Postreading

SELF-ASSESSMENT
AND CME

Self-Assessment
and CME Test
By Douglas J. Gelb, MD, PhD, FAAN; D. Joanne Lynn, MD, FAAN

PERIPHERAL NERVE AND MOTOR NEURON DISORDERS

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POSTREADING TEST

ARTICLE 1: A STRUCTURED APPROACH TO THE DIAGNOSIS OF PERIPHERAL

NERVOUS SYSTEM DISORDERS

1 A lesion of which of the following peripheral nerves would be most

likely to result in a pure sensory syndrome with no motor
manifestations?

A common fibular (peroneal)

B deep fibular (peroneal)
C obturator
D saphenous
E superficial fibular (peroneal)

2 In a patient with monomelic amyotrophy (Hirayama disease), which

of the following muscles is likely to be strongest?

A abductor digiti minimi

B brachioradialis
C extensor digitorum
D first dorsal interosseous
E flexor pollicis longus

3 A mononeuropathy of which of the following nerves would be

expected to cause a pure motor syndrome?

A axillary
B obturator
C posterior interosseous
D tibial
E ulnar

4 Which of the following neuropathic conditions presents with a

proximal and distal asymmetric pattern of involvement?

A acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

B brachial amyotrophic diplegia
C Charcot-Marie-Tooth disease
D chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
E vasculitic neuropathy

1410 OCTOBER 2020

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ARTICLE 2: DIABETES AND METABOLIC DISORDERS AND THE PERIPHERAL
NERVOUS SYSTEM

5 Which type of peripheral neuropathy is most common in patients with

diabetes?

A acute diabetic radiculoplexopathy

B autonomic neuropathy
C length-dependent axonal polyneuropathy
D mononeuropathy
E treatment-induced neuropathy of diabetes

6 What risk factor for neuropathy is most important to control in

type 1 diabetes?

A abdominal obesity
B hyperglycemia
C hypertension
D hypertriglyceridemia
E low high-density lipoprotein levels

7 A 62-year-old man with type 2 diabetes develops the acute onset of

burning pain in his right hip and low back that spreads to involve the
whole leg and then to the left hip and leg. Over several weeks, he also
develops significant proximal weakness to the point that he ambulates
with a cane. This is associated with a 13.6-kg (30-lb) weight loss. EMG
shows involvement of lumbosacral roots, plexus, and nerves. What is
the most likely pathophysiology of this neuropathy?

A abnormal sphingolipid metabolism

B inflammation secondary to dyslipidemia
C microvasculitis
D mitochondrial dysfunction
E oxidative stress

8 Which of the following autonomic neuropathic complications of

diabetes is associated with the highest mortality?

A bladder dysfunction
B gastroparesis
C gustatory sweating
D obstipation
E orthostatic hypotension

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POSTREADING TEST

ARTICLE 3: GUILLAIN-BARRÉ SYNDROME

9 In a patient for whom the diagnosis of acute inflammatory

demyelinating polyradiculoneuropathy (AIDP) is being considered
because of progressive sensory and motor symptoms over several
days, which of the following features would most likely suggest an
alternate diagnosis?

A back pain at onset of symptoms

B bilateral facial weakness
C lightheadedness when standing from a seated position
D onset of weakness in proximal muscles
E severe urinary sphincter dysfunction at onset of symptoms

10 One week after hospitalization for Guillain-Barré syndrome, which of

the following parameters has the most value in predicting the
probability that a patient will be able to walk independently in
6 months?

A CSF protein level

B deep fibular (peroneal) distal motor latency
C oxygen saturation level
D serum antiganglioside antibody titer
E severity of weakness

11 A 58-year-old man began having trouble walking 2 days ago, and it has
progressed to the point where he cannot ambulate unassisted. He has
also started to experience diplopia. On examination, he can only
abduct each eye 20 degrees, and he is unable to move his left eye up or
down. He has diffuse truncal and appendicular ataxia, and he is
areflexic. Antibodies to which of the following gangliosides are likely
to be present in his serum?

A Ga1NAc-GD1a
B GD1a
C GM1
D GM1b
E GQ1b

1412 OCTOBER 2020

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ARTICLE 4: CHRONIC INFLAMMATORY DEMYELINATING
POLYRADICULONEUROPATHY AND ITS VARIANTS

12 A unique finding of both acute and chronic demyelinating

polyneuropathies is predominant involvement of the upper extremity
sensory nerves with relative sparing of which of the following nerves?

A axillary
B lateral plantar
C saphenous
D sural
E ulnar

13 A 58-year-old man presents with progressive painless weakness in a

distal greater than proximal pattern. Electrophysiologic studies
demonstrate an asymmetric multifocal process. CSF analysis
demonstrating elevated protein and a pleocytosis should raise concern
for which of the following?

A amyotrophic lateral sclerosis

B chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
C infectious or neoplastic polyradiculitis
D multifocal acquired demyelinating sensory and motor neuropathy
(MADSAM)
E multifocal motor neuropathy (MMN)

14 A 50-year-old man develops progressive generalized weakness and

distal-predominant sensory loss of subacute onset consistent with
chronic inflammatory demyelinating polyradiculoneuropathy
(CIDP). Clinical features include tremor and sensory ataxia. His
electrophysiologic studies demonstrate prolonged and absent F
waves, multiple areas of partial motor conduction block, and a sural
sparing pattern. Blood work for monoclonal gammopathy and
anti–myelin associated glycoprotein (MAG) antibodies are negative.
He has not responded to IV immunoglobulin (IVIg), corticosteroids,
or plasma exchange. What additional tests should be obtained?

A anti–tissue transglutaminase antibodies

B endomysial antibodies
C ganglioside GD1b antibody
D ganglioside GM1 antibody
E nodal and paranodal antibodies

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POSTREADING TEST

15 Which of the following differentiates multifocal motor neuropathy

(MMN) from amyotrophic lateral sclerosis?

A asymmetric weakness
B conduction block
C cramps
D fasciculations
E lack of pain

ARTICLE 5: CHARCOT-MARIE-TOOTH DISEASE AND OTHER HEREDITARY

NEUROPATHIES

16 Which of the following features would be the most compelling

evidence that a patient with peripheral polyneuropathy has an
acquired demyelinating neuropathy rather than a hereditary
demyelinating neuropathy?

A absence of affected family members

B absent ankle reflexes
C ankle plantar flexion weakness
D patchy slowing and abnormal temporal dispersion on nerve
conduction studies
E ulnar motor conduction velocity less than 38 m/s

17 Charcot-Marie-Tooth disease is most often caused by mutations in the

gene for which of the following proteins?

A connexin 32
B mitofusin 2
C myelin protein zero
D peripheral myelin protein 22
E voltage-gated sodium channel Nav1.7

18 Deletions in the gene responsible for Charcot-Marie-Tooth disease

type 1A can cause which of the following hereditary conditions?

A abetalipoproteinemia
B familial erythromelalgia
C hereditary brachial plexus neuropathy
D hereditary neuropathy with liability to pressure palsies (HNPP)
E phytanic acid deficiency

1414 OCTOBER 2020

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19 A US Food and Drug Administration (FDA)–approved antisense
oligonucleotide is available for which of the following
hereditary conditions?

A cerebrotendinous xanthomatosis
B Fabry disease
C giant axonal neuropathy 1
D Tangier disease
E transthyretin amyloidosis

ARTICLE 6: PERIPHERAL NEUROPATHIES ASSOCIATED WITH VASCULITIS

AND AUTOIMMUNE CONNECTIVE TISSUE DISEASE

20 Which of the following disorders is classified as a medium vessel

vasculitis?

A eosinophilic granulomatosis with polyangiitis

B giant cell arteritis
C granulomatosis with polyangiitis
D microscopic polyangiitis
E polyarteritis nodosa

21 Which of the following disorders should be considered when a

patient’s serum is positive by immunoassay for both myeloperoxidase
and proteinase 3 antineutrophil cytoplasmic antibody autoantibodies?

A drug-induced vasculitis
B eosinophilic granulomatosis with polyangiitis
C essential mixed cryoglobulinemia
D microscopic polyangiitis
E vasculitis associated with rheumatoid arthritis

22 Which of the following characteristics in combination with other

findings support a diagnosis of polyarteritis nodosa?

A adult-onset asthma
B antineutrophil cytoplasmic antibody antibodies
C cryoglobulinemia
D glomerulopathy
E serum positive for hepatitis B virus antigen

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POSTREADING TEST

23 A 64-year-old man presents with generalized weakness, palpable

purpura, diffuse joint pain, and peripheral neuropathy. The
neuropathy is distal and symmetric. Which of the following disorders
is most likely to present with this constellation of symptoms?

A cryoglobulinemic vasculitis
B eosinophilic granulomatosis with polyangiitis
C giant cell arteritis
D granulomatosis with polyangiitis
E microscopic polyangiitis

24 A 29-year-old woman presents with a mononeuritis multiplex type

neuropathy that has progressed over 3 months. She has experienced
constitutional symptoms of weight loss, fatigue, and diffuse muscle
aches over this time. Approximately 6 months ago, she was diagnosed
with new-onset asthma. Myeloperoxidase antineutrophil
cytoplasmic antibody test is positive. Which of the following is the
most likely diagnosis?

A cryoglobulinemic vasculitis
B eosinophilic granulomatosis with polyangiitis
C microscopic polyangiitis
D mixed connective tissue disease
E polyarteritis nodosa

ARTICLE 7: PERIPHERAL NEUROPATHIES DUE TO VITAMIN AND MINERAL

DEFICIENCIES, TOXINS, AND MEDICATIONS

25 Metformin can cause reduced absorption of which of the following

nutrients?

A copper
B vitamin B1 (thiamine)
C vitamin B6 (pyridoxine)
D vitamin B12 (cobalamin)
E vitamin E (α-tocopherol)

26 Excessive intake of which of the following nutrients can cause a

sensory ganglionopathy?

A copper
B vitamin B1 (thiamine)
C vitamin B6 (pyridoxine)
D vitamin B12 (cobalamin)
E vitamin E (α-tocopherol)

1416 OCTOBER 2020

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27 A motor-predominant upper extremity phenotype is most common
with neuropathy due to which of the following toxins?

A arsenic
B cisplatin
C lead
D paclitaxel
E thallium

28 Cold-induced hyperalgesia can be a manifestation of toxicity from

which of the following medications?

A amiodarone
B bortezomib
C colchicine
D isoniazid
E oxaliplatin

29 A 58-year-old woman developed severe nausea, abdominal pain,

and diarrhea 4 hours after returning from a seafood restaurant. Two
days later, she began to notice that when she touched cold objects,
they felt unbearably hot. She most likely ingested which of the
following toxins?

A brevetoxin
B ciguatoxin
C saxitoxin
D scombrotoxin
E tetrodotoxin

ARTICLE 8: MANAGEMENT OF NEUROPATHIC PAIN IN POLYNEUROPATHY

30 Which of the following symptoms is suggestive of opioid-induced

hyperalgesia in the setting of increased pain despite treatment with
escalating opioid doses?

A acral paresthesia
B diffuse allodynia
C hyperhidrosis
D migratory erythema
E urticaria

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POSTREADING TEST

31 A 45-year-old man with painful diabetic peripheral neuropathy

would like to try something to alleviate his pain but prefers a
nutraceutical option. Which of the following agents has the most
evidence of symptomatic benefit for painful diabetic neuropathy in
clinical studies?

A α-lipoic acid
B curcumin
C evening primrose
D ginkgo biloba
E omega-3 fatty acids

ARTICLE 9: AMYOTROPHIC LATERAL SCLEROSIS AND OTHER MOTOR

NEURON DISEASES

32 Patients with amyotrophic lateral sclerosis are most likely to develop

symptoms of which of the following disorders?

A behavioral variant frontotemporal dementia

B limbic-predominant age-related transactive response
DNA-binding protein 43 encephalopathy
C logopenic variant primary progressive aphasia
D progressive nonfluent aphasia
E semantic variant primary progressive aphasia

33 Mutations in which of the following genes are the most common cause
of familial amyotrophic lateral sclerosis?

A C9orf72
B FUS
C SOD1
D TARDBP
E VCP

34 A 56-year-old man with diffuse weakness and fasciculations,

prominent facial twitching, and gynecomastia should be tested for
mutations in the gene for which of the following?

A androgen receptor
B insulin like growth factor-1 (IGF1)
C progranulin
D survival of motor neuron 1, telomeric (SMN1)
E vascular endothelial growth factor (VEGF)

1418 OCTOBER 2020

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ARTICLE 10: SPINAL MUSCULAR ATROPHY

35 The majority of individuals affected with spinal muscular atrophy

have which type?

A spinal muscular atrophy type 0

B spinal muscular atrophy type 1
C spinal muscular atrophy type 2
D spinal muscular atrophy type 3
E spinal muscular atrophy type 4

36 What is the therapeutic action for onasemnogene abeparvovec-xioi

for the treatment of spinal muscular atrophy?

A deletion of SMN2 exon 7

B enhancement of SMN protein production
C gene replacement of SMN1
D improved transcription of SMN1 gene
E promotion of muscle growth

37 What is the route of administration for nusinersen?

A inhalation
B intramuscular
C intrathecal
D intravenous
E oral

ARTICLE 11: PERIPHERAL NEUROPATHIES ASSOCIATED WITH

MONOCLONAL GAMMOPATHIES

38 A 59-year-old man has had progressively worsening balance

problems over the past year. He also reports numbness in his hands
and feet, clumsiness of his hands, and fluctuating double vision, all of
which have gradually progressed. His examination is notable for
marked limitation of eye movements in all directions, impaired
vibration and position sensation in all limbs with a distal-to-proximal
gradient, truncal and appendicular ataxia, and areflexia. Testing
reveals an IgM gammopathy. The antibodies are most likely to be
directed against which of the following?

A gangliosides with a disialosyl epitope

B N-methyl-D-aspartate (NMDA) receptors
C vascular endothelial growth factor (VEGF)
D voltage-gated calcium channels
E voltage-gated potassium channels

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POSTREADING TEST

39 A 50-year-old woman began to experience numbness, tingling, and

weakness in her feet 3 months ago, and it rapidly progressed to the
point where she now has severe weakness in all limbs, bilateral
footdrop, and calf atrophy. Her examination is notable for skin
hyperpigmentation and hypertrichosis, hepatomegaly,
lymphadenopathy, diffuse weakness, distal sensory loss, and
areflexia. Electrodiagnostic studies are consistent with demyelinating
polyradiculoneuropathy. Serum protein electrophoresis reveals an
IgG lambda M protein. Which of the following blood tests is most
likely to be abnormal?

A angiotensin-converting enzyme
B antiphospholipid antibodies
C rheumatoid factor
D tissue transglutaminase antibodies
E vascular endothelial growth factor (VEGF)

40 Which of the following is the most common cause of polyneuropathy

in patients with multiple myeloma?

A autoantibodies
B chemotherapy
C diffuse compression of peripheral nerves
D diffuse infiltration of peripheral nerves
E diffuse ischemia of peripheral nerves

1420 OCTOBER 2020

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