270. A 21-year-old, right-handed female student was working in the photography lab 1 week ago, which required standing all day. After that, she experienced a cold sensation in the left foot and her entire left leg fell asleep. The feeling lasted 4-to-5 days and then slowly went away. Her right lower extremity was fine. Coughing, sneezing, and the Valsalva maneuver did not worsen her symptoms. She had a slight back pain, which she thought was due to using a poor mattress. Past history includes an episode of optic neuritis in the left eye 2 years ago. At that time, she was reportedly depressed and was sleeping constantly. One day, her left eye became blurred and her vision went out. In 1 week, her vision returned to normal. Her vision now is 20/20. She has not had a repeat episode since then. She had an magnetic reasonance imaging (MRI) of her brain, which was normal at that time. She drinks alcohol occasionally and does not use any illicit drugs. Her only medication is birth control pills. Examination is significant for brisk reflexes and sustained clonus at the right ankle. Babinski sign is present on the right. Testing is positive for oligoclonal bands. Which of the following is the most likely diagnosis in this case?
a. Seizure
b. Transient ischemic attack
c. Anaplastic astrocytoma
d. Multiple sclerosis (MS)
e. Parkinson disease
271. A patient has brought some test results from an outside doctor with her today. One of the results indicates that oligoclonal bands were positive. What are oligoclonal bands?
a. Wave frequency changes on the electroencephalogram (EEG) during sleep
b. Markings about the iris
c. Pathologic features of Alzheimer disease
d. Chromosomal markings found with MS
e. Immunoglobulin patterns in the cerebrospinal fluid (CSF) with MS
272. A 39-year-old woman with multiple sclerosis reports symptoms consistent with bladder spasticity and has clonus of the lower extremities. On briskly flexing her neck forward, which of the following is she most likely to report?
a. Dystonic posturing of the legs
b. An electrical sensation radiating down the spine or into the legs
c. Bilateral wristdrop
d. Spontaneous evacuation of the bladder and bilateral extensor plantar responses
e. Rapidly evolving hemifacial pain
273. A 19-year-old man had an episode of left optic neuritis, which resolved over several weeks. Two years later there was a month-long episode of bladder dysfunction. The patient underwent many tests and was told that he had multiple sclerosis. The CSF in persons with multiple sclerosis will typically exhibit which of the following?
a. Glucose content of less than 20% of the serum content
b. Persistently elevated total protein content
c. Persistently elevated immunoglobulin G (IgG) content
d. Mononuclear cell counts of greater than 100 cells per μL
e. Erythrocyte counts of greater than 10 cells per μL
274. A 35-year-old man with multiple sclerosis initially presented 4 years ago with left eye optic neuritis. He did not receive steroids at that time. Two years ago he had loss of sensation in his hands that progressed over weeks to motor involvement, limiting his ability to write with the left hand. He received steroids at that time. Four years ago, he began interferon β-1A. One year ago, he developed right leg weakness, constipation, and urinary urgency. He received steroids at that time as well. He now presents with symptoms that concern him about the possible start of a new flare. Two days ago, he noticed decreased sensation in the palm of his right hand that is worse when he exercises. This has gotten a little worse over the past 2 days. Yesterday, he noticed diminished sensation along the lower right trunk in the front and back. He has no pain, tingling, exacerbation of symptoms with neck movement, neck injury, incontinence, gait disturbance, diplopia, fever, chills, nausea, or vomiting. Examination findings include full visual fields with a left afferent pupillary defect. Bulk, strength, and tone are normal. Light touch is decreased over the left trunk and back over roughly the T8-to-T12 dermatomes. Finger tapping, rapid alternating movements, finger-nose-finger, and heel tapping to shin are normal. Which of the following is the most appropriate pharmacological treatment for this patient at this time?
a. Interferon β-1B
b. Corticosteroids
c. Gabapentin
d. Glatiramer
e. Pramipexole
275. A 30-year-old man was recently diagnosed with multiple sclerosis. The patient and his wife have many questions. Among them, they would like know how common multiple sclerosis is. You might tell them that multiple sclerosis is the most common demyelinating disease in the United States, affecting approximately one person in how many?
a. 100
b. 500
c. 1000
d. 5000
e. 10,000
276. A patient with suspected MS undergoes multimodality evoked potentials, EEG, MRI, and CSF testing. Which of the following evoked response patterns is most often abnormal in patients with early MS?
a. Brainstem auditory evoked response (BAER)
b. Far-field somatosensory evoked response (SSER)
c. Visual evoked response (VER)
d. Jolly test
e. Sensory nerve conduction test
277. A 37-year-old woman with progressive multiple sclerosis is being admitted for intravenous glucocorticoid therapy. She was diagnosed with multiple sclerosis 10 years ago after presenting with bilateral decreased visual acuity. She had an abnormal MRI at that time. She has been hospitalized approximately nine times since presentation, with her flares commonly consisting of increasing bilateral lower extremity weakness and decreased sensation manifested as a heavy feeling, waxing and waning generalized fatigue, bilateral hand tingling, and occasional nondescript speech changes that make her sound as though she has a slight accent. She has also had bilateral optic neuritis and one transient episode of aphasia in the past. She was last hospitalized 3 years ago. For the past 2 years she has been on cyclophosphamide and methylprednisolone, originally every 4 weeks, and now every 6 weeks, with the last treatment 1 month ago. She has tried and failed interferon β therapy. For the 2 months prior to admission, the patient has had worsening bilateral lower extremity weakness/heaviness, increased fatigue, and mild low back numbness, as well as intermittent and alternating decreased hearing in both ears at work. She has also noticed mild unsteadiness when walking. Which of the following should be included among her admission orders?
a. Heart-healthy diet
b. Ranitidine 150 mg bid
c. Neurological checks every hour for the first 48 hours
d. Placement of central venous line
e. Stat head computed tomography (CT) for change in mental status
278. A 29-year-old man contracted HIV-1 through homosexual activity 5 years ago. He had been doing well on highly active antiretroviral therapy, but stopped taking his medications 8 months ago because he thought that he would be better off. Two months ago, he was successfully treated for Pneumocystis carinii pneumonia. A papovavirus infection of the central nervous system (CNS) in this person would be most likely to produce which of the following?
a. Adrenoleukodystrophy
b. Multiple sclerosis
c. Subacute sclerosing panencephalitis (SSPE)
d. Progressive multifocal leukoencephalopathy (PML)
e. Metachromatic leukodystrophy
279. A 3-month-old child has a rapid regression of psychomotor function and loss of sight. There is increased urinary excretion of N-acetyl-L-aspartic acid. A preliminary diagnosis of Canavan disease (Canavan-van Bogaert-Bertrand disease; spongy degeneration of infancy) is made. This is a demyelinating disease that produces retardation in infants, is inherited in an autosomal recessive pattern, and results in which of the following?
a. Anencephaly
b. Microcephaly
c. Porencephaly
d. Macrocephaly
e. Dolichocephaly
280. A 58-year-old man with a basilar tip aneurysm is referred by a neuro-surgeon. He has a 4-year history of progressive spastic paraparesis. He has recently had urge incontinence of urine. He also has numbness in the right toes more than the left and pain in the thighs and back. There have been some gradual fluctuations, but no clear, discrete episodes of deterioration. He has had no disturbances of vision, eye movement, or motor control of the upper extremities. He was referred when surgical clipping of the aneurysm 3 months ago failed to help his symptoms. Which of the following is the most appropriate next diagnostic test?
a. Cerebral angiography
b. Spinal angiography
c. MRI of the spinal cord
d. Spinal cord biopsy
e. Visual evoked potential
281. A 41-year-old man has had relapsing-remitting multiple sclerosis for nearly 20 years. Over that time his irreversible neurological deficits have gradually accumulated, and he now has decreased visual acuity, poor coordination, and a spastic paraparesis. Cystometrographic analysis of bladder function in this patient is likely to show which of the following abnormalities?
a. Bladder hypotonia
b. Large residual volume of urine
c. Premature bladder emptying
d. Good voluntary control of bladder emptying
e. Urinary tract infection
282. A patient with multiple sclerosis has worsening leg weakness. He has severe spasms of his legs bilaterally and is increasingly unable to ambulate because of this. A reasonable symptomatic treatment option would be which of the following?
a. Cyclophosphamide
b. Baclofen
c. Gabapentin
d. Amitriptyline hydrochloride
e. Propranolol
283. You are counseling a 22-year-old woman with the recent diagnosis of multiple sclerosis. She wants to know what, if any, lifestyle changes she may have to make. Which of the following factors might be expected to worsen multiple sclerosis symptoms?
a. Bright lights
b. Red wine
c. Tyramine-containing compounds
d. Hot weather
e. Amantadine
Questions 284 to 289
For each patient, select the most likely diagnosis. Each lettered option may be used once, more than once, or not at all.
a. Neuromyelitis optica (Devic disease)
b. Central pontine myelinolysis
c. Marchiafava-Bignami disease
d. Acute disseminated encephalomyelitis
e. Pelizaeus-Merzbacher disease
f. Leber optic atrophy
g. Alexander disease
h. Adrenoleukodystrophy
i. Canavan disease
284. A 23-year-old woman awakens with bilateral leg weakness and numbness, urinary retention, and impaired bowel control. She has had several episodes of blurred vision over the previous 2 years, but these had always been attributed to idiopathic papillitis.
285. Two weeks after recovering from a febrile illness associated with a productive cough, a 19-year-old man complains of headache and neck stiffness. These complaints are associated with fever and are soon followed by deteriorating cognitive function. He becomes disoriented, lethargic, and increasingly unresponsive. MRI reveals widespread damage to the white matter of the cerebral hemispheres.
286. A 24-year-old man has progressive loss of vision over the course of 5 years. A visual field examination reveals a centrocecal scotoma. Two of his cousins have similar problems with visual loss. Both of the affected relatives are male and in their twenties. Genetic testing reveals a mutation of mitochondrial DNA.
287. Two brothers, 4 and 7 years of age, exhibit limb ataxia, nystagmus, and mental retardation. MRI of their brains reveals areas of abnormal signal in the white matter. Cerebellar involvement is substantial. Both boys also have abnormally low serum cortisol levels.
288. A 3-month-old boy exhibits nystagmus and limb tremors unassociated with seizures. Over the next few years, he develops optic atrophy, choreoathetotic limb movements, seizures, and gait ataxia. He dies during status epilepticus and at autopsy is found to have widespread myelin breakdown with myelin preservation in islands about the blood vessels. The pathologist diagnoses a sudanophilic leukodystrophy to describe the pattern of staining observed on slides prepared to look for myelin breakdown products.
289. A 54-year-old alcoholic man is brought to the emergency room with profound agitation. He is believed to have delirium tremens and is treated with thiamine and intravenous fluids. His serum sodium is noted to be markedly depressed, and intravenous supplements are adjusted to rapidly correct this hyponatremia. He becomes acutely quadriplegic and unresponsive and dies within 24 hours.