers from denervation. Polyphasic units (greater than four phases) are abnormal, and can be seen in both neurogenic and myogenic disorders. In summary, with neurogenic disorders, motor units appear of longer duration and higher amplitude than normal potentials and are usually polyphasic. Myopathic potentials are just the opposite, with shorter durations and smaller amplitudes than normal potentials and are also usually polyphasic. Fibrillations, positive sharp waves, and increased insertional activity can be seen with denervated muscle (Merritt, pp. 75-76; Youmans, pp. 3856- 3857).

Question 66

Select one or more than one answer with which it is most closely associated: Sleep spindles and K complexes A. Stage 1 B. Stage 2 C. Stage 3 D. Stage 4 E. Rapid eye movement (REId) F. None of the above

Answer 66

A **B ** **C** D E F

Question 67

Select one or more than one answer with which it is most closely associated: > 50% delta waves A. Stage 1 B. Stage 2 C. Stage 3 D. Stage 4 E. Rapid eye movement (REId) F. None of the above

Answer 67

A B C **D** E F

Question 68

Select one or more than one answer with which it is most closely associated: Transition from C/. waves to slow low-voltage activity A. Stage 1 B. Stage 2 C. Stage 3 D. Stage 4 E. Rapid eye movement (REId) F. None of the above

Answer 68

**A** B C D E F

Question 69

Select one or more than one answer with which it is most closely associated: Obstructive sleep apnea can be associated with this stage of sleep A. Stage 1 B. Stage 2 C. Stage 3 D. Stage 4 E. Rapid eye movement (REId) F. None of the above

Answer 69

A B C D **E** F

Question 70

Select one or more than one answer with which it is most closely associated: Night terrors A. Stage 1 B. Stage 2 C. Stage 3 D. Stage 4 E. Rapid eye movement (REId) F. None of the above

Answer 70

A B **C D** E F

Question 71

Select one or more than one answer with which it is most closely associated: The presence of this stage of sleep during daytime electroencephalography (EEG) suggests sleep deprivation , alcohol withdrawal, or narcolepsy A. Stage 1 B. Stage 2 C. Stage 3 D. Stage 4 E. Rapid eye movement (REId) F. None of the above

Answer 71

A B C D **E** F

Question 72

This electroencephalographic (EEG) finding (Figure 3.74Q) is most consistent with what abnormality? 4.A. Creutzfeldt-Jakob disease B. Hepatic encephalopathy C. Massive cerebral infarction D. Focal motor seizures E. Brain death

Answer 72

A **B** C D E ## Footnote Triphasic waves are a type of generalized, pseudoperiodic pattern consisting of sharp discharges occurring at typical frequencies of 1 to 2 per second. This is a nonspecific pattern and has to be distinguished from other generalized periodic patterns (CJD, status epilepticus, and hypoxicischemic injury). Approximately 50% of patients with triphasic waves have hepatic encephalopathy; the other half have other toxic-metabolic encephalopathies, including renal failure. Creutzfeldt-Jalcob disease is characterized by generalized periodic sharp waves, an acute destructive cerebral lesion (stroke) often results in periodic lateralizing epileptiform discharges (PLEDs) on EEG, and brain death is marked by no activity on EEG (Merritt, pp. 64-65).

Question 73

All of the following are characteristics of narcolepsy EXCEPT? A. Associated with excessive daytime sleepiness, sleep paralysis, cataplexy, and hypnagogic hallucinations B. Narcoleptics may experience paralysis of voluntary muscles during the initial and terminal periods of the sleep cycle, which often involve the extraocular muscles C. Hypnagogic hallucinations often occur at the beginning of the sleep cycle D. May exhibit decreased levels of orexin E. Associated with the DR2 allele

Answer 73

A. Associated with excessive daytime sleepiness, sleep paralysis, cataplexy, and hypnagogic hallucinations **B. Narcoleptics may experience paralysis of voluntary muscles during the initial and terminal periods of the sleep cycle, which often involve the extraocular muscles ** C. Hypnagogic hallucinations often occur at the beginning of the sleep cycle D. May exhibit decreased levels of orexin E. Associated with the DR2 allele ## Footnote Narcolepsy is a sleep disorder characterized by excessive daytime sleepiness, sleep paralysis, cataplexy, and hypnagogic hallucinations. Narcolepsy affects males and females equally, is associated with the DR2 allele, and usually presents between the ages of 15 and 30 years. Patients with narcolepsy experience increased daytime sleepiness and irresistible sleep attacks during the day that last between 5 and 30 minutes. Patients with narcolepsy sleep more frequently than normal people, but the amount of total sleep is the same in narcoleptics and healthy adults. Narcoleptics also display attacks of cataplexy, which is the abrupt loss of muscle tone and is often precipitated by emotion. Cataplexy reflects RE~I'! sleep paralysis during periods of normal wakefulness. RE~I'! periods occur early in the sleep cycles of patients with narcolepsy (sleep-onset REM), and this is diagnostic of the condition. Narcoleptics may experience paralysis of voluntary muscles (sleep paralysis) during the initial and terminal periods of the sleep cycle, often with sparing of the extraocular muscles. Hypnagogic hallucinations occur at the beginning of the sleep cycle in narcoleptics; these hallucinations can be auditory or visual. Abnormalities in the hypothalamic projecting system are common in narcolepsy, and patients with this condition exhibit decreased levels of orexin. The trea tment of narcolepsy involves adrenergic stimulants, such as methylphenidate (Ritalin), pemoline, and amphetamines. Cataplexy can often be effectively controlled with antidepressants (tricyclics and 88RIs), which suppress RE~I'! sleep (Merritt, pp. 843-844)

Question 74

The most likely clinical finding associated with the EEG (Figure 3.76- 3. 78Q) in this lO-year-old girl would include? A. Drop attacks B. Infantile spasms C. Staring spells D. Oral-alimentaryautisms E. Myoclonic jerks

Answer 74

A B **C** D E ## Footnote The absence seizure is characterized by a generalized, symmetric, and synchronous 3-Hz spikeslow-wave discharge. This is diagnostic of an absence seizure and is best treated with medications such as ethosuximide (Zarontin) or sodium valproate (Depakote). The common activating procedures usually performed on patients with suspected seizures are hyperventilation, photic stimulation, and sleep. As soon as 3-Hz spike-and-wave bursts stop and the seizure ceases, the EEG returns to its interictal state immediately with no postictal depression or slowing (Merritt, p. 66; Rolak, pp. 365- 366).

Question 75

In general, what seizure-activating procedures can be employed to induce seizure activity? A. Hyperventilation B. Sleep deprivation C. Photic stimulation D. 1 and 3 only E. All of the above

Answer 75

A. Hyperventilation B. Sleep deprivation C. Photic stimulation D. 1 and 3 only **E. All of the above** ## Footnote The absence seizure is characterized by a generalized, symmetric, and synchronous 3-Hz spikeslow-wave discharge. This is diagnostic of an absence seizure and is best treated with medications such as ethosuximide (Zarontin) or sodium valproate (Depakote). The common activating procedures usually performed on patients with suspected seizures are hyperventilation, photic stimulation, and sleep. As soon as 3-Hz spike-and-wave bursts stop and the seizure ceases, the EEG returns to its interictal state immediately with no postictal depression or slowing (Merritt, p. 66; Rolak, pp. 365- 366).

Question 76

As soon as the seizure stops in this patient, the EEG activity is expected to A. Return to the interictal state immediately with no postictal depression or slowing B. Return to the interictal state after a brief period of lateralizing discharges C. Return to the interictal state after a brief period of paroxysmal bi-occipital activity D. Resume with normal mu rhythm often seen with this disorder E. Return to the interictal state with marked depression of background activity in all regions

Answer 76

**A. Return to the interictal state immediately with no postictal depression or slowing ** B. Return to the interictal state after a brief period of lateralizing discharges C. Return to the interictal state after a brief period of paroxysmal bi-occipital activity D. Resume with normal mu rhythm often seen with this disorder E. Return to the interictal state with marked depression of background activity in all regions ## Footnote The absence seizure is characterized by a generalized, symmetric, and synchronous 3-Hz spikeslow-wave discharge. This is diagnostic of an absence seizure and is best treated with medications such as ethosuximide (Zarontin) or sodium valproate (Depakote). The common activating procedures usually performed on patients with suspected seizures are hyperventilation, photic stimulation, and sleep. As soon as 3-Hz spike-and-wave bursts stop and the seizure ceases, the EEG returns to its interictal state immediately with no postictal depression or slowing (Merritt, p. 66; Rolak, pp. 365- 366).

Question 77

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 77

A B C D **E** F G ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V(Merritt, pp. 68- 69).

Question 78

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 78

** A** B C D E F G ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V (Merritt, pp. 68- 69).

Question 79

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 79

A B V D E **F** G ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V (Merritt, pp. 68- 69).

Question 80

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 80

A B **C** D E F G ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V (Merritt, pp. 68- 69).

Question 81

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 81

A **B** C D E F G ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V (Merritt, pp. 68- 69).

Question 82

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 82

A B C D E F **G** ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V (Merritt, pp. 68- 69).

Question 83

The questions below relate to brain-stem auditory evoked potentials. Select one answer with which it is most closely associated: A. Wave I B. Wave II C. Wave III D. Wave IV E. Wave V F. Wave VI G. Wave VII

Answer 83

A B C **D** E F G ## Footnote Brainstem auditory evoked potentials (BAEPs) are elicited with specific auditory stimuli and result from several important components of the auditory pathway. There are generally seven peaks in the BAEP, which result from the auditory nerve (wave I), cochlear nuclei (wave II), superior olive or trapezoid body (wave III), lateral lemniscus (wave IV), inferior colliculus (wave V) , medial geniculate body (wave VI), and auditory radiations (wave VII). BilliPs are usually preserved with metabolic abnormalities and exhibit prolonged latencies with structural lesions of the brainstem or auditory nerves. BAEPs are abnormal in 90% of patients with acoustic neuromas and usually consist of delayed conduction from CN VIII to the lower pons (prolongation of waves I to III). BilliPs are also abnormal in 33% of multiple sclerosis patients and usually involve increased interpeak latency of waves III to V (Merritt, pp. 68- 69).

Question 84

How can one attempt to normalize the rhythm depicted by this EEG (Figure 3.86Q)? A. Dilantin administration B. Surgical procedure C. Phenobarbital administra tion D. ACTH administration E. This rhythm has classically been unresponsive to any medical or surgical intervention

Answer 84

A. Dilantin administration **B. Surgical procedure ** C. Phenobarbital administra tion D. ACTH administration E. This rhythm has classically been unresponsive to any medical or surgical intervention ## Footnote A breach rhythm is a rhythmic sharp wave pattern that appears in the region of a skull defect. The skull acts as a "low-pass filter" and screens out high frequencies generated by the cortex. The skull defect allows these higher frequencies to appear. This is the EEG of a man with a history of head trauma and a surgical scar noted within the right frontocentral region (F4 , C4) (Rolal~, pp. 359-360).

Question 85

The primary center for bladder function that synchronizes bladder contraction with urethral sphincter relaxation is believed to reside in what location ? A. Midbrain tegmentum B. Hypothalamus C. Amygdala D. Pons E. Thalamus

Answer 85

A. Midbrain tegmentum B. Hypothalamus C. Amygdala **D. Pons ** E. Thalamus

Question 86

Which of the following bladder abnormalities is most likely to result from acute cauda equina syndrome? A. Detrusor hyperreflexia B. Detrusor areflexia C. Decreased bladder compliance D. Increased urinary flow rate E. None of the above

Answer 86

A. Detrusor hyperreflexia **B. Detrusor areflexia ** C. Decreased bladder compliance D. Increased urinary flow rate E. None of the above

Question 87

All of the following can be used to treat detrusor hyperreflexia EXCEPT? A. Propantheline (Pro-Banthine) B. Oxybutynin (Ditropan) C. Tolterodine (Detrol) D. Flavoxate (Urispas) E. Bethanecol (Urecholine)

Answer 87

A. Propantheline (Pro-Banthine) B. Oxybutynin (Ditropan) C. Tolterodine (Detrol) D. Flavoxate (Urispas) **E. Bethanecol (Urecholine)**

Question 88

The components of the somatosensory evoked potential (SSEP) tracing of the median nerve include all of the followingEXCEPT? A. Brachial plexus (Erb's point) B. Central gray of the spinal cord (Nl3) C. Caudal medial lemniscus (Pl4) D. Rostral brainstem (NI8) E. Brodmann's areas 5 and 7 (N24)

Answer 88

A. Brachial plexus (Erb's point) B. Central gray of the spinal cord (Nl3) C. Caudal medial lemniscus (Pl4) D. Rostral brainstem (NI8) **E. Brodmann's areas 5 and 7 (N24)** ## Footnote Somatosensory evoked potentials (SSEPs) result from stimulation of peripheral nerves (median and posterior tibial) and are generated by components of the dorsal column sensory pathways. The components of the SSEP tracing of the median nerve include Erb's point (brachial plexus), NI3 (central gray of cervical spinal cord), PI4 (caudal medial lemniscus), NIB (rostral brainstem), and N20 (primary sensory cortex), but not Brodmann's areas 5 and 7, which are located posterior to the primary sensory cortex. SSEPs are affected by many different pathological conditions that influence the somatosensory system, including strokes, syringomyelia, spondylosis, subacute combined deficiency, and multiple sclerosis. SSEPs are frequently utilized intraoperatively during neurosurgical procedures to monitor the integrity of the spinal cord. Some anesthetics that suppress SSEPs include benzodiazepines, halothane, and thiopental sodium (Pentothal) (Merritt, p. 70).

Question 89

A 52-year-old male with a history of diabetes mellitus and hypertension presents to a loca l emergency department with left hemiparesis ai1d an evolving infarct involving portions of the right middle cerebral artery (lvrCA) distribution. A four-vessel angiogram of the brain reveals occlusion of the right MCA proximal to the bifurcation. Data obtained from the National Institute of Neurologic Disorders and Stroke (NINDS) trial showed that administering t-PA within how many hours of stroke resulted in improved functional outcome at 3 months and 1 year? A. 3 hours B. 6 hours C. 8 hours D. 12 hours E. 24 hours

Answer 89

**A. 3 hours ** B. 6 hours C. 8 hours D. 12 hours E. 24 hours ## Footnote The safety and efficacy of thrombolytic therapy has been extensively studied in acute stroke. Data obtained from the National Institute of Neurologic Disorders and Stroke (NINDS) trial showed that administering t-PA within 3 hours of symptom onset resulted in improved functional outcome at 3 months and 1 year. The benefit was seen across all stroke types and was not affected by age, sex, or ethnicity. Although symptomatic intracerebral hemorrhage was higher in the t-PA group, there was no difference in mortality at 3 months. Other trials have assessed the safety of other thrombolytic agents with or without the addition of neuroprotective agents with mixed results. Data from the ATLA.l'IJTIS trial have provided further information concerning the efficacy and safety of administering t-PA beyond the 3-hour window established by the NINDS trial. Although the drug was not shown to be effective beyond the 3-hour window, the safety profile was similar to that of the NIlImS trial. This information provides physicians with some flexibility in administering t-PA beyond the 3-hour window, although CHAPTER 3 Neurology Answers 73 most still advoca te its administration in a time frame similar to that established by NINDS (Youmans, pp. 1595-1502).

Question 90

Since other factors can influence velocities, including blood pressure and overall cerebral blood flow, distinguishing vasospastic from hyperemic increases in MCA blood now velocities by transcranial Doppler is best achieved by

Answer 90

A B C **D** E ## Footnote Since other factors can influence velocities, including blood pressure and overall cerebral blood flow , distinguishing vasospastic from hyperemic increases in MCA blood !low velocities by transcranial Doppler is best achieved by measuring the cervical carotid artery velocities in addition to the intracranial blood velocities. A "Lindegaard ratio" of V~I C/VI C" greater than 3 is consistent with vasospasm, as hyperemia is associated with increased velocities in both the MCA and ICA, so that the ratio remains the same. Repeating the Doppler study within 1 hour to confirm the reading, administering a vasodilating agent in conjunction with the Doppler study to evaluate the percentage change in flow and plotting the velocity versus vessel diameter on a logarithmic scale will not differentiate between hyperemia and vasospasm (Youmans, pp. 1850-1851).

Question 91

Match each of the following breathing patterns with the most likely lesion site: Cheyne-Stokes breathing A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 91

**A. Diencephalon/bilateral cerebral hemispheres** B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 92

Match each of the following breathing patterns with the most likely lesion site: Central neurogenic hyperventilation A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 92

A. Diencephalon/bilateral cerebral hemispheres **B. Pons ** C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 93

Match each of the following breathing patterns with the most likely lesion site: Cluster breathing A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 93

A. Diencephalon/bilateral cerebral hemispheres B. Pons **C. I-ligh medulla/lower pons ** D. Medulla E. Low 1l1idbrain/high pons F. None of the above ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 94

Match each of the following breathing patterns with the most likely lesion site: Apneustic breathing A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 94

A. Diencephalon/bilateral cerebral hemispheres **B. Pons ** C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 95

Match each of the following breathing patterns with the most likely lesion site: Ataxic breathing A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 95

A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons **D. Medulla ** E. Low 1l1idbrain/high pons F. None of the above ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 96

Match each of the following breathing patterns with the most likely lesion site: Stertorous breathing A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 96

A B C D E **F** ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 97

Match each of the following breathing patterns with the most likely lesion site: Ondine's curse A. Diencephalon/bilateral cerebral hemispheres B. Pons C. I-ligh medulla/lower pons D. Medulla E. Low 1l1idbrain/high pons F. None of the above

Answer 97

A B C **D** E F ## Footnote Cheyne-Stokes respiration consists of briefs periods of hyperpnea alternating with even shorter periods of apnea and often results from large supratentorial lesions affecting either the cerebral hemispheres or diencephalon . The respiratory drive with this respiratory pattern is highly dependent on the pC Oz. and accumulation causes hyperpnea , which in turn induces a drop in the pC02. With this drop in pC0l> the respiratory stimulus ceases, and a period of apnea ensues. Central neurogenic hyperventilation most often results from pontine lesions in which patients have prolonged and rapid breathing. This breathing pattern can also result from midbrain/ high pontine lesions. It may be responsive to morphine or methadone. Apneustic breathing is characterized by a long inspiratory pause, after which the air is retained for several seconds before it is released. It appears to result from lesions of the lateral tegmentum of the pons. Cluster breathing is characterized by a series of breaths following each other in an irregular sequence, which can result from low pontine or high medullary lesions. Ataxic breathing (Biot breathing) has a completely irregular pattern in which breaths with diverse amplitude and length are mixed with periods of apnea, which often follows damage to the dorsomedial medulla. Loss of automatic breathing with preserved voluntary breathing ("Ondine's curse") can also occur with medullary lesions, although they tend to occur in a somewhat lower location than those causing ataxic breathing. Ondine's curse has also been noted to occur after lesions involving the ventrolateral high cervical spinal cord , a location transmitting fib ers from the primary medullary respiratory centers. Stertorous breathing is a sign of airway obstruction (Brazis, pp. 568-571; Greenberg, p. 121; Merritt, p. 18).

Question 98

1\ poorly controlled hypertensive patient had a sudden onset of hemiplegia and reduced touch and position sense, all of which affected the right side, with bilateral preservation of pain and temperature sensation. There was also weakness on the right side of the tongue and upbeat nystagmus, but no facial droop was noted. An infarct causing this symptomatology would most likely reside in what location? A. Pontine tegmentum B. Dorsolateralmedulla C. Midbrain tegmentum D. Paramedian medulla E. Posterior limb of the internal capsule

Answer 98

A B C **D** E ## Footnote The tracts that were most lil{ely affected in this patient run along a paramedian plane in the medulla. In ventrodorsal order they consist of the pyramidal tract, medial lemniscus, medial longitudinal fasciculus, and hypoglossal nucleus. Tllis region receives blood from the paramedian branches of the vertebrobasilar system and anterior spinal artery. The face was spared because corticobulbar fibers to the facial nucleus typically depart before reaching the medulla . Pain and temperature were spared because the lateral spinothalamic tracts run in the lateral parts of the medulla, which is supplied by the circumferential arteries. This patient is suffering from medial medullary syndrome (Dejerine's anterior bulbar syndrome) (Carpenter pp. 115-150; Brazis, pp.345- 346).

Question 99

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Mixed transcortical A.A B.B C.C D.D E.E F.F G.G

Answer 99

A.A B.B C.C D.D E.E **F.F** G.G

Question 100

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Conduction A.A B.B C.C D.D E.E F.F G.G

Answer 100

A.A B.B **C.C** D.D E.E F.F G.G

Question 101

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Transcortical motor A.A B.B C.C D.D E.E F.F G.G

Answer 101

A.A B.B C.C **D.D** E.E F.F G.G

Question 102

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Global A.A B.B C.C D.D E.E F.F G.G

Answer 102

A.A B.B C.C D.D E.E F.F **G.G**

Question 103

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Broca A.A B.B C.C D.D E.E F.F G.G

Answer 103

**A.A** B.B C.C D.D E.E F.F G.G

Question 104

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Transcortical sensory A.A B.B C.C D.D E.E F.F G.G

Answer 104

A.A **B.B** C.C D.D E.E F.F G.G

Question 105

Match the type of aphasia (numbered items) with the associated clinical findings (lettered options in Table 3.101-3.107Q), using each answer once. (-) indicates abnormality, (+) indicates no abnormality, (+/-) indicates abnormality may be present. Wernicke A.A B.B C.C D.D E.E F.F G.G

Answer 105

A.A **B.B** C.C D.D E.E F.F G.G

Question 106

Bilateral internuclear ophthalmoplegia can be differentiated from a bilateral medial rectus nuclear lesion by A. The presence of normal medial movement with saccades in bilateral INO B. The presence of normal medial movement with vestibular stimulation in bilateral INO C. The presence of normal convergence in bilateral INO D. The presence of normal lateral movement in midline medial recti nuclear lesion E. The presence of normal lateral movement in bilateral INO

Answer 106

A. The presence of normal medial movement with saccades in bilateral INO B. The presence of normal medial movement with vestibular stimulation in bilateral INO **C. The presence of normal convergence in bilateral INO ** D. The presence of normal lateral movement in midline medial recti nuclear lesion E. The presence of normal lateral movement in bilateral INO ## Footnote It is extremely difficult to differentiate between bilateral INO and bi

Question 107

Oculomasticatory myorhythmia is most frequently seen with what abnormality? A. Hallervorden-Spatz disease B. Progressive supranuclear palsy C. Whipple's disease D. Arnold-Chiari malformation E. Creutzfeldt-lakob disease pendular vergence o

Answer 107

A. Hallervorden-Spatz disease B. Progressive supranuclear palsy **C. Whipple's disease ** D. Arnold-Chiari malformation E. Creutzfeldt-lakob disease ## Footnote OCtl[o/11C1sticatory myorhythmia refers to acquired

Question 108

'Yhat is the best way to differentiate central from peripheral vestibular nystagmus? A. The suppression of peripheral nystagmus with fL\:ation B. The accentuation of central nystagmus with fL\:ation C. The presence of a torsional component with central n ys tagm us D. The presence of increased nystagmus with gaze directed toward the slow phase of nystagmus E. The presence of concomitant myoclonic jerks with peripheral nystagmus from central nystagm

Answer 108

**A. The suppression of peripheral nystagmus with fL\:ation ** B. The accentuation of central nystagmus with fL\:ation C. The presence of a torsional component with central n ys tagm us D. The presence of increased nystagmus with gaze directed toward the slow phase of nystagmus E. The presence of concomitant myoclonic jerks with peripheral nystagmus ## Footnote Two main differences that differentiate peripheral

Question 109

Amantadine may be classified as what type of medica tion? A. Dopamine receptor agonist B. Dopamine receptor antagonist C. tvIuscarinic blocker D. Muscarinic activator E. None of the above augments dopamine rel

Answer 109

A B C D **E** ## Footnote Amantadine is an indirect dopaminergic agent that

Question 110

Match the following muscles with the associated eye moveluent, using each answer once, more than once, or not at all. Pure vertical upward movement of the pupil when it is positioned 51 degrees toward the nose (adducted) A. Superior rectus muscle B. Inferior rectus muscle C. Lateral rectus muscle D. Medial rectus muscle E. Superior oblique muscle F. Inferior oblique muscle

Answer 110

A B C D E **F** ## Footnote The globe of the eye is moved by the action of the superior and inferior oblique muscles as well as the lateral, medial, superior, and inferior recti. The superior rectus muscle elevates the eye when it is abducted (23 degrees), while the inferior rectus muscle depresses the eye when the globe is abducted (23 degrees). By contrast, when the eye is adducted, the superior rectus muscle intorts the eye (moves it counterclockwise in the case of the left eye), while the inferior rectus muscle extorts the eye (moves the left eye clockwise). The oblique muscles have a similar and complementary action in moving the eyes in a vertical plane. They move the eyeball in a vertical plane when the eye is adducted (51 degrees) and act as rotators (intort, extort) when it is abducted (39 degrees). Unlike the rectus muscles, however, they function as would be expected from their insertional points on the globe. The superior oblique muscle depresses the adducted eye or twists it inwardly when the eye is abducted (counterclockwise in the case of the left eye), and the inferior oblique muscle elevates the adducted eye or extorts it when abducted (moves the left eye clockwise). The action of the lateral and medial rectus muscles causes the eyes to deviate laterally and medially, respectively (Brazis, pp. 156-157).

Question 111

Match the following muscles with the associated eye moveluent, using each answer once, more than once, or not at all. Pure down movement with the eye abducted 23 degrees A. Superior rectus muscle B. Inferior rectus muscle C. Lateral rectus muscle D. Medial rectus muscle E. Superior oblique muscle F. Inferior oblique muscle

Answer 111

A **B** C D E F ## Footnote The globe of the eye is moved by the action of the superior and inferior oblique muscles as well as the lateral, medial, superior, and inferior recti. The superior rectus muscle elevates the eye when it is abducted (23 degrees), while the inferior rectus muscle depresses the eye when the globe is abducted (23 degrees). By contrast, when the eye is adducted, the superior rectus muscle intorts the eye (moves it counterclockwise in the case of the left eye), while the inferior rectus muscle extorts the eye (moves the left eye clockwise). The oblique muscles have a similar and complementary action in moving the eyes in a vertical plane. They move the eyeball in a vertical plane when the eye is adducted (51 degrees) and act as rotators (intort, extort) when it is abducted (39 degrees). Unlike the rectus muscles, however, they function as would be expected from their insertional points on the globe. The superior oblique muscle depresses the adducted eye or twists it inwardly when the eye is abducted (counterclockwise in the case of the left eye), and the inferior oblique muscle elevates the adducted eye or extorts it when abducted (moves the left eye clockwise). The action of the lateral and medial rectus muscles causes the eyes to deviate laterally and medially, respectively (Brazis, pp. 156-157).

Question 112

Match the following muscles with the associated eye moveluent, using each answer once, more than once, or not at all. Pure down movement with the eye adducted 51 degrees A. Superior rectus muscle B. Inferior rectus muscle C. Lateral rectus muscle D. Medial rectus muscle E. Superior oblique muscle F. Inferior oblique muscle

Answer 112

A B C D **E** F ## Footnote The globe of the eye is moved by the action of the superior and inferior oblique muscles as well as the lateral, medial, superior, and inferior recti. The superior rectus muscle elevates the eye when it is abducted (23 degrees), while the inferior rectus muscle depresses the eye when the globe is abducted (23 degrees). By contrast, when the eye is adducted, the superior rectus muscle intorts the eye (moves it counterclockwise in the case of the left eye), while the inferior rectus muscle extorts the eye (moves the left eye clockwise). The oblique muscles have a similar and complementary action in moving the eyes in a vertical plane. They move the eyeball in a vertical plane when the eye is adducted (51 degrees) and act as rotators (intort, extort) when it is abducted (39 degrees). Unlike the rectus muscles, however, they function as would be expected from their insertional points on the globe. The superior oblique muscle depresses the adducted eye or twists it inwardly when the eye is abducted (counterclockwise in the case of the left eye), and the inferior oblique muscle elevates the adducted eye or extorts it when abducted (moves the left eye clockwise). The action of the lateral and medial rectus muscles causes the eyes to deviate laterally and medially, respectively (Brazis, pp. 156-157).

Question 113

Match the following muscles with the associated eye moveluent, using each answer once, more than once, or not at all. Pure upward movement with the eye abducted 23 degrees A. Superior rectus muscle B. Inferior rectus muscle C. Lateral rectus muscle D. Medial rectus muscle E. Superior oblique muscle F. Inferior oblique muscle

Answer 113

**A** B C D E F ## Footnote The globe of the eye is moved by the action of the superior and inferior oblique muscles as well as the lateral, medial, superior, and inferior recti. The superior rectus muscle elevates the eye when it is abducted (23 degrees), while the inferior rectus muscle depresses the eye when the globe is abducted (23 degrees). By contrast, when the eye is adducted, the superior rectus muscle intorts the eye (moves it counterclockwise in the case of the left eye), while the inferior rectus muscle extorts the eye (moves the left eye clockwise). The oblique muscles have a similar and complementary action in moving the eyes in a vertical plane. They move the eyeball in a vertical plane when the eye is adducted (51 degrees) and act as rotators (intort, extort) when it is abducted (39 degrees). Unlike the rectus muscles, however, they function as would be expected from their insertional points on the globe. The superior oblique muscle depresses the adducted eye or twists it inwardly when the eye is abducted (counterclockwise in the case of the left eye), and the inferior oblique muscle elevates the adducted eye or extorts it when abducted (moves the left eye clockwise). The action of the lateral and medial rectus muscles causes the eyes to deviate laterally and medially, respectively (Brazis, pp. 156-157).

Question 114

Match the following muscles with the associated eye moveluent, using each answer once, more than once, or not at all. Pure intorsion with the eye abducted 39 degrees A. Superior rectus muscle B. Inferior rectus muscle C. Lateral rectus muscle D. Medial rectus muscle E. Superior oblique muscle F. Inferior oblique muscle

Answer 114

A B C D **E** F ## Footnote The globe of the eye is moved by the action of the superior and inferior oblique muscles as well as the lateral, medial, superior, and inferior recti. The superior rectus muscle elevates the eye when it is abducted (23 degrees), while the inferior rectus muscle depresses the eye when the globe is abducted (23 degrees). By contrast, when the eye is adducted, the superior rectus muscle intorts the eye (moves it counterclockwise in the case of the left eye), while the inferior rectus muscle extorts the eye (moves the left eye clockwise). The oblique muscles have a similar and complementary action in moving the eyes in a vertical plane. They move the eyeball in a vertical plane when the eye is adducted (51 degrees) and act as rotators (intort, extort) when it is abducted (39 degrees). Unlike the rectus muscles, however, they function as would be expected from their insertional points on the globe. The superior oblique muscle depresses the adducted eye or twists it inwardly when the eye is abducted (counterclockwise in the case of the left eye), and the inferior oblique muscle elevates the adducted eye or extorts it when abducted (moves the left eye clockwise). The action of the lateral and medial rectus muscles causes the eyes to deviate laterally and medially, respectively (Brazis, pp. 156-157).

Question 115

Match the following muscles with the associated eye moveluent, using each answer once, more than once, or not at all. Pure extorsion with the eye abducted 39 degrees A. Superior rectus muscle B. Inferior rectus muscle C. Lateral rectus muscle D. Medial rectus muscle E. Superior oblique muscle F. Inferior oblique muscle

Answer 115

A B C D E **F** ## Footnote The globe of the eye is moved by the action of the superior and inferior oblique muscles as well as the lateral, medial, superior, and inferior recti. The superior rectus muscle elevates the eye when it is abducted (23 degrees), while the inferior rectus muscle depresses the eye when the globe is abducted (23 degrees). By contrast, when the eye is adducted, the superior rectus muscle intorts the eye (moves it counterclockwise in the case of the left eye), while the inferior rectus muscle extorts the eye (moves the left eye clockwise). The oblique muscles have a similar and complementary action in moving the eyes in a vertical plane. They move the eyeball in a vertical plane when the eye is adducted (51 degrees) and act as rotators (intort, extort) when it is abducted (39 degrees). Unlike the rectus muscles, however, they function as would be expected from their insertional points on the globe. The superior oblique muscle depresses the adducted eye or twists it inwardly when the eye is abducted (counterclockwise in the case of the left eye), and the inferior oblique muscle elevates the adducted eye or extorts it when abducted (moves the left eye clockwise). The action of the lateral and medial rectus muscles causes the eyes to deviate laterally and medially, respectively (Brazis, pp. 156-157).

Question 116

In a 1'6 paraplegic (complete cord section), the presence of hypertension, headache, diaphoresis, and bradycardia should be treated with A. Propranolol B. Bladder catheterization C. Arfonad (ganglionic blocker) D. 1000 cc bolus of normal saline E. Nitroglycerin

Answer 116

A **B** C D E ## Footnote Bladder retention and autonomic instability in a T6 paraplegic patient typically resolve with bladder catheterization (Brazis, p. 89; Merritt, p. 422).

Question 117

Small, pinpoint pupils may be seen in all of the following EXCEPT? A. Pontine tegmental lesions B. Bilateral diencephalic dysfunction C. Narcotic intoxication D. Oculomotor nerve compression E. Cholinergic drugs

Answer 117

A B C **D** E ## Footnote Oculomotor nerve compression typically causes a dilated pupil, whereas a pontine hemorrhage, bilateral diencephalic injury, narcotic administration, and cholinergic drug therapy typically result in small pupils (Merritt, pp.18-19).

Question 118

Severe shoulder pain followed in several days by proximal arm weakness characterizes the A. Erb-Duchenne syndrome B. Parsonage-Turner syndrome C. Dejerine-Klumpke syndrome D. Thoracic outlet syndrome E. None of the above

Answer 118

A **B** C D E ## Footnote Neurogenic amyotrophy (Parsonage-Turner syndrome) is characterized by severe, acute pain located in the shoulder and radiating into the arm, neck, and back. To prevent pain, movement of the ann is avoided and the arm is held in a position of tlexion at the elbow and adduction at the shoulder (tlexion-adduction sign). The muscles innervated by the axillary, suprascapular, and long thoracic nerves are most often affected. The pain usually disappears but is then followed by paresis of the shoulder and proximal musculature (Brazis, p. 57).

Question 119

All of the following involve the lower motor CN VII EXCEPT? A. Ramsay-Hunt syndrome B. Ieige's syndrome C. Foville's syndrome D. Benedikt's syndrome E. Millard-Gubler syndrome

Answer 119

A B C **D** E ## Footnote Benedikt's syndrome consists of ipsilateral oculomotor paresis, usually with a dilated pupil, as well as contralateral involuntary movements including intenti hemichorea, or hemiathetosis due to red nucleus damage. Ramsay-Hunt syndrome, Meige's syndrome, Foville's syndrome, and the Millard-Gubler syndrome involve the facial nerve (CN VII) (Brazis, pp. 359- 360).

Question 120

Galactorrhea may be due to all of the following EXCEPT? A. Irritative lesions of the anterior chest wall B. Levodopa/carbidopa C. Hypothyroidism D. Pituitary chromophobe adenomas E. Contraceptive drugs

Answer 120

A **B** C D E ## Footnote Levodopa/carbidopa does not typically produce galactorrhea (Merritt, pp. 687- 691).

Question 121

The "paramedian diencephalic syndrome" includes all of the following EXCEPT? A. Hypersomnolence B. Memory loss C. Horizontal gaze palsy D. Apathy E. Hemiataxia

Answer 121

A B **C** D E ## Footnote Infarcts involving the paramedian region of the midbrain and pons may result in decreased level of conciousneness, behavioral changers (agitation, confusion, lack of initiative, apathy), memory loss, vertical gaze and convergence disorders, contralateral hemiatR.xia, asterLxis, motor weakness, and action tremor in the contralateral limbs. Lateral gaze palsies are not typically associated with this syndrome (Brazis, pp. 408- 409).

Question 122

The syndrome of "Dejerine and Roussy" occurs most commonly with lesions of the A. Ventral lateral (VL) nuclei of the thalamus B. Ventral posterior (VP) nuclei of the thalamus C. Intralaminar nuclei of the thalamus D. Geniculate nuclei E. Subthalamic nuclei

Answer 122

A **B** C D E ## Footnote The syndrome of "Dejerine and Roussy" is characterized by contralateral sensory loss to a ll modalities, dysesthesias of the involved side (thalamiC pain), vasomotor disturbances, transient contralateral hemiparesis, and choreoathetoid or ballistic movements. It is most often the result of infarction in the ventral posterior (VP) nuclei of the thalamus, which are supplied by the penetrating branches of the posterior communicating artery (Brazis, p. 548).

Question 123

Vitreous hemorrhage associated with subarachnoid hemorrhage is 1m own as A. Carson syndrome B. Terson syndrome C. Colla ret syndrome D. Vicard syndrome E. Heubner syndrome

Answer 123

A **B** C D E ## Footnote Subarachnoid hemorrhage (or any intracranial hemorrhage) may result in vitreous hemorrhage, also Imown as Terson's syndrome (Brazis, p. 559).

Question 124

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Myelin proteolipid A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 124

A B C **D** E F G

Question 125

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Galactocerebrosidase A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 125

**A** B C D E F G

Question 126

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Aryl sulfatase A A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 126

A **B** C D E F G

Question 127

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Arginase A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 127

A B C D E F **G**

Question 128

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Aspartoacylase A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 128

A B C D E F **G**

Question 129

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. ATP-binding transporter A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 129

A B **C** D E F G

Question 130

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Long-chain fatty acid metabolism A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 130

A B C D E **F** G

Question 131

Match the disorder (leukodystrophy) with the enzyme abnormality using each answer once, more than once, or not at all. Copper ATPase defiCiency A. Krabbe's disease B. Metachromatic leukodystrophy C. Adrenoleulwdystrophy D. Pelizaeus-Merzbacher disease E. Alexander's disease F. Zellweger's syndrome G. None of the above

Answer 131

A B C D E F **G**

Question 132

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all Sphingomyelinase A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 132

A B C D **E** F G H I J K

Question 133

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all a-galactosidase A A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 133

A B **C** D E F G H I J K

Question 134

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all B~-glucocerebrosidase A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 134

A B C **D** E F G H I J K

Question 135

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all B~-galactosidase A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 135

A B C D E F G H **I** J K

Question 136

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all Hexosaminidase A absent; hexosaminidase B increased A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 136

**A** B C D E F G H I J K

Question 137

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all Hexosaminidase A and B deficient A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 137

A **B** C D E F G H I J K

Question 138

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all Acid ceramidase A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 138

A B C D E F G H I **J** K

Question 139

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all Acid lipase A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 139

A B C D E **F** G H I J K

Question 140

Match the disorder (sphingolipidosis) with the associated enzyme abnormality, using each answer once, more than once, or not at all Palmitoylprotein thioesterase A. 'fay-Sachs disease (G~1 gangliosidosis) B. Sand hoff's disease C. Fabry's disease D. Gaucher's disease E. Niemann-Pick disease F. Wolman's disease G. Batten's disease (neuronal ceroid Iipofuscinosis) H. Cerebrotendinous xanthomatosis I. G~IJ gangliosidosis J. Farber's disease K. None of the above

Answer 140

A B C D E F **G** H I J K

Question 141

What is the inheritance pattern of hereditary hemorrhagic telangiectasia (I-II-IT) ? A. Autosomal recessive B. Autosomal dominant C. X-linlled D. Sporadic E. None of the above

Answer 141

A **B** C D E ## Footnote I-H-IT or Rendu-Osler-Weber disease, is an autosomal dominant neurocutaneous syndrome that involves mutations in the TGF-~ receptor gene. Patients with HHT develop arteriovenous malformations of the liver, lungs, brain, and spine, in descending order of frequency. Patients with HI-IT also exhibit telangiectasias of the skin and mucosa, and often present with epista.'(is. One key distinguishing feature of I-IHT is the presence of nail bed telangiectasias. The pulmonary shunts that are commonly associated with I-H-IT put patients at significant risk for the development of brain abscesses. Patients with HHT also experience paradoxical cerebral emboli, strokes, and subarachnoid hemorrhage (due to an increased risk of intracranial aneurysms) (Merritt, p. 371; Osborn DN, pp. 106-107).

Question 142

I-II-IT is associated with mutations of what gene? A. EGFR B. TNF-cz C. TGF-~ D. Superoxide disl11utase E. None of the above

Answer 142

A B **C** D E ## Footnote I-H-IT or Rendu-Osler-Weber disease, is an autosomal dominant neurocutaneous syndrome that involves mutations in the TGF-~ receptor gene. Patients with HHT develop arteriovenous malformations of the liver, lungs, brain, and spine, in descending order of frequency. Patients with HI-IT also exhibit telangiectasias of the skin and mucosa, and often present with epista.'(is. One key distinguishing feature of I-IHT is the presence of nail bed telangiectasias. The pulmonary shunts that are commonly associated with I-H-IT put patients at significant risk for the development of brain abscesses. Patients with HHT also experience paradoxical cerebral emboli, strokes, and subarachnoid hemorrhage (due to an increased risk of intracranial aneurysms) (Merritt, p. 371; Osborn DN, pp. 106-107).

Question 143

Patients with I-II-IT are at high risk for the developing? A. Brain abscesses B. Multiple sclerosis C. Lymphoma D. Hypoglycemia E. Myoglobinuria

Answer 143

**A** B C D E ## Footnote I-H-IT or Rendu-Osler-Weber disease, is an autosomal dominant neurocutaneous syndrome that involves mutations in the TGF-~ receptor gene. Patients with HHT develop arteriovenous malformations of the liver, lungs, brain, and spine, in descending order of frequency. Patients with HI-IT also exhibit telangiectasias of the skin and mucosa, and often present with epista.'(is. One key distinguishing feature of I-IHT is the presence of nail bed telangiectasias. The pulmonary shunts that are commonly associated with I-H-IT put patients at significant risk for the development of brain abscesses. Patients with HHT also experience paradoxical cerebral emboli, strokes, and subarachnoid hemorrhage (due to an increased risk of intracranial aneurysms) (Merritt, p. 371; Osborn DN, pp. 106-107).

Question 144

What is the I-year stroke risk of asymptomatic 80% carotid artery stenosis? A. 3%

Answer 144

A. 3% ## Footnote Symptomatic carotid artery stenosis and significant asymptomatic carotid artery stenosis are associated with an increased risk of stroke. The North American Symptomatic Carotid Endarterectomy Trial (NASCET) provided strong evidence for the benefit of carotid endarterectomy over maximum medical management of patients with symptomatic ipsilateral carotid artery stenosis between 70 and 99%. In the NASCET study group, patients with mild strokes or ipsilateral hemispheric or retinal TIAs within the past 120 days exhibited a 17% decrease in the rate of all ipsilateral strokes (26% in medical group versus 9% in surgical group), and a 10.6% deCl'ease in major or fatal ipsilateral stroke (13.1% in medical group versus 2.5% in surgical group) at 2 years compared to medical management. Symptomatic patients with less than 50% carotid stenosis should not undergo carotid endarterectomy, and stenosis between 50 and 69% is controverSial, with a 6.5% reduction in stroke rate with surgery. Asymptomatic carotid artery stenosis of greater than 75% is associated with a 3.3% risk of stroke per year (2.5% risk of ipsilateral stroke), and stenosis of less than 75% is associated with a 1.3% risk of stroke per year. The Asymptomatic Carotid Atherosclerosis Study (ACAS) concluded that patients with asymptomatic carotid artery stenosis greater than 60% have reduced stroke rates with carotid endarterectomy compared to medical management at 5 years (11.0% stroke rate in medical group versus 5.1% stroke rate in surgical group). However, these results apply to patients in reasonable health and to medical centers that perform carotid endarterectomy with less than 3% perioperative morbidity and mortality (Executive committee for ACAS, pp. 1421-1428; NASCET, pp. 445-453; Merritt, pp. 228, 257).

Question 145

All of the following disorders are associated with defective DNA repair EXCEPT? A. Xeroderma pigmen tosa B. Fanconi's anemia C. Bloom's syndrome D. Ataxia telangiectasia E. IUinefelter's syndrome

Answer 145

A B C D **E** ## Footnote Cockayne's syndrome, ata.xia telangiectasia, xeroderma pigmentosa, Fanconi's anemia , and Blooms syndrome are associated with defective DNA repair, whereas I

Question 146

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Miller-Dieker syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 146

A B C D E F G **H** I J

Question 147

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Down's syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 147

**A** B C D E F G H I J

Question 148

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Cri du chat syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 148

A B C D E F **G** H I J

Question 149

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Patau syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 149

A B **C** D E F G H I J

Question 150

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Prader-Willi syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 150

A B C D **E** F G H I J

Question 151

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Edward's syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 151

A **B** C D E F G H I J

Question 152

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Fragile X syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 152

A B C D E F G H **I** J

Question 153

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Turner syndrome A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 153

A B C D E F G H **I** J

Question 154

lvlatch the gene abnormality with the associated syndrome, using each answer once, more than once, or not at all. Associated with Dandy-WallIeI' syndrome in some cases A. Trisomy 21 B. Trisomy 18 C. Trisomy 13 D. Trisomy 9 E. Long arm deletion, chromosome 15 F. Short arm deletion, chromosome 4 G. Short ann deletion, chromosome 5 H. Short arm deletion, chromosome 17 I. Decrease in the number of sex chromosomes J. None of the above

Answer 154

A B C **D** E F G H I J

Question 155

All of the following disorders are associated with "cherry red" spots EXCEPT? A. Tay-Sachs disease B. Sandhoff's disease C. Sialidosis D. Niemann Pick disease E. Galactosemia

Answer 155

A B C D **E** ## Footnote Galactosemia is associated with cataracts, not "cherry-red" spots (Geyer, p. 114).

Question 156

Increased skin pigmentation is associated with what disorder? A. Adrenoleukodystrophy B. Hurler's disease C. Hunter's disease D. Cockayne's syndrome E. Homocystinuria

Answer 156

**A** B C D E ## Footnote Increased skin pigmentation is one of the features of adrenoleukodystrophy, while Hurler's syndrome, homocystinuria , and Cockayne's syndrome are associated with corneal clouding (Geyer, pp. 113- 114

Question 157

Match the clinical feature with the associated disorder, using each answer once, more than once, or not at Atrophic tongue A. Bulbar palsy B. Pseudobulbar palsy C. Both D. None of the above

Answer 157

**A** B C D ## Footnote Patients with bulbar p alsy typically have an atrophic tongue with fasciculations, flaccid speech, a weak face, absent jaw-jerk and gag ret1exes, and diminished extraocular movements. Patients with pseudobulbar palsy have a normal-sized tongue with spastic speech, a weak face, emotional lability, the presence of jaw-jerk and gag (hyperactive) reflexes, diminished extraocular movements, and the absence of fasciculations (Brazis, p. 321; Merritt, p. 239; Geyer, p. 133).

Question 158

Match the clinical feature with the associated disorder, using each answer once, more than once, or not at Weak face A. Bulbar palsy B. Pseudobulbar palsy C. Both D. None of the above

Answer 158

A B **C** D ## Footnote Patients with bulbar p alsy typically have an atrophic tongue with fasciculations, flaccid speech, a weak face, absent jaw-jerk and gag ret1exes, and diminished extraocular movements. Patients with pseudobulbar palsy have a normal-sized tongue with spastic speech, a weak face, emotional lability, the presence of jaw-jerk and gag (hyperactive) reflexes, diminished extraocular movements, and the absence of fasciculations (Brazis, p. 321; Merritt, p. 239; Geyer, p. 133).

Question 159

Match the clinical feature with the associated disorder, using each answer once, more than once, or not at Absent jaw-jerk retlex A. Bulbar palsy B. Pseudobulbar palsy C. Both D. None of the above

Answer 159

**A** B C D ## Footnote Patients with bulbar p alsy typically have an atrophic tongue with fasciculations, flaccid speech, a weak face, absent jaw-jerk and gag ret1exes, and diminished extraocular movements. Patients with pseudobulbar palsy have a normal-sized tongue with spastic speech, a weak face, emotional lability, the presence of jaw-jerk and gag (hyperactive) reflexes, diminished extraocular movements, and the absence of fasciculations (Brazis, p. 321; Merritt, p. 239; Geyer, p. 133).

Question 160

Match the clinical feature with the associated disorder, using each answer once, more than once, or not at Decreased extraocular movements A. Bulbar palsy B. Pseudobulbar palsy C. Both D. None of the above

Answer 160

A B **C** D ## Footnote Patients with bulbar p alsy typically have an atrophic tongue with fasciculations, flaccid speech, a weak face, absent jaw-jerk and gag ret1exes, and diminished extraocular movements. Patients with pseudobulbar palsy have a normal-sized tongue with spastic speech, a weak face, emotional lability, the presence of jaw-jerk and gag (hyperactive) reflexes, diminished extraocular movements, and the absence of fasciculations (Brazis, p. 321; Merritt, p. 239; Geyer, p. 133).

Question 161

Match the clinical feature with the associated disorder, using each answer once, more than once, or not at Emotional lability A. Bulbar palsy B. Pseudobulbar palsy C. Both D. None of the above

Answer 161

A **B** C D ## Footnote Patients with bulbar p alsy typically have an atrophic tongue with fasciculations, flaccid speech, a weak face, absent jaw-jerk and gag ret1exes, and diminished extraocular movements. Patients with pseudobulbar palsy have a normal-sized tongue with spastic speech, a weak face, emotional lability, the presence of jaw-jerk and gag (hyperactive) reflexes, diminished extraocular movements, and the absence of fasciculations (Brazis, p. 321; Merritt, p. 239; Geyer, p. 133).

Question 162

Match the clinical feature with the associated disorder, using each answer once, more than once, or not at Hyperactive gag A. Bulbar palsy B. Pseudobulbar palsy C. Both D. None of the above

Answer 162

A **B** C D ## Footnote Patients with bulbar p alsy typically have an atrophic tongue with fasciculations, flaccid speech, a weak face, absent jaw-jerk and gag ret1exes, and diminished extraocular movements. Patients with pseudobulbar palsy have a normal-sized tongue with spastic speech, a weak face, emotional lability, the presence of jaw-jerk and gag (hyperactive) reflexes, diminished extraocular movements, and the absence of fasciculations (Brazis, p. 321; Merritt, p. 239; Geyer, p. 133).

Question 163

lvlatch the virus with the associated genetic content, using each answer once, more than once, o Poliovirus A. DNA virus B. RNA virus C. Both D. None of the above

Answer 163

A **B** C D ## Footnote Enteroviruses (polioVirus, coxsackievirus, and echovirus), arboviruses (tlaviviruses, bunyaviruses, alphaviruses, rubiviruses), rhabdoviruses (rabies virus), and arenaviruses (lymphocytic choriomeningitis) are R.J\TA viruses, while the herpesviruses CHAPTER 3 Neurology Answers 77 (HSV-l, HSV-2, varicella zoster virus, cytomegalovirus, and HHV-6) are DNA viruses. Viral infections of the CNS can result in meningitis, ventriculitis, encephalitis, and myelitis. CSF in patients with viral syndromes of the CNS reveals increased pressure, lymphocytic pleocytosis, mild elevations in protein, and normal glucose levels. Viral (aseptic) meningitis usually peaks in the summer and fall seasons, whereas bacteria l meningitis is more common during the winter. The most common causes of viral meningitis are the enteroviruses, but togaviruses are also frequent pathogens. Encephalitis often results from infections with herpes simplex virus, mumps, or arboviruses (Merritt, pp. 134- 174).

Question 164

lvlatch the virus with the associated genetic content, using each answer once, more than once, o Echovirus A. DNA virus B. RNA virus C. Both D. None of the above

Answer 164

A **B** C D ## Footnote Enteroviruses (polioVirus, coxsackievirus, and echovirus), arboviruses (tlaviviruses, bunyaviruses, alphaviruses, rubiviruses), rhabdoviruses (rabies virus), and arenaviruses (lymphocytic choriomeningitis) are R.J\TA viruses, while the herpesviruses CHAPTER 3 Neurology Answers 77 (HSV-l, HSV-2, varicella zoster virus, cytomegalovirus, and HHV-6) are DNA viruses. Viral infections of the CNS can result in meningitis, ventriculitis, encephalitis, and myelitis. CSF in patients with viral syndromes of the CNS reveals increased pressure, lymphocytic pleocytosis, mild elevations in protein, and normal glucose levels. Viral (aseptic) meningitis usually peaks in the summer and fall seasons, whereas bacteria l meningitis is more common during the winter. The most common causes of viral meningitis are the enteroviruses, but togaviruses are also frequent pathogens. Encephalitis often results from infections with herpes simplex virus, mumps, or arboviruses (Merritt, pp. 134- 174).

Question 165

lvlatch the virus with the associated genetic content, using each answer once, more than once, o Herpes simplex 1 virus A. DNA virus B. RNA virus C. Both D. None of the above

Answer 165

**A** B C D ## Footnote Enteroviruses (polioVirus, coxsackievirus, and echovirus), arboviruses (tlaviviruses, bunyaviruses, alphaviruses, rubiviruses), rhabdoviruses (rabies virus), and arenaviruses (lymphocytic choriomeningitis) are R.J\TA viruses, while the herpesviruses CHAPTER 3 Neurology Answers 77 (HSV-l, HSV-2, varicella zoster virus, cytomegalovirus, and HHV-6) are DNA viruses. Viral infections of the CNS can result in meningitis, ventriculitis, encephalitis, and myelitis. CSF in patients with viral syndromes of the CNS reveals increased pressure, lymphocytic pleocytosis, mild elevations in protein, and normal glucose levels. Viral (aseptic) meningitis usually peaks in the summer and fall seasons, whereas bacteria l meningitis is more common during the winter. The most common causes of viral meningitis are the enteroviruses, but togaviruses are also frequent pathogens. Encephalitis often results from infections with herpes simplex virus, mumps, or arboviruses (Merritt, pp. 134- 174).

Question 166

lvlatch the virus with the associated genetic content, using each answer once, more than once, *Arenavirus* A. DNA virus B. RNA virus C. Both D. None of the above

Answer 166

A **B** C D ## Footnote Enteroviruses (polioVirus, coxsackievirus, and echovirus), arboviruses (tlaviviruses, bunyaviruses, alphaviruses, rubiviruses), rhabdoviruses (rabies virus), and arenaviruses (lymphocytic choriomeningitis) are R.J\TA viruses, while the herpesviruses CHAPTER 3 Neurology Answers 77 (HSV-l, HSV-2, varicella zoster virus, cytomegalovirus, and HHV-6) are DNA viruses. Viral infections of the CNS can result in meningitis, ventriculitis, encephalitis, and myelitis. CSF in patients with viral syndromes of the CNS reveals increased pressure, lymphocytic pleocytosis, mild elevations in protein, and normal glucose levels. Viral (aseptic) meningitis usually peaks in the summer and fall seasons, whereas bacteria l meningitis is more common during the winter. The most common causes of viral meningitis are the enteroviruses, but togaviruses are also frequent pathogens. Encephalitis often results from infections with herpes simplex virus, mumps, or arboviruses (Merritt, pp. 134- 174).

Question 167

lvlatch the virus with the associated genetic content, using each answer once, more than once, *Flavivirus* A. DNA virus B. RNA virus C. Both D. None of the above

Answer 167

A **B** C D ## Footnote Enteroviruses (polioVirus, coxsackievirus, and echovirus), arboviruses (tlaviviruses, bunyaviruses, alphaviruses, rubiviruses), rhabdoviruses (rabies virus), and arenaviruses (lymphocytic choriomeningitis) are R.J\TA viruses, while the herpesviruses CHAPTER 3 Neurology Answers 77 (HSV-l, HSV-2, varicella zoster virus, cytomegalovirus, and HHV-6) are DNA viruses. Viral infections of the CNS can result in meningitis, ventriculitis, encephalitis, and myelitis. CSF in patients with viral syndromes of the CNS reveals increased pressure, lymphocytic pleocytosis, mild elevations in protein, and normal glucose levels. Viral (aseptic) meningitis usually peaks in the summer and fall seasons, whereas bacteria l meningitis is more common during the winter. The most common causes of viral meningitis are the enteroviruses, but togaviruses are also frequent pathogens. Encephalitis often results from infections with herpes simplex virus, mumps, or arboviruses (Merritt, pp. 134- 174).

Question 168

lvlatch the virus with the associated genetic content, using each answer once, more than once, *Coxsackievirus* A. DNA virus B. RNA virus C. Both D. None of the above

Answer 168

A **B** C D ## Footnote Enteroviruses (polioVirus, coxsackievirus, and echovirus), arboviruses (tlaviviruses, bunyaviruses, alphaviruses, rubiviruses), rhabdoviruses (rabies virus), and arenaviruses (lymphocytic choriomeningitis) are R.J\TA viruses, while the herpesviruses CHAPTER 3 Neurology Answers 77 (HSV-l, HSV-2, varicella zoster virus, cytomegalovirus, and HHV-6) are DNA viruses. Viral infections of the CNS can result in meningitis, ventriculitis, encephalitis, and myelitis. CSF in patients with viral syndromes of the CNS reveals increased pressure, lymphocytic pleocytosis, mild elevations in protein, and normal glucose levels. Viral (aseptic) meningitis usually peaks in the summer and fall seasons, whereas bacteria l meningitis is more common during the winter. The most common causes of viral meningitis are the enteroviruses, but togaviruses are also frequent pathogens. Encephalitis often results from infections with herpes simplex virus, mumps, or arboviruses (Merritt, pp. 134- 174).

Question 169

What is the normal velocity of a human peripheral nerve when tested by electromyography? A. 5-10 meters per second B. 10-20 meters per second C. 20-30 met. :rs per second D. 30-40 met , fS ')er second E. 40-60 meters per second

Answer 169

A B C D **E** ## Footnote The normal conduction velocity of a human peripheral nerve, as tested by electromyography, is approximately 40 to 60 mis (Adams, p. 1023).

Question 170

What is neurotransmitter A? A. Glutamate B. GABA C. Acetylcholine D. Dopamine E. Substance P

Answer 170

**A** B C D E ## Footnote This is a highly Simplified depiction of the basal ganglia circuitry. The neurotransmitters utilized by these neurons are as follows: A, D, and G utilize glutamate; B, C, and E utilize GABA, while F neurons utilize dopamine (Youmans, p. 2684).

Question 171

What is neurotransmitter C? A. Glutamate B. GABA C. Acetylcholine D. Dopamine E. Substance P

Answer 171

A. Glutamate **B. GABA ** C. Acetylcholine D. Dopamine E. Substance P ## Footnote This is a highly Simplified depiction of the basal ganglia circuitry. The neurotransmitters utilized by these neurons are as follows: A, D, and G utilize glutamate; B, C, and E utilize GABA, while F neurons utilize dopamine (Youmans, p. 2684).

Question 172

What is neurotransmitter E? A. Glutamate B. GABA C. Acetylcholine D. Dopamine E. Substance P

Answer 172

A. Glutamate **B. GABA ** C. Acetylcholine D. Dopamine E. Substance P ## Footnote This is a highly Simplified depiction of the basal ganglia circuitry. The neurotransmitters utilized by these neurons are as follows: A, D, and G utilize glutamate; B, C, and E utilize GABA, while F neurons utilize dopamine (Youmans, p. 2684).

Question 173

Wha t is neurotransmitter G? A. Glutamate B. GABA C. Acetylcholine D. Dopamine E. Substance P

Answer 173

**A. Glutamate ** B. GABA C. Acetylcholine D. Dopamine E. Substance P ## Footnote This is a highly Simplified depiction of the basal ganglia circuitry. The neurotransmitters utilized by these neurons are as follows: A, D, and G utilize glutamate; B, C, and E utilize GABA, while F neurons utilize dopamine (Youmans, p. 2684).