SECTION III

Review Questions

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 SECTION III: REVIEW QUESTIONS 467

Review Question Notes: The following are strategically designed review

questions to assess whether the student is able to integrate the information
presented in the cases. The explanations to the answer choices describe the
rationale, including which cases are relevant.

REVIEW QUESTIONS

R-1. A 62-year-old man is noted to have weight loss, cough, and night sweats
of 1-month duration. He also complains of weakness of the legs, especially
difficulty climbing stairs, and some double vision. On examination, the
lower extremity strength is 3/5 bilaterally. Which of the following is the
most likely mechanism for his neurologic condition?
A. Acetylcholine receptor antibodies
B. Decreased levels of serum calcium
C. Inhibition acetylcholine degradation
D. Voltage-gated calcium channel antibodies
R-2. A 56-year-old woman is noted by her daughter to be moving slowly, walk-
ing with a shuffling gait, and having a noticeable tremor. On examination,
the patient shows little facial expression, and her extremities have some
rigidity to passive movement. Which of the following medications should
be considered as initial therapy in this case?
A. Coenzyme Q10
B. Dopamine agonists
C. Levodopa
D. Monoamine oxidase B inhibitors
E. Catechol-o-methyl transferase inhibitor
R-3. A 31-year-old woman is being evaluated for abnormal uterine bleeding. She
had a T4 spinal cord transection 4 years previously due to a motor vehicle
accident and is paraplegic. During the insertion of the vaginal speculum,
the patient developed the acute onset of a severe headache and flushing of
her face. Her blood pressure is noted to be 180/110 mm Hg and heart rate
(HR) is 140 beats/min. Which of the following is the most likely diagnosis?
A. Autonomic dysreflexia
B. Allergic reaction to lubricant
C. Preeclampsia
D. Panic attack

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R-4. A 58-year-old man is noted to have the acute onset of aphasia, and right arm
and leg weakness. On examination, he is found to have swelling and redness
of the right lower extremity. If the neurologic problems occur due to the con-
dition in his leg, which of the following is most likely present in this patient?
A. Atrial fibrillation
B. Berry aneurysm of the brain
C. Carotid bruit
D. Atrial septal defect
R-5. A 43-year-old man is being evaluated by his physician for multiple syncopal
episodes. He notes that he passes out when he is shaving, and also when he
turns his head while wearing a tight collared shirt. He has no past medical
problems. His vital signs are normal. Which of the following is the most
likely pathophysiology of his syncope?
A. Baroreceptor hypersensitivity
B. Sinus node dysfunction
C. Atrioventricular (AV) nodal heart block
D. AV nodal reentry
E. Ventricular arrhythmia
R-6. A 62-year-old woman is being evaluated by her physician for problems with
concentration and memory, and problems losing urine. On examination,
she is found to have a shuffling gait. Computed tomography (CT) imaging
shows dilated cerebral ventricles and no mass effect. Which of the follow-
ing is the most likely diagnosis?
A. Alzheimer dementia
B. Creutzfeldt-Jakob disease
C. Lewy body dementia
D. Normal pressure hydrocephalus
R-7. A 24-year-old man complains of photophobia, headache, and neck stiff-
ness. On examination, his temperature is 100.8°F. He appears lethargic and
has nuchal rigidity. There is a normal neurologic examination otherwise. If
the patient has herpes simplex virus (HSV) meningoencephalitis, which of
the following cerebrospinal fluid (CSF) findings would be expected?

CSF Protein
WBC/mL Type of Cells CSF Glucose (mg/dL) Erythrocytes/mm3
A 2000 Neutrophils Low 300 5
B 50 Lymphocytes Normal 50 2
C 260 Lymphocytes Normal 80 120
D 500 Lymphocytes Low 140 0

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 SECTION III: REVIEW QUESTIONS 469

See also Case 25 (Acute Disseminated Encephalomyelitis) and Case 26

(Viral Meningitis)
R-8. A 45-year-old woman complains of right hand pain. She states that she
is dropping items such as mugs and her phone. She complains of numb-
ness of the thumb and index finger. On examination, she has weakness of
opposition of the right thumb. Which of the following is the most likely
mechanism of her condition?
A. Cerebral white matter disease
B. Plexopathy
C. Spinal cord anterior horn degeneration
D. Peripheral nerve entrapment
R-9. A 4-year-old boy is brought into the pediatrician’s office due to not speak-
ing much over the past 2 months. The child had met most developmental
milestones until recently and has stopped interacting with other children
or even family members. On examination, the child is sitting in the middle
of the room and does not respond to his name or questions. He is play-
ing with a toy car. When his mother attempts to touch him, he shrugs
away and does not look at her. Which of the following is the most likely
diagnosis?
A. Autism spectrum disorder
B. Down syndrome
C. Impaired hearing
D. Fetal alcohol syndrome
R-10. A 9-month-old male infant is brought into the emergency department due
to fever and seizures. The seizure was described as thrashing arms and legs
and lasting for 2 minutes. The infant is noted to have a temperature of
101.8°F rectally. There is no nuchal rigidity and no neurologic abnormali-
ties. Which of the following is the best therapy for this patient?
A. Antipyretics
B. CT imaging
C. Lumbar puncture (LP)
D. Magnetic resonance imaging (MRI)

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 470 CASE FILES: NEUROLOGY

ANSWERS

R-1. D. This patient likely has lung cancer and weakness due to Lambert-Eaton
syndrome (LES), which is caused by antibodies to presynaptic voltage-gated
calcium channels resulting in a decrease release of acetylcholine. In this case,
it is a paraneoplastic syndrome, similar to myasthenia gravis (MG). How-
ever, as opposed to MG, LES is associated with more lower extremity weak-
ness. Answer A would be the mechanism of MG, in which upper extremity
and eye weakness is prominent. Low levels of calcium can induce weakness,
but it is usually generalized with muscle twitching. Answer C would be the
mechanism by which pyridostigmine delays degradation of acetylcholine
and improves weakness in MG and LEMS patients.
See also Case 3 (NMDA Encephalitis), Case 27 (Infantile Botulism), and
Case 36 (Ptosis [Myasthenia Gravis]).
R-2. D. This patient likely has Parkinson disease (PD). Currently, there are no
known medications that impact clinical disease process. Thus, medications
are prescribed as symptomatic therapy. Thus, the medications are consid-
ered to be symptomatic. Levodopa slows down the onset of symptoms,
although with long-term use, motor fluctuation and dyskinesias often
develop. Monoamine oxidase (MAO) B inhibitors have better side-effect
profiles and are useful early in the disease and in younger patients with
milder disease, since they have only a modest effect on the motor symptoms
of PD. COMT inhibitors extend the benefit of levodopa by reducing “off ”
symptoms between doses. Thus, COMT inhibitors as a monotherapy, have
no effect on Parkinson’s symptoms.
See also Case 4 (Parkinson Disease), Case 1 (Essential Tremor), and Case 2
(Huntington Disease).
R-3. A. In a patient who has a spinal cord lesion above T6, stimulation below the
level (such as pelvic examination, rectal examination, full bladder) can lead
to an uninhibited sympathetic discharge, so-called autonomic dysreflexia.
Because of the spinal cord discontinuity, parasympathetic stimulation (inhib-
iting the sympathetic discharge) cannot occur. The presentation is the acute
onset of headache, nasal congestion, flushing or goose bumps above the level
of the lesion, profuse sweating, and dangerously elevated blood pressure and
HR. This complication can be life-threatening. An allergic reaction to lubri-
cant would lead to hives, itching, and hypotension. There is no indication of
pregnancy or retention of fetal tissue and would not present acutely in asso-
ciation with pelvic examination. A panic attack would typically present as
hyperventilation, anxiety, and sweating, but the BP would not be so elevated.
See also Case 7 (Spinal Cord Injury, Traumatic).

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 SECTION III: REVIEW QUESTIONS 471

R-4. D. This is a patient with a probable cerebrovascular accident (or transient

ischemic attack [TIA]). These are typically ischemic events, although they
can be hemorrhagic, or more rarely embolic. Embolic strokes can be caused
by arterial atherosclerotic plaques such as those involving the carotid artery,
or thrombi within the left atrium (atrial fibrillation). This patient has what
appears to be a deep vein thrombosis (DVT) (venous system). Emboliza-
tion of the thrombus would cause pulmonary emboli unless the patient has
a right-to-left shunt, such as an atrial septal defect. Such an intracardiac
defect can allow a venous thrombus to enter the systemic circulation and
cause a stroke.
See also Case 11 (Acute Cerebral Infarct), Case 12 (Subarachnoid
Hemorrhage), and Case 13 (Stroke in a Young Patient [Acute Ischemic]).
R-5. A. This patient likely has carotid hypersensitivity. The history of syncope
with shaving and turning one’s head especially with a tight-fitting collar
is very suggestive of this condition. To confirm the condition, a carotid
massage may be performed prior to auscultation of the carotid arteries to
ensure that no bruits are present. The patient has normal vital signs, which
rules out a sinus node dysfunction or AV nodal heart block. The patient
has no symptoms of palpitations, which makes AV nodal reentry or ven-
tricular arrhythmia unlikely.
See also Case 16 (Cardiogenic Syncope).
R-6. D. This patient has the classic triad of dementia, gait abnormality, and uri-
nary incontinence. Additionally, the CT scan shows dilated cerebral ven-
tricles. The dementia can be characterized with memory or concentration.
The difficulty with gait can be a shuffling or wide-based gait, or balance
issues related to apraxia (loss of previously learned motor function). The
urinary complaints can be urinary frequency or frank incontinence. Nor-
mal pressure hydrocephalus (NPH) is demonstrated by dilated ventricles
with a normal opening pressure on lumbar puncture (LP). Treatment such
as with ventriculoperitoneal (VP) shunt can reverse the symptoms.
See also Case 20 (Alzheimer Dementia) and Case 21 (Lewy Body Dementia).
R-7. C. The CSF characteristics associated with HSV meningoencephalitis usu-
ally show a moderately increased number of white blood cells (10-1000/
mm3), normal CSF glucose, elevated CSF protein, and numerous erythro-
cytes (10-500/mm3). A bloody CSF is distinctive. Answer A is more typical
of a bacterial meningitis with a large number of PMN cells, low-to-normal
glucose, high protein (>100 mg/dL), and few erythrocytes. Viral meningitis
is associated with less than 100 cells/mm3, usually with lymphocyte predom-
inance, normal glucose and protein, and no erythrocytes. Tuberculous (TB)
meningitis is associated with a moderate number of cells (10-1000), usually
of lymphocyte predominance, low glucose, elevated protein, but no RBCs.
See also Case 12 (Subarachnoid Hemorrhage), Case 25 (Acute Dissemi-
nated Encephalomyelitis), and Case 26 (Viral Meningitis).

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 472 CASE FILES: NEUROLOGY

R-8. D. This patient has median nerve entrapment, also known as carpal tunnel
syndrome. The carpal tunnel at the wrist impinges on the median nerve,
leading to motor weakness of the opposition of the thumb, and sensory
dysfunction of the palmar surface of the thumb, index finger, and middle
finger. An example of spinal cord anterior horn degeneration is amyotrophic
lateral sclerosis (ALS). A brachial plexopathy could involve the same nerve
roots, but there nerve roots contribute to other nerves and would involve
other dermatomes and muscles, not specifically innervated by the median
nerve.
See also Case 41 (Amyotrophic Lateral Sclerosis), Case 42 (Median Nerve
Mononeuropathy), and Case 43 (Foot Drop).
R-9. A. This child most likely has autism spectrum disorder. The history is typi-
cal with regression of developmental milestones, especially lack of social
interaction. Often there is also lack of verbal ability and stereotypical non-
purposeful behavior. Down syndrome would lead to developmental delay
from birth. Hearing loss must be ruled out in this case but would not fully
explain the lack of social interaction, stereotypical non-purposeful behav-
ior, or lack of eye contact. Fetal alcohol syndrome has a classic facies and
mental retardation from birth.
See also Case 51 (Autism Spectrum Disorder).
R-10. A. This is a very classic example of a simple febrile seizure, in which a child
has a single tonic-clonic seizure less than 15 minutes in duration, is aged
6 months to 6 years, is without neurologic findings, and is associated with
a fever that is not due to meningitis. In these cases, further imaging of the
brain or LP are not necessary. The use of prophylactic antiseizure medica-
tion is controversial.
See also Case 45 (Febrile Seizures).

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