CME

Multiple-Choice
Questions—Preferred
Responses

Following are the preferred responses for the Multiple-Choice Questions in

this issue. The questions and answer options are repeated, and
the preferred response appears in bold print, followed by an explanation
and a reference with which you may seek more specific information. You are
encouraged to review the responses and explanations carefully to evaluate your
general understanding of the course material. The comments and references
included with each question are intended to encourage independent study.

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
The correct answer is C. A typical neuroimaging feature of neurosarcoidosis,
but not neuromyelitis optica, is meningeal involvement with focal or diffuse
thickening of the meninges that typically enhances with gadolinium. All of the
other answer options may occur in both neuromyelitis optica and
neurosarcoidosis. For more information, refer to the article
‘‘Autoimmune Myelopathies’’ and to the following additional article.
Pickuth D, Heywang-Köbrunner SH. Neurosarcoidosis: evaluation with MRI. J Neuroradiol
2000;27(3):185Y188.

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 MCQs—Preferred Responses

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
The correct answer is A. The history and findings are consistent with autosomal
dominant hereditary spastic paraparesis with early onset and uncomplicated
phenotype. This is most commonly due to mutations in the atlastin GTPase 1
gene, ATL1 (SPG3A), encoding the atlastin-1 protein. For more information,
refer to the article ‘‘Hereditary Myelopathies’’ and to the
following additional article.
Dürr A, Camuzat A, Colin E, et al. Atlastin1 mutations are frequent in young-onset autosomal
dominant spastic paraplegia. Arch Neurol 2004;61(12):1867Y1872.

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
The correct answer is E. This patient most likely has polyradiculopathy related
to porphyria. The important clues in the history include the onset of symptoms
preceded by use of a sulfonamide, the presence of autonomic instability
(hypertension and tachycardia), constipation and ileus, and the presence of
CNS symptoms (drowsiness). Although the other answer options may have
some of the symptoms associated with polyradiculopathy, none would be
expected to have all of these features. For more information, refer to the
article ‘‘Acute and Chronic Polyradiculopathies.’’

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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 levels 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
The correct answer is C. The patient has a spinal epidural abscess. The most
common causative agent is Staphylococcus aureus. For more information,
refer to the article ‘‘Infectious Myelopathies’’ and to the
following additional articles.
Kaufman DM, Kaplan JG, Litman N. Infectious agents in spinal epidural abscesses. Neurology
1980;30(8):844Y850.

Baker AS, Ojemann RG, Swartz MN, Richardson EP Jr. Spinal epidural abscess. N Engl J Med
1975;293(10):463Y468.

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
The correct answer is C. When breast and lung cancer produce brachial
plexopathy from direct tumor invasion, the lower trunk and medial cord
are typically affected initially. Radiation injury tends to affect the upper trunk
and lateral cord of the plexus because of the protective effect the clavicle has
on the lower plexus during radiotherapy. A Horner syndrome is rare in
radiation plexopathy but is not always present with malignant plexopathy.

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 MCQs—Preferred Responses

Lymphedema may be present in any patient who has received prior radiation
to the axilla, and its presence does not exclude malignancy. Both radiation
injury and tumor recurrence may appear years after the initial cancer
diagnosis. Malignant plexopathy tends to be more painful than radiation
plexopathy, but this is not a universal finding. For more information, refer
to the article ‘‘Metastases Involving Spinal Cord, Roots,
and Plexus.’’

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
The correct answer is C. The presence of sensory ataxic neuropathy with
autonomic involvement is highly suggestive of Sjögren syndrome (SS).
Peripheral nervous system disease often precedes the clinical diagnosis of
SS. The MRI in patients with a sensory ataxia may show T2 hyperintensity
in the cervical spinal cord. Anatomicopathologic evidence suggests that
ganglioneuritis may be the cause of the sensory ataxic form of neuropathy
seen in SS. For more information, refer to the article
‘‘Diagnostic Approach to Myeloneuropathy’’ and to the following
additional articles.
Mori K, Iijima M, Koike H, et al. The wide spectrum of clinical manifestations in Sjögren’s syndrome-associated
neuropathy. Brain 2005;128(pt 11):2518Y2534.

de Seze J, Delalande S, Fauchais AL, et al. Myelopathies secondary to Sjögren’s syndrome: treatment with
monthly intravenous cyclophosphamide associated with corticosteroids. J Rheumatol 2006;33(4):709Y711.

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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
The correct answer is E. The history and findings indicating involvement of the
posterior and lateral columns resembling subacute combined degeneration is
typical of vacuolar myelopathy. This slowly progressive myelopathy in adult
patients with is commonly associated with other neurologic manifestations,
including cognitive deficits and peripheral neuropathy. Vacuolar myelopathy
is a diagnosis of exclusion after considering other causes, such as
opportunistic infections and lymphoma. For more information, refer to the
article ‘‘Infectious Myelopathies’’ and to the following
additional article.
Petito CK, Navia BA, Cho ES, et al. Vacuolar myelopathy pathologically resembling subacute combined
degeneration in patients with the acquired immunodeficiency syndrome. N Engl J Med 1985;312(14):
874Y879.

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

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 MCQs—Preferred Responses

The correct answer is A. The combination of lower extremity spasticity

and prominent amyotrophy of distal hand and foot muscles is commonly
designated as Silver syndrome. It is differentiated from motor neuron
disorders by lack of denervation of paraspinal and proximal muscles. Silver
syndrome has been linked to mutation in the Berardinelli-Seip congenital
lipodystrophy 2 (seipin) gene, BSCL2. For more information, refer to the
article ‘‘Hereditary Myelopathies’’ and to the following
additional article.
Auer-Grumbach M, Schlotter-Weigel B, Lochmüller H, et al. Phenotypes of the N88S Berardinelli-Seip
congenital lipodystrophy 2 mutation. Ann Neurol 2005;57(3):415Y424.

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
The correct answer is E. This patient has numb chin syndrome, which is most
likely due to a bony metastasis to the mandible producing injury to the mental
nerve. A more proximal lesion of cranial nerve V (ie, at the foramen ovale
or cerebellopontine angle) is less likely because of the lack of weakness
in the muscles of mastication. A standard MRI of the brain will not include
the mandible. Dental examination usually is normal in these patients. The
audiogram would not be abnormal in a patient with numb chin syndrome.
For more information, refer to the article ‘‘Metastases Involving
Spinal Cord, Roots, and Plexus.’’

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

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 The correct answer is C. Approximately 10% of patients with NMO
antibodies have brain lesions involving the lateral, third, and fourth
ventricles and the hypothalamus. These areas are characterized by high
expression of aquaporin-4. For more information, refer to the
article ‘‘Autoimmune Myelopathies’’ and to the following
additional article.
Pittock SJ, Weinshenker BG, Lucchinetti CF, et al. Neuromyelitis optica brain lesions localized at sites of
high aquaporin 4 expression. Arch Neurol 2006;63(7):964Y968.

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
The correct answer is D. Based on the clinical history, MRI and CT
findings, and CSF analysis, this patient probably has neurosarcoidosis. The
diagnosis likely can be confirmed with transbronchial biopsy of the lymph
nodes or mediastinoscopy with biopsy. The established therapy for this
disease is corticosteroids given intravenously initially and then orally. The
CSF findings are not consistent with a diagnosis of multiple sclerosis. Neither
vasculitis nor multiple sclerosis would have lymphadenopathy, and
tubercular infection of the cord is highly unlikely. For more information,
refer to the article ‘‘Treatment of Acute Transverse Myelitis
and Its Early Complications.’’

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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
The correct answer is C. Copper deficiency that occurs in the setting of
malabsorption due to small bowel disease produces a myeloneuropathy
resembling that caused by vitamin B12 deficiency. While copper deficiency may
also cause sideroblastic anemia and other hematologic abnormalities, this does
not always occur. For more information, refer to the article
‘‘Diagnostic Approach to Myeloneuropathy’’ and to the following additional
articles.
Kumar N, Gross JB, Ahlskog JE. Copper deficiency myelopathy produces a clinical picture like subacute
combined degeneration. Neurology 2004;63(1):33Y39.

Goodman BP, Bosch EP, Ross MA, Hoffman-Snyder C, et al. Clinical and electrodiagnostic findings in
copper deficiency myeloneuropathy. J Neurol Neurosurg Psychiatry 2009;80(5):524Y527.

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
The correct answer is D. This patient most likely has POEMS syndrome
(polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy,
and skin changes). This disorder is associated with osteosclerotic myeloma,
which can typically be seen on a bone survey. The other answer options would

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 not be helpful in establishing the diagnosis of osteosclerotic myeloma. For
more information, refer to the article ‘‘Acute and Chronic
Polyradiculopathies.’’

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
The correct answer is C. The greatest concern in this clinical setting is a
pulmonary embolism (PE). Venous thromboembolic events are a common cause
of morbidity and mortality in patients with myelopathy, particularly if they are
nonambulatory. Spiral CT is a useful diagnostic tool in the diagnosis of PE, with
approximately 90% sensitivity and specificity. Only 30% of patients with PE have a
documented deep vein thrombosis by venous ultrasound, so a normal venous
ultrasound does not rule out PE. Based on the level of the lesion, this patient
would not be expected to have respiratory muscle weakness. It is highly unlikely
that the patient has Pneumocystis jiroveci pneumonia given the fact that he is
not immunocompromised and has a normal chest x-ray. Echocardiogram is likely
to be normal and is not useful in the diagnosis of PE. For more information, refer
to the article ‘‘Rehabilitation in Transverse Myelitis.’’

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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 MCQs—Preferred Responses

The correct answer is A. This patient most likely has leptomeningeal spread
of her small cell lung cancer. As many as 2% to 5% of patients with lung
cancer develop this complication. The relative absence of pain and bilateral
signs and symptoms makes a bilateral lumbosacral plexopathy less likely.
Involvement of the cauda equina with leptomeningeal metastases is
particularly common because of the settling of malignant cells in the
dependent areas of the spine. All levels of the CNS may be involved at
diagnosis or later in the course. MRI with contrast may demonstrate
leptomeningeal enhancement, although a negative study does not exclude
this disorder. The best confirmatory test is a CSF examination, which typically
demonstrates elevated protein, low glucose, and lymphocytic pleocytosis.
Eighty percent of patients will have a positive CSF cytology on the first
lumbar puncture. For more information, refer to the article
‘‘Metastases Involving Spinal Cord, Roots, and Plexus.’’

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
The correct answer is E. This patient’s history and examination are consistent
with adrenomyeloneuropathy, an X-linked disorder caused by mutations of
the ABCD1 gene encoding an ATP-binding cassette peroxisomal transporter,
which causes abnormal deposition of very long-chain fatty acids.
"-Galactosidase levels are reduced in Fabry disease, which produces a small
fiber neuropathy. Low vitamin D levels are not associated with myeloneuropathy.
GJB1 mutations produce X-linked Charcot-Marie-Tooth disease. CAG repeat
expansion of the androgen receptor gene causes bulbospinal muscular
atrophy (Kennedy disease). For more information, refer to the
article ‘‘Diagnostic Approach to Myeloneuropathy’’ and to the following
additional article.
Moser HW. Adrenoleukodystrophy, phenotypes, genetics, pathogenesis and therapy. Brain
1997;120(pt 8):1485Y1508.

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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
The correct answer is A. In patients with HIV or patients who are otherwise
immunosuppressed, a painful polyradiculopathy with cauda equina syndrome
and polymorphonuclear pleocytosis in the CSF is highly suggestive of
cytomegalovirus infection. For more information, refer to the
article ‘‘Infectious Myelopathies’’ and to the following additional article.
Fux CA, Pfister S, Nohl F, Zimmerli S. Cytomegalovirus-associated acute transverse myelitis in
immunocompetent adults. Clin Microbiol Infect 2003;9(12):1187Y1190.

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
The correct answer is C. This patient most likely has multiple sclerosis.
Neuromyelitis optica and systemic vasculitis are less likely. The best initial
therapy for this condition is IV methylprednisolone (1 g IV daily for 3 to 5 days).
Plasma exchange may be used for patients who fail to respond to IV steroids.
There is no established role for IV immunoglobulin or cyclophosphamide in
treating multiple sclerosis. Interferon beta reduces the risk of subsequent
relapse but does not treat the acute episode of demyelination. For more
information, refer to the article ‘‘Treatment of Acute Transverse
Myelitis and Its Early Complications.’’

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 MCQs—Preferred Responses

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
The correct answer is C. Involvement of the area postrema or other
circumventricular organs expressing high levels of aquaporin-4 is characteristic
of neuromyelitis optica spectrum disorders. Nausea, hiccups, or intractable
vomiting may be the sole manifestation of the presence of neuromyelitis
optica antibodies. For more information, refer to the article
‘‘Autoimmune Myelopathies’’ and to the following additional articles.
Apiwattanakul M, Popescu BF, Matiello M, et al. Intractable vomiting as the initial presentation of
neuromyelitis optica. Ann Neurol 2010;68(5):757Y761.

Misu T, Fujihara K, Nakashima I, et al. Intractable hiccup and nausea with periaqueductal lesions in
neuromyelitis optica. Neurology 2005;65(9):1479Y1482.

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
The correct answer is D. The history and findings are consistent with autosomal
dominant hereditary spastic paraparesis of late onset and uncomplicated
phenotype. The most common cause is mutations of the spastin gene, SPAST,
on chromosome 2p22 (SPG4), encoding the spastin protein. For more
information, refer to the article ‘‘Hereditary Myelopathies’’
and to the following additional article.
Hazan J, Fonknechten N, Mavel D, et al. Spastin, a new AAA protein, is altered in the most frequent
form of autosomal dominant spastic paraplegia. Nat Genet 1999;23(3):296Y303.

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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
The correct answer is B. Neurologic complications of Mycobacterium
tuberculosis remain common in developing countries. Myelopathy may
occur in the setting of tuberculous spondylitis (Pott disease), which typically
affects the anterior portions of the vertebral bodies. Although the other
answer options can produce myelopathy, bone involvement is not a typical
feature. For more information, refer to the article ‘‘Infectious
Myelopathies’’ and to the following additional article.
Schlesinger N, Lardizabal A, Rao J, McDonald R. Tuberculosis of the spine: experience in an inner city
hospital. J Clin Rheumatol 2005;11(7):17Y20.

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
The correct answer is D. Folate deficiency should be suspected in patients with a
history of alcohol abuse or use of drugs, such as phenytoin, that affect either
absorption or metabolism of folic acid. Folate deficiency can produce a
myeloneuropathy that resembles subacute combined degeneration. An anemia with
macrocytosis and hypersegmented polymorphonuclear leukocytes is typical. Plasma
homocysteine is elevated in folate deficiency and is useful to confirm the presence of
folate deficiency and to monitor the response to folate supplementation. For more

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 MCQs—Preferred Responses

information, refer to the article ‘‘Diagnostic Approach to

Myeloneuropathy’’ and to the following additional articles.
Kumar N. Metabolic and toxic myelopathies. Continuum Lifelong Learning Neurol 2008;14(3):91Y115.

Kumar N. Neurologic presentations of nutritional deficiencies. Neurol Clin 2010;28(1):107Y170.

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
The correct answer is E. The combination of bilateral Babinski signs and the
lower extremity areflexia with impaired vibration sensation together with
kyphoscoliosis and pes cavus is characteristic of Friedreich ataxia.
However, vitamin E deficiency caused by mutations in "-tocopherol transfer
protein and Bassen-Kornzweig syndrome (abetalipoproteinemia) leading to
malabsorption of lipid-soluble vitamins may resemble Friedreich ataxia. Vitamin
E supplementation may partially reverse the clinical phenotype. For more
information, refer to the article ‘‘Hereditary Myelopathies’’ and
to the following additional article.
Ouahchi K, Arita M, Kayden H, et al. Ataxia with isolated vitamin E deficiency is caused by mutations
in the alpha-tocopherol transfer protein. Nature Genet 1995;9(2):141Y145.

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
The correct answer is B. The degree of permanent disability a patient with
transverse myelitis may experience is difficult to predict at disease onset. Some
surrogate markers have been identified to guide this prediction. For example,
acute illness requiring ventilatory assistance is related to later impairment in
ambulation. Additionally, an increased number of white blood cells in the
CSF predicts worse functional mobility, as does age less than 3 years at disease
onset. The other answer options are not specifically associated with a worse
prognosis for recovery in transverse myelitis. For more information, refer to the
article ‘‘Rehabilitation in Transverse Myelitis.’’

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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
The correct answer is D. Vitamin E deficiency may produce a spinocerebellar
syndrome resembling Friedreich ataxia and clinically manifests with signs of a
cerebellar ataxia, posterior column sensory loss, pyramidal signs, and signs of
peripheral sensory loss. Somatosensory-evoked potentials typically indicate
central slowing, and EMG studies confirm an axonal, primarily sensory
peripheral neuropathy. Acquired causes of vitamin E deficiency include chronic
gastrointestinal disorders that result in malabsorption, including pancreatic
insufficiency. For more information, refer to the article ‘‘Diagnostic
Approach to Myeloneuropathy’’ and to the following additional articles.
Kumar N. Metabolic and toxic myelopathies. Continuum Lifelong Learning Neurol 2008;14(3):91Y115.
Kumar N. Neurologic presentations of nutritional deficiencies. Neurol Clin 2010;28(1):107Y170.

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
The correct answer is A. This patient most likely has Guillain-Barré syndrome (also
known as acute inflammatory demyelinating polyneuropathy [AIDP]). This is an
immune-mediated disorder that is often associated with an antecedent
infection that triggers an inflammatory response targeted against the nerves.

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 MCQs—Preferred Responses

A variety of preceding infections have been described in AIDP, including upper

respiratory infections (49%), gastrointestinal illness (10%), Epstein-Barr virus,
cytomegalovirus, and HIV. In patients with AIDP who have experienced a
preceding gastrointestinal illness, infections with Campylobacter jejuni are the
most common and are present in 15% to 38% of patients with AIDP overall. It is
believed that antibodies targeted against the lipopolysaccharide coat of C. jejuni
cross-react with structures on the peripheral nerve. For more information, refer to
the article ‘‘Acute and Chronic Polyradiculopathies.’’

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
The correct answer is E. The clinical picture is that of diffuse involvement
of lower motor neurons. While effective vaccination has made poliomyelitis due
to poliovirus very rare in the Western world, West Nile virus may also produce
acute flaccid paralysis. For more information, refer to the article
‘‘Infectious Myelopathies’’ and to the following additional article.
Sejvar JJ, Marfin AA. Manifestations of West Nile neuroinvasive disease. Rev Med Virol 2006;16(4):209Y224.

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
The correct answer is D. In patients with acute demyelination who do not
improve with IV methylprednisolone, plasma exchange is a reasonable next
step. A small study of plasma exchange in steroid-refractory patients with acute
inflammatory demyelinating events, including multiple sclerosis (MS),

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 demonstrated statistically significant benefits in terms of outcome compared
with those patients who received sham exchanges. It is unlikely that a slight
increase in oral corticosteroids would be beneficial. There is no known role for
IV immunoglobulin, cyclophosphamide, or infliximab in the treatment of acute
MS exacerbations. Infliximab, a tumor necrosis factor inhibitor, can actually
worsen MS. For more information, refer to the article
‘‘Treatment of Acute Transverse Myelitis and Its Early Complications.’’

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
The correct answer is C. The clinical picture suggests a complicated X-linked
hereditary spastic paraplegia referred to with the acronym CRASH (corpus
callosum hypoplasia, retardation, adducted thumbs, spastic paraplegia, and
hydrocephalus). This syndrome is caused by mutations of the L1 cell adhesion
molecule gene, L1CAM. For more information, refer to the
article ‘‘Hereditary Myelopathies’’ and to the following additional article.
Weller S, Gärtner J. Genetic and clinical aspects of X-linked hydrocephalus (L1 disease): mutations of
L1CAM gene. Hum Mutat 2001;18(1):1Y12.

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
The correct answer is B. This patient most likely has diabetic lumbosacral
radiculoplexus neuropathy (DLRPN), also known as diabetic amyotrophy.
This disorder is typically painful and affects upper lumbar segments. It is
often associated with weight loss and rapidly developing atrophy. DLRPN

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 MCQs—Preferred Responses

tends to occur during periods of relatively good diabetic control and is

believed to be caused by a diabetes-associated microvasculitis with associated
nerve ischemia. Although therapies have not been proven, many clinicians
have used IV immunoglobulin and short courses of IV methylprednisolone.
The focality and associated weight loss are not typical for acute inflammatory
demyelinating polyneuropathy, chronic inflammatory demyelinating
polyneuropathy, or spinal arteriovenous fistula. Another vasculitic process
would typically be more generalized. For more information, refer to the
article ‘‘Acute and Chronic Polyradiculopathies.’’

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)
The correct answer is B. The clinical syndrome and symmetric tract-specific
changes on MRI are characteristic of paraneoplastic myelopathies. Neural
autoantibodies recognized most frequently with paraneoplastic myelitis include
collapsin response-mediator protein-5 (CRMP-5)-IgG and amphiphysin-IgG. For
more information, refer to the article ‘‘Autoimmune
Myelopathies’’ and to the following additional article.
Flanagan EP, McKeon A, Lennon VA, et al. Paraneoplastic isolated myelopathy: clinical course and
neuroimaging clues. Neurology 2011;76(24):2089Y2095.

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
The correct answer is B. This patient most likely has a lung apex tumor
(Pancoast tumor), likely of lung primary. Lymphoma is less likely but remains

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 a possibility. Lung apex lesions typically invade the lower trunk, medial cord
of the brachial plexus, or the C8-T1 roots, producing pain, weakness, and
paresthesia in a C8-T1 distribution. The absence of a Horner syndrome
suggests that the lesion is likely distal to the C8-T1 roots. An EMG would
yield no additional information as to the underlying etiology of the nerve
injury. A CSF examination and bone marrow biopsy are of low yield. The
patient requires a chest CT followed by a lesional biopsy to confirm the
diagnosis. For more information, refer to the article
‘‘Metastases Involving Spinal Cord, Roots, and Plexus.’’

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
The correct answer is E. This patient has infection with varicella-zoster virus,
producing a focal L5 radiculopathy. Varicella-zoster virus typically affects the
dorsal root ganglion, but motor fibers may be involved. The hallmark vesicular
rash is typically located within the sensory dermatome and may be delayed
several days from the onset of symptoms. The other answer options are unlikely
to produce a monoradiculopathy with the rash described. For more information,
refer to the article ‘‘Acute and Chronic Polyradiculopathies.’’

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

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 MCQs—Preferred Responses

The correct answer is E. The main features that distinguish multiple sclerosis
(MS) from neuromyelitis optica (NMO) are the presence of a short
asymmetric spinal cord lesion (as opposed to longitudinally extensive
transverse myelitis in NMO) and the presence of NMO immunoglobulin G
antibodies. While proprioceptive loss is a prominent feature of MS,
it may also occur in NMO; painful spasms are more frequent in NMO
than in MS. Lymphocytic pleocytosis occurs in both MS and NMO,
although polymorphonuclear pleocytoses should indicate NMO. For more
information, refer to the article ‘‘Autoimmune Myelopathies’’
and to the following additional article.
Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol
2007;6(9):805Y815.

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
The correct answer is C. Intramedullary spinal cord metastases (ISCM) are rare
complications of systemic cancer. They often coexist with brain parenchymal
and leptomeningeal metastases. Occasionally, ISCM is the presenting
manifestation of systemic malignancy. In these patients, cancer of the lung is
usually the underlying malignancy. A search for the underlying malignancy
with CT of the chest and abdomen is the next appropriate diagnostic step.
For more information, refer to the article ‘‘Metastases Involving
Spinal Cord, Roots, and Plexus.’’

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

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b 36. Nausea, cystitis, thrombocytopenia
The correct answer is A. Cyclophosphamide is an alkylating agent with side
effects that include nausea, vomiting, hair loss, cytopenia, opportunistic
infection, and hemorrhagic cystitis. There is a small risk of delayed malignancy,
particularly leukemias. For more information, refer to the
article ‘‘Treatment of Acute Transverse Myelitis and Its Early Complications.’’

b 37. Aseptic meningitis, nephrotoxicity, anaphylaxis

The correct answer is B. IV immunoglobulin can cause headache, allergic
reactions (including anaphylaxis), pulmonary edema, venous thrombosis,
aseptic meningitis, acute renal failure, and dermatitis. For more information,
refer to the article ‘‘Treatment of Acute Transverse Myelitis
and Its Early Complications.’’

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

The correct answer is E. Patients with T2 injuries and above typically cannot
achieve ambulation. For more information, refer to the
article ‘‘Rehabilitation in Transverse Myelitis.’’

b 39. T5 level lesion

The correct answer is D. Patients with T3 to T11 injuries are able to use
braces for physiologic standing and therapeutic ambulation. For more
information, refer to the article ‘‘Rehabilitation in Transverse
Myelitis.’’

b 40. T12 level lesion

The correct answer is B. Patients with T12 to L2 injuries will likely be
able to achieve household ambulation. For more information, refer to the
article ‘‘Rehabilitation in Transverse Myelitis.’’

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