Self-Assessment and CME

Postreading Self-Assessment and CME Test

D. Joanne Lynn, MD, FAAN; Joseph E. Safdieh, MD, FAAN

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b 1. A 56-year-old man who is 3 months postYcadaveric kidney transplant develops fever, headache, confusion,
visual complaints, and nuchal rigidity over several weeks. Funduscopic examination shows findings consistent with
retinitis. His brain MRI shows ventriculomegaly and diffuse ependymal enhancement best visualized on the
diffusion-weighted images. Which of the following is the mostly likely etiology for this patients condition?
A. Cytomegalovirus (CMV)
B. progressive multifocal leukoencephalopathy
C. tuberculosis
D. varicella-zoster virus
E. West Nile virus

b 2. A 58-year-old man with long-standing rheumatoid arthritis and neck pain develops acute quadriparesis after
intubation in the emergency department during an acute episode of anaphylaxis caused by a newly prescribed
antibiotic. Which of the following is the most likely etiology of the quadriparesis?
A. atlantoaxial subluxation
B. nitrous oxide myelopathy
C. spinal cord infarction
D. subaxial subluxation
E. vertical subluxation

b 3. A 53-year-old man with Hodgkin lymphoma develops several months of progressive confusion, paranoid
ideation, and memory loss. Brain MRI shows increased T2 signal in the bilateral hippocampi. Antibodies against
which of the following targets are most likely to be associated with this syndrome?
A. glutamic acid decarboxylase
B. glycine receptor
C. metabotropic glutamate receptor 5
D. voltage-gated calcium channel
E. Yo

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 Postreading Test

b 4. A 68-year-old woman presents with a 1-week history of progressive diplopia and gait instability. She has
been undergoing chemotherapy for breast cancer, complicated by intractable vomiting. Her family notes that
she has seemed confused over the past 2 days. On examination, she demonstrates disorientation to date, 0/3
delayed recall, nystagmus, bilateral dysmetria, and an ataxic gait. MRI of the brain demonstrates nonenhancing
T2 hyperintensities in the periaqueductal gray matter and lateral to the third ventricle bilaterally. Which of the
following is the most likely diagnosis?
A. beriberi
B. metastatic disease
C. pellagra
D. subacute combined degeneration
E. Wernicke-Korsakoff syndrome

b 5. A 32-year-old high school teacher with no significant past medical history develops uncharacteristic anxiety and
agitation. Over several days, she develops cognitive changes with decreased memory and becomes less active;
she spends most of her time sitting in the living room chair. After she has a seizure witnessed by her husband,
she is taken to the hospital. There she is noted to have a central hypoventilation respiratory pattern and
intermittent orofacial and upper limb dyskinesia. At times, her dyskinesia resembles someone trying to play
the piano. What is the most common neoplasm associated with this clinical syndrome?
A. breast carcinoma
B. Hodgkin lymphoma
C. neuroblastoma
D. small cell lung cancer
E. teratoma

b 6. Which of the following is the most common neurologic complication of inflammatory bowel disease?
A. encephalopathy
B. myelopathy
C. myopathy
D. peripheral neuropathy
E. stroke

b 7. A 68-year-old woman undergoes evaluation after suffering a right occipital infarct 3 days ago. She has a history
of well-controlled hypertension and has been afebrile. Workup thus far has revealed a wedge-shaped right
occipital infarct and cutoff of the P1 segment of the right posterior cerebral artery on brain magnetic resonance
angiography (MRA). MRA of the neck, transthoracic echocardiogram, and 24-hour cardiac telemetry are normal.
Which of the following is the most appropriate next diagnostic step?
A. conventional cerebral angiogram
B. CT perfusion
C. EEG
D. long-term cardiac rhythm monitoring
E. no additional investigation is indicated

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b 8. A 32-year-old woman presents with 2 days of rapidly worsening headache and visual loss. The patient has a
known history of a nonfunctioning pituitary macroadenoma that has been stable on annual imaging for the past
4 years. On examination, she is afebrile and has bitemporal hemianopia as well as mild nuchal rigidity. Which
of the following is the most likely diagnosis?
A. bacterial meningitis
B. cavernous sinus thrombosis
C. mucormycosis
D. pituitary apoplexy
E. subarachnoid hemorrhage

b 9. Joint pain is a hallmark of which of these environmentally induced disorders?

A. acute radiation syndrome
B. decompression sickness type I
C. electrical injury
D. high-pressure neurologic syndrome
E. hypothermia

b 10. The face of the giant panda sign on T2-weighted images has been described as a brain MRI finding in which of
the following hepatic diseases?
A. Budd-Chiari syndrome
B. hemochromatosis
C. hepatitis A
D. non-Wilsonian hepatocerebral degeneration
E. Wilson disease

b 11. Treatment with which of the following antiepileptic medications would most likely require supplemental
dosing after dialysis?
A. carbamazepine
B. lamotrigine
C. levetiracetam
D. phenytoin
E. valproic acid

b 12. Which of the following features of aortic atheromas suggests a higher risk of embolization?
A. calcification
B. high lipid content
C. nonmobile
D. smaller size (less than 4 mm)
E. smooth contour

b 13. Central nervous system prophylaxis is used routinely in the treatment of which of the following types
of lymphoma?
A. Burkitt lymphoma
B. follicular lymphoma
C. Hodgkin lymphoma
D. mantle cell lymphoma
E. small lymphocytic lymphoma

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 Postreading Test

b 14. Which of the following is the most common central nervous system manifestation in patients with systemic
lupus erythematosus?
A. cerebrovascular disease
B. cognitive impairment
C. headache
D. mood disorders
E. seizures

b 15. Which of the following neurologic complications is associated with toxicity of vitamin A?
A. dementia
B. intracranial hypertension
C. myelopathy
D. peripheral neuropathy
E. seizures

b 16. A 56-year-old man presents to the emergency department after having a witnessed generalized tonic-clonic
seizure in the waiting room of the medicine clinic. He had been experiencing tingling in his fingers and around
his lips for the prior week as well as painful finger spasms. He underwent total thyroidectomy 1 month ago
for thyroid cancer. He has no prior neurologic history, and his only medication is levothyroxine. On examination,
he is alert and fully oriented with normal cognitive function. When percussing his parotid region on either side,
ipsilateral facial contractions are noted. Which of the following is the most likely diagnosis?
A. hypocalcemia
B. hypoglycemia
C. hypokalemia
D. hyponatremia
E. hypothyroidism

b 17. A 60-year-old man with moderate chronic renal failure is admitted to the hospital for treatment of a urinary
tract infection. On the third day, he becomes confused and appears sedated. The following day, he develops
myoclonus and is witnessed to have a generalized seizure. Which of the following medications is most likely
to cause this constellation of symptoms?
A. cefazolin
B. cefepime
C. ceftaroline
D. ceftriaxone
E. cephalexin

b 18. A 46-year-old man presents with cognitive deterioration over the past 2 months. He has a long-standing history
of alcoholism and has been treated over the past year for multiple dental infections in the setting of poor
oral hygiene. He also has been experiencing abdominal pain and diarrhea. On physical examination, he has
a reddish-brown rash on his face, chest, and the dorsum of his hands and feet. Neurologic examination reveals
the inability to perform serial 7s and poor recall. Deficiency of which of the following nutrients is most likely
responsible for his presentation?
A. cobalamin
B. folate
C. niacin
D. pyridoxine
E. thiamine

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b 19. A 24-year-old woman presents with headaches, nausea, and ataxia and is found to have a cerebellar
hemangioblastoma. Her family history is notable for a pheochromocytoma in her mother. Periodic screening for
which of the following conditions should be performed in this patient?
A. Fanconi syndrome
B. glomerular nephritis
C. lymphoma
D. renal cell carcinoma
E. vertebrobasilar aneurysm

b 20. Which of the following clinical features should prompt a clinician to consider an autoimmune cause for
seizures?
A. frontal lobe epileptic focus
B. normal CSF examination
C. olfactory aura
D. positive family history of epilepsy
E. resistance to antiepileptic drug treatment

b 21. Which of the following is the most likely mechanism of stroke occurring as a complication of inflammatory
bowel disease?
A. atherosclerosis
B. cardiac embolism
C. hypercoagulability
D. lipohyalinosis
E. reversible cerebral vasoconstriction syndrome

b 22. A 65-year-old man with a long history of alcoholism and chronic liver disease presents for evaluation of
fluctuating neurologic symptoms, including lethargy, disorientation, and apathy. His wife describes recent
episodes of inappropriate behavior, such as walking around the house naked when his adult daughter is
visiting. His examination shows lethargy, disorientation for time, asterixis, and mild dyspraxia. What grade of
hepatic encephalopathy under the West Haven criteria does he demonstrate?
A. grade I
B. grade II
C. grade III
D. grade IV
E. minimal

b 23. Which of the following is the most common neurologic manifestation of type A aortic dissection?
A. cerebral infarction
B. Horner syndrome
C. recurrent laryngeal nerve palsy
D. spinal cord ischemia
E. subarachnoid hemorrhage

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 Postreading Test

b 24. A 56-year-old man with chronic liver disease due to viral hepatitis develops painful peripheral neuropathy.
Electrodiagnostic studies are consistent with a predominantly axonal distal symmetric sensory neuropathy.
Laboratory evaluation is remarkable for the presence of mixed cryoglobulinemia. Which of the following etiologies
of viral hepatitis is most likely present in this patient?
A. hepatitis A
B. hepatitis B
C. hepatitis C
D. hepatitis D
E. hepatitis E

b 25. A 49-year-old woman develops a progressive encephalomyelitis syndrome over 3 weeks. She is hospitalized
because of the development of rigidity and myoclonus in her lower extremities that prevent safe ambulation.
This is associated with painful spasms in her legs and low back. Admission physical examination by her internist
discloses a firm nonmobile 3-cm diameter mass in her left breast. Which of the following antibodies are
most likely to be associated with this clinical presentation?
A. amphiphysin
B. antineural nuclear autoantibody type 2 (ANNA-2)
C. Ma
D. N-methyl-D-aspartate (NMDA) receptor
E. Purkinje cell antibody (PCA)-1 (anti-Yo)

b 26. A 20-year-old man presents with 24 hours of acute-onset polyuria, polydipsia, vomiting, generalized weakness,
and confusion. For the past 2 weeks, he has been aggressively training for a football tryout and has been
taking steroids and amphetamines that he obtained from a classmate. In the emergency department, his
finger-stick blood glucose reads as over 400 mg/dL, and his initial labs demonstrate hyperglycemia, metabolic
acidosis, and the presence of ketone bodies in his serum. Which of the following is the most appropriate initial
step in management of this patient?
A. emergent dialysis
B. fluid resuscitation
C. glucagon administration
D. insulin administration
E. IV methylprednisolone

b 27. A 54-year-old man presents with 3 weeks of numbness in his hands and feet as well as worsening balance.
He has a history of peptic ulcer disease and takes omeprazole. On examination, he has reduced pinprick in
a stocking-glove distribution, reduced vibratory and joint position sense at the toes, bilateral Babinski signs,
and a positive Romberg sign. Deficiency of which of the following nutrients is most likely responsible for
his presentation?
A. cobalamin
B. folate
C. niacin
D. pyridoxine
E. thiamine

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b 28. A 68-year-old man is evaluated for a sensorimotor predominantly demyelinating neuropathy. His examination
shows hepatosplenomegaly, hypothyroidism, and areas of dermal hyperpigmentation. His laboratory studies
are remarkable for thrombocytosis and a lambda light chain monoclonal gammopathy. Which of the following
disorders is the most likely cause of his peripheral neuropathy?
A. AL amyloidosis
B. chronic inflammatory demyelinating polyradiculopathy (CIDP)
C. neurolymphomatosis
D. polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS)
syndrome
E. Waldenstrom macroglobulinemia

b 29. A 50-year-old man with diabetes mellitus reports feeling nauseated while on an airplane. The flight has been
delayed by 2 hours, during which time the airplane was sitting on the tarmac awaiting clearance for takeoff.
He then becomes diaphoretic and tremulous and develops palpitations. He notifies the flight attendant, and a
physician who happens to be on the flight is summoned. Over the next 3 minutes, the patient becomes
unresponsive. Which of the following is the most likely diagnosis?
A. acute stroke
B. diabetic ketoacidosis
C. hypercalcemia
D. hyperglycemic hyperosmolar state
E. hypoglycemia

b 30. A 41-year-old man develops an acute confusional state with dense anterograde and patchy retrograde amnesia
1 month after hematopoietic stem cell transplantation for treatment of chronic lymphocytic leukemia associated with
moderately severe graft versus host disease. He is unable to remember his chronic lymphocytic leukemia diagnosis and
asks why he has a central venous access catheter in place. He is witnessed to have several complex partial seizures.
Laboratory evaluation is remarkable for moderate hyponatremia. A brain MRI is abnormal, with hyperintense signal in
the medial temporal lobes bilaterally on T2-weighted images that do not enhance on postcontrast T1-weighted images.
CSF shows a mild pleocytosis. What is the most likely etiology of this patients syndrome?
A. Cytomegalovirus
B. enterovirus
C. human herpesvirus 6
D. JC virus
E. varicella-zoster virus

b 31. Which of the following is the most common neurologic manifestation of Sjogren syndrome?
A. aseptic meningitis
B. encephalopathy
C. myelopathy
D. myopathy
E. peripheral neuropathy

b 32. Which of the following solid organ transplantations are associated with the highest risk for Toxoplasma gondii
infection?
A. heart
B. kidney
C. liver
D. lung
E. pancreas

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 Postreading Test

b 33. A 15-year-old girl presents with bilateral optic neuritis and features of myelitis localizable to the conus. Her
brain MRI is remarkable for multiple lesions with a cotton wool appearance with poorly demarcated margins.
Which of the following antibodies is most likely to be found in this patient?
A. antineuronal nuclear antibody type 1 (ANNA-1) (anti-Hu)
B. anti-Ma
C. antibodies to myelin oligodendrocyte glycoprotein (MOG)
D. Purkinje cell antibody (PCA)-tr
E. recoverin (anti-CAR)

b 34. Which of the following vitamins can cause peripheral neuropathic symptoms in both a state of deficiency
or excess?
A. vitamin B1
B. vitamin B6
C. vitamin B12
D. vitamin D
E. vitamin E

b 35. A 35-year-old man presents with 2 months of progressive cognitive deterioration, weight loss, and diarrhea.
On examination, he has impaired delayed recall, poor attention, impaired upgaze, and pendular convergence
nystagmus with synchronous contractions of the jaw muscles. Which of the following is the most likely diagnosis?
A. neuro-Behet disease
B. neurosarcoidosis
C. neurosyphilis
D. progressive supranuclear palsy
E. Whipple disease

b 36. A 61-year-old man develops dyspnea on exertion and pedal edema. Abnormalities noted on his examination
include periorbital purpura, hepatomegaly, and macroglossia. He also has multiple features of autonomic failure,
including orthostatic hypotension, erectile dysfunction, and gastroparesis. Which of the following diagnostic
procedures, in combination with bone marrow biopsy, would be the most useful to confirm the etiology of
this disease?
A. abdominal subcutaneous fat aspiration
B. liver biopsy
C. muscle biopsy
D. pericardial biopsy
E. salivary gland biopsy

b 37. Keraunoparalysis is transient paralysis caused by which of the following environmental-related conditions?
A. acute mountain sickness
B. decompression sickness
C. hyperthermia
D. hypothermia
E. lightning strike

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b 38. A 44-year-old man presents with acute left-sided weakness. He has been febrile for the past week. He has a
history of IV drug abuse. On examination, he is febrile and has a cardiac murmur and left lower face and
arm weakness. MRI of the brain demonstrates an acute right frontal infarct. Magnetic resonance angiography
(MRA) of the brain demonstrates two small distal middle cerebral artery aneurysms. Erythrocyte sedimentation
rate is 86 mm/hr. Transthoracic echocardiogram demonstrates vegetations on the mitral valve. Which of
the following is the most appropriate next therapeutic step?
A. antibiotics
B. anticoagulation
C. aspirin
D. endovascular occlusion of the distal aneurysms
E. mitral valve replacement

b 39. A 45-year-old man presents with diplopia and right eye pain for 3 weeks. On examination, he has proptosis and
ophthalmoparesis of the right eye. MRI of the brain demonstrates patchy pachymeningeal enhancement as well
as thickening and enhancement of right periorbital muscles. What is the most likely diagnosis?
A. antiphospholipid antibody syndrome
B. Behet disease
C. IgG4-related disease
D. mixed connective tissue disease
E. polyarteritis nodosa

b 40. A 77-year-old woman is brought to the emergency department with lethargy that has been worsening
over the past 3 days. She has reported feeling cold and unwell for the prior 2 weeks. On examination, she
is hypothermic to 34.8-C (94.6-F). She has nonpitting edema in her limbs and face, is severely lethargic and
barely arousable, and has generalized hyporeflexia. Which of the following endocrine disorders is most likely?
A. addisonian crisis
B. hyperglycemic hyperosmolar state
C. myxedema coma
D. pituitary apoplexy
E. thyroid storm

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