Self-Assessment and CME

Postreading
Self-Assessment and
CME Test
Adam Kelly, MD; D. Joanne Lynn, MD, FAAN

The Continuum Postreading Self-Assessment and CME Test is an integral

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b 1. Which of the following white matter disorders is most likely to initially

manifest with neurobehavioral symptoms and evolve to include a progressive
dementia in some cases?
A. AARS2-related leukoencephalopathy
B . adult-onset polyglucosan body disease
C. Fabry disease
D. hereditary diffuse leukoencephalopathy with spheroids
E . Kearns-Sayre syndrome

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 Postreading Test

b 2. A 25-year-old woman is involved in a motor vehicle accident and has a

closed head injury. She has persistent headaches after the accident and is
referred for a brain MRI. Her MRI is abnormal, with five ovoid periventricular
and subcortical white matter lesions that are suggestive of demyelination,
one of which is enhancing. She has no history of neurologic symptoms other
than the posttraumatic headaches. What is this patients approximate risk
of developing multiple sclerosis (MS) within the next 5 years?
A. 5%
B . 10%
C. 33%
D. 66%
E . 90%
b 3. Approximately what percentage of people with multiple sclerosis have their
first clinical symptom as a child (before the age of 18 years)?
A. 7%
B. 14%
C. 21%
D. 28%
E. 35%
b 4. Which of the following disease-modifying therapies for relapsing multiple
sclerosis is most strongly associated with an increase in herpetic infections,
including varicella reactivation?
A. dimethyl fumarate
B . fingolimod
C. glatiramer acetate
D. natalizumab
E . teriflunomide
b 5. A 34-year-old woman is seen in the emergency department for 4 days of
worsening right eye pain and visual loss in the right eye. Examination is
notable for blurring of the disc margin on the right and a right relative
afferent pupillary defect. Which of the following best describes the evidence
for treatment in this setting?
A. adrenocorticotropic hormone gel is more effective than IV steroids
B . IV methylprednisolone is more effective than low-dose oral prednisone
C. low-dose oral steroids are equally effective as IV steroids
D. no treatment has been shown to be more effective than placebo
E . plasma exchange is considered first-line therapy

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b 6. Which of the following disease-modifying therapies would require an
accelerated elimination procedure in the event of an unexpected pregnancy?
A. dimethyl fumarate
B . fingolimod
C. natalizumab
D. pegylated Interferon beta
E . teriflunomide
b 7. During which of the following pregnancy-related stages should treatment
of multiple sclerosis relapses with steroids be avoided, if possible, because of
a possible increase in the risk to the fetus or newborn?
A. first 3 months postpartum
B . first trimester
C. second trimester
D. third trimester
E . throughout the entire pregnancy
b 8. Which of the following MRI findings is relatively specific to cerebral
autosomal dominant arteriopathy with subcortical infarcts and
leukoencephalopathy (CADASIL) as compared to other genetic
leukoencephalopathies?
A. elevated lactate of affected areas on magnetic resonance spectroscopy (MRS)
B . enlarged perivascular spaces
C. prominent T2 hyperintensity of the external capsules
D. radiating stripes (tigroid pattern) of white matter abnormalities
E . T2-weighted hyperintensity of the dentate nuclei
b 9. Which of the following characteristics would be more commonly seen in
white matter hyperintensities from demyelinating disease as compared to
nonspecific white matter hyperintensities?
A. heterogeneous in signal intensity
B . indistinct borders
C. ovoid shape
D. punctate in size
E . subcortical location
b 10. Which of the following clinical or imaging characteristics may be helpful in
the early determination of patients with a more highly active (aggressive)
subtype of multiple sclerosis (MS)?
A. degree of T2 burden
B . higher burden of brainstem lesions
C. history of multifocal attacks affecting activities of daily living
D. presence of cortical atrophy
E . presence of T1 black holes

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 Postreading Test

b 11. Which of the following best describes the pattern of relapses during and
immediately following pregnancy as seen in the Pregnancy in Multiple
Sclerosis (PRIMS) study?
A. no observed relationship exists between pregnancy and relapse risk
B . relapse risk is decreased in the postpartum period
C. relapse risk is increased in the first trimester of pregnancy
D. relapse risk is increased in the postpartum period
E . relapse risk is increased throughout pregnancy
b 12. What is the mean age in years for onset of progressive multiple sclerosis?
A. 30
B . 35
C. 40
D. 45
E . 50
b 13. Which of the following characteristics is more likely to be seen in optic
neuritis in the setting of neuromyelitis optica (NMO) as opposed to optic
neuritis as a manifestation of multiple sclerosis (MS)?
A. absence of pain with eye movements
B . altitudinal visual field defects
C. excellent response to oral corticosteroids
D. mild visual impairment
E . unilateral presentation
b 14. A 63-year-old woman has an 18-year history of multiple sclerosis (MS).
She had multiple exacerbations earlier in her disease course. She has been
treated with interferon beta for many years; however, she has not had any
recent exacerbations in over 5 years and has had a slowly progressive
worsening of her gait over the past 2 years. She asks if she can stop her
interferon beta treatment. Which of the following factors would most
strongly support continued treatment with a disease-modifying agent?
A. current gadolinium-enhancing lesions
B . Expanded Disability Status Scale (EDSS) score
C. patient age
D. patient sex
E . presence of cerebral atrophy

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b 15. A 50-year-old woman with relapsing multiple sclerosis was recently
hospitalized after she developed diplopia and right-sided ataxia. An MRI of
the brain at that time showed an enhancing lesion in the pons, and she was
treated with 5 days of IV methylprednisolone. She is seen in clinic 2 weeks
later and reports no subjective improvement in her symptoms; her
examination is also unchanged. Which of the following is the best next option
in management?
A. additional 3 days of IV steroids
B . IVIg
C. natalizumab
D. oral prednisone therapy
E . plasma exchange
b 16. Which of the following disease-modifying therapies for relapsing multiple
sclerosis has been associated with the development of macular edema?
A. dimethyl fumarate
B . fingolimod
C. glatiramer acetate
D. natalizumab
E . teriflunomide
b 17. A 43-year-old woman is seen in clinic after an MRI ordered by her
primary care physician demonstrated multiple nonspecific white matter
hyperintensities. She had one episode of vertigo 4 years previously that was
attributed to vestibular neuritis but has not had other spells of neurologic
dysfunction. Which of the following imaging or clinical features would be most
suggestive of demyelinating disease as the cause of her imaging findings?
A. confluent white matter changes
B . family history of similar imaging findings
C. normal cervical and thoracic spinal cord imaging
D. presence of at least two oligoclonal bands on CSF testing
E . prominent involvement of external capsule
b 18. A 36-year-old woman with relapsing multiple sclerosis presents with
lancinating pain involving her right jaw. The pain is paroxysmal and is
triggered by speaking, chewing, touch to the area, or even the breeze from a
fan. Which of the following medications is supported by Level A evidence of
efficacy for the treatment of this clinical manifestation of multiple sclerosis?
A. baclofen
B . carbamazepine
C. gabapentin
D. lamotrigine
E . oxcarbazepine

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 Postreading Test

b 19. A 7-year-old girl presents with right leg weakness that developed several
days into a febrile illness marked by cough, sore throat, and rhinitis. The
weakness worsened over several days, and she developed plegia of the distal
right leg with loss of reflexes. MRI demonstrates a longitudinally extensive
thoracic spinal cord lesion primarily involving the gray matter and anterior
horn regions. Brain MRI is normal. This patients clinical scenario is most
compatible with which of the following clinical syndromes?
A. acute flaccid myelitis
B . acute idiopathic polyradiculoneuritis (Guillain-Barre syndrome)
C. monophasic acute disseminated encephalomyelitis
D. neuromyelitis optica (NMO) spectrum disorder
E . pediatric multiple sclerosis
b 20. Which of the following CSF patterns would be more likely in patients with
neuromyelitis optica (NMO) as opposed to relapsing multiple sclerosis (MS)?
A. absent oligoclonal bands
B . elevated opening pressure
C. lymphocytic cellular predominance
D. mild pleocytosis (less than 15 cells/mL)
E . normal protein level
b 21. Which of the following diagnostic changes occurred as a result of the
adoption of the 2010 revisions of the McDonald criteria for the diagnosis of
multiple sclerosis (MS)?
A. elimination of use of CSF examination in MS diagnosis
B . increased cost of diagnosis because of the requirement of more frequent
MRI scans
C. increased use of evoked potentials as an ancillary diagnostic tool
D. lengthening of time to diagnosis of definite relapsing MS
E . possibility of diagnosing MS decisively at the time of the first clinical attack
if MRI criteria are met
b 22. A 27-year-old woman is seen in clinic for management of newly diagnosed
multiple sclerosis. She has recently married and plans to start her family soon;
she would like to use a disease-modifying therapy that is considered safest
during pregnancy. Which of the following options has the best available
evidence regarding safety during pregnancy?
A. fingolimod
B . glatiramer acetate
C. interferon
D. natalizumab
E . teriflunomide

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b 23. Which of the following is a feature of the current concept of no evidence
of disease activity (NEDA) as a desired treatment goal in the assessment of
multiple sclerosis disease-modifying therapies?
A. demonstrated regression of MRI lesions
B . lack of disability progression
C. lack of fatigue or subjective neuropsychiatric symptoms
D. no limitation of mobility
E . no new brain atrophy beyond that expected for age
b 24. Which of the following leukoencephalopathies is related to expansion
of a trinucleotide repeat and therefore subject to anticipation (earlier
disease presentation in future generations) with transmission to
subsequent generations?
A. adrenoleukodystrophy
B . dentatorubral-pallidoluysian atrophy
C. Fabry disease
D. Kearns-Sayre syndrome
E . metachromatic leukodystrophy
b 25. Which of the following characteristics is the strongest predictor of the
time for patients with multiple sclerosis (MS) to reach Expanded Disability
Status Scale (EDSS) scores of 6.0 and 8.0?
A. age at MS diagnosis
B . lack of oligoclonal bands on CSF analysis
C. nonwhite race
D. sensory symptoms as initial MS symptoms
E . time to reach an EDSS score of 3.0
b 26. A 34-year-old patient with relapsing multiple sclerosis (MS) is asked to
consider smoking cessation because of the deleterious effects of smoking on
MS course. Which of the following is one of the known mechanisms of
tobacco use in worsening of MS?
A. decrease in Fas (CD95) expression on lymphocytes
B . decrease in matrix metalloproteinase
C. increase in antiapoptotic milieu
D. injury of the blood-brain barrier
E . reduction of oxidative stress

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 Postreading Test

b 27. A 12-year-old boy presents with lower extremity numbness that started
in his right foot, spreading over 3 days to involve his left leg and ascending
onto the trunk. His deficits reached a plateau after the third day. He has difficulty
starting his urinary stream. His examination is remarkable for moderate
weakness of both lower extremities and a T4 sensory level. Spinal cord MRI
shows an enhancing lesion involving the cervical spinal cord from the
C5 to C7 vertebral levels without significant cord expansion. His brain MRI
is unremarkable. CSF is notable for a mild lymphocytic pleocytosis and
moderate elevation of total protein. Which of the following clinical
characteristics is associated with a higher risk of disability and subsequent
relapse of this patients syndrome?
A. male sex
B . maximal severity at 3 days
C. partial cord syndrome
D. presence of a CSF pleocytosis
E . spinal cord lesion with gadolinium enhancement
b 28. Which of the following medications carries an indication for treatment of
secondary progressive multiple sclerosis (MS)?
A. fingolimod
B . glatiramer acetate
C. methotrexate
D. mitoxantrone
E . natalizumab
b 29. A 42-year-old man with relapsing multiple sclerosis presents to the
emergency department with 5 days of progressive bilateral lower extremity
weakness. MRI demonstrates an active area of demyelination in the upper
thoracic spinal cord. He is prescribed 1 g/d IV methylprednisolone. Which of
the following treatments should also be considered in this patient?
A. calcium plus vitamin D for prevention of bone density loss
B . furosemide for prevention of hyperkalemia
C. ranitidine for peptic ulcer prevention
D. tamsulosin for bladder dysfunction
E . trimethoprim-sulfamethoxazole for infection prophylaxis

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b 30. In patients with a radiologically isolated syndrome, which of the
following features is most likely to be associated with a high risk of
future dissemination in time?
A. contrast enhancement of one or more lesions
B . juxtacortical location
C. lesion size smaller than 3 mm
D. patient age older than 50
E . periventricular location
b 31. The area postrema syndrome (intractable hiccups, nausea, and vomiting)
is most commonly encountered as a presentation of which of the following
autoimmune conditions?
A. neuromyelitis optica (NMO)
B . primary angiitis of the central nervous system
C. relapsing multiple sclerosis
D. Sjogren syndrome
E . Whipple disease
b 32. Which of the following best describes the course and outcomes of
pregnancy in women with multiple sclerosis (MS)?
A. children of women with MS have lower Apgar scores
B . children of women with MS have lower birth weight
C. epidural anesthesia is associated with higher relapse risk
D. women with MS are at higher risk for eclampsia
E . women with MS are at higher risk for gestational diabetes
b 33. Which of the following best describes the results of the Cochrane
Systematic Review and other studies describing the evidence behind
treatment with oral versus IV steroids for patients with acute multiple
sclerosis relapses?
A. high-dose oral and IV methylprednisolone appear equally effective
B . high-dose oral prednisone is more effective than high-dose
IV methylprednisolone
C. IV methylprednisolone is more effective than oral methylprednisolone
D. low-dose IV methylprednisolone (40 mg/d) is equally effective as higher doses
E . oral steroid tapers are associated with improved functional outcomes

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 Postreading Test

b 34. A 62-year-old woman with a long-standing history of relapsing-remitting

multiple sclerosis (MS) maintained on interferon beta-1a presents with a
1-year history of gradual worsening of her gait with increasing leg spasms
and deteriorating balance. Her neurologic examination is remarkable for
4/5 strength in hip flexors and 4/5 strength in her knee flexors and ankle
dorsiflexors. Her sensory examination is notable for patchy decrease in
pinprick sensation in her right distal leg and foot and a moderate decrease
in vibration sense in both legs up to the knees. Hyperreflexia, spasticity,
and extensor plantar responses are present bilaterally. Her recent brain MRI
shows stable MS lesion load; cervical spine MRI shows no evidence of critical
stenosis or other superimposed conditions that might cause myelopathy.
Which of the following is the best next step in management?
A. change her disease-modifying therapy to fingolimod
B . check for interferon beta neutralizing antibodies
C. increase vitamin D3 supplementation dose
D. order an antiYJC virus antibody
E . order serum vitamin B12 level
b 35. The prognosis and outcome of which of the following leukoencephalopathies
can be improved with disease-modifying interventions?
A. adult-onset Alexander disease
B. cerebral autosomal dominant arteriopathy with subcortical infarcts and
leukoencephalopathy (CADASIL)
C. cerebrotendinous xanthomatosis
D. hereditary diffuse leukoencephalopathy with spheroids
E . vanishing white matter disease
b 36. Which of the following factors is unassociated with increased rates of
brain atrophy?
A. diabetes mellitus
B . exercise
C. higher BMI
D. hypertension
E . smoking
b 37. A 29-year-old man who was diagnosed with multiple sclerosis at age 25
has experienced multiple clinical relapses and evidence of additional disease
activity on imaging studies despite initial treatment with glatiramer acetate,
followed by fingolimod. Treatment with alemtuzumab is considered. For
which of the following adverse effects specific to alemtuzumab should he
monitored very closely?
A. cardiotoxicity
B . emergence of other autoimmune conditions
C. increased risk of bladder cancer
D. progressive multifocal leukoencephalopathy
E . toxic epidermal necrolysis

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b 38. Which of the following is the mechanism of action for teriflunomide as a
disease-modifying agent for relapsing multiple sclerosis?
A. inhibition of pyrimidine synthesis
B . lymphocyte sequestration
C. shift of cytokine milieu from TH1 to TH2
D. stabilization of blood-brain barrier
E . upregulation of T lymphocytes
b 39. Fingolimod may be associated with bradycardia, hypotension, and other
effects on atrioventricular conduction. Aside from initiation of fingolimod
treatment for the first time, which of these situations requires another first
dose observation protocol with monitoring of clinical status, vital signs, and
ECG before resumption of regular dosing?
A. missing a dose for 1 day during the second week of treatment
B . missing a dose for 1 day during the third week of treatment
C. missing doses for 4 days during the fourth week of treatment
D. missing doses for 7 days during the second month of treatment
E . missing 10 days during the third month of treatment
b 40. Which of the following best describes observed data and recommendations
for breast-feeding in women with multiple sclerosis?
A. glatiramer acetate should be avoided in women electing to breast-feed
B . monoclonal antibodies do not enter breast milk and thus are safe to use
C. regardless of disease-modifying therapy, breast-feeding should be discouraged
D. relapse rates appear to be decreased in women who breast-feed
E . women who are breast-feeding should not receive steroid treatment
during relapses

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