CME

Multiple-Choice Questions

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TYPE A QUESTIONS (SINGLE BEST ANSWER)

b 1. A 38-year-old man is evaluated for progressive leg weakness over the past
4 months. He had a previous cervical spinal cord lesion that transiently
improved after a 3-day course of IV methylprednisolone. He now presents
for reevaluation because of recent worsening of his symptoms despite
treatment with interferon beta for the presumptive diagnosis of multiple
sclerosis. A cervical cord MRI shows a T2 hyperintense lesion with patchy
gadolinium enhancement extending from C5 to T4. CSF examination shows
a lymphocytic pleocytosis (20 cells/dL, 80% monocytes, 20% lymphocytes)
and elevated protein concentration (80 mg/dL). Which of the following
findings is most likely to support the diagnosis of neurosarcoidosis as
opposed to neuromyelitis optica as the cause of this patient’s longitudinally
extensive transverse myelitis?
A. absence of supernumerary oligoclonal bands on CSF
B. bilateral involvement of the optic nerves
C. contrast enhancement in the meninges
D. improvement with prolonged use of high-dose prednisone
E. presence of T2 hyperintense lesion in the hypothalamus

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 Multiple-Choice Questions

b 2. An 8-year-old boy who was initially diagnosed with cerebral palsy is

evaluated for progressive difficulty walking over the past 5 years. He has
always had a tendency to stumble and difficulty running because of
stiffness in his legs. His 40-year-old father started having similar symptoms
by age 10 but can walk independently with a cane. Examination reveals
spastic paraparesis with hyperreflexia and bilateral Babinski signs. The
rest of the neurologic examination is normal. MRI of the head is
unremarkable. His IQ is normal for his age. The most likely cause of
this patient’s condition is a mutation of a gene encoding which of the
following proteins?
A. atlastin-1
B. paraplegin
C. probable helicase senataxin
D. seipin
E. spastin

b 3. A 25-year-old woman presents to the emergency department with

abdominal pain, constipation, vomiting, and extremity weakness. One week
ago she was diagnosed with a urinary tract infection and was prescribed
sulfamethoxazole. Three days ago she noted diffuse colicky abdominal pain
and distention with constipation and vomiting. Today she notes weakness
of all four limbs with prominent bilateral footdrop. On examination her
blood pressure is 170/100 mm Hg, her heart rate is 110 beats/min, and her
temperature is 37-C (99-F). She is drowsy but oriented. She has 4/5 diffuse
weakness. Deep tendon reflexes are absent, and the plantar responses are
flexor. Her abdomen is distended but not tender. Bowel sounds are absent.
The EMG demonstrates a diffuse axonal polyradiculopathy. Which of the
following conditions is the most likely diagnosis in this patient?
A. diphtheritic neuropathy
B. Guillain-Barré syndrome
C. lead toxicity
D. neurosarcoidosis
E. porphyria

b 4. A 45-year-old man with a history of diabetes and IV drug use is

evaluated for fever, back pain, leg weakness, and urinary hesitancy over
the past 3 days. Examination reveals paraparesis, absent knee jerks,
exaggerated ankle jerks, and bilateral Babinski signs. An MRI of the lumbar
spine reveals a contrast-enhancing epidural lesion at the T10 to T12 level
with associated swelling of the spinal cord. Which of the following organisms
is most likely to be involved in this patient’s condition?
A. Listeria monocytogenes
B. Pseudomona aeruginosa
C. Staphylococcus aureus
D. Staphylococcus epidermidis
E. Streptococcus pneumoniae

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b 5. A 45-year-old woman presents with a 3-week history of left arm
weakness, hand paresthesia, and aching shoulder discomfort. She was
diagnosed with stage II adenocarcinoma of the left breast 2 years ago.
She underwent lumpectomy, breast radiation (35 Gy), and six cycles of
adjuvant chemotherapy and is now believed to be in remission. Three
weeks ago she noted aching discomfort of the left shoulder (which
she reports as being 4/10 in severity), paresthesia of the left index finger
and thumb, and weakness of shoulder abduction and elbow flexion. On
examination, she has 4/5 strength in the left biceps, deltoid, supinator,
and pronator. The left biceps reflex is absent. All other muscles are strong
with 2/2 reflexes. Sensory examination is normal. She has mild
lymphedema of the left arm. Which of the following clinical features of this
patient is most suggestive of a radiation plexopathy as opposed to
malignant invasion of the plexus?
A. absence of a Horner syndrome
B. absence of severe pain
C. distribution of the weakness and paresthesia
D. presence of lymphedema
E. time interval from the original cancer diagnosis

b 6. A 45-year-old woman is evaluated for progressive numbness in the

hands and feet, gait difficulties, heat intolerance, and occasional postural
dizziness over the past 3 weeks. Examination shows severe sensory ataxia
with Romberg sign. Mental status, cranial nerve function, and motor
strength in all limbs are normal. The patient has hyporeflexia in the upper
limbs and areflexia in the lower limbs. Plantar responses are flexor. She has
symmetric loss of point position and vibration sense in the toes with mild
decrease in pinprick and temperature sensation at the toes, ankles, and
fingers. EMG shows reduced sensory nerve action potential amplitude in all
nerves tested; nerve conduction velocities and needle examination are
normal. MRI of the cervical spine shows an area of increased T2 signal in
the cervical spinal cord. Her serum vitamin B12, folate, and vitamin E levels
are normal. Autonomic testing shows impaired sweating in the limbs and
orthostatic hypotension. Which of the following conditions is the most likely
diagnosis for this patient?
A. adrenomyeloneuropathy
B. monoclonal immunoglobulin G paraproteinemia
C. Sjögren ganglionopathy
D. vitamin B6 deficiency
E. vitamin D deficiency

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 Multiple-Choice Questions

b 7. A 32-year-old man with HIV infection reports progressive leg paresthesia,

leg weakness, gait unsteadiness, and urinary incontinence. He has also
noticed difficulties with concentration and memory over the past 2 months.
He was started on antiretroviral therapy 1 month ago. Examination shows
spastic paraparesis, sensory ataxia, and loss of joint position and vibration
sense in the toes. He has no sensory level. Tibial somatosensory potentials are
absent. EMG reveals reduced amplitude of sensory and motor compound
action potentials and fibrillations in distal leg muscles. An MRI of the spinal
cord is normal. CSF shows 10 mononuclear cells, a protein concentration
of 50 mg/dL, and a glucose concentration of 60 mg/dL. PCR for HIV is
positive, but PCR and culture for herpes simplex virus types 1 and 2,
varicella-zoster virus, cytomegalovirus, Epstein-Barr virus, human T-cell
lymphotropic virus types I and II, and JC virus are negative. Which of the following
pathologic substrates is the most likely cause of this patient’s symptoms?
A. antiretroviral drugYinduced myeloradiculopathy
B. demyelinating polyradiculopathy
C. meningeal B-cell lymphomatosis
D. tuberculous meningovasculitis
E. vacuolar myelopathy

b 8. A 22-year-old man is evaluated for possible familial ALS. Over the past 2
years, he has developed progressive gait difficulties, hand weakness, and
footdrop. His mother died at age 65 after having developed progressive leg
weakness that caused her to be wheelchair dependent during the last 15 years
of her life. Neurologic examination shows spasticity in the lower limbs and
prominent atrophy of intrinsic hand and foot muscles. MRI of the head and
spine is normal. EMG shows denervation of C8 through T1 and distal L5
and S1 innervated muscles without denervation of proximal upper or lower
limb or paraspinal muscles. This syndrome has been linked to mutations
in which of the following genes?
A. Berardinelli-Seip congenital lipodystrophy 2 (seipin)
B. cytochrome P450, family 7, subfamily B, polypeptide 1
C. fatty acid 2-hydroxylase
D. proteolipid protein 1
E. survival of motor neuron 1, telomeric

b 9. An 80-year-old man with a history of metastatic prostate cancer presents

to the neurologist with a 4-week history of painless anesthesia over his left
chin. He has no other neurologic symptoms other than interscapular pain
from thoracic vertebral metastases for which he is currently undergoing
radiotherapy. Examination of the cranial nerves reveals a well-circumscribed
patch of reduced sensation to pinprick over the skin of his left chin. Which of
the following is most likely to be abnormal in this patient?
A. audiometry
B. dental examination
C. CSF cytology
D. MRI of the brain
E. panorex x-ray of the mandible

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b 10. A 44-year-old woman is evaluated for subacute onset of bilateral hand
numbness, leg weakness, and urinary retention. Examination shows spastic
paraparesis with bilateral Babinski signs and loss of vibration sense in the fingers and
toes. A cervical cord MRI shows a T2 hyperintense lesion with patchy gadolinium
enhancement extending from C6 to T2. The patient has positive neuromyelitis
optica (NMO) antibodies. Involvement of which of the following regions on head
MRI would be consistent with the diagnosis of an NMO spectrum disorder?
A. anterior thalamic nucleus
B. mesial temporal lobe
C. periventricular hypothalamus
D. pontine tegmentum
E. splenium of the corpus callosum

b 11. A previously healthy 35-year-old man presents to the emergency department

with a 3-week history of progressive leg weakness, ascending paresthesia, and
urinary retention. On examination he has 3/5 weakness in the lower extremities
with 4+ reflexes at the knee and ankle and bilateral extensor plantar reflexes.
Sensation to pinprick and temperature is present at the level of the nipples, and
vibration and joint position sense are absent at the level of the knees. The
bladder is distended. Urinary catheterization drains 700 mL of urine. An MRI of
the spine demonstrates an enhancing lesion within the cord at the T4 level.
The CSF examination shows a protein of 100 mg/dL, glucose of 30 mg/dL, white
blood cell count of 75 (100% lymphocytes), absent oligoclonal bands, negative
cytology, and negative culture and stains for bacteria and fungi. A CT of the chest
and abdomen reveals enlarged hilar lymph nodes. Which of the following
treatments is likely to be the best long-term regimen for this patient’s condition?
A. antitubercular therapy
B. cyclophosphamide
C. interferon beta
D. oral corticosteroids
E. plasma exchange

b 12. A 35-year-old woman with biopsy-proven celiac disease is evaluated for a 2-month
history of progressive gait unsteadiness and paresthesia in the fingertips and feet.
Neurologic examination shows sensory ataxia, mild spastic gait, and difficulty walking
on heels bilaterally. Romberg sign is present. Mental status, cranial nerve function,
and motor strength in the upper limbs are normal. She has mild weakness in foot
dorsiflexors bilaterally. Muscle stretch reflexes are normal in the upper extremities,
brisk at the knees, and absent at the ankles. Babinski signs are present bilaterally.
Vibration and joint position senses are absent at the toes and ankles bilaterally. The
rest of the neurologic examination is normal. MRI of the cervical spine reveals symmetric
T2 hyperintensity in the posterior columns without gadolinium enhancement. Blood
smear shows a megaloblastic anemia with neutropenia. Which of the following
tests is most likely to provide the diagnosis of this patient’s condition?
A. antigliadin antibodies
B. anti-Ro (SSA) and anti-La (SSB) antibodies
C. serum ceruloplasmin levels
D. serum ferritin levels
E. serum vitamin D levels

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 Multiple-Choice Questions

b 13. A 60-year-old man presents with a 2-month history of hand and foot
paresthesia and distal lower extremity weakness. He has noted increased
pigmentation of the skin and erectile dysfunction over the same time period. His
examination reveals hyperpigmented skin, mild gynecomastia, and hepatomegaly.
The neurologic examination is remarkable for diffuse distal-more-than-proximal
weakness and areflexia. The EMG shows a mixed axonal and demyelinating
polyradiculoneuropathy. Serum protein electrophoresis demonstrates a
monoclonal immunoglobulin G paraprotein. Which of the following studies is
most useful in confirming the probable diagnosis in this patient?
A. CSF cytology
B. fat aspirate for amyloid staining
C. liver biopsy
D. metastatic bone survey
E. muscle biopsy

b 14. A 40-year-old man with a 1-month history of progressive midthoracic

myelopathy is admitted to the neurology service. Based on the results of MRI,
CSF evaluation, and a lymph node biopsy, he is diagnosed with neurosarcoidosis
and started on IV methylprednisolone. After the first dose of steroids, the patient
develops acute dyspnea without cough or chest pain. On examination, his
blood pressure is 130/80 mm Hg, his heart rate is 110 beats/min, his temperature
is 37-C (98.6-F), and his respiratory rate is 20 breaths/min. The chest is clear to
auscultation. Heart sounds are normal. He has no swelling or tenderness in the
lower extremities. The neurologic examination demonstrates spastic paraparesis
with a sensory level at T6. Chest x-ray is normal, and the EKG shows only sinus
tachycardia. Serum troponins are normal. Arterial blood gas shows a respiratory
alkalosis and mild hypoxemia. Which of the following is the most important next
step in diagnosis of this patient?
A. obtain an echocardiogram
B. obtain lower extremity venous ultrasound
C. obtain spiral CT of the chest
D. perform Pneumocystis PCR on induced sputum
E. perform pulmonary function testing

b 15. A 68-year-old woman with small cell lung cancer reports a 2-week history
of painless right footdrop, bilateral foot paresthesia, and urinary retention.
On examination, the cranial nerves are normal. Upper extremity strength is
preserved. In the lower extremity, she has 3/5 strength in the right hamstring,
anterior tibialis, posterior tibialis, and peronei. Deep tendon reflexes are
absent in the lower extremities but are 2/2 in the arms. The plantar reflexes
are flexor, and she has saddle numbness to pin and temperature.
Anal tone is reduced. An MRI of the lumbosacral spine is normal. Which of the
following is the most appropriate next step in diagnosis of this patient?
A. CSF examination
B. CT of the pelvis
C. EMG/nerve conduction study
D. MRI of the cervical and thoracic spine
E. PET scan

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b 16. A 40-year-old man is evaluated for an 8-year history of progressive gait
difficulties. He had been very physically active up until the onset of his symptoms.
He started becoming increasingly clumsy and ultimately stopped running 4 years
ago because of frequent tripping. Over the past 2 years, he started experiencing
numbness in both legs and feet as well as urinary urgency. Family history is negative
for neurologic disease. Neurologic examination shows a spastic gait and a Romberg
sign. Mental status, cranial nerve function, and upper limb examination are normal.
He has symmetric weakness and atrophy in the leg and foot muscles, spasticity,
hyperreflexia, and Babinski signs. Sensation to pinprick, light touch, vibration, and
proprioception are symmetrically reduced below the knees. Spinal cord MRI shows
diffuse atrophy without signal changes in the cervical and thoracic spinal cord. EMG
shows diffuse mixed axonal-demyelinating peripheral neuropathy. Which of the
following determinations should be made to confirm a diagnosis in this patient?
A. !-galactosidase levels in leukocytes
B. CAG repeats in the androgen receptor gene
C. mutations in the GJB1 gene
D. plasma vitamin D levels
E. ratios of very long-chain fatty acids

b 17. A 28-year-old man with HIV infection is evaluated for bilateral leg pain,
paresthesia, leg weakness, and urinary retention that developed over the past
5 days. Examination shows weakness, areflexia, and sensory loss to all modalities
in the lower limbs and perianal anesthesia. Postvoid urinary residual is 300 mL.
CSF shows polymorphonuclear-predominant pleocytosis. Which of the following
infections is the most likely cause of this patient’s symptoms and clinical and
laboratory findings?
A. cytomegalovirus
B. Epstein-Barr virus
C. herpes simplex virus type 1
D. Toxoplasma gondii
E. Treponema pallidum

b 18. A 30-year-old woman presents to the emergency department with a 3-day history
of bilateral leg paresthesia and saddle anesthesia. Today she notes difficulty walking
and difficulty emptying her bladder. She has a prior history of right eye pain and visual
loss that resolved spontaneously 1 year ago. At that time, she was told the visual
loss was due to a viral infection. On examination, she has right optic disc pallor.
Strength is 4/5 in L5- and S1-innervated muscles. The deep tendon reflexes are brisk
throughout, and the plantar responses are extensor. She has saddle anesthesia and
reduced anal tone. The bladder is distended. An MRI of the spine shows a small,
minimally enhancing lesion at the conus. An MRI of the brain demonstrates several
nonenhancing periventricular white matter lesions. CSF examination reveals a protein
of 70 mg/dL, glucose of 60 mg/dL, white blood cell count of three, and five oligoclonal
bands. Which of the following is the best initial treatment for this condition?
A. cyclophosphamide
B. interferon beta
C. IV corticosteroids
D. IV immunoglobulin
E. plasma exchange

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 Multiple-Choice Questions

b 19. A 34-year-old man with a history of cigarette smoking and

rheumatoid arthritis is evaluated for intractable hiccups and vomiting
over the past 4 days. An MRI of the head shows an area of increased
T2 signal involving the area postrema and extending to the upper
cervical cord. CSF examination shows mild lymphocytic pleocytosis and a
moderate increase in protein concentration. Which of the following
tests is the most appropriate to conduct as a next step in diagnosing
this patient?
A. CT of the chest
B. ganglionic acetylcholine receptor antibody titers in CSF
C. serum neuromyelitis optica antibodies
D. serum rheumatoid factor and complement
E. visual-evoked potentials

b 20. A 42-year-old man is evaluated for progressive leg stiffness and

difficulty walking that began insidiously approximately 10 years ago. He
denies sensory loss or bladder difficulties. He has a 32-year-old sister
who recently noticed that she tends to stumble and fall easily. His
mother is also affected by similar symptoms that began in her thirties,
and she now requires a cane. Neurologic examination reveals weakness
in the iliopsoas, hamstring, and anterior tibialis muscles, hyperreflexia
at the knees and ankles, and bilateral Babinski signs. He has decreased
vibration sense in the toes. The rest of the examination is unremarkable.
MRI of the head and spine and CSF are normal. The most likely cause of
this patient’s condition is a mutation of a gene encoding which of the
following proteins?
A. atlastin-1
B. paraplegin
C. spartin
D. spastin
E. spatacsin

b 21. A 35-year-old Ethiopian woman is evaluated for a 3-week history of fever,

progressive back pain, leg weakness, and urinary hesitancy. Examination
reveals spastic paraparesis with a sensory level at T8. An MRI of the spine
shows anterior wedging of the T6 and T7 vertebrae with loss of disk space and
anterior epidural spinal cord compression. Which of the following agents is
the most likely cause of this patient’s condition?
A. Aspergillus fumigatus
B. Mycobacterium tuberculosis
C. Schistosoma mansoni
D. Taenia solium
E. Treponema pallidum

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b 22. A 44-year-old man with a history of alcohol abuse and epilepsy on chronic
treatment with phenytoin is evaluated for a 2-month history of progressive
gait unsteadiness and paresthesia in the fingertips and feet. Neurologic
examination shows sensory ataxia and difficulty walking on heels
bilaterally. Romberg sign is present. Mental status, cranial nerve function, and
motor strength in the upper limbs are normal. He has mild postural tremor
in the hands and mild weakness in foot dorsiflexors. Muscle stretch reflexes
are normal in the upper extremities, brisk at the knees, and absent at the
ankles. Babinski signs are present bilaterally. Vibration and joint position
senses are absent at the toes and ankles bilaterally. MRI of the cervical spine
reveals symmetric T2 hyperintensity in the posterior columns without
gadolinium enhancement. Blood smear shows a megaloblastic anemia and
neutropenia with hypersegmented neutrophils. Vitamin B12 (cobalamin)
and ceruloplasmin levels are normal. Which of the following laboratory
abnormalities is most likely to provide a clue about the cause of this
patient’s symptoms?
A. decreased methylmalonic acid levels
B. decreased vitamin D levels
C. decreased vitamin E levels
D. increased homocysteine levels
E. increased serum zinc levels

b 23. A 16-year-old girl is evaluated for progressive imbalance over the

past 3 years. Her family has no history of balance difficulties. Examination
shows kyphoscoliosis and bilateral pes cavus. She has mild ataxic dysarthria,
gait ataxia, absent knee and ankle jerks, bilateral Babinski signs, and absent
vibration and joint position sense in the toes and ankles. Motor and sensory
functions in the upper limbs are normal. MRI of the head and cervical spine
shows mild atrophy of the cervical cord. Genetic testing for a frataxin gene,
mutation is negative. Mutations of which of the following genes may also
produce a similar phenotype?
A. arylsulfatase A, ARSA
B. ATP-binding cassette, sub-family D (ALD), member 1, ABCD1
C. cytochrome P450, family 27, subfamily A, polypeptide 1, CYP27A1
D. galactosidase, alpha, GLA
E. tocopherol (alpha) transfer protein, TTPA

b 24. Which of the following clinical features or laboratory results predicts

an increased risk of permanent disability in a patient recovering from
transverse myelitis?
A. age greater than 40 years at disease onset
B. age less than 3 years at disease onset
C. elevated CSF protein level
D. increased red blood cells in the CSF
E. presence of oligoclonal bands in the CSF

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 Multiple-Choice Questions

b 25. A 44-year-old man with a history of chronic pancreatitis and steatorrhea is

evaluated for slowly progressive gait instability. Neurologic examination
shows severe gait ataxia and Romberg sign. Mental status is normal. He has
mild dysarthria but no nystagmus. Motor strength is normal. Finger-to-nose
testing is normal, but heel-to-knee testing shows dysmetria. Muscle stretch
reflexes are normal in the upper extremities, brisk at the knees, and absent at
the ankles. Babinski signs are present bilaterally. Vibration sense is absent,
and joint position sense is reduced at the toes and ankles bilaterally. MRI of
the head and cervical spine shows mild cervical cord atrophy. Vitamin B12,
folate, and ceruloplasmin levels are normal. Which of the following tests is the
most appropriate to perform next in this patient?
A. blood smear
B. serum homocysteine
C. serum vitamin D
D. serum vitamin E
E. visual-evoked potentials

b 26. A 22-year-old woman presents to the emergency department with a 4-day

history of tingling paresthesia in her hands and feet associated with aching
discomfort in her low back. For the past 2 days she has noted progressive
weakness of the legs, and she fell twice in the shower this morning. She
reports no change in bowel or bladder function. Two weeks ago she had a
diarrheal illness with fever, which has resolved. On examination, she is slightly
tachypneic. The cranial nerve examination reveals mild bilateral facial
weakness. She has 4/5 weakness proximally and distally in the upper and
lower extremities. The deep tendon reflexes are absent, and the plantar
responses are flexor. The sensory examination is normal. Evidence of which
of the following infectious agents is most likely to be present in this patient?
A. Campylobacter jejuni
B. Corynebacterium diphtheria
C. cytomegalovirus
D. Epstein-Barr virus
E. HIV

b 27. A 70-year-old man who emigrated from Mexico 10 years ago is evaluated
for low back pain and progressive asymmetric arm and leg weakness over the
past 6 days. He had visited his grandson at a summer camp 2 weeks ago.
Examination shows asymmetric flaccid weakness and reduced muscle stretch
reflexes in all limbs. Sensory examination is normal. EMG shows fibrillation
potentials in proximal and distal muscles in the four limbs. Nerve conduction
velocities are normal. CSF shows lymphocytic pleocytosis (50 cells/dL) and
mildly elevated protein concentration (60 mg/dL). Which of the following
additional tests on CSF would have the highest diagnostic yield in this case?
A. Blastomyces serology
B. ELISA assay for Cysticercus antigen
C. Lyme serology
D. Venereal Disease Research Laboratory
E. West Nile virus PCR

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b 28. A 30-year-old woman with a 2-year history of multiple sclerosis
presents with progressive lower extremity weakness, urinary retention, and
reduced sensation to pin and temperature at the level of the umbilicus. Lower
extremity strength is 3/5 with hyperreflexia and extensor plantar responses.
An MRI demonstrates an enhancing lesion within the cord at the T9 level.
She does not improve and even worsens slightly after 3 days of IV
methylprednisolone (1 g/d). She is placed on a tapering dose of oral
prednisone, starting at 60 mg/d, with plans to taper over 14 days. Which
of the following is the most appropriate next step in management of
this patient?
A. begin IV cyclophosphamide
B. begin IV immunoglobulin
C. begin IV infliximab
D. begin plasma exchange
E. increase prednisone to 80 mg/d and taper over 8 weeks

b 29. A 3-year-old boy is evaluated for mental retardation and delayed motor
milestones. Examination reveals lower extremity spasticity and adducted
thumbs. He has a similarly affected brother and two unaffected sisters.
Which of the following findings is most likely to be revealed on MRI?
A. band heterotopy
B. communicating hydrocephalus
C. corpus callosum hypoplasia
D. diffuse hypomyelination
E. syringobulbia

b 30. A 55-year-old woman with type 2 diabetes mellitus presents with a

3-week history of pain, paresthesia, and weakness in her left leg. During
the same period, she has lost 4.5 kg (10 lb) without trying. Her diabetes
has been reasonably well controlled. She describes burning pain in her left
flank and anterior thigh and has had difficulty walking, with the left knee
tending to buckle. On examination, she has marked wasting of the left
quadriceps muscle. She has 3/5 strength in the left iliopsoas, quadriceps,
and adductor muscles. The deep tendon reflexes are reduced throughout,
but the left quadriceps reflex is absent. She has reduced pinprick and
temperature sensation spanning the left T12-L3 dermatomes. An MRI of the
thoracic and lumbar spine is normal. Her fasting glucose is 148 mg/dL, and
her hemoglobin A1C is 6%. EMG demonstrates a predominantly axonal
polyradiculoneuropathy. Which of the following disorders is the most
likely diagnosis for this patient?
A. chronic inflammatory demyelinating polyneuropathy
B. diabetic amyotrophy
C. Guillain-Barré syndrome (acute inflammatory demyelinating
polyneuropathy)
D. spinal arteriovenous fistula
E. vasculitic mononeuritis multiplex

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 Multiple-Choice Questions

b 31. A 55-year-old man with a history of diabetes mellitus, rheumatoid

arthritis, and cigarette smoking is evaluated for progressive leg weakness
and stiffness over the past 7 months. Examination shows spastic
paraparesis, hyperreflexia, and bilateral Babinski signs. An MRI of the
spine demonstrates moderate cord swelling and T2 hyperintensity from
the T2 to T11 segments on sagittal view; coronal view shows symmetric
contrast enhancement restricted to the lateral columns. Serum
vitamin B12 and ceruloplasmin are within normal levels. Autoantibodies
against which of the following proteins are most likely to be associated with
this syndrome?
A. aquaporin-4
B. collapsin response-mediator protein-5 (CRMP-5)
C. glutamic acid decarboxylase
D. potassium channel complex
E. rho (SSA)/La (SSB)

b 32. A 60-year-old man presents with a 1-month history of burning pain

(which he reports as being 8/10 in severity) and paresthesia in the right
shoulder, medial forearm, and ulnar two fingers. More recently he has
noted right hand wasting and weakness. The pain keeps him awake at
night. On examination, no evidence of a Horner syndrome is present.
He has 3/5 weakness of the right thenar, hypothenar, and interossei
and 4/5 weakness in the right finger extensors and triceps muscles.
The right triceps reflex is reduced. The remainder of his examination is
normal. Which of the following studies is most likely to establish a diagnosis
in this patient?
A. bone marrow biopsy
B. chest CT
C. CSF examination
D. EMG/nerve conduction study
E. MRI of the cervical spine

b 33. A 75-year-old woman presents to her physician with burning

dysesthesias in the left buttock and posterior thigh that began 4 days
ago. Today she notes a mild left footdrop. On examination, she has 4/5
weakness in the left hamstring, gluteus medius, anterior tibialis, peronei,
and posterior tibialis muscles. The left ankle reflex is reduced. The
remainder of her neurologic examination is normal. A red macular rash
with a few vesicles is observed on her left buttock and posterior thigh.
Which of the following infectious agents is most likely the cause of this
patient’s symptoms?
A. Borrelia burgdorferi
B. Corynebacterium diphtheria
C. cytomegalovirus
D. herpes simplex virus
E. varicella-zoster virus

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b 34. A 24-year-old woman with a history of migraines is evaluated for numbness
and lack of dexterity in the right hand that developed over the course of
2 days. She has also noticed moderate gait imbalance, urinary urgency, and
intermittent painful spasms in her right arm lasting 20 to 45 seconds.
Examination shows sensory ataxia, severe proprioceptive loss on the right hand,
and moderate upper motor neuron distribution weakness in the right leg
with a Babinski sign. MRI studies reveal a well-circumscribed T2 hyperintense
lesion at the C5 level of the right spinal cord spanning one vertebral body, with
gadolinium enhancement on T1-weighted images. CSF examination shows 10
mononuclear cells/dL and elevated protein concentration (50 mg/dL). Which of
the following findings most strongly supports the diagnosis of multiple sclerosis
as opposed to neuromyelitis optica as the cause of myelopathy in this patient?
A. lymphocytic pleocytosis on CSF
B. mildly elevated CSF protein
C. painful spasms
D. sensory ataxia
E. short, asymmetric signal abnormality on cervical spinal MRI

b 35. A 60-year-old woman presents with a 2-week history of progressive

neck pain radiating into the shoulders bilaterally. She also notes paresthesia
in the feet, unsteady gait, and mild urinary retention. Her examination reveals 4/5
corticospinal distribution weakness in the legs with spasticity, hyperreflexia, and
extensor plantar responses. There is a subtle pin and temperature level to T2.
An MRI shows an enhancing lesion within the spinal cord at the T1 level. A CSF
examination is positive for malignant cells consistent with adenocarcinoma.
Which of the following is the most likely primary site of her malignancy?
A. breast
B. colon
C. lung
D. ovary
E. salivary gland

TYPE R QUESTIONS (EXTENDED MATCHING)

Theme: Treatments
Focus: Adverse effects associated with specific treatments

Match the adverse effects described below with the most likely associated
treatment. Answer options may be used more than once or not at all.

A. IV cyclophosphamide
B. IV immunoglobulin
C. IV methylprednisolone
D. oral azathioprine
E. plasma exchange

b 36. Nausea, cystitis, thrombocytopenia

b 37. Aseptic meningitis, nephrotoxicity, anaphylaxis

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 Multiple-Choice Questions

Theme: Level of spinal cord injury

Focus: Degree of ambulation achieved with rehabilitation

For each of the following levels of spinal cord injury, match the most likely
degree of ambulation that can be achieved with rehabilitation. Answer options
may be used more than once or not at all.

A. ability to ambulate in the community (more than 150 feet at a time)

B. ability to ambulate in the household
C. ability to ambulate without an assistive device
D. ability to use braces for physiologic standing
E. no ambulation

b 38. T1 level lesion

b 39. T5 level lesion

b 40. T12 level lesion

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