Continnum Oct 2020

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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
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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
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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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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
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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
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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
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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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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
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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
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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
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C cryoglobulinemia
D glomerulopathy
E serum positive for hepatitis B virus antigen

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

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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)
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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
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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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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
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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
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Postreading
Self-Assessment
and CME Test—Preferred
Responses
By Douglas J. Gelb, MD, PhD, FAAN; D. Joanne Lynn, MD, FAAN
PERIPHERAL NERVE AND MOTOR NEURON DISORDERS

Following are the preferred responses to the questions in the Postreading
Self-Assessment and CME Test in this Continuum issue. The preferred
response is 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 article topic. The comments and references included
with each question are intended to encourage independent study.
US PARTICIPANTS: Upon completion of the Postreading Self-Assessment and
CME Test and issue evaluation online at continpub.com/CME, participants
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may earn up to 20 AMA PRA Category 1 CreditsTM toward SA-CME. US
participants have up to 3 years from the date of publication online to earn
SA-CME credits.
CANADIAN PARTICIPANTS:This program is an Accredited Self-Assessment

Program (Section 3) as defined by the Maintenance of Certification
Program of the Royal College of Physicians and Surgeons of Canada and
approved by the University of Calgary Office of Continuing Medical
Education and Professional Development. Canadian participants should
visit MAINPORT (www.mainport.org) to record learning and outcomes.
Canadian participants can claim a maximum of 20 hours per issue (credits
are automatically calculated).
ARTICLE 1: A STRUCTURED APPROACH TO THE DIAGNOSIS OF PERIPHERAL

NERVOUS SYSTEM DISORDERS
1 The preferred response is D (saphenous). The saphenous nerve has no motor

component, so a focal lesion of the saphenous nerve results in a pure sensory
syndrome. Lesions of mixed nerves can also cause pure or predominantly
sensory syndromes, especially in the early stages, in situations in which
preferential involvement of sensory fascicles exists. For more information, refer
SELF-ASSESSMENT
AND CME

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to page 1137 of the Continuum article “A Structured Approach to the Diagnosis of

Peripheral Nervous System Disorders.”
2 The preferred response is B (brachioradialis). Patients with monomelic

amyotrophy (Hirayama disease) primarily have involvement of muscles
innervated by the C7, C8, and T1 roots, resulting in progressive painless
weakness in the hand and forearm, with sparing of the brachioradialis muscle,
which derives its innervation from the C5 and C6 roots. For more information,
refer to page 1150 of the Continuum article “A Structured Approach to the
Diagnosis of Peripheral Nervous System Disorders.”
3 The preferred response is C (posterior interosseous). Most mononeuropathies

will be associated with both sensory and motor manifestations. The most
common mononeuropathies to cause a pure motor syndrome are those involving
nerves without sensory components, such as the posterior interosseous,
anterior interosseous, or suprascapular nerves. Occasionally, entrapment or
other pathologic processes involving a mixed nerve will result in a pure motor
presentation because of preferential sparing of fascicles serving sensation. For
more information, refer to page 1136 of the Continuum article, “A Structured
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Approach to the Diagnosis of Peripheral Nervous System Disorders.”
4 The preferred response is E (vasculitic neuropathy). Mononeuritis multiplex

generally presents with significant initial asymmetry affecting multiple individual
nerves in both distal and proximal distributions. Vasculitis and other systemic
inflammatory disorders, lymphoma, diabetes, and viral infection are common
causes of multiple mononeuropathies. Acute inflammatory demyelinating
polyradiculoneuropathy (AIDP) and chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP) most often present as symmetric distal and
proximal distributions. Charcot-Marie-Tooth disease would be expected to
present in a distal symmetric pattern and brachial amyotrophic diplegia in a
proximal symmetric pattern. For more information, refer to page 1139 of the
Continuum article, “A Structured Approach to the Diagnosis of Peripheral
Nervous System Disorders.”
ARTICLE 2: DIABETES AND METABOLIC DISORDERS AND THE PERIPHERAL

NERVOUS SYSTEM
5 The preferred response is C (length-dependent axonal polyneuropathy). The

most common type of diabetic neuropathy is length-dependent axonal
polyneuropathy, which can predominantly affect small-diameter or
large-diameter axons, or both. This type of neuropathy accounts for
approximately 50% to 75% of cases of diabetic neuropathy. Mononeuropathies are
common, and diabetes causes nerves to be more vulnerable to compression.
Autonomic neuropathies are not uncommon and may have multiple different
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manifestations. Acute diabetic radiculoplexopathies and treatment-induced

neuropathy are relatively uncommon neurologic manifestations of diabetes. For
more information, refer to page 1162 of the Continuum article “Diabetes and
Metabolic Disorders and the Peripheral Nervous System.”
6 The preferred response is B (hyperglycemia). For type 1 diabetes, the risk of

neuropathy is directly linked to glycemic control. Optimal glycemic control in
type 1 diabetes reduces the relative risk of development of neuropathy by
almost 80%. In contrast, tight glycemic control is much less effective at reducing
the risk of development of neuropathy in type 2 diabetes, and other individual
components of the metabolic syndrome appear to contribute significantly to
the overall risk. For more information, refer to page 1163 of the Continuum article
“Diabetes and Metabolic Disorders and the Peripheral Nervous System.”
7 The preferred response is C (microvasculitis). This case is most consistent with

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diabetic lumbosacral radiculoplexus neuropathy or diabetic amyotrophy. This
entity is notable for severe pain starting in one lower limb and frequently
progressing to the other associated with the development of proximal weakness
and relative sparing of sensory functions. It is often associated with weight loss.
The underlying pathophysiology has been shown to be a microvasculitic process
causing ischemic nerve injury. It typically progresses over months and takes 1 to
2 years for incomplete recovery to occur. For more information, refer to
page 1171 of the Continuum article “Diabetes and Metabolic Disorders and the
Peripheral Nervous System.”
8 The preferred response is E (orthostatic hypotension). Diabetic cardiovascular

autonomic neuropathy includes manifestations of increased resting heart rate,
diminished heart rate response to physiologic stress, decreased cardiac output,
and orthostatic hypotension. Related to vagus nerve denervation, diabetic
cardiovascular autonomic neuropathy is associated with increased mortality,
especially when orthostatic hypotension is present. For more information, refer
to page 1173 of the Continuum article “Diabetes and Metabolic Disorders and the
Peripheral Nervous System.”
ARTICLE 3: GUILLAIN-BARRÉ SYNDROME
9 The preferred response is E (severe urinary sphincter dysfunction at onset of

symptoms). Most patients with acute inflammatory demyelinating
polyradiculoneuropathy (AIDP) present with back pain, radicular pain, painful
paresthesia, or other sensory symptoms. Cranial nerve symptoms, including
facial weakness, are common. Orthostatic hypotension and other features of
autonomic dysfunction may also occur in AIDP. Although ascending weakness is
the classic pattern, the weakness may start proximally. Severe sphincter
dysfunction at presentation is unusual and suggests the possibility of spinal

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cord, conus medullaris, or cauda equina dysfunction. For more information, refer
to page 1186 of the Continuum article “Guillain-Barré Syndrome.”
10 The preferred response is E (severity of weakness). A functional outcome

model based on age, the presence or absence of preceding diarrhea, and
severity of weakness (quantified using the Medical Research Council [MRC]
sum score) predicts the probability of walking independently at 1, 3, and
6 months. For more information, refer to page 1190 of the Continuum article
“Guillain-Barré Syndrome.”
11 The preferred response is E (GQ1b). This patient has ophthalmoparesis, ataxia,

and areflexia, the classic triad of the Miller Fisher variant of Guillain-Barré
syndrome. More than 80% of patients with Miller Fisher syndrome have
anti-GQ1b antibodies in their serum. For more information, refer to page 1189 of
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the Continuum article “Guillain-Barré Syndrome.”
ARTICLE 4: CHRONIC INFLAMMATORY DEMYELINATING

POLYRADICULONEUROPATHY AND ITS VARIANTS
12 The preferred response is D (sural). The presence of the sural sparing pattern
on electrophysiologic studies is a hallmark of chronic inflammatory
demyelinating polyradiculoneuropathy (CIDP) and other acquired primary
demyelinating neuropathies. For more information, refer to page 1208
of the Continuum article “Chronic Inflammatory Demyelinating
Polyradiculoneuropathy and Its Variants.”
13 The preferred response is C (infectious or neoplastic polyradiculitis). Chronic

inflammatory demyelinating polyradiculoneuropathy (CIDP) and most of its
variants are associated with a CSF albuminocytologic dissociation with
elevated protein but normal cell count or a normal profile as in multifocal
CIDP. A significant CSF pleocytosis should raise concern for the possibility
of an infectious or neoplastic cause of symptoms such as human
immunodeficiency virus (HIV), lymphoma, or leptomeningeal carcinomatosis.
For more information, refer to page 1207 of the Continuum article “Chronic
Inflammatory Demyelinating Polyradiculoneuropathy and Its Variants.”
14 The preferred response is E (nodal and paranodal antibodies).

Approximately 10% of patients with chronic inflammatory demyelinating
polyradiculoneuropathy (CIDP) will have autoantibodies directed against
paranodal or nodal antigens. These nodo-paranodopathies should be
suspected if the presentation includes distal-predominant presentation,
sensory ataxia, prominent tremor, and poor response to the usual beneficial
treatments. Some patients with this variant have been observed to respond
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to rituximab. For more information, refer to page 1217 of the Continuum article
“Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Its Variants.”
15 The preferred response is B (conduction block). Multifocal motor neuropathy

(MMN) generally presents as a painless asymmetric progressive weakness
associated with cramps and fasciculations. These findings are also common in
amyotrophic lateral sclerosis. However, conduction block in multiple nerves is
the hallmark of MMN. For more information, refer to page 1214 of the Continuum
article “Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Its
Variants.”
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ARTICLE 5: CHARCOT-MARIE-TOOTH DISEASE AND OTHER HEREDITARY
NEUROPATHIES
16 The preferred response is D (patchy slowing and abnormal temporal

dispersion on nerve conduction studies). Inherited demyelinating neuropathies
usually cause uniform slowing of conduction velocities, although exceptions
exist. In contrast, patchy slowing, partial or total conduction blocks, and
abnormal temporal dispersion are common in acquired demyelinating
neuropathies. Patients with inherited neuropathies often have no affected
family members because of de novo mutations. Areflexia, weakness of ankle
dorsiflexion and plantar flexion, and slow conduction velocities are typical of
both inherited and acquired demyelinating neuropathies. For more information,
refer to page 1229 of the Continuum article “Charcot-Marie-Tooth Disease and
Other Hereditary Neuropathies.”
17 The preferred response is D (peripheral myelin protein 22). The most common
form of Charcot-Marie-Tooth disease (CMT) is type 1A, which accounts for
approximately 70% of all CMT1 cases and more than 50% of all CMT cases.
CMT1A is caused by mutations in the gene for peripheral myelin protein 22. For
more information, refer to page 1230 of the Continuum article “Charcot-Marie-

Tooth Disease and Other Hereditary Neuropathies.”
18 The preferred response is D (hereditary neuropathy with liability to pressure

palsies [HNPP]). In most patients with HNPP, the genetic cause is a deletion in
the gene for peripheral myelin protein 22, the same gene responsible for
Charcot-Marie-Tooth disease type 1A (CMT1A). For more information, refer to
page 1239 of the Continuum article “Charcot-Marie-Tooth Disease and Other
Hereditary Neuropathies.”
19 The preferred response is E (transthyretin amyloidosis). Two small

oligonucleotide-based gene therapies have been approved by the US Food and
Drug Administration (FDA) for transthyretin amyloidosis. One (patisiran) is a small

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interfering RNA, and the other (inotersen) is an antisense oligonucleotide. For
more information, refer to page 1243 of the Continuum article “Charcot-Marie-

Tooth Disease and Other Hereditary Neuropathies.”
ARTICLE 6: PERIPHERAL NEUROPATHIES ASSOCIATED WITH VASCULITIS

AND AUTOIMMUNE CONNECTIVE TISSUE DISEASE
20 The preferred response is E (polyarteritis nodosa). Polyarteritis nodosa

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is a vasculitis that affects medium blood vessels such as arterioles. Polyarteritis
nodosa is one of the vasculitides that is most frequently associated with
neuropathy. For more information, refer to page 1258 of the Continuum article
“Peripheral Neuropathies Associated With Vasculitis and Autoimmune
Connective Tissue Disease.”
21 The preferred response is A (drug-induced vasculitis). Antineutrophil

cytoplasmic antibody (ANCA) positivity is common in multiple types of
vasculitis, including those associated with infections, inflammatory bowel
disease, and other autoimmune diseases. ANCA specificity to myeloperoxidase
is typically seen in microscopic polyangiitis and eosinophilic granulomatosis
with polyangiitis. Proteinase 3 specificity is typically seen in granulomatosis with
polyangiitis. If positivity for both myeloperoxidase and proteinase 3 is present,
then drug-induced vasculitis should be suspected. For more information, refer
to page 1265 of the Continuum article “Peripheral Neuropathies Associated
With Vasculitis and Autoimmune Connective Tissue Disease.”
22 The preferred response is E (serum positive for hepatitis B virus antigen). The
American College of Rheumatology classification criteria require that patient
presentations fulfill three or more of 10 criteria for the identification of
polyarteritis nodosa vasculitis patients for research purposes. The presence of
hepatitis B virus antigen is one characteristic supportive of a diagnosis of
polyarteritis nodosa. Antineutrophil cytoplasmic antibody antibodies,
glomerulopathy, adult-onset asthma, and cryoglobulinemia are generally not
present in patients with polyarteritis nodosa. For more information, refer to
page 1266 of the Continuum article “Peripheral Neuropathies Associated With
Vasculitis and Autoimmune Connective Tissue Disease.”
23 The preferred response is A (cryoglobulinemic vasculitis). The majority of

patients with cryoglobulinemia present with the classic triad of generalized
weakness, palpable purpura, and diffuse joint pain. Associated neuropathy may
occur in several different forms: distal and symmetric, small fiber predominant,
or multifocal neuropathy. Diagnosis requires careful testing for cryoglobulins,
and treatment must be focused at the underlying causative condition. For more
information, refer to page 1267 of the Continuum article “Peripheral
1426 OCTOBER 2020

is prohibited.
Neuropathies Associated with Vasculitis and Autoimmune Connective Tissue

Disease.”
24 The preferred response is B (eosinophilic granulomatosis with polyangiitis).

This presentation of subacute multifocal neuropathy with adult-onset asthma is
Page 16
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most consistent with eosinophilic granulomatosis with polyangiitis. Eosinophilic
granulomatosis with polyangiitis is accompanied by peripheral blood
eosinophilia and extravascular granulomatosis, which can affect multiple
organs. Patients with neuropathy have a high incidence of myeloperoxidase
antineutrophil cytoplasmic antibody positivity. For more information, refer to
page 1265 of the Continuum article “Peripheral Neuropathies Associated with
Vasculitis and Autoimmune Connective Tissue Disease.”
ARTICLE 7: PERIPHERAL NEUROPATHIES DUE TO VITAMIN AND MINERAL

DEFICIENCIES, TOXINS, AND MEDICATIONS
25 The preferred response is D (vitamin B12 [cobalamin]). Long-term metformin

use has been associated with poor absorption of vitamin B12 (cobalamin). For
more information, refer to page 1281 of the Continuum article “Peripheral
Neuropathies Due to Vitamin and Mineral Deficiencies, Toxins, and
Medications.”
26 The preferred response is C (vitamin B6 [pyridoxine]). Vitamin B6 toxicity

causes direct damage to dorsal root ganglion sensory neurons.
For more information, refer to page 1285 of the Continuum article “Peripheral
Medications.”
27 The preferred response is C (lead). Lead toxicity classically produces a

neuropathy with a motor-predominant upper extremity phenotype. For more
information, refer to page 1290 of the Continuum article “Peripheral
Medications.”
28 The preferred response is E (oxaliplatin). Toxicity from cisplatin, oxaliplatin,

or
carboplatin manifests as a sensory ganglionopathy; in addition, oxaliplatin causes
acute cold-induced hyperalgesia, which is probably due to increased neuronal
excitability resulting from oxaliplatin’s effect on voltage-gated sodium
channels. For more information, refer to page 1293 of the Continuum article
“Peripheral Neuropathies Due to Vitamin and Mineral Deficiencies, Toxins, and
Medications.”

is prohibited.
29 The preferred response is B (ciguatoxin). Ciguatoxin (ciguatera toxin) is

produced by a dinoflagellate and concentrated as it moves up the food chain
to reef-dwelling fish, such as barracuda, grouper, and snapper. It produces
gastrointestinal symptoms and a neuropathy, which often has the distinctive
feature of temperature inversion, in which cold objects are perceived as very
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hot. For more information, refer to page 1296 of the Continuum article
“Peripheral Neuropathies Due to Vitamin and Mineral Deficiencies, Toxins, and
Medications.”
ARTICLE 8: MANAGEMENT OF NEUROPATHIC PAIN IN POLYNEUROPATHY
30 The preferred response is B (diffuse allodynia). Opioid-induced hyperalgesia

should be considered when patients report worsening pain rather than
improvement with opioid treatment and experience diffuse allodynia. The
underlying mechanisms are thought to involve neuroplastic changes and opioid
activation of immune cells with resultant sensitization of opioid receptors. For
more information, refer to page 1310 of the Continuum article “Management of
Neuropathic Pain in Polyneuropathy.”
31 The preferred response is A (α-lipoic acid). α-Lipoic acid is an antioxidant that

has been shown to provide greater symptomatic relief compared to placebo
for patients with painful diabetic neuropathy, although several large clinical
trials have yielded conflicting evidence. α-Lipoic acid is generally well tolerated
and is a good option for use as second-line or adjunctive therapy and for
patients who are averse to conventional pharmacotherapeutics. Anecdotal
reports of improvement in neuropathy symptoms exist for curcumin. For more
information, refer to page 1311 of the Continuum article “Management of
Neuropathic Pain in Polyneuropathy.”
ARTICLE 9: AMYOTROPHIC LATERAL SCLEROSIS AND OTHER MOTOR

NEURON DISEASES
32 The preferred response is A (behavioral variant frontotemporal dementia). Of

the 5% to 15% of patients with amyotrophic lateral sclerosis who have frank
frontotemporal dementia, the majority have the behavioral variant. For more
information, refer to page 1326 of the Continuum article “Amyotrophic Lateral
Sclerosis and Other Motor Neuron Diseases.”
33 The preferred response is A (C9orf72). Hexanucleotide expansions in the

noncoding region of C9orf72 are the most common cause of familial
amyotrophic lateral sclerosis, accounting for 40% of familial cases in a European
amyotrophic lateral sclerosis population. For more information, refer to
1428 OCTOBER 2020

is prohibited.
page 1335 of the Continuum article “Amyotrophic Lateral Sclerosis and Other
Motor Neuron Diseases.”
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{10461234747337885226
34 The preferred response is A (androgen receptor). Spinal bulbar muscular
atrophy, an X-linked disorder caused by a trinucleotide repeat expansion in the
androgen receptor gene, typically presents in adulthood. It is characterized by
weakness, fasciculations, prominent facial twitching, and symptoms of
androgen sensitivity, including gynecomastia and reduced fertility. Most
patients also have sensory nerve involvement. For more information, refer to
page 1330 of the Continuum article “Amyotrophic Lateral Sclerosis and Other
Motor Neuron Diseases.”
ARTICLE 10: SPINAL MUSCULAR ATROPHY
35 The preferred response is B (spinal muscular atrophy type 1). Spinal muscular
atrophy (SMA) type 1 is the most common form of SMA and represents
approximately 60% of infants born with a SMA genotype. Infants affected with
SMA type 1 present in the first few months of life with muscle weakness and
delay or absence of early motor milestones. For more information, refer to
page 1352 of the Continuum article, “Spinal Muscular Atrophy.”
36 The preferred response is C (gene replacement of SMN1). Approved

by the US Food and Drug Administration (FDA) in 2019, onasemnogene
abeparvovec-xioi is an adeno-associated virus 9–delivered SMN1 gene
replacement therapy administered as a single IV dose. Clinical trials
demonstrated therapeutic benefits, including attainment of ability to sit
unassisted for at least 5 seconds in 92% of treated infants and improved
survival free of respiratory intervention. For more information, refer to
page 1357 of the Continuum article, “Spinal Muscular Atrophy.”
37 The preferred response is C (intrathecal). Nusinersen is an antisense

oligonucleotide that improves the inclusion of exon 7 during splicing of the SMN2
gene and is dosed intrathecally every 14 days for the first three loading doses,
followed by a dose 1 month later and then every 4 months for life. For more
information, refer to page 1363 of the Continuum article, “Spinal Muscular Atrophy.”
ARTICLE 11: PERIPHERAL NEUROPATHIES ASSOCIATED WITH MONOCLONAL

GAMMOPATHIES
38 The preferred response is A (gangliosides with a disialosyl epitope). This

patient’s clinical presentation, with gradually progressive sensory loss, ataxia,

is prohibited.
and ophthalmoparesis, is typical of CANOMAD (chronic ataxic neuropathy,

ophthalmoplegia, IgM paraprotein, cold agglutinins, and disialosyl antibodies)
syndrome. For more information, refer to page 1375 of the Continuum article
“Peripheral Neuropathies Associated With Monoclonal Gammopathies.”
Page 19
{10461234747337885226
39 The preferred response is E (vascular endothelial growth factor [VEGF]). This

patient has polyneuropathy, organomegaly, monoclonal gammopathy, and skin
changes, four of the features of POEMS (polyneuropathy, organomegaly,
endocrinopathy, monoclonal plasma cell disorder, and skin changes)
syndrome. Most patients with this syndrome have levels of VEGF that are more
than 5 times normal values. For more information, refer to page 1376 of the
Continuum article “Peripheral Neuropathies Associated With Monoclonal
Gammopathies.”
40 The preferred response is B (chemotherapy). Chemotherapy-induced

peripheral neuropathy is the most frequent cause of polyneuropathy in
multiple myeloma. For more information, refer to page 1375 of the Continuum
article “Peripheral Neuropathies Associated with Monoclonal Gammopathies.”
Page 20

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ARTICLE 1: A STRUCTURED APPROACH TO THE DIAGNOSIS OF PERIPHERAL

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

1410 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

ARTICLE 2: DIABETES AND METABOLIC DISORDERS AND THE PERIPHERAL

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

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

ARTICLE 3: GUILLAIN-BARRÉ SYNDROME

9 In a patient for whom the diagnosis of acute inflammatory

1412 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

ARTICLE 4: CHRONIC INFLAMMATORY DEMYELINATING

12 A unique finding of both acute and chronic demyelinating

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

15 Which of the following differentiates multifocal motor neuropathy

16 Which of the following features would be the most compelling

1414 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

19 A US Food and Drug Administration (FDA)–approved antisense

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

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

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

25 Metformin can cause reduced absorption of which of the following

1416 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

27 A motor-predominant upper extremity phenotype is most common

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

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

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

32 Patients with amyotrophic lateral sclerosis are most likely to develop

1418 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

ARTICLE 10: SPINAL MUSCULAR ATROPHY

35 The majority of individuals affected with spinal muscular atrophy

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

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

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

1420 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

Downloaded from http://journals.lww.com/continuum by

PERIPHERAL NERVE AND MOTOR NEURON DISORDERS

CANADIAN PARTICIPANTS:This program is an Accredited Self-Assessment

ARTICLE 1: A STRUCTURED APPROACH TO THE DIAGNOSIS OF PERIPHERAL

1 The preferred response is D (saphenous). The saphenous nerve has no motor

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

to page 1137 of the Continuum article “A Structured Approach to the Diagnosis of

2 The preferred response is B (brachioradialis). Patients with monomelic

3 The preferred response is C (posterior interosseous). Most mononeuropathies

4 The preferred response is E (vasculitic neuropathy). Mononeuritis multiplex

ARTICLE 2: DIABETES AND METABOLIC DISORDERS AND THE PERIPHERAL

5 The preferred response is C (length-dependent axonal polyneuropathy). The

POSTREADING TEST—PREFERRED RESPONSES

1422 OCTOBER 2020

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

manifestations. Acute diabetic radiculoplexopathies and treatment-induced

6 The preferred response is B (hyperglycemia). For type 1 diabetes, the risk of

7 The preferred response is C (microvasculitis). This case is most consistent with

8 The preferred response is E (orthostatic hypotension). Diabetic cardiovascular

9 The preferred response is E (severe urinary sphincter dysfunction at onset of

Copyright © American Academy of Neurology. Unauthorized reproduction of this article

10 The preferred response is E (severity of weakness). A functional outcome

11 The preferred response is E (GQ1b). This patient has ophthalmoparesis, ataxia,

ARTICLE 4: CHRONIC INFLAMMATORY DEMYELINATING