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					Drugs that can cause parkinsonism

-metoclopramide (can be used to treat headaches)

-prochlorperazine, haloperidol, amiodarone, valproic acid

Essential Tremor – gabapentin, primidone, beta blockers, topamax

Restless Legs – Gabapentin, Levodopa, RPP

Huntington’s: worry about depression, Halo, fluphenazine, reserpine

Wilson’s: Confirm—Liver biopsy

       Screen—Urine copper, slit lamp, MRI (t2 signal in basal ganglia, thalamus, red

Common peripheral neuropathies: DM, Alcohol, GBS(CIDP) –albumincytologic
dissociation, Amyloid, Uremia

        Dysautonomia prevalent

Parenchymal mets – brain radiation

Leptomeningeal mets (likes periventricular/arachnoid granulations) - chemo

Neurology:Question 1
A 39-year-old executive comes to the office for a follow-up visit. She had a generalized tonic-
clonic seizure 1 week ago, witnessed by her husband, who reports that the seizure started
suddenly. She had tonic extension and stiffening of her whole body followed by rhythmic jerking
movements of her arms, trunk, and legs. The patient had no warning prior to the seizure, and
afterward fell into a deep sleep. She was hospitalized. Wake and sleep electroencephalography
and magnetic resonance imaging of the brain with contrast were normal, and she was sent home
on a maintenance dose of phenytoin. The patient states that, for the 4 nights prior to the seizure,
she slept no more than 2 hours a night in order to finish an assignment. There is no personal or
family history of seizures. Neurologic examination is normal.
Which of the following is the most appropriate next step in management?
A. Continue phenytoin
B. Discontinue phenytoin
C. Discontinue phenytoin and begin carbamazepine
D. Discontinue phenytoin and begin valproic acid
E. Discontinue phenytoin and begin gabapentin

Neurology:Question 2
A 73-year-old woman has transient left hemiparesis lasting 20 minutes and calls your office that
same day to inform you. She has a history of intermittent nonvalvular atrial fibrillation, angina
pectoris, and hypertension. At the time of her call, she is completely asymptomatic. Her only
antithrombotic therapy is aspirin, 81 mg daily.
What is the optimal initial management for this patient?
A. Schedule an office appointment within 1 week.
B. Schedule transesophageal echocardiography.
C. Schedule Holter monitoring.
D. Schedule outpatient carotid duplex ultrasonography.
E. Obtain emergency computed tomography.

Neurology:Question 3
A 35-year-old woman comes to the office with a 1-day history of blurred vision and retro-orbital
pain with movement of the right eye. Past medical history is unremarkable. On examination, she
has a right afferent pupillary defect. Visual acuity is 20/200 in the right eye with a dense central
scotoma, and 20/25 in the left eye. Funduscopic examination is normal bilaterally. The rest of the
neurologic examination is normal. Magnetic resonance imaging of the brain shows one
nonenhancing periventricular white matter lesion. The patient refuses lumbar puncture for
examination of cerebrospinal fluid. Treatment with intravenous methylprednisolone improves her
optic neuritis.
Which of the following should be done to monitor for development of multiple sclerosis?
A. Repeat examination only when patient has a new neurologic symptom
B. Serial visual evoked potential studies in 6 months
C. Formal visual field studies in 6 months
D. Follow-up magnetic resonance imaging of the brain with gadolinium in 6 months
E. Serial nerve conduction velocity examinations in 6 months

Neurology:Question 4
A 42-year-old man comes to the emergency department because he had a 10-minute generalized
tonic-clonic seizure that occurred during sleep 2 hours earlier. He reports a 1-month history of
constant, progressively worsening headache, mild right-hand clumsiness, and occasional word-
finding difficulty. There is no significant past medical history. Neurologic examination reveals
mild expressive language difficulties and a subtle right hemiparesis with right-sided
hyperreflexia. The remainder of the physical examination is normal. Chest radiograph is normal.
Computed tomographic scan is shown.
Which of the following is the most likely cause of this patients findings?
A. Brain metastasis
B. Glioblastoma multiforme
C. Toxoplasmosis
D. Lymphoma of the central nervous system
E. Bacterial abscess

Neurology:Question 5
A 36-year-old woman with a 15-year history of headaches comes to the office for a follow-up
visit. Initially, she had moderate to severe bilateral pulsatile, frontal headaches accompanied by
nausea, light and noise sensitivity, and occasional vomiting. They occurred one to two times each
month and lasted 24 to 36 hours. In the last 2 years, she has developed a daily mild to moderate
bilateral frontal headache that is present when she awakens and lasts all day. Approximately once
per week she has a headache that is similar to her previous episodic headaches but less severe.
She takes amitriptyline, 75 mg at bedtime, as a prophylactic agent. She takes
acetaminophen/caffeine/butalbital, 4 tablets/d, and rizatriptan, 10 to 20 mg, one to two days per
week for more severe headaches. Physical and neurologic examinations are normal.
What is the most appropriate next step in managing this patients headache disorder?
A. Discontinue amitriptyline and initiate treatment with divalproex sodium.
B. Discontinue acetaminophen/caffeine/butalbital.
C. Discontinue rizatriptan.
D. Increase dose of amitriptyline.

Neurology:Question 6
A 65-year-old man has weakness and wasting of his left hand and muscle twitching involving his
and thighs. The symptoms have progressed over 5 months. There is no associated pain or
paresthesia. Neurologic examination reveals severe weakness and atrophy of the left thenar and
hypothenar muscles, and mild weakness of left foot dorsiflexion. Muscle stretch reflexes are
hyperactive and symmetric. Sensation is normal. Magnetic resonance imaging of the cervical
spine was obtained by an orthopedic surgeon 2 weeks earlier and is normal.
Which of the following is the most appropriate next step?
A. Muscle biopsy
B. Nerve biopsy
C. Serum creatine kinase determination
D. Electromyography
E. Genetic testing

Neurology:Question 7
A 46-year-old woman comes to the office for management of a 4-mm left middle cerebral artery
aneurysm. The aneurysm was found when the patient had magnetic resonance angiography and
imaging for evaluation of a chronic headache disorder. Medical history is limited to chronic
migraine headaches and smoking. Neurologic examination is normal.
Which of the following is the most appropriate next step in this patient‟s management?
A. Arteriography
B. Computed tomographic angiography
C. Follow-up magnetic resonance angiography in 6 months
D. Aneurysm clipping

Neurology:Question 8
A 71-year-old man comes to the office because of slowness, tremor, stiffness, and difficulty with
dexterity, such as buttoning clothes. The problems developed insidiously over the last few years,
and have caused him to curtail his activities severely. On examination, he has a shuffling gait,
tremor of one hand while walking, and reduced arm swing (right more than left). Rapid
alternating movements are very slow, especially in the right limbs. Passive movement of his
limbs reveals rigidity. Neurologic examination is otherwise normal.
Which of the following is the most appropriate management for this patient?
A. No treatment
B. Selegiline
C. Amantadine
D. Carbidopa/levodopa
E. Entacapone

Neurology:Question 9
A 68-year-old man is under evaluation for memory difficulty that, according to his wife, began
insidiously 3 or 4 years earlier. He has difficulty remembering recent events. For example, he
forgets appointments and recent conversations, and forgot that a close relative had recently died.
He is no longer able to manage his own checkbook or operate his car without getting lost.
Medical history is unremarkable. Physical examination is normal. Mental status examination
shows prominent memory loss and difficulty drawing a complex figure. Magnetic resonance
imaging of the brain shows only mild cerebral atrophy.
Which of the following is the most likely diagnosis?
A. Alzheimer‟s disease
B. Dementia with Lewy bodies
C. Vascular dementia
D. Frontotemporal dementia
E. Creutzfeldt-Jakob disease

Neurology:Question 10
A 23-year-old woman comes to the office with her husband for evaluation of recent onset of
unusual behavior. While providing the clinical history, the patient begins to cry. Shortly after that
the patients arms start shaking. Gradually, the shaking becomes more pronounced and involves
her legs and head. The patients breathing becomes deep and fast. This continues on and off for
several minutes. The shaking stops gradually. The patient opens her eyes and states that she has a
headache and is very tired and dizzy. Family history is positive for seizures in the patients cousin.
There has been a significant amount of family stress recently.
Which of the following is the most likely diagnosis?
A. Syncope
B. Nonepileptic event (pseudoseizure)
C. Partial seizure with secondary generalization
D. Generalized tonic-clonic seizure
E. Basilar migraine

Neurology:Question 11
A 65-year-old woman comes to the emergency department because of mild left hem iparesis and
mild left-sided sensory deficit that began suddenly 2 days earlier. She has a history of myocardial
infarction and takes aspirin, 81 mg daily.
Which of the following is the most appropriate next step in the management of this patient?
A. Increase the aspirin dose to 325 mg daily.
B. Discontinue aspirin and begin clopidogrel, 75 mg daily.
C. Change aspirin to combined low-dose aspirin/extended-release dipyridamole.
D. Discontinue aspirin and begin warfarin (target international normalized ratio, 2.0 to 3.0).
E. Initiate inpatient diagnostic evaluation.

Neurology:Question 12
A 74-year-old man with a history of hypertension is brought to the emergency department after
being found unresponsive by his wife in bed. He is intubated for airway protection. Blood
pressure is 210/90 mm Hg; pulse, 80/min; and temperature, 37 C (98.6 F). When his eyes are
opened by the examiner, there are intermittent spontaneous downward eye movements. There are
no spontaneous horizontal eye movements, nor are there horizontal movements in response to the
doll‟s head maneuver. The pupils are very small but reactive. He does not move any extremities
on command. There is occasional spontaneous extensor posturing in his arms and legs, and
Babinski‟s signs are present bilaterally. He blinks his eyes three times in a row on command.
Which of the following is the most likely cause of this patient‟s condition?
A. Anoxic encephalopathy
B. Left middle cerebral artery stroke
C. Pontine infarct
D. Cerebellar infarct

Neurology:Question 13
A 42-year-old man with secondary progressive multiple sclerosis comes to the local urgent care
facility because of increased weakness and spasticity of the legs, with increased hip flexor tone.
Over the past 3 days he has also noted an increase in urinary urgency and frequency. Until 3 days
ago, he had been fairly stable, with a slowly progressive course, and could walk at least 500 feet
with one cane; spasticity had been well controlled with baclofen, 20 mg three times a day orally.
On examination, blood pressure is 110/70mm Hg; pulse, 92/min; and temperature, 38.2 C (100.8
F). He has bilateral internuclear ophthalmoplegia. Hip flexion is weak bilaterally. Spasticity is
severe in the legs, with sustained clonus at both ankles. Urinalysis shows 50 to 100
leukocytes/hpf, and 2+ leukocyte esterase.
In addition to treatment of the urinary tract infection, which of the following is the most
appropriate treatment?
A. Acetaminophen
B. Oral prednisone
C. Intravenous methyiprednisolone
D. Interferon beta
E. Glatiramer acetate

Neurology:Question 14
A 34-year-old woman comes to the office because of a 2-year history of bifrontal, throbbing
headaches that occur two to four times per month and last 8 to 16 hours. During a headache, she
tolerates noise and sound poorly and prefers to lie down. She sometimes has associated blurred
vision, eye tearing, and rhinorrhea, and she is occasionally unable to work during a headache. The
headache worsens with physical activity, such as brisk walking. Physical and neurologic
examinations, including funduscopic examination, are normal.
Which of the following is the most likely diagnosis?
A. Episodic tension-type headache
B. Cluster headache
C. Idiopathic intracranial hypertension
D. Migraine without aura
E. Sinus headache

Neurology:Question 15
A 55-year-old woman presents to her physician with a 4-week history of occipital headache with
morning vomiting and a 1-week history of difficulty with peripheral vision to the left. She reports
no other neurologic symptoms. The patient has a history of node-positive breast cancer.
Neurologic examination reveals papilledema, left homonymous hemianopia, mild left
hemiparesis, and subtle gait ataxia. Chest radiograph shows multiple nodules.
Which of the following is the most likely cause of this patient‟s findings?
A. Parenchymal brain metastases
B. Leptomeningeal metastases
C. Paraneoplastic encephalitis
D. Cerebral infarction

Neurology:Question 16
A 25-year-old woman comes to the office for a follow-up visit. She has a 10-year history of
complex partial seizures with occasional secondary generalization. The patient reports that she is
14 weeks pregnant and that she has had no seizures since her last visit more than 1 year ago.
Currently, she is on carbamazepine monotherapy, 200 mg three times daily. Her carbamazepine
blood level today is 8.2 μglmL (therapeutic range, 8 to 12 μglmL).
Which of the following is the most appropriate next step in management?
A. Continue carbamazepine at the current dose
B. Increase the carbamazepine dose
C. Add another antiepileptic agent
D. Change carbamazepine to lamotrigine
E. Discontinue carbamazepine

Neurology:Question 17
A 67-year-old man comes to the emergency department because of sudden onset of severe
vertigo, headache, nausea, vomiting, and inability to walk. He has a history of hypertension. On
examination, his blood pressure is 190/110 mm Hg; pulse, 100/min, respiratory rate, 18/mm, and
temperature, 36.7 C (98 F). He is in distress from severe headache and nausea. He has dysarthria,
prominent nystagmus on lateral gaze, and clumsiness of the left arm on finger-to-nose maneuver,
and he falls to the left when he tries to sit up.
Which of the following is the most likely cause of this patient‟s presentation?
A. Menieres disease
B. Vestibular neuronitis
C. Cerebellar hemorrhage
D. Benign positional vertigo

Neurology:Question 18
A 38-year-old man comes to the office because of paresthesias in his feet that began
approximately 8 months ago. He now also has bilateral foot drop and weakness of the right hand.
On examination, he has severe weakness in the foot dorsiflexors bilaterally and moderate
weakness of foot eversion, inversion, and plantar flexion. He has mild weakness of hip flexion
and is unable to rise from a chair without using his arms. There is also weakness in the ulnar-
innervated hand muscles, slightly more pronounced on the right than on the left. There are no
obtainable reflexes, and he has moderate proprioceptive loss in the toes.
Which of the following is the most likely diagnosis?
A. Guillain-Barré syndrome
B. Chronic inflammatory demyelinating polyneuropathy
C. Polymyositis
D. Myasthenia gravis

Neurology:Question 19
An 85-year-old man arrives in the emergency department within 30 minutes of sudden-onset left
hemiparesis. Blood pressure is 240/124 mm Hg on arrival and 2 hours after the onset of his
He has severe left hemiparesis, neglect (left hemi-inattention), and a left hemisensory deficit.
Electrocardiography, laboratory tests, and cerebral computed tomography are normal.
Which of the following is the most appropriate management for this patient?
A. Give intravenous labetalol to achieve blood pressure below 185/110 mm Hg and then give
intravenous tissue plasminogen activator (t-PA).
B. Give intravenous nitroprusside to achieve blood pressure below 185/110 mm Hg and then give
intravenous t-PA.
C. Do not give antihypertensive medication or intravenous t-PA.
D. Do not give antihypertensive medication but do give intravenous t-PA.
E. Give antihypertensive medication to achieve blood pressure below 185/110 mm Hg and then
give intra-arterial t-PA.

Neurology:Question 20
A 23-year-old woman comes to the office for re-evaluation of migraine headaches. She was seen
2 weeks earlier because of recurrent generalized, moderate to severe pulsatile headaches
associated with nausea and light and noise sensitivity. The headaches typically last 1 to 2 days,
and she was having approximately 10 headache days per month. Amitriptyline was begun at a
dose of 10 mg at bedtime, with instructions to increase the dose in 10-mg increments every week
toward an initial target dose of 50 mg. The current dose is 30 mg at bedtime. She tolerates the
amitriptyline well. Today she has developed a typical attack that is treated with subcutaneous
sumatriptan in the office.
What plan should be pursued for prophylactic treatment?
A. Continue amitriptyline at the current dose.
B. Continue increasing amitriptyline as planned.
C. Discontinue all prophylaxis.
D. Discontinue amitriptyline and begin propranolol.
E. Discontinue amitriptyline and begin divalproex sodium.

Neurology:Question 21
A 55-year-old man reports that his handwriting has been getting smaller, and his wife states that
he has been walking with a slow, shuffling gait for about 2 years. He otherwise feels well. He has
diabetes, diabetic gastroparesis, a 3-year history of celiac disease responsive to medications and a
gluten-free diet, and depression. Daily medications are glyburide, ranitidine, metoclopramide, and
bupropion. On examination, he has facial masking, a slightly shuffling gait, bilateral reduced arm
swing, mild diffuse limb rigidity, and reduced speed when performing rapid alternating
What is the most appropriate management of his current symptoms?
A. Discontinue metoclopramide.
B. Discontinue ranitidine.
C. Discontinue bupropion.
D. Begin carbidopa/levodopa.
E. Begin pramipexole.

Neurology:Question 22
A 60-year-old woman with a history of adenocarcinoma of the lung presents to her physician with
a 3-week history of horizontal diplopia, dysphagia, and right leg pain with foot drop. Examination
reveals weakness of right eye abduction, left facial weakness, reduced gag reflex, and weakness
of the right leg muscles supplied by the L5 nerve root. Computed tomography of the head with
contrast shows only enlargement of the entire ventricular system (communicating
Which of the following is the most likely cause of this patient‟s findings?
A. Parenchymal brain metastases
B. Venous sinus thrombosis
C. Paraneoplastic encephalitis
D. Leptomeningeal metastases
E. Herpes zoster infection of the central nervous system

Neurology:Question 23
A 21-year-old woman comes to the office with her mother because of recurrent blackouts. The
patient has had five episodes in her life, the last one a week ago. She describes feeling dizzy,
lightheaded, nauseated, and warm prior to blacking out. According to her mother, the patient
becomes pale, then falls suddenly to the ground and has several jerks of her limbs. Her mother
states that the patient recovers consciousness almost immediately, without any confusion or
disorientation. During one event, the patient was incontinent of urine, and did not recover for at
least 30 seconds. Most episodes have occurred in church after prolonged standing; the latest
occurred when the patient witnessed a medical procedure while visiting a relative in the hospital.
Which of the following is the most likely diagnosis?
A. Juvenile myoclonic epilepsy
B. Complex partial seizures with secondary generalization
C. Syncope
D. Nonepileptic events (pseudoseizures)
E. Atonic seizures

Neurology:Question 24
A 29-year-old woman with multiple sclerosis comes to the office because of overwhelming
fatigue. She has had three exacerbations of multiple sclerosis in the past 8 years and is now in
remission. Her fatigue is particularly severe in mid afternoon; she often naps if possible or finds it
difficult to concentrate on work. She has no problem falling asleep at night and does not wake up
before she wishes. She sleeps 10 hours a day. She has no symptoms to suggest depression.
Neurologic examination and thyroid function studies are normal.
Which of the following would be a reasonable medication to prescribe for her pathologic fatigue?
A. Thyroid supplement
B. Amphetamine
C. Amantadine
D. Fluoxetine
E. Pemoline

Neurology:Question 25
A 78-year-old man is evaluated in the office for progressive impairment of memory and other
aspects of intellect, which has developed insidiously over 3 years. The patient recalls none of
three words after a 3-minute delay, and has a Mini-Mental State Examination score of 22 of 30.
There are no other
significant findings on physical examination.
Which of the following is most likely to improve this patient‟s symptoms?
A. Galantamine
B. Gingko biloba
C. Vitamin E
D. Selegiline

Neurology:Question 26
A 67-year-old man who lives alone is found on the floor of his home by his son and is brought to
the emergency department within 30 minutes. When last seen by his son 3 days earlier, the patient
was in full health. In the emergency department, his blood pressure is 180/100 mm Hg. He is
mildly lethargic and has global aphasia, a dense right visual field deficit, right hemiplegia, and no
response to painful stimuli on the right side. Computed tomography of the head shows that much
of the left hemisphere is dark (hypodense) with mass effect (compression of sulci and the left
lateral ventricle).
Which of the following is the most appropriate next step in management of this patient?
A. Do not give acute therapy.
B. Give intravenous tissue plasminogen activator.
C. Give intra-arterial tissue plasminogen activator.
D. Give intravenous corticosteroids.
E. Give intravenous heparin.

Neurology:Question 27
A 60-year-old woman comes to the office for evaluation of fainting episodes. She feels
lightheaded when she stands, and she has fainted three times within the past 2 months. She has
imbalance and a shuffling gait that developed slowly over the past 2 years. She is sleepy during
the day, and her husband describes high-pitched snoring (screeching‟) while she sleeps. She has
urinary incontinence. Her medical history is otherwise unremarkable. She takes no medications.
Her older sister has a gait disorder. On examination, blood pressure is 120/80 mm Hg while
sitting and 80/50 mm Hg when standing; pulse is 80 beats/min in both positions. Eye movements
are normal. Finger-to-nose and heel-to-shin movements are uncoordinated, but there is no tremor.
Movements are slow and there is limb rigidity. Deep tendon reflexes are brisk, and Babinski‟s
sign is present.
Which of the following is the most likely diagnosis?
A. Parkinson‟s disease
B. Creutzfeldt-Jakob disease
C. Spinocerebellar degeneration
D. Progressive supranuclear palsy
E. Multiple system atrophy

Neurology:Question 28
A 46-year-old man comes to the office because of a 1-year history of a slowly progressive gait
disorder. He describes his legs as heavy, and he tires quickly when walking. He has spasms in his
legs especially when they are tired or when he tries to sleep at night, On examination, he has mild
weakness in both hip flexors and ankle dorsiflexors, diffuse hyperreflexia, and bilateral extensor
plantar responses. He has severe loss of proprioception and vibration sense in the legs. Pinprick
sensation is normal throughout. Magnetic resonance imaging of the cervical and thoracic spine is
normal; cerebrospinal fluid is normal, and there are no oligoclonal bands.
Which of the following is the most likely diagnosis?
A. Diabetic neuropathy
B. Polymyositis
C. Vitamin B 12 deficiency
D. Chronic inflammatory demyelinating polyradiculopathy
E. Lambert-Eaton myasthenic syndrome

Neurology:Question 29
A 29-year-old computer programmer is brought to the emergency department by friends after he
suddenly falls to the ground at a party. He has severe headache, photophobia, and vomiting. He
has no significant medical history. On examination, blood pressure is 210/110 mm Hg; pulse rate,
110/min; respiratory rate, 22/min; and temperature, 37.2 C (99 F). He is in moderate distress from
headache. He has moderate meningismus but no focal neurologic findings. Routine laboratory
tests are normal. Urine drug screen is positive for cocaine. Computed tomography of the brain
shows a moderate-sized left thalamic hemorrhage with some intraventricular extension of blood.
The patient is stabilized and his blood pressure is controlled with intravenous nicardipine. He is
admitted to the intensive care unit for monitoring.
What additional diagnostic testing is most appropriate?
A. None
B. Electroencephalography
C. Echocardiography
D. Cerebral angiography

Neurology:Question 30
An obese 66-year-old man has had increasing pain and numbness of the right anterior thigh over
the past 3 weeks. He has not seen a physician in more than 20 years. On examination, he has
moderate weakness of right knee extension, thigh adduction, and hip flexion. The right knee jerk
reflex is absent; the left knee jerk is 1+. Achilles reflexes are absent bilaterally. He has sensory
loss in the right anterior thigh region and a stocking-glove distribution of sensory loss in the distal
legs. Magnetic resonance imaging of the lumbosacral spine and lumbosacral plexus is normal.
Which of the following studies is most likely to be diagnostic?
A. Creatine kinase level
B. Hemoglobin A 10 level
C. Thyroid function
D. Lumbar puncture
E. Erythrocyte sedimentation rate

Neurology:Question 31
A 68-year-old woman comes to the office because of a 2-year history of falls and imbalance that
has progressed insidiously. She also has trouble reading and doing needlework because of poor
vision. Family history is negative for neurologic disease. She has a staring facial expression.
Visual acuity and visual fields are normal, but she has impaired upward/downward gaze. She
moves stiffly and slowly, and nearly falls over while walking. Her neck and, to a lesser degree,
limbs are rigid. Rapid alternating movements are markedly slowed.
Which of the following is the most likely diagnosis?
A. Parkinson‟s disease
B. Generalized dystonia
C. Multiple system atrophy
D. Progressive supranuclear palsy
E. Cerebellar degeneration

Neurology:Question 32
A 57-year-old man with a clerical job is brought to the office by his wife for evaluation of
changes in memory, personality, and behavior. Over the past 3 years, he has become
progressively insensitive to his wife‟s concerns, to the point that she has considered separation.
He makes loud, inappropriate comments in public, and recently received a poor review from his
work supervisor. Past medical history is unremarkable; he takes no medications. His mother, a
maternal uncle, and the maternal grandfather had dementia. Physical examination is normal. The
Mini-Mental State Examination score is 28 of 30.
Which of the following is the most likely diagnosis?
A. Mild cognitive impairment
B. Alzheimer‟s disease
C. Dementia with Lewy bodies
D. Frontotemporal dementia
E. Creutzfeldt-Jakob disease

Neurology:Question 33
A 70-year-old man presents to his physician with a 2-month history of progressive cognitive
abnormalities and right-sided weakness. He has difficulty dressing and bathing himself and is
frequently disoriented with respect to time and place. For the last week he is dragging his right
foot when walking. On examination, he is alert but inattentive. He is disoriented with respect to
time and place. Papilledema is present. There is a mild right hemiparesis affecting the right face,
arm, and leg. Computed tomography of the head with contrast shows multiple, homogeneously
enhancing lesions in the periventricular white matter of both cerebral hemispheres (left more than
right) with surrounding edema and mass effect. A biopsy of one of the lesions is performed.
Postoperatively, dexamethasone, 4 mg three times daily, is administered; after 2 days, the patient
is alert and fully oriented, and his right-sided strength is normal. Computed tomography of the
head is repeated and shows marked improvement in all lesions with reduced enhancement and
mass effect.
Which of the following is the most likely cause of this patient‟s findings?
A. Metastatic carcinoma
B. Multiple sclerosis
C. Glioblastoma multiforme
D. Central nervous system vasculitis
E. Primary central nervous system lymphoma

Neurology:Question 34
A 40-year-old woman with secondary progressive multiple sclerosis continues to have a gradual
decline in neurologic status despite treatment with interferon beta. Two years ago she could walk
200 meters without a cane or rest. She has recently worsened to the point that she can walk no
further than 25 meters with a walker. On examination, she has bilateral lower-extremity weakness
and hyperreflexia. Magnetic resonance imaging of the brain shows multiple white matter lesions
in a periventricular distribution.
Which of the following treatments would be most appropriate instead of interferon beta?
A. Chronic oral prednisone
B. Bone marrow transplantation
C. Glatiramer acetate
D. Plasmapheresis
E. Mitoxantrone

Neurology:Question 35
A 24-year-old woman comes to the office because of severe generalized, throbbing headaches
associated with nausea, occasional vomiting, and light and noise sensitivity. The headaches occur
two to three times weekly and last 12 to 24 hours. She misses approximately 1 workday each
month. She has been taking zolmitriptan, 5 mg orally, for acute attacks, with good benefit
approximately two thirds of the time. She has insomnia and a past history of childhood asthma.
Which of the following medications is most appropriate to prevent the headaches?
A. Amitriptyline
B. Nadolol
C. Naratriptan
D. Propranolol
E. Sertraline

Neurology:Question 36
A 38-year-old man comes for a follow-up visit. Four years earlier, he had three complex partial
seizures and began treatment with phenytoin. He has had no seizures since beginning treatment
and tolerates the medication well. He has no previous personal or family history of seizures or
other neurologic illness. Neurologic examination is normal. The patients electroencephalograms
(EEGs) consistently show right temporal spikes and sharp waves; his last EEG was 3 months ago.
Magnetic resonance imaging (MRI) of the brain is normal. He asks whether he still needs to take
Which of the following would you recommend for this patient?
A. Taper phenytoin
B. Continue phenytoin at current dose
C. Change phenytoin to carbamazepine
D. Obtain single photon emission computed tomography (SPECT)
E. Repeat EEG and MRI of brain

Neurology:Question 37
A 50-year-old woman presents to her physician with a 4-month history of progressive right-sided
headache that sometimes awakens her from sleep. She has noted a non-tender swelling over the
right side of her head for the past year. This is mostly apparent to her when she combs her hair.
Neurologic examination is normal except for hyperreflexia of her left upper arm. There is
asymmetry of the head, with a fullness of the right side of the skull. Computed tomography of the
head reveals a dural-based, calcified, enhancing mass measuring 6 cm along the right cerebral
convexity. There is hyperostosis of the skull contiguous with the lesion.
Which of the following is most likely responsible for the clinical and radiographic findings?
A. Low-grade glioma
B. Glioblastoma multiforme
C. Meningioma
D. Subdural hematoma
E. Dural metastasis

Neurology:Question 38
An 87-year-old previously healthy woman awakens at 5:00 a.m., makes coffee, and then falls to
the kitchen floor unable to speak or move her right side, as witnessed by her daughter. In the
emergency department, her blood pressure is 174/70 mm Hg. She has a dense global aphasia,
right visual field deficit, right hemiplegia, and right hemisensory loss. Electrocardiography,
routine laboratory tests, and computed tomography of the head are normal. At 6:15 a.m., the
patients daughter asks about whether the patient should receive intravenous tissue plasminogen
activator (t-PA).
Which of the following is the most appropriate response?
A. Intravenous t-PA is contraindicated because of the patients age and size of stroke.
B. Intravenous t-PA offers the patient the best chance of recovery.
C. Intra-arterial t-PA is safer than intravenous t-PA.
D. Intravenous t-PA and oral aspirin given simultaneously offer the best chance for recovery.

Neurology:Question 39
A 33-year-old man is brought to the emergency department by ambulance after having three
generalized tonic-clonic seizures over 30 minutes without recovering consciousness between
seizures. His friend states that the patient has a history of epilepsy but is not compliant with his
medications. During examination, the patient has another generalized seizure. He becomes
cyanotic despite oxygen supplementation. His pulse is 110/min and blood pressure is 150/90 mm
Hg. He continues to have seizures despite receiving 8 mg of intravenous lorazepam en route to
the hospital. The patient weighs approximately 80 kg (176 Ib).
Which of the following intravenous medications should be administered next?
A. Fosphenytoin 1600 mg phenytoin-equivalent
B. Phenobarbital 1600 mg
C. Phenytoin 1000 mg
D. Valproic acid 1600 mg

Neurology:Question 40
A 75-year-old man is brought to the office because he is confused on a regular basis, particularly
at night, and sometimes moment to moment during the day. He woke up thinking he was tied to
the bed. He hallucinates that people are in the house, and sometimes believes his wife has been
replaced by a look-alike. On some days he needs help with basic activities. Medical history is
significant for a confusional episode due to prescription of meclizine for acute peripheral
vestibulopathy 2 years earlier. Meclizine was discontinued, and at a follow-up office visit 1
month later the confusion was much improved. He currently takes no medications. There is no
family history of dementia or parkinsonism. Masked facies, mild bradykinesia, and rigidity are
evident. His score on the Mini-Mental State Examination is 20 of 30. The remainder of the
examination is normal.
Which of the following is the most likely diagnosis?
A. Mild cognitive impairment
B. Alzheimer‟s disease
C. Frontotemporal dementia
D. Dementia with Lewy bodies

Neurology:Question 41
A 35-year-old man comes to the office for evaluation of almost constant, involuntary movement
of his hands and, to a lesser extent, his feet. The movements have developed insidiously over the
past 3 years. He denies feeling nervous, and perceives that he has little control over the
movements. He takes no medications and has otherwise been in excellent health. His wife
mentions recent personality change.
Abnormal involuntary movements are seen throughout the examination. There is no pattern to the
movements. His hands move continuously in rapidly flowing gyrations; he tends to sit on them
when talking to prevent them from moving. He walks with dancing movements that sometimes
cause him to misstep. He has an abnormal cognitive score on the office mental status
Which of the following is the most likely cause of the movements?
A. Huntington‟s chorea
B. Parkinson‟s disease
C. Creutzfeldt-Jakob disease
D. Tourette‟s syndrome

Neurology:Question 42
A 45-year-old woman with multiple sclerosis comes to the office as a new patient. Her disease
began 15 years ago with an attack of optic neuritis that resolved after 4 months. She was
asymptomatic until 6 years ago, when she had weakness of the right leg that responded to
treatment with intravenous corticosteroids. Over the next 5 years she had a series of exacerbations
that were less responsive to treatment with intravenous corticosteroids and left her with residual
neurologic deficits. In the past year, she has had gradual bilateral deterioration in leg strength,
such that she now requires a walker.
Which of the following best describes this patient‟s course of multiple sclerosis?
A. Monosymptomatic demyelination
B. Relapsing-remitting
C. Primary progressive
D. Secondary progressive
E. Benign

Neurology:Question 43
A 75-year-old man comes to the emergency department because he has an ischemic stroke
manifested by mild expressive aphasia and a mild right hemiparesis especially involving his face
and arm. He has a history of hypertension, hypercholesterolemia, and cigarette smoking.
Noninvasive cardiac evaluation reveals no cardioembolic source. Carotid duplex ultrasonography
shows 80% to 99% stenosis of the left internal carotid artery (LICA) just above the carotid
bifurcation. Catheter angiography reveals 70% stenosis of the extracranial LICA and no other
extracranial or intracranial stenosis.
Which of the following is the most appropriate management for this patient?
A. Intravenous heparin
B. Aspirin
C. Aspirin plus dipyridamole
D. Left carotid endarterectomy
E. Clopidogrel

Neurology:Question 44
A 75-year-old woman presents to the emergency department after a fall at home. She struck her
head and was briefly unconscious. She is now alert and fully oriented. Neurologic examination is
normal. Computed tomography of the head is negative for hemorrhage, but shows a 1-cm dural-
based, calcifed, enhancing lesion over her left cerebral convexity.
Which of the following is the most appropriate management of this patients lesion?
A. Gross total resection
B. Biopsy
C. Gamma-knife radiosurgery
D. Observation

Neurology:Question 45
A 72-year-old woman is admitted to the hospital after a cardiac arrest at home. Minutes after the
event, paramedics discern no heartbeat, but she regains sinus rhythm after a 30-minute
resuscitative effort. An acute myocardial infarction is diagnosed on admission. Five weeks after
the cardiac arrest, she remains unresponsive to voice and does not follow any commands. Her
pupillary responses are intact, she has full lateral eye movements to the doll‟s head maneuver,
and she triggers the ventilator with spontaneous respirations. Her eyes are open some of the time,
and she appears to have sleep-wake cycles. There is no evidence of purposeful or voluntary
response to visual, auditory, tactile, or noxious stimuli, but she flexes her arms to noxious stimuli.
Which of the following is the most appropriate description of this patients neurologic condition?
A. Brain death
B. Locked-in syndrome
C. Minimally conscious state
D. Persistent vegetative state

Neurology:Question 46
A 29-year-old woman comes to the office because of gradual loss of vision in her left eye over 5
days. The problem started as a “smudge” in her central visual field and gradually worsened such
that she cannot read with her left eye. She has pain with left eye movements, and she is finding it
difficult to work because of the impairment.
Neurologic examination is unremarkable except for eye findings. Visual acuity is 20/20 in the
right eye and 20/400 in the left eye. Visual field testing shows a dense central scotoma on the left.
Funduscopic examination is normal. There is a left afferent pupillary defect. Magnetic resonance
imaging of the brain is normal.
What is the best course of treatment?
A. Intravenous methylprednisolone
B. Interferon beta
C. Glatiramer acetate
D. Aspirin
E. Oral prednisone

Neurology:Question 47
A 26-year-old woman comes to the office with her husband, who provides the clinical history.
According to him, for the last couple of months the patient has been having episodes of sudden
“freezing and looking around, during which she is unresponsive if people talk to her. The
episodes last between 5 and 15 seconds, and occur 3 to 6 times a week. The patient does not
recall these events and accuses her husband of making them up. Physical examination is normal.
Which one of the following medications should be started while diagnostic work-up is in
A. Lamotrigine
B. Phenobarbital
C. Gabapentin
D. Carbamazepine
E. Ethosuximide

Neurology:Question 48
A 55-year-old woman has noticed a tremor of her hands for the past 2 to 3 years. Initially, it was
apparent when she held a cup of coffee or wrote, but it now also interferes with her use of eating
utensils and computer keyboarding. She requests treatment. Medical and family history is
negative and she takes no medications. Examination is remarkable only for a moderately severe,
bilateral postural and action tremor of both hands. Routine blood studies, including thyroid-
stimulating hormone, are normal.
Which of the following would be appropriate therapy?
A. Atenolol
B. Carbidopa/levodopa
C. Primidone
D. Valproic acid
E. Pramipexole

Neurology:Question 49
A 73-year-old woman is brought to the emergency department because of sudden onset of
moderate right hemiparesis and hemisensory deficit involving her face, arm, and leg equally. The
patient has no relevant medical history and takes no medications. She has no other signs or
symptoms. She is admitted for inpatient etiologic evaluation. Her average blood pressure during
hospitalization is 138/82 mm Hg. Carotid duplex ultrasonography shows nonstenotic plaque
bilaterally. Transthoracic echocardiography shows diastolic dysfunction but no potential
cardioembolic source.
Which of the following should be recommended to the patient for blood pressure management?
A. Begin angiotensin-converting enzyme inhibitor in 2 weeks.
B. Begin oral β-blocker immediately.
C. Begin oral calcium channel blocker immediately.
D. Recommend diet and lifestyle changes only.

Neurology:Question 50
A 37-year-old woman comes to the office because of recurrent headaches for 5 years. Initially,
the headaches were of moderate intensity and occurred approximately four times per year. In the
past year, she has had right-sided severe, pulsatile pain associated with light and noise sensitivity
and nausea without vomiting; the headaches last 12 to 16 hours and occur once or twice monthly.
The headaches cause her to miss work and other activities occasionally. She uses acetaminophen
without benefit. Physical and neurologic examinations are normal.
Which of the following is most likely to be effective for acute treatment of this patients
A. Acetaminophen/ASA/caffeine combination
B. Acetaminophen/butalbital/caffeine combination
C. Aspirin
D. Naproxen
E. Zolmitriptan

Neurology:Question 51
A 30-year-old right-handed man with known HIV infection presents to the emergency department
with a 3-day history of progressive right hemiparesis and aphasia. Magnetic resonance imaging of
the brain reveals a homogeneously enhancing left frontal lesion. There is central necrosis and
mass effect in the white matter of the left cerebral hemisphere. A biopsy is performed and reveals
diffuse B-cell lymphoma.
Genomic information from which of the following viruses is most likely to be found in this
patients tumor cells?
A. Cytomegalovirus
B. Epstein-Barr virus
C. Herpes simplex virus
D. Herpes zoster virus

Neurology:Question 52
A 44-year-old woman with a history of hypertension presents to the emergency department after
sudden onset of the “worst headache” of her life. On examination, blood pressure is 190/95 mm
Hg; pulse, 112/min ; respiratory rate, 24/mm; and temperature, 36.8 C (98.2 F). She is mildly
drowsy but arouses easily and is cooperative. She has meningismus but no focal neurologic
deficits. Computed tomography of the brain shows a moderate amount of subarachnoid blood.
After basic medical care, which of the following is the most important acute treatment at this
A. Oral nimodipine
B. Intravenous labetalol
C. Intravenous dexamethasone
D. Intravenous enalapril

Neurology:Question 53
A previously healthy 38-year-old man has numbness in the right anterolateral leg that began 3
weeks ago and is associated with a deep aching pain in the thigh. Ten days ago, he started to drag
his right foot. Yesterday, he became aware of numbness in the fourth and fifth digits of the left
hand. He has lost 10 lb in the past month, and has noted frequent night sweats. On examination,
there is evidence of a right peroneal mononeuropathy and left ulnar mononeuropathy. Serum
chemistries, including fasting blood glucose level, are normal.
Which of the following is the most likely diagnosis?
A. Amyloid polyneuropathy
B. Vasculitic neuropathy
C. Guillain-Barré syndrome
D. Diabetic neuropathy

Neurology:Question 54
An 18-year-old college student comes to the emergency department because of a seizure that
occurred at 8:30 this morning. According to her roommate, the patient woke up at her usual time
and had several episodes of brief arm jerks before she fell and lost consciousness. Her whole
body stiffened for 5 to 10 seconds, and then she had rhythmic clonic jerking of her arms, legs,
and trunk for about 2 to 3 minutes. She remained unresponsive for 10 minutes and then gradually
regained complete consciousness over the next hour. The patient remembers the arm jerks but has
no recollection of the seizure. She states that she has been having arm jerks in the morning for
several months and that this has become worse recently. She has been staying up late every night
for the last week studying for final examinations. Neurologic examination is normal.
Which of the following is the most likely diagnosis?
A. Partial seizure with secondary generalization
B. Absence epilepsy
C. Syncope
D. Juvenile myoclonic epilepsy
E. Nonepileptic event (pseudoseizure)

Neurology:Question 55
A 26-year-old woman with clinically definite multiple sclerosis comes to the office because she is
unexpectedly pregnant and wants to discuss how to proceed with disease management. She is
maintained on interferon beta 1a. She has had one acute exacerbation in the past 2 years.
Neurologic examination shows right afferent pupillary defect with mild weakness of the right leg
and mild right-sided hyperreflexia. She asks how her multiple sclerosis and treatment will affect
the pregnancy.
Which of the following is the most appropriate counsel to share with her?
A. Continue interferon
B. Discontinue interferon beta
C. Change to glatiramer acetate
D. Recommend therapeutic abortion

Neurology:Question 56
A 42-year-old woman comes to the office because of a 3-year history of daily headaches that
involve the left retro-orbital, temporal, and parietal areas. The headaches begin when she awakens
and persist all day. The pain fluctuates from mild to moderate; at its most intense, she has left eye
lacrimation and left-sided nasal congestion and rhinorrhea. She occasionally has nausea. The
patient takes propranolol 160 mg/d without benefit. Physical and neurologic examinations are
What is the most likely diagnosis?
A. Chronic tension-type headache
B. Chronic migraine
C. Cluster headache
D. Hemicrania continua

Neurology:Question 57
A 68-year-old woman comes for a routine physical examination. She describes a 2-year history of
insomnia, which she attributes to an inability to get comfortable in bed because of a “creepy-
crawly” sensation in her lower limbs. The sensation is absent during most of the day, but it has
begun to bother her in the evening when she sits and is especially troublesome in bed. Walking
relieves the sensation. Physical examination, including neurologic examination, is normal.
Which of the following studies should be done next?
A. Serum ceruloplasmin level
B. Serum ferritin level
C. Urine porphyrin level
D. Nerve conduction study
E. Magnetic resonance imaging of the brain

Neurology:Question 58
A 60-year-old woman is brought to the emergency department for evaluation of word-finding
difficulty. She called her husband‟s attention to the problem 3 weeks ago. Two weeks ago, the
difficulty became apparent to others, and she began to have difficulty finding her way around the
offices at work. One week ago she had difficulty navigating in her own home, confusing the
garage, closet, and basement doors, and had difficulty with arithmetic calculations and vision.
Past medical and family history is unremarkable. On examination, she has occasional myoclonic
jerks of the upper extremities and facial musculature, worse on the right side. Her score is 15 of
30 on the Mini-Mental State Examination. Electroencephalography shows mild diffuse slowing.
Magnetic resonance imaging of the brain is normal.
What is the next step in testing?
A. Lumbar puncture
B. Presenilin-1 test
C. Positron emission tomography/single photon emission computed tomography
D. Repeat electroencephalography in 1 week
E. Brain and leptomeningeal biopsy

Neurology:Question 59
A 50-year-old previously healthy woman presents to her physician with a 3-week history of
progressive gait and limb ataxia, dysarthria, and blurred vision. Examination reveals nystagmus,
dysarthria, and severe gait and limb ataxia. The remainder of the neurologic examination is
normal. Magnetic resonance imaging of the head with contrast is normal. A blood test reveals
positive serology for anti-Yo antibodies (anti-Purkinje cell antibody).
Which of the following malignancies is most likely present in this patient?
A. Non-Hodgkin‟s lymphoma
B. Small cell lung cancer
C. Ovarian cancer
D. Melanoma

Neurology:Question 60
A 34-year-old man comes to the office because of a 1-month history of episodes during which he
is suddenly unable to speak. The episodes last between 20 and 30 seconds. During some episodes,
the patient has twitching of the right side of his face; on one occasion the twitching progressed to
involve his right arm. He has had five events, the last three during the past week. No warning
precedes the spells. The patient states that he is fully aware of his surroundings during the
episodes and has no other symptoms. Neurologic examination is normal.
Which of the following is the most likely diagnosis?
A. Transient ischemic attack
B. Anxiety attack
C. Frontal lobe seizure
D. Hemiplegic migraine
E. Hypoglycemia

Neurology:Question 61
A 24-year-old woman comes to the emergency department because of a 3-day history of severe
headache. She states that while she was at work she had sudden onset of excruciating, diffuse
headache associated with nausea and vomiting. She was unable to continue working, and a
colleague drove her home. Over the next 2 days the headache remained too severe for her to go to
work. She has no significant past medical history except tobacco use. On examination, blood
pressure is 144/90 mm Hg; pulse, 104/min; respiratory rate, 18/mm; and temperature, 37.2 C
(98.9 F). She is mildly agitated and has photophobia and minimal neck stiffness. There are no
focal neurologic deficits. Computed tomography of the brain is normal.
What is the most appropriate next step in the management of this patient?
A. Emergent magnetic resonance imaging
B. Lumbar puncture
C. Cerebral arteriography
D. Treatment with sumatriptan
E. Treatment with ketorolac

Neurology:Question 62
A 35-year-old woman comes to the office because of episodic, severe headaches with left retro-
orbital and temporal pain and light and noise sensitivity; they are typically accompanied by
severe nausea and vomiting shortly after onset. The headaches most commonly awaken her from
sleep in the early morning and last 16 to 24 hours. They have occurred approximately one or two
times per month for the past 4 years. There are no headache triggers. She has used over-the-
counter medications and oral naratriptan without benefit. Physical and neurologic examinations
are normal.
Which of the following is the most appropriate acute treatment for this patients headaches?
A. Oral almotriptan
B. Oral rizatriptan
C. Intravenous dihydroergotamine
D. Subcutaneous sumatriptan

Neurology:Question 63
A 75-year-old man comes for an office visit because he is concerned about memory loss. He
reports a family history of Alzheimers disease, with dementia affecting three first-degree relatives
in their 50s. His difficulties are not apparent to his spouse or close friends, and have not affected
his ability to perform his daily activities. He is an active and successful financial officer. Past
medical history is remarkable for treated hypertension and osteoarthritis. There is no evidence of
depression. Physical examination is normal. Mental status examination discloses mild to
moderate memory impairment and no abnormalities in other areas of cognition. Levels of thyroid-
stimulating hormone and vitamin B12 are normal, as is magnetic resonance imaging of the brain.
Which of the following is the most appropriate next step?
A. Presenilin-1 test
B. Follow-up evaluation in 1 year
C. Positron emission tomography/single photon emission computed tomography
D. Lumbar puncture
E. Diffusion-weighted magnetic resonance imaging

Neurology:Question 64
A 28-year-old woman comes to the office because of diplopia on lateral gaze in either direction
that has worsened over several days. She has no history of neurologic problems. Neurologic
examination is normal except for paresis of the adducting eye with nystagmus of the abducting
eye on horizontal gaze in either direction. Magnetic resonance image of the brain is shown.

Which of the following long-term treatments should be considered?
A. Methotrexate
B. Cyclophosphamide
C. Interferon beta
D. Aspirin
E. Clopidogrel

Neurology:Question 65
A 35-year-old woman comes to the office because she cannot prevent her neck from turning to
the right. The condition developed insidiously over 2 years and is very painful. She is the
secretary for a small law firm and is under pressure at work. She recalls no trauma and is
otherwise in good health. On examination, she sits with her head turned 45 degrees to the right;
walking exacerbates the condition. She is unable to rotate her head to the left. Cervical paraspinal
muscles are tense. The remainder of the examination is normal.
Which of the following is most likely to be helpful?
A. Diazepam
B. Botulinum toxin injections
C. Carbidopa/levodopa
D. Cyclobenzaprine
E. Psychiatry referral

Neurology:Question 66
A 26-year-old woman comes to the office because of recurrent episodes of unusual behavior. She
usually has a warning—a “weird” feeling in her stomach —prior to the episodes. Her husband has
witnessed most of the events, and states that the patient suddenly freezes, looks around, smacks
her lips, and moves her fingers repetitively. The episodes usually last between 30 seconds and 2
minutes. She has no recollection of the events after the warning. Afterward, the patient is
confused and disoriented for as long as 10 minutes. The patient has had at least 10 episodes over
the last 2 years.
Which of the following is the most likely diagnosis?
A. Complex partial seizures
B. Absence seizures
C. Nonepileptic events (pseudoseizures)
D. Migraine
E. Transient global amnesia

Neurology:Question 67
A65-year-oldwoman presentsto herphysicianwith a 1-month historyof proximal muscleweakness,
with difficulty lifting her arms above her head and rising from a low chair. She also notes dry
mouth and eyes and postural light-headedness. She has recently completed chemotherapy for
small cell lung cancer. Examination reveals symmetric proximal muscle weakness that improves
with repeated effort. Deep tendon reflexes are absent. The remainder of the neurologic
examination is normal. Blood pressure in the right arm is 125/80 mm Hg lying down and 100/50
mm Hg standing. Pulse rate remains 70/min in either position.
Which of the following is the most likely diagnosis?
A. Metastatic spinal cord compression
B. Myasthenic syndrome
C. Polymyositis
D. Polyneuropathy

Neurology:Question 68
A 52-year-old woman comes to the emergency department because of a severe generalized,
pulsatile headache accompanied by light and noise sensitivity, nausea, and repeated vomiting.
The headache began 16 hours earlier. She has a 25-year history of recurrent migraine headaches.
She takes amitriptyline for migraine prophylaxis and has used oral rizatriptan today. Pulse rate is
120/min and regular; brachial blood pressure is 160/94 mm Hg. Physical and neurologic
examinations are otherwise normal.
In addition to intravenous fluid and electrolyte replacement, what is the most appropriate next
step in management?
A. Intravenous dihydroergotamine
B. Intravenous dihydroergotamine and metoclopramide
C. Intravenous chlorpromazine
D. Oral codeine
E. Subcutaneous sumatriptan

Neurology:Question 69
A 73-year-old man has sudden onset of right hem iparesis and expressive aphasia that improves
nearly to baseline by the time he arrives in the emergency department. He is admitted to the
hospital for rapid etiologic evaluation. Laboratory studies are normal except for a low-density
lipoprotein level of 110 mg/dL. Carotid duplex ultrasonography shows left carotid stenosis of
Which of the following is the most appropriate management for this patient‟s serum cholesterol?
A. Begin pravastatin.
B. Begin niacin.
C. Recommend diet and lifestyle changes only.
D. Recommend no further intervention.

Neurology:Question 70
A 25-year-old man with a 15-year history of seizures comes to the office for routine follow-up.
He has never been seizure free for more than a few weeks. A feeling of déjà vu precedes the
seizures. He then has lip smacking and repetitive movements of his left fingers that continue for
less than a minute and are followed by a generalized tonic-clonic seizure. He has had many
injuries due to seizures. The patient has tried several combinations of antiepileptic drugs with
varying success. Currently, he takes a combination of valproic acid and lamotrigine. Serum levels
are in the therapeutic range. The patient has had several electroencephalograms showing focal
right temporal lobe epileptiform activity. Magnetic resonance imaging of the brain performed 6
months ago is consistent with right mesial temporal sclerosis.
Which of the following is the most appropriate next management step?
A. Repeat magnetic resonance imaging of the brain
B. Refer for consideration of temporal lobe surgery
C. Order positron emission tomography
D. Treat with vagal nerve stimulation
E. Add a third antiepileptic drug

Neurology:Question 71
A 79-year-old woman comes to the emergency department because of sudden onset of headache
and right-sided weakness. She has no significant medical history and has had no medical follow-
up for many years. On admission, her blood pressure is 220/110mm Hg; pulse rate, 80/min;
respiratory rate, 22/min; and temperature, 37.3 C (99.2 F). She has mild dysarthria and a right
hemiparesis. Electrocardiography shows normal sinus rhythm, no ST segment changes, and left
ventricular hypertrophy by voltage criteria. Computed tomographic scan of the head is shown.
What is the most likely cause of the finding on computed tomography?
A. Chronic hypertension
B. Ruptured arteriovenous malformation
C. Ruptured aneurysm
D. Amyloid angiopathy

Neurology:Question 72
A 27-year-old man comes to the office for advice about treatment of his recently diagnosed
multiple sclerosis. Three years ago he had an episode of diplopia that resolved entirely after 2
months. Six months ago, he had mild weakness and numbness of the right leg. Magnetic
resonance imaging of the brain at that time showed multiple cerebral white matter lesions in a
periventricular distribution classic for multiple sclerosis. His leg weakness resolved without
treatment. He is now asymptomatic and has a normal neurologic examination.
Which of the following is the best treatment recommendation at this point?
A. High-dose intravenous methylprednisolone
B. Chronic oral prednisone
C. Azathioprine
D. Interferon beta
E. Observation

Neurology:Question 73
A 25-year-old woman comes to the office because of a 4-month history of generalized,
nonpulsatile headaches of moderate severity. The headaches are associated with intermittent
nausea and episodes during which the vision in her right eye fades for several seconds. She takes
naproxen sodium 3 days per week. Physical examination is normal exceptfora body mass index of
32.1. Neurologic examination shows bilateral swelling of the optic discs. Magnetic resonance
imaging of the brain and magnetic resonance venography are normal. Examination of CSF shows
an opening pressure of 35 cm H2O, total protein of 35 mg/dL, glucose of 64 mg/dL, and 2
leukocytes/μL; the fluid is negative for VDRL. What is the most likely diagnosis?
A. Hydrocephalus
B. Chronic migraine
C. Borrelia burgdorferi meningitis
D. Idiopathic intracranial hypertension
E. Medication overuse headache

Neurology:Question 74
A 72-year-old woman is brought to the office by her daughter for evaluation of mental status
changes. The patient had a stroke 1 month earlier, with mild residual left-sided weakness. She had
one seizure while in the hospital; the seizure was considered grand mal, and she was treated with
phenytoin. According to the daughter, the patient‟s left face and arm twitch frequently, and her
left side is weaker than when she left the hospital. For the last few days the patient has been
confused and disoriented, “like in a daze.” Her current medications include phenytoin and aspirin.
On examination, vital signs are normal. The patient is somewhat lethargic and has frequent
twitching of her left face and arm. There is left facial weakness and left hemiparesis involving the
face, arm, and leg. Complete blood count, serum electrolytes, and urinalysis are normal.
Which of the following is the most likely diagnosis?
A. Stroke in evolution
B. Partial status epilepticus
C. Phenytoin toxicity
D. Creutzfeldt-Jakob disease

Neurology:Question 75
A 78-year-old man is brought to the office by his family because of memory decline and
confusion. The family reports progressive confusion over 6 months. The family noticed his
symptoms at first mainly in the morning, but they now seem to occur throughout the day. He does
not recognize his spouse at times, and thinks someone is chasing him. He wanders from the
house, has visual hallucinations, and has difficulty using household appliances. Medical history is
significant for diabetes with painful peripheral neuropathy, coronary artery disease, and
congestive heart failure. Medications include glyburide, nortriptyline, digoxin, lorazepam,
diltiazem, and lisinopril. There is no family history of neurologic disease. On examination, the
patient has mild asterixis and findings consistent with peripheral neuropathy. He is mildly
lethargic and inattentive, and not oriented to time or place. He recalls two of three words after a
delay. The Mini-Mental State Examination score is 13 of 30. Electrolyte levels, oxygen
saturation, liver function, and renal function are normal. Computed tomography of the head
without contrast is normal.
Which of the following conditions is most likely to account for his cognitive impairment?
A. Adverse effect of medication
B. Dementia with Lewy bodies
C. Cerebrovascular disease
D. Depression

Neurology:Question 1
The correct answer is B
Educational Objectives
Manage a patient with a single seizure.
Most neurologists would not start antiepileptic drug therapy after a single seizure unless the
patient has one or more risk factors associated with seizure recurrence: abnormal neurologic
examination, abnormal electroencephalography or magnetic resonance imaging of the brain,
partial onset, a history of neurologic injury (such as stroke, hemorrhage, or tumor), family history
of seizures, or postictal motor paralysis. This patient has no risk factor for recurrence of seizures.
Her seizure was provoked by sleep deprivation. Seizures caused by external events (drug toxicity,
drug withdrawal, sleep deprivation, hypoglycemia, hypoxia, hyponatremia) are considered
provoked and do not require chronic antiepileptic drug treatment. Patients with a single provoked
seizure should have electroencephalography and magnetic resonance imaging to rule out
underlying focal pathology that could lower the seizure threshold and precipitate a seizure. Not
ordering any tests and just treating the patient with an antiepileptic drug can lead to missing an
early diagnosis of brain tumor, small stroke, hemorrhage, or other focal cortical abnormality.

Neurology:Question 2
The correct answer is E
Educational Objectives
Initiate urgent workup for a patient with intermittent atrial fibrillation and transient ischemic
Patients with transient ischemic attacks (TIA5) should be evaluated and treated urgently. Among patients
who come to the emergency department with TIA, 5% will have a stroke within 2 days, and 25% will have
a recurrent event within 3 months. Patients with atrial fibrillation and TIA or stroke should receive life-long
oral anticoagulation to prevent cerebral ischemia from cardioembolism. Emergency computed tomography
should be done in this patient to rule out tumor or hemorrhage as the cause of hemiparesis. Once bleeding
is ruled out, anticoagulation should be initiated urgently. Warfarin benefits patients with chronic or
intermittent atrial fibrillation regardless of left atrium size, so findings on Holter monitoring and
transesophageal echocardiography would not affect initial management. It is appropriate to obtain carotid
duplex ultrasonography, since 5% to 10% of patients with atrial fibrillation have concurrent carotid
stenosis, but this is less immediately urgent.

Neurology:Question 3
The correct answer is D
Educational Objectives
Monitor disease activity in a patient with a monosymptomatic demyelinating event.
According to the new McDonald criteria for diagnosis of multiple sclerosis, new disease activity
demonstrable by new T2 white matter lesions, or new gadolinium-enhancing lesions on serial brain or
spinal cord magnetic resonance imaging (MRI) at least 3 months after an initial scan qualify as
dissemination of demyelination across time. Serial MRI is the best way to monitor for ongoing disease
activity. Much of the activity of multiple sclerosis is subclinical and shows up only on MRI without
changes in neurologic examination or new symptoms. Monitoring of visual system function would not help
because new disease activity must be disseminated across space (in multiple areas of the central nervous
system) and the optic nerves have already been affected. Nerve conduction velocities test for abnormalities
in the peripheral nervous system, not the central nervous system, which is affected by multiple sclerosis.

Neurology:Question 4
The correct answer is B
Educational Objectives
Recognize the clinical presentation of high-grade glioma.
This patient had a generalized seizure in the setting of progressive focal neurologic deficit and
symptoms of raised intracranial pressure. Headache, seizure, and hemiparesis are the most
common presenting signs and symptoms of brain tumor. Computed tomography scan shows a
solitary, space-occupying lesion with evidence of necrosis and peripheral enhancement. A
solitary enhancing lesion in an otherwise healthy patient with a normal chest radiograph is most
likely to be a high-grade glioma. Although brain metastases are more common than primary brain
tumors, they are usually multiple and are associated with an abnormal chest radiograph (revealing
a primary lung tumor or lung metastases) in most patients. Most brain metastases occur in
patients with a known history of systemic malignancy. Moreover, brain metastases typically have
more surrounding edema than gliomas. Bacterial and parasitic infection and primary central
nervous system lymphoma are rare causes of mass lesions, and are unlikely in immunocompetent
patients or in patients with no evidence of systemic infection.

Neurology:Question 5
The correct answer is B
Educational Objectives
Recognize medication overuse as a cause of chronic daily headache.
This patient has developed a form of daily headache known as chronic migraine (transformed
migraine). In many patients the change in headache pattern is caused by overuse of analgesics
(more than 2 to 3 days per week). Combination analgesics are particularly likely to lead to
chronic daily headache. Accordingly, the first intervention is to discontinue use of the
combination analgesic. Discontinuing the offending agent usually results in improvement within
1 month, but occasionally takes longer. Patients should be advised that headaches may transiently
worsen when the medication is discontinued. Amitriptyline may prove to be an effective
preventive agent once the medication overuse headache has been eliminated. Increased
amitriptyline or substitution with divalproex sodium is not likely to improve the daily headaches
as long as the overuse of analgesics persists. Overuse headache from triptan agents is unlikely to
occur when the medication is taken at a frequency of 2 days per week, as in this patient.

Neurology:Question 6
The correct answer is D
Educational Objectives
Recognize the clinical presentation of amyotrophic lateral sclerosis.
This patient has progressive, painless weakness and atrophy in the absence of sensory symptoms.
He has upper and lower motor neuron signs in the symptomatic left arm, and essentially
asymptomatic weakness in the left leg. The presentation suggests motor neuron disease
(amyotrophic sclerosis). EMG would most likely confirm the diagnosis with findings of
denervation in multiple extremities. As only 5% to 10% of cases of amyotrophic lateral sclerosis
are familial, genetic testing at this stage is not indicated. The distribution of weakness is not
proximal, as would occur with most myopathies, so muscle biopsy would not be helpful.
Although creatine kinase levels may be elevated in rapidly progressive motor neuron disease, it is
a nonspecific finding. The absence of sensory symptoms or signs argues against a peripheral
neuropathy, so nerve biopsy, while likely showing axonal loss, would not be of high yield.

Neurology:Question 7
The correct answer is C
Educational Objectives
Manage incidental unruptured aneurysm.
This patient has a 4-mm asymptomatic intracranial aneurysm. The natural history of
asymptomatic aneurysms less than 10 mm in diameter is usually benign, with a very low risk of
rupture. In general, these patients should probably not undergo surgical therapy unless the
aneurysm enlarges or changes shape. Periodic noninvasive testing is appropriate. Computed
tomographic angiography has little to add to the magnetic resonance angiography that has already
been performed in this patient. Catheter angiography is associated with additional risk and does
not improve assessment of asymptomatic aneurysm.

Neurology:Question 8
The correct answer is D
Educational Objectives
Initiate treatment of symptomatic Parkinson‟s disease.
This man has signs and symptoms of parkinsonism, and very likely Parkinson‟s disease, that are
causing him to restrict his lifestyle. Treatment is indicated to keep him engaged in activities
appropriate for his age. Carbidopa/levodopa is the appropriate first choice of medication.
Dopamine agonist medications, such as pramipexole, ropinirole, pergolide, or bromocriptine, are
not as effective as carbidopa/levodopa and may be insufficient to counter his increasing
parkinsonian symptoms. Selegiline and amantadine are unlikely to have a major impact upon his
symptoms. Entacapone is ineffective in the absence of levodopa treatment and hence has no role
in initial treatment.

Neurology:Question 9
The correct answer is A
Educational Objectives
Recognize Alzheimer‟s disease.
This patients progressive cognitive impairment affects both multiple aspects of intellect and his
ability to perform daily tasks. The findings are consistent with a progressive dementia. Prominent
recent memory loss suggests early involvement of the temporal lobes. The lack of other
neurologic signs suggests sparing of the basal ganglia and primary motor and sensory areas. The
findings are most compatible with dementia due to Alzheimer‟s disease. Frontotemporal dementia
may cause a similar profile, but usually has more prominent early personality change and less
visuospatial impairment. Imaging often shows focal frontotemporal brain atrophy. Dementia with
Lewy bodies is accompanied by parkinsonism, hallucinations, and fluctuating symptoms, none of
which is present here. Creutzfeldt-Jakob disease has a much more rapid course and is usually
associated with motor signs such as rigidity, ataxia, or myoclonus. Vascular dementia is unlikely
in the absence of vascular risk factors, focal abnormalities on imaging, or a stroke-like (abrupt or
stepwise) course of illness.

Neurology:Question 10
The correct answer is B
Educational Objectives
Recognize the clinical presentation of nonepileptic events (pseudoseizures).
This is a typical presentation of a nonepileptic event (pseudoseizure). Pseudoseizures usually start in an
emotionally charged setting, with a gradual buildup of symptoms. Symptoms and duration are not
stereotypical. The movements are not synchronous and are usually prolonged, starting and stopping many
times throughout the event. Women are more likely than men to have nonepileptic events; associated
factors include health care occupation, major life stressors, medical history of unexplained physical
symptoms, psychiatric comorbidity, and traumatic childhood, including history of abuse. Many patients
with nonepileptic episodes have family members or friends with seizures or have witnessed a seizure.
Syncope often causes tonic posturing and myoclonic jerks, but the episodes are brief and there is fast and
full recovery of function (no postictal confusion). True generalized tonic-clonic seizures are characterized
by tonic extension of the whole body, followed by synchronous, rhythmic clonic movements of the limbs.
Partial seizures with secondary generalization have a focal onset. Basilar migraine is characterized by
recurrent stereotypical episodes of transient brainstem symptoms such as vertigo or diplopia, and is usually
associated with headache.

Neurology:Question 11
The correct answer is E
Educational Objectives
Initiate etiologic evaluation to determine appropriate therapy for secondary stroke prevention.
The choice of antithrombotic agent for secondary stroke prevention depends on the cause of stroke;
initiating therapy without taking into account the cause of stroke is not appropriate. Patients with atrial
fibrillation benefit most from chronic anticoagulation with warfarin (target INR [international normalized
ratio] between 2.0 and 3.0). In patients with large- or small-artery disease, antiplatelet therapy decreases the
risk of stroke and death, with no additional benefit from warfarin.

Neurology:Question 12
The correct answer is C
Educational Objectives
Recognize the „locked-in‟ syndrome that can occur due to acute pontine lesions.
This patient is not comatose, but is in a “locked-in” state. Patients in a locked-in state are quadriplegic,
have paralysis of horizontal eye movements, and are unable to speak because of paralysis of bulbar
muscles; communication is possible only through the patient‟s ability to move the eyes vertically and blink.
The locked-in syndrome occurs with lesions of the base of the pons, usually infarction (such as from basilar
artery occlusion) or hemorrhage. The finding of brisk downward eye movements is consistent with ocular
bobbing, a kind of spontaneous eye movement that can be seen in large pontine lesions. Although not
entirely specific, another clue to a brainstem localization of this patient‟s findings is the presence of
bilateral extensor (decerebrate) posturing. The bilaterally small pupils are also commonly associated with
pontine lesions. A left middle cerebral artery stroke would cause aphasia and a right hemiparesis, not the
bilateral signs or the eye findings seen in this patient. An anoxic encephalopathy would cause diffuse
cerebral, and sometimes brainstem, dysfunction but would not explain this patient‟s eye movements or the
intact alertness in the presence of quadriplegia. The typical findings of a cerebellar infarct are headache,
nausea, vomiting, and ataxia; brainstem (including pontine) dysfunction can occur as a secondary
phenomenon due to mass effect from infarct-related edema, which is why neurosurgical consultation is
important for large cerebellar infarcts as well as for cerebellar hemorrhages.

Neurology:Question 13
The correct answer is A
Educational Objectives
Recognize effect of fever on multiple sclerosis.
Neurologic function often deteriorates when patients with multiple sclerosis are exposed to ambient
heat or fever, because raised temperature decreases conduction through demyelinated nerves. This is
considered a pseudoexacerbation and is not necessarily evidence of a new exacerbation. Sudden
worsening of lower-extremity strength and increased spasticity in any patient with multiple sclerosis,
especially if associated with fever, should prompt investigation for urinary tract infection, decubitus
ulcer, cellulitis, arthritis, or other irritative conditions. Any painful or irritating stimulus can worsen
spasticity. Often the course improves over several days with hydration, appropriate antibiotic therapy,
and aggressive reduction of fever. The other treatment options will not directly address this patient‟s
deterioration due to fever.

Neurology:Question 14
The correct answer is D
Educational Objectives
Recognize the clinical features of migraine headaches.
This patients symptoms fulfill the criteria for migraine without aura, despite the bilateral pain and
absence of nausea. Rhinorrhea and tearing are autonomic symptoms that frequently accompany
migraine and do not usually represent sinus disease. Most patients with a diagnosis of sinus headache
in fact have migraine. Acute sinusitis is accompanied by fever and purulent nasal discharge. Episodic
tension-type headaches are not pulsatile, do not worsen with physical activity, and are not associated
with photophobia and phonophobia. Cluster headaches are unilateral (most commonly retro-orbital),
last 1 to 2 hours, and occur in „clusters” of recurrent attacks lasting weeks to months. Most patients
are agitated and prefer not to lie down during a cluster headache. This patient has no signs or
symptoms, such as visual obscurations, that suggest a diagnosis of idiopathic intracranial
hypertension, and there is no papilledema on funduscopic examination.

Neurology:Question 15
The correct answer is A
Educational Objectives
Recognize brain metastases.
This patients history of node-positive breast cancer places her at high risk for development of
recurrent and metastatic tumor. She presents with progressive signs and symptoms of focal neurologic
deficit and raised intracranial pressure. She most likely has multiple brain metastases. Magnetic
resonance imaging of the head with contrast would be the most appropriate diagnostic test for this
patient. Parenchymal brain metastases are by far the most common neurologic complication of
systemic cancer, occurring in 20% to 40% of patients. Leptomeningeal and subdural metastases are
                  with leptomeningeal metastases typically
less common. Patients
present with headache, multiple cranial nerve palsies, and
multiple spinal radiculopathies. Patients with subdural metastases and dural sinus
thrombosis primarily present with headache, hemiparesis, and sometimes drowsiness. Hemianopia
would be distinctly unusual with a lesion not directly involving brain parenchyma. Paraneoplastic
encephalitis is rare. It presents with seizures and rapidly progressive cognitive and personality
changes; it is not usually associated with headache or signs of raised intracranial pressure. A cerebral
infarct presents as an acute-onset neurologic deficit without symptoms or signs of raised intracranial

Neurology:Question 16
The correct answer is A
Educational Objectives
Manage antiepileptic drug therapy in a pregnant patient.
During pregnancy, seizure frequency increases in approximately one third of women with
epilepsy. This is a result of increased hepatic metabolism of most antiepileptic drugs and an
increased volume of distribution. Therefore, drug levels should be monitored during pregnancy to
make sure they remain therapeutic. This patient appears to have good seizure control on the
current dose of carbamazepine. There is no need to change the dose or add or switch to another
antiepileptic drug at the present time. If the patient starts to have breakthrough seizures, the dose
of carbamazepine should be increased instead of adding or changing to another antiepileptic drug.
Changing antiepileptic drug therapy during pregnancy can be harmful because during the
transition from one drug to another there is a risk of toxicity or breakthrough seizures. Ideally, the
patient would have discussed plans to become pregnant in advance, so any necessary changes to
her antiepileptic drug regimen could be made before the patient conceives. Since often
pregnancies are not planned, every woman of childbearing age should take folate supplements to
reduce the risk of neural tube defects from anticonvulsant therapy.
The goal is no seizures on the minimal effective dose of a single antiepileptic drug. Combination
of antiepileptic drugs should be avoided if possible since there is an increased risk of congenital
malformations and side effects. For many women, withdrawal of antiepileptic drugs before
pregnancy results in recurrence or exacerbation of seizures, which can be dangerous for both
mother and fetus.

Neurology:Question 17
The correct answer is C
Educational Objectives
Recognize cerebellar hemorrhage.
This patient has classic signs and symptoms of an acute left cerebellar insult, including headache,
nausea, vomiting, vertigo, and left-sided ataxia. The etiology is either cerebellar hemorrhage or
cerebellar infarction. He requires emergent computed tomography to check for cerebellar
hemorrhage; if hemorrhage is present, he would need urgent neurosurgical consult. Cerebellar
hemorrhages larger than 3 cm or with mass effect on the brainstem usually require
decompression, which is often lifesaving, and patients can recover with minimal neurologic
deficits. The other options are peripheral vestibular disorders that are unlikely to cause headache
or the cerebellar findings in this patient.

Neurology:Question 18
The correct answer is B
Educational Objectives
Diagnose chronic inflammatory demyelinating polyneuropathy.
The diagnostic features of chronic inflammatory demyelinating polyneuropathy include roughly
symmetric weakness and sensory loss progressing over at least 2 months, with evidence of
segmental demyelination on nerve conduction studies. Although results of electrodiagnostic
testing are not given in this case, chronic inflammatory demyelinating polyneuropathy is the most
likely diagnosis of the options given. Polymyositis and myasthenia gravis would not produce
sensory signs or symptoms. The progression of symptoms over 8 months is too long for Guillain-
Barré syndrome, which evolves over 4 to 6 weeks.

Neurology:Question 19
The correct answer is C
Educational Objectives
Treat patient with acute ischemic stroke and persistently elevated blood pressure.
Management of blood pressure is especially complex in patients with acute ischemic stroke who
are otherwise eligible for intravenous tissue plasminogen activator (t-PA). In stroke patients who
receive intravenous t-PA, elevated blood pressure carries an increased risk for intracranial
hemorrhage, hence persistent systolic blood pressure above 185 mm Hg or persistent diastolic
blood pressure above 110 mm Hg is a contraindication to intravenous t-PA. However, acute
hypertension at the time of stroke is most often a result of—not a cause of—stroke, and is
necessary to supply the reversibly ischemic brain (ischemic penumbra) with enough blood to
survive. Since the goal of intravenous t-PA therapy is to save the penumbra, it is
counterproductive to lower blood pressure excessively in order to administer t-PA. Thus, the need
for aggressive therapy to achieve blood pressure below 185/110 mm Hg is also a contraindication
to intravenous t-PA. In this patient, the systolic value would need to be lowered by 55 mm Hg in
order to administer intravenous t-PA within the next hour. This is considered aggressive therapy.
Intra-arterial t-PA is not currently approved by the U.S. Food and Drug Administration for
ischemic stroke; at this time, it is used in specialized centers for patients who present within 3 to
6 hours of onset of symptoms. Recommendations regarding blood pressure management are the
same for intra-arterial and intravenous t-PA.

Neurology:Question 20
The correct answer is B
Educational Objectives
Manage prophylaxis for migraine headaches.
The patient is tolerating the amitriptyline well; since the dose is currently low, it is appropriate to
continue to increase it gradually as planned. Maximal benefit of a prophylactic agent may not be
recognized for 2 to 3 months after attaining the target dose. Accordingly, it would be an error to
continue with the current dose or to discontinue amitriptyline. Propranolol and divalproex sodium
are both useful prophylactic agents, but should not be substituted for amitriptyline at this time.
They might be considered if amitriptyline is ultimately not effective or tolerated.

Neurology:Question 21
The correct answer is A
Educational Objectives
Recognize that antiemetic drugs with dopamine antagonist properties may induce parkinsonism.
This patient has neurologic symptoms consistent with parkinsonism. Metoclopramide, a
commonly prescribed antiemetic that blocks dopamine receptors, may cause parkinsonism and
could be responsible for his symptoms.
Gluten sensitivity has been associated with peripheral neuropathies and cerebellar ataxia, but is
not a likely cause of parkinsonism. Neither ranitidine nor bupropion has been associated with
development of parkinsonism. Selective serotonin reuptake inhibitors (SSRI5) have been reported
to cause parkinsonism, but this is extremely rare; they are not contraindicated and usually are well
tolerated by patients with Parkinson‟s disease. Symptomatic treatment with carbidopa/levodopa
or pramipexole may be appropriate later, if discontinuation of metoclopramide fails to resolve

Neurology:Question 22
The correct answer is D
Educational Objectives
Recognize leptomeningeal metastases.
This patient has known lung cancer and presents with subacute multifocal neurologic deficits
affecting multiple levels of the nervous system (ventricular system, cranial nerves, lumbar root).
This is the classic presentation of leptomeningeal tumor, which occurs in 3% to 8% of all patients
with cancer. Communicating hydrocephalus is common and due to obstruction of the arachnoid
granulations. Multifocal cranial neuropathies and radiculopathies are frequent and due to nerve
infiltration by tumor cells within the cerebrospinal fluid.
Multiple parenchymal metastases could produce a similar clinical picture but generally would be
manifest on computed tomography. Paraneoplastic neuropathy and herpes zoster infection of the
central nervous system are extremely rare complications of cancer; paraneoplastic neuropathy
would not cause cranial nerve deficits. Venous sinus thrombosis would not produce multifocal
cranial neuropathy and lumbar radiculopathy.

Neurology:Question 23
The correct answer is C
Educational Objectives
Distinguish seizures from syncope.
This patient‟s symptoms are most compatible with vasovagal syncope. There is usually a brief
prodrome that may include lightheadedness, warmth, diaphoresis, nausea, pallor, and blurred
vision. Abnormal movements (mainly myoclonic jerks or tonic posturing) and urinary
incontinence may occur during unconsciousness. Once recumbent, the patient usually recovers
consciousness rapidly but may be confused for up to 30 seconds.
Juvenile myoclonic epilepsy is a genetic syndrome characterized by myoclonic jerks and absence
and generalized tonic-clonic seizures. Affected patients have no warning prior to the generalized
tonic-clonic seizure. Afterward, the patient falls into a deep sleep or is tired and confused for
minutes to hours. Complex partial seizures with secondary generalization are associated with
auras, but patients are not likely to be pale or diaphoretic or to feel warm. Postictal confusion
lasts minutes to hours. Pseudoseizures are not stereotypical in presentation and usually last
longer. Atonic seizures consist of sudden loss of postural tone without any prodrome or warning
and without loss of consciousness.

Neurology:Question 24
The correct answer is C
Educational Objectives
Treat pathologic fatigue of multiple sclerosis.
Amantadine is the first-line pharmacologic agent for treatment of multiple sclerosis fatigue,
which is common and disabling. Amantadine affects several neurotransmitter systems and
enhances neurotransmitter release; however, the exact mechanism by which it improves fatigue of
multiple sclerosis is not known. It is well tolerated in younger patients with multiple sclerosis
despite anticholinergic effects at doses such as 100 mg orally each morning or twice a day
(morning and afternoon).
Other causes of severe fatigue should be sought in all patients with multiple sclerosis. Typically,
patients should be screened for thyroid dysfunction, but thyroid supplementation is used only if
laboratory abnormalities suggest thyroid disease. Amphetamines and agents such as ritalin are not
first-line therapy because of their addiction potential.
No firm data support use of selective serotonin reuptake inhibitors for fatigue of multiple
sclerosis. One study suggests an overlap between depression and fatigue in multiple sclerosis, but
they are believed to be due to different mechanisms. Pemoline has been used for multiple
sclerosis fatigue in the past, but it is now used infrequently because frequent liver function tests
are needed to check for hepatotoxicity. Modafinil, a nonamphetamine stimulant used extensively
in narcolepsy, is useful in fatigue of multiple sclerosis as well. Its primary limitation is cost.
Other approaches to fatigue include energy conservation, graded exercise programs to promote
stamina, and elimination of medications that may contribute to sedation.
Neurology:Question 25
The correct answer is A
Educational Objectives
Use cholinergic augmentation in Alzheimers disease.
This patient has symptoms consistent with progressive dementia, most likely Alzheimer‟s
        Clinical trials consistently demonstrate modest efficacy
of cholinesterase inhibitors, including galantamine, tacrine,
donepezil, and rivastigmine, in mild to moderate Alzheimers disease, on both
cognitive and global function scales. In addition, cholinesterase inhibitors act favorably on
psychiatric symptoms, including agitation, apathy, and hallucinations. There is no documented
efficacy of these drugs in delaying the natural history of Alzheimer‟s disease. Gingko biloba
probably exerts modest effects on cognitive symptoms, but has not been shown to improve global
or psychiatric function. Although some studies suggest that vitamin E and selegiline delay the
progression of Alzheimer‟s disease, they do not directly relieve cognitive or psychiatric

Neurology:Question 26
The correct answer is A
Educational Objectives
Administer intravenous tissue plasminogen activator (t-PA)to ischemic stroke patients only
within 3
hours of stroke onset.
Intravenous t-PA decreases the risk of disability in patients with acute ischemic stroke only when
given according to the protocol established in 1995 in studies by the National Institute of
Neurological Disorders and Stroke (NINDS). Chief among the inclusion criteria is stroke onset
within 3 hours of when the patient was last known to be normal. Since patients with acute stroke
often cannot give accurate histories, the histories obtained from witnesses and caregivers are
essential. According to protocol criteria, this patient‟s stroke onset was 3 days earlier. The
computed tomography findings lend further evidence that the stroke was not acute: a hypodense
(dark) area with mass effect suggests a subacute (days-old) infarction.
Corticosteroids are not indicated and may be harmful in patients with ischemic stroke. The
cytotoxic cerebral edema that occurs with ischemic stroke is greatest 2 to 5 days after stroke
onset, and does not respond to corticosteroids. Corticosteroids are effective for vasogenic edema
due to mass lesions such as tumors. Despite its frequent use in the United States, there is no
evidence that intravenous heparin is safe and effective in patients with acute ischemic stroke; in
fact, this therapy carries increased risk of hemorrhagic transformation of the infarction, especially
in large subacute infarctions. Intra-arterial t-PA is not currently approved by the U.S. Food and
Drug Administration for ischemic stroke; at this time, it is used in specialized centers for patients
who present within 3 to 6 hours of onset of symptoms.

Neurology:Question 27
The correct answer is E
Educational Objectives
Recognize multiple system atrophy.
This patient has evidence of dysautonomia (with orthostatic hypotension and possible neurogenic
bladder), parkinsonism, corticospinal tract signs, and ataxia. The constellation of findings is
typical of multiple system atrophy. The “screeching” sounds during the night are worrisome for
nocturnal stridor, which is highly suggestive of multiple system atrophy. Polysomnography
should be obtained to confirm nocturnal stridor because patients with multiple system atrophy
and stridor may die during the night, presumably from dysfunction of the brainstem breathing
center. Stridor can be treated with tracheostomy or possibly with continuous positive airway
pressure. Although Parkinson‟s disease is often associated with dysautonomia, the pronounced
autonomic symptoms, including symptomatic orthostatic hypotension, are atypical. Further,
corticospinal tract signs and ataxia are not expected in Parkinson‟s disease. Creutzfeldt-Jakob
disease is unlikely, given the clinical picture and the 2-year course.
Although spinocerebellar degenerations may manifest with parkinsonism, corticospinal tract
signs, and ataxia, they do not include prominent dysautonomia. Progressive supranuclear palsy
manifests with parkinsonism and often corticospinal tract signs, but not with severe dysautonomia
with symptomatic orthostatic hypotension. Also, the patient does not have the vertical
(downward) gaze palsy suggesting progressive supranuclear palsy.

Neurology:Question 28
The correct answer is C
Educational Objectives
Recognize vitamin B12 deficiency.
This man has signs and symptoms of progressive spinal cord dysfunction. He has dense posterior
column dysfunction (vibration and proprioception) as well as corticospinal tract motor signs and
deficits. This combination suggests subacute combined degeneration, as seen in vitamin B12
deficiency. The normal magnetic resonance image of the spinal cord rules out cord compression.
However, other causes of progressive myelopathy should be considered.
The other options are not upper motor neuron syndromes and are not associated with
hyperreflexia or extensor plantar responses.

Neurology:Question 29
The correct answer is D
Educational Objectives
Evaluate intracranial hemorrhage.
This patient has an intracerebral hemorrhage with intraventricular extension. Although the
location of the hemorrhage is typical for a hypertensive bleed, patients younger than 45 years who
have no prior history of hypertension are at much higher risk for a potential vascular anomaly
underlying the hemorrhage. Cocaine use could be solely responsible for the stroke; however,
patients with cocaine-related hemorrhage are also more likely to have a vascular anomaly. This
patient needs aggressive evaluation for a possible underlying vascular malformation, and the
definitive test is cerebral angiography. Electroencephalography is not indicated unless the history
raises the possibility of seizure. Echocardiography would provide information about the
possibility of chronic hypertension in this patient, but patients younger than 45 years should have
vascular imaging after intracranial hemorrhage, even if they have known hypertension.

Neurology:Question 30
The correct answer is B
Educational Objectives
Diagnose acute diabetic proximal neuropathy.
This patients signs and symptoms are consistent with a lesion of the right upper lumbar root or right
lumbar plexus. The normal findings on magnetic resonance imaging argue against a structural or
compressive cause. The most likely noncompressive cause of right upper lumbar radiculoneuropathy
is diabetes. Diabetic proximal neuropathy (diabetic amyotrophy) may be symmetric or asymmetric,
and presents with subacute proximal leg muscle weakness associated with severe pain. Symptoms
usually begin after longstanding uncontrolled hyperglycemia and weight loss. The pathogenesis is
unclear; nerve biopsy studies have shown a vasculitic inflammatory infiltrate in involved nerves.
Obtaining a creatine kinase level would be appropriate if primary muscle disease were suspected, but
this is not likely given the presence of neuropathic pain and sensory loss. Thyroid function tests are
often obtained in cases of peripheral neuropathy, but thyroid derangement would not cause a lumbar
radiculopathy or plexopathy. Lumbar puncture may help confirm an inflammatory process in the
cerebrospinal fluid, but would not result in a specific diagnosis. Similarly, elevations of the
erythrocyte sedimentation rate are nonspecific.

Neurology:Question 31
The correct answer is D
Educational Objectives
Diagnose progressive supranuclear palsy.
This patient‟s early falls and vertical gaze paresis make progressive supranuclear palsy the likely
diagnosis. Patients with progressive supranuclear palsy often have vision complaints, yet their
optometrists are unable to document problems with either visual acuity or peripheral vision. Impaired
eye movements, especially the inability to look down, underlie these complaints. Many routine tasks,
such as reading and needlework, require down gaze and visual tracking.
Patients with idiopathic Parkinson‟s disease do not fall early in the course of disease. Although there
are clues that might suggest dystonia, multiple system atrophy, or cerebellar degeneration, the overall
clinical picture fits best with progressive supranuclear palsy.

Neurology:Question 32
The correct answer is D
Educational Objectives
Recognize frontotemporal dementia.
This patients progressive impairment of cognition with prominent personality and behavior changes is
most consistent with frontotemporal dementia. Brain imaging would be appropriate to exclude a mass
lesion. Brain imaging in patients with frontotemporal dementia typically shows pronounced atrophy of
the frontal and temporal lobes. Despite the patients high score on the Mini-Mental State Examination
(MMSE), a diagnosis of mild cognitive impairment is inappropriate, since the patient is clearly
impaired in his interpersonal and occupational function, and not only in memory. In fact, the
paradoxically high score is a clue to the diagnosis, because the MMSE is relatively insensitive to
executive dysfunction, the predominant impairment in frontotemporal dementia. The positive family
history suggests an autosomal-dominant disorder, which is more likely in frontotemporal dementia
than in Alzheimer‟s disease. None of the features of dementia with Lewy bodies (hallucinations,
parkinsonism, fluctuating symptoms) is present. Creutzfeldt-Jakob disease evolves more rapidly, and
usually causes myoclonus and other neurologic signs.

Neurology:Question 33
The correct answer is E
Educational Objectives
Recognize the effect of corticosteroids on primary lymphoma of the CNS.
This is an elderly patient with subacute progressive neurologic deficits referable to the
supratentorial level and signs of raised intracranial pressure. The clinical history and imaging
suggest neoplasm. Corticosteroid therapy produces rapid clinical and radiographic improvement.
This clinical picture is most characteristic of primary central nervous system lymphoma. If
primary central nervous system lymphoma is suspected, it is important to refrain from using
corticosteroids prior to biopsy, unless the patients clinical situation absolutely requires it.
Corticosteroids exert an oncolytic effect on lymphomas and may
render the biopsy nondiagnostic.
Glioblastoma and metastatic carcinoma are unlikely to have such a dramatic radiographic and
clinical response to corticosteroids. Although vasculitis or multiple sclerosis may respond rapidly
to corticosteroids, neither disorder is likely to develop in a 70-year-old patient, and neither would
generally present as a mass lesion on brain imaging.

Neurology:Question 34
The correct answer is E
Educational Objectives
Treat secondary progressive multiple sclerosis (MS).
Mitoxantrone is approved by the U.S. Food and Drug Administration (FDA) to slow progression
of secondary progressive or severe relapsing-remitting MS. The speed of this patient‟s decline
merits consideration of aggressive immunosuppression. The primary concern about treatment
with mitoxantrone is that dose-related cardiotoxicity prohibits use beyond a cumulative dose of
120 to 140 mg/m2.
No benefit has been shown for chronic prednisone treatment and this is not recommended. Bone
marrow transplantation is investigational only. There is some evidence that plasmapheresis is
useful for acute devastating attacks of demyelination that do not respond quickly to standard
intravenous corticosteroid treatment, but there is little evidence of benefit for progressive disease.
Glatiramer acetate can reduce the frequency of relapses in patients with relapsing-remitting MS,
but is not indicated for progressive disease. Results are inconsistent concerning whether
interferon beta slows secondary progressive MS. Other agents that have been used for secondary
progressive disease include cyclophosphamide, intravenous immunoglobulin, azathioprine, and
methotrexate, although none are FDA approved for this purpose.

Neurology:Question 35
The correct answer is A
Educational Objectives
Select prophylaxis for migraine headache.
This patient has frequent attacks of migraine without aura. She is at risk for developing rebound
headaches from overuse of acute medication. Accordingly, a preventive medication is indicated.
Amitriptyline is a first-line agent for migraine prophylaxis; its sedating effect may also help her
insomnia. Nadolol and propranolol are contraindicated because they may trigger her asthma.
Naratriptan is used for treatment of acute migraine and is not approved for prophylactic therapy.
Selective serotonin reuptake inhibitors have limited effectiveness in migraine prophylaxis.

Neurology:Question 36
The correct answer is B
Educational Objectives
Determine when to withdraw antiepileptic drug treatment.
According to recent studies of adult patients who are seizure free for more than 2 years, approximately
one third relapse following termination of antiepileptic drugs. Risk factors associated with recurrence
of seizures include prolonged duration and high frequency of seizures before they were controlled,
abnormal neurologic examination, abnormal magnetic resonance imaging (MRI) of the brain,
consistently abnormal electroencephalograms (EEG5), and mental retardation. This patient has
consistently abnormal EEGs. He should continue antiepilepsy treatment despite having been seizure
free for 4 years. Ordering another EEG and MRI would not change the outcome. Single photon
emission computed tomography (SPECT) is not helpful when clinical history, EEG, or MRI provide
sufficient localization. Changing to another agent such as carbamazepine would be appropriate if the
patient had side effects from phenytoin.

Neurology:Question 37
The correct answer is C
Educational Objectives
Recognize the typical clinical presentation of cerebral meningioma.
This 50-year-old patient has a chronic progressive history of a scalp/skull mass and symptoms of
raised intracranial pressure. Computed tomography demonstrates an extra-axial calcified enhancing
mass with overlying skull hyperostosis, a classic picture for meningioma. Meningiomas derive from
the arachnoid granulations of the dura, hence their usual occurrence over the cerebral convexities,
skull base, or falx cerebri. Calcification is typical and reflects the slow-growing, chronic nature of the
tumor. Meningiomas often invade adjacent bone and may produce a palpable or visible hyperostotic
mass. Meningiomas most commonly affect middle-aged and elderly women.
Gliomas, including glioblastoma multiforme, are intra-axial tumors that involve the parenchyma of the
brain and not the dural surface. Dural metastases are by definition malignant tumors and usually
present more acutely. They do not calcify. While chronic subdural hematomas may calcify, they
usually do not enhance, nor do they produce hyperostosis of the overlying bone.

Neurology:Question 38
The correct answer is B
Educational Objectives
Treat an elderly patient with a large cerebral infarction.
Intravenous tissue plasminogen activator (t-PA) decreases the risk of disability in patients with
ischemic stroke when administered within 3 hours of stroke onset (National Institute of
Neurological Disorders and Stroke [NINDS] protocol). Although older patients have a higher risk
of intracerebral hemorrhage than younger patients, persons within the same age group who
receive intravenous t-PA fare better than those who do not. The same is true for patients with
large cerebral infarctions (for example, infarcts involving more than one third of the middle
cerebral artery territory). At this time, there is insufficient evidence to recommend intra-arterial
over intravenous t-PA within 3 hours of stroke onset, and intra-arterial t-PA is not currently
approved by the U.S. Food and Drug Administration for ischemic stroke. The NINDS protocol
forbids administration of aspirin or any other antithrombotic agent within 24 hours after t-PA
administration. To date, all clinical trials assessing emergent administration of aspirin plus
thrombolysis in stroke patients have shown poor outcomes.

Neurology:Question 39
The correct answer is A
Educational Objectives
Manage status epilepticus.
The patient has already received a full dose of lorazepam (0.1 mg/kg). The most appropriate next
step is to administer a full loading dose of fosphenytoin or phenytoin (20 mg/kg of body weight;
the phenytoin equivalence in option C is too low). Fosphenytoin is preferred over phenytoin
because it can be administered faster than phenytoin and has a lower incidence of hypotension
and local thrombophlebitis at the site of administration (“purple glove syndrome‟). Intravenous
infusion of fosphenytoin (ordered in phenytoin-equivalent doses) should be initiated while
lorazepam is still being administered because it takes approximately 15 minutes for fosphenytoin
to take effect. If there is no risk of respiratory suppression (patient is intubated) and the patient is
hemodynamically stable, additional doses of lorazepam (beyond a full dose) can be given if
necessary. Appropriate loading doses (mg/kg of body weight) of phenobarbital (after intubation)
or valproic acid can be administered if the patient continues to have seizures despite treatment
with lorazepam and fosphenytoin. The common practice of routinely giving 1 gram of phenytoin
may result in under- or overdosing. Phenytoin should be administered according to patient

Neurology:Question 40
The correct answer is D
Educational Objectives
Recognize dementia with Lewy bodies.
The patient manifests the cardinal features of dementia with Lewy bodies, including cognitive
impairment, fluctuating symptoms, hallucinations, and parkinsonism. Patients who have dementia
with Lewy bodies often also have a cholinergic deficit, as suggested in this case by the episode of
confusion associated with an anticholinergic medication. The fluctuating and psychiatric
symptoms warrant treatment. Treatment with a cholinesterase inhibitor, such as donepezil, may
be beneficial.
Mild cognitive impairment is not an appropriate diagnosis, because the disorder is severe enough
to impair his daily function, and because the deficits go beyond memory impairment. A primary
diagnosis of Alzheimers disease is not appropriate because fluctuating sensorium and
parkinsonism are not core features of this disease, as they are in dementia with Lewy bodies.
However, a component of Alzheimer pathology is commonly superimposed on the pathology of
dementia with Lewy bodies. For similar reasons, a primary diagnosis of frontotemporal dementia
is also not appropriate.

Neurology:Question 41
The correct answer is A
Educational Objectives
Distinguish chorea from other hyperkinetic movement disorders.
Rapidly flowing, dancing-like involuntary movements occurring in a chaotic, nonstereotyped
fashion define chorea. The differential diagnosis for chorea includes disorders that are inherited
(for example, Huntingtons disease), immune-mediated (for example, antiphospholipid antibody or
paraneoplastic syndrome), endocrinologic (for example, hyperthyroidism), and hematologic (for
example, polycythemia vera or neuroacanthocytosis). In this case, the patients age, chronicity of
symptoms, and abnormal mental status score suggest Huntington‟s disease. This could be
confirmed by genetic testing, if he agrees.
Chorea does not occur in Parkinsons disease unless provoked by medications (he is taking none).
The 3-year course makes Creutzfeldt-Jakob disease unlikely. Tourettes syndrome starts earlier in
life with stereotyped movements (tics). This patient‟s adventitious movements are chaotic and not
stereotyped like tics.

Neurology:Question 42
The correct answer is D
Educational Objectives
Classify course of multiple sclerosis.
This woman‟s course began with acute exacerbations followed by remissions of variable duration, so
her disease was initially relapsing-remitting. However, in the past year, she has had progression of
neurologic deficits between acute relapses. In at least 50% of patients with relapsing-remitting
multiple sclerosis, disease will evolve to a secondary progressive course, such as in this case. Even a
single remote attack followed a decade later by the onset of progression is classified as a secondary
progressive course.
Monosymptomatic demyelination refers to a single clinical episode of demyelination, such as isolated
optic neuritis. Relapsing-remitting disease refers to multiple attacks of demyelination. Primary
progressive multiple sclerosis starts as an insidious spinal cord dysfunction or as ataxia, progressing
without distinct acute exacerbations; it tends to have fewer cerebral lesions (as seen on magnetic
resonance imaging) than other forms of multiple sclerosis. Benign multiple sclerosis refers to a course
in which all neurologic systems are fully functional 15 years after disease onset.

Neurology:Question 43
The correct answer is D
Educational Objectives
Manage symptomatic extracranial carotid atherosclerosis.
Sudden right hemiparesis with expressive aphasia suggests a left cortical infarction in the territory of
the left middle cerebral artery, which is supplied by the left internal carotid artery. Carotid stenosis of
50% or more that is ipsilateral to the infarction implies that the stroke is due to large artery disease.
Carotid endarterectomy decreases risk of future stroke in patients with symptomatic stenosis of 50%
or more, and especially in patients with 70% to 99% stenosis, as compared with antiplatelet agents
alone. Aspirin, 81 or 325 mg daily, decreases the risk of stroke after endarterectomy. There is no
evidence that intravenous heparin is safe and effective in patients with acute ischemic stroke.

Neurology:Question 44
The correct answer is D
Educational Objectives
Manage small, asymptomatic meningiomas.
This 75-year-old woman has what is most probably a small, asymptomatic convexity
meningioma. The calcification suggests that the meningioma has been present for months or
years. Calcified meningiomas are less likely to progress than noncalcified lesions. In this setting,
the appropriate management is follow-up imaging in 6 months to assess the stability of the lesion,
with consideration of further follow-up at increasing intervals if no growth is seen. If the lesion
enlarges or becomes symptomatic, then surgical resection is indicated, assuming the patient is a
suitable candidate. Biopsy and radiosurgery are not appropriate for patients with incidental,
asymptomatic meningioma. In autopsy series, small, asymptomatic meningiomas have been
found in 1% to 2% of individuals.

Neurology:Question 45
The correct answer is D
Educational Objectives
Recognize the persistent vegetative state.
In this patient, severe cerebral anoxia from her cardiac arrest led to diffuse cortical injury with
relative preservation of brainstem function, and the development of a vegetative state.         A
vegetative state is a clinical condition of complete unawareness of
self or the environment, accompanied by sleep-wake cycles and
preservation of brainstem and hypothalamic autonomic functions.
According to the conclusions of a multi-society task force, persistent vegetative state is diagnosed
if a vegetative state is present 1 month after acute traumatic or nontraumatic brain injury. Unlike
a vegetative state, in which there is no evidence of consciousness, in the minimally conscious
state there is severely altered consciousness with minimal but definite behavioral evidence of self
or environmental awareness. In the locked-in syndrome, patients are conscious because of normal
cortical and upper brainstem function, but are quadriplegic and can communicate only through
the ability to move their eyes vertically and blink; this syndrome is caused by lesions at the base
of the pons. Brain death is the complete absence of all cerebral hemispheric and brainstem
functions, including the absence of respiratory drive.

Neurology:Question 46
The correct answer is A
Educational Objectives
Treat optic neuritis.
This patient has signs and symptoms consistent with left optic neuritis. Although most patients
have good recovery in 6 months with or without treatment, a course of intravenous
methylprednisolone (1 g daily for 3 days) followed by an oral prednisone taper speeds recovery
of visual acuity in optic neuritis. The decision on whether to use corticosteroids or hold treatment
depends on patient preference, severity of the visual deficit, health of the fellow eye, and
vocational requirements.
This patient has a single clinical demyelinating event, with no
history of previous clinical neurologic events or evidence of silent
demyelinating lesions on brain imaging. Therefore, a diagnosis of
multiple sclerosis and treatment with disease-modifying therapies
(interferon beta or glatiramer acetate) are not warranted. However,
surveillance may be appropriate with follow-up magnetic resonance imaging of the brain to watch
for development of cerebral white matter lesions. One major study of optic neuritis found that
there is no benefit to treatment with oral prednisone alone over no treatment; indeed, the study
found an increased risk of optic neuritis in the fellow eye in the next 6 to 12 months with oral
prednisone treatment. The presentation is not suggestive of an ischemic event and there is no
indication for treatment with aspirin.

Neurology:Question 47
The correct answer is D
Educational Objectives
Manage complex partial seizures.
This patients episodes are most likely complex partial seizures of frontal lobe origin. Frontal lobe
seizures are brief, usually occur in clusters, and usually are not associated with aura or postictal
confusion. Appropriate anticonvulsant management should be initiated, even during the
diagnostic work-up. Carbamazepine is approved by the U.S. Food and Drug Administration
(FDA) for treatment of partial seizures and is an appropriate initial choice. Phenobarbital is not a
first-line agent because of possible associated sedation and cognitive impairment.
Gabapentin is approved for adjunctive treatment of partial seizures but should not be used in
monotherapy. Ethosuximide is used to treat absence seizures, which this patient does not have.
Lamotrigine is FDA approved for adjunctive treatment of partial seizures; many neurologists use
it with success as second-line monotherapy.

Neurology:Question 48
The correct answer is C
Educational Objectives
Treat essential tremor.
This patient has a postural and action tremor (essential tremor), rather than a resting tremor
typical of Parkinson‟s disease. First-line drugs include primidone or a nonselective β-adrenergic
blocker, such as propranolol. Selective β1 blockers, such as atenolol, are not effective against
tremor, at least in the lower doses where the β1 effect is predominant. As the clinical description
is not that of parkinsonian tremor, carbidopa/levodopa or pramipexole would not be helpful.
Vaiproic acid may induce tremor and is not used to treat it.

Neurology:Question 49
The correct answer is A
Educational Objectives
Prescribe an angiotensin-converting enzyme (ACE) inhibitor and a diuretic for patients who have
had stroke or transient ischemic attack, regardless of baseline blood pressure.
In PROGRESS, patients with a recent stroke or transient ischemic attack (TIA) had decreased
risk of future stroke, heart attack, or death if they took the ACE inhibitor perindopril and the
diuretic indapamide—even if they had no previous history of hypertension or if their pretreatment
blood pressure levels were in the so-called „normotensive” range. However, it is best not to lower
blood pressure aggressively soon after a stroke in order to preserve the penumbra and because
cerebral autoregulation is dysfunctional. In PROGRESS, low-dose perindopril was begun 2
weeks after the stroke, and gradual increases in antihypertensive therapy were made at 2-week
intervals. The results of recent clinical outcome-based trials such as PROGRESS have made it
increasingly clear that optimal target blood pressure levels are lower than we previously believed.
The target blood pressure for patients with diabetes or end-organ damage, including stroke
patients, is 130/80 mm Hg. There are no data regarding the efficacy of β -blockers or calcium
channel blockers for secondary stroke prevention.

Neurology:Question 50
The correct answer is E
Educational Objectives
Select the appropriate medication for acute treatment of migraine.
This patient has episodic severe migraine without aura. Zolmitriptan and other triptans are more
likely to be effective for severe migraine headaches than nonspecific agents such as combination
analgesics and nonsteroidal anti-inflammatory drugs. Nonspecific agents may be effective for
mild to moderate headaches.
Neurology:Question 51
The correct answer is B
Educational Objectives
Recognize the association of Epstein-Barr infection with primary lymphoma of the central
nervous system in immunocompromised patients.
Primary central nervous system (CNS) lymphoma develops in approximately 2% of patients with
HIV infection, usually late in the course of infection. Unlike the lesions of immunocompetent
patients with primary CNS lymphoma, the lesions in HIV-infected patients often manifest central
necrosis with a ring-enhancing appearance that is difficult to distinguish from CNS infections
such as toxoplasmosis. Nearly all primary CNS lymphomas in HIV-infected individuals contain
Epstein-Barr viral genome. Cerebrospinal fluid polymerase chain reaction is typically negative
for Epstein-Barr virus. In the presence of reduced T-cell function, the Epstein-Barr-infected B
cells become immortalized and are driven toward a monoclonal malignant B-cell population.
Epstein-Barr viral genome is rare in primary CNS lymphoma in immunocompetent patients.
Patients with primary CNS lymphoma may be treated with high-dose methotrexate and brain
radiation. Unfortunately, treatment of primary CNS lymphoma in patients with HIV is rarely
successful, and most patients die within 6 months of diagnosis (usually from complications of
other opportunistic infections). Cytomegalovirus, herpes zoster, and herpes simplex may infect
the brain of an immunocompromised patient, but none is associated with CNS lymphoma.

Neurology:Question 52
The correct answer is A
Educational Objectives
Treat a patient with subarachnoid hemorrhage.
This patient has a subarachnoid hemorrhage, likely due to aneurysm rupture. In terms of the
hemorrhage, the most important intervention at this time is to initiate treatment with nimodipine.
Nimodipine helps reduce subsequent development of vasospasm and has been shown to improve
outcome in clinical trials. Other antihypertensive medications, such as labetalol and enalapril, are
not necessary because nimodipine has an antihypertensive effect. There is no role for
corticosteroids in patients with subarachnoid hemorrhage.

Neurology:Question 53
The correct answer is B
Educational Objectives
Diagnose mononeuropathy multiplex.
This patient has a right peroneal mononeuropathy and a left ulnar mononeuropathy. By definition,
this is a case of multiple mononeuropathies (mononeuritis multiplex). Mononeuritis
multiplex is usually due to vascular infarction of multiple nerves.
Causes of mononeuritis multiplex include diabetes (unlikely in this
case because of the normal fasting glucose), connective tissue
diseases (such as systemic lupus erythematosus or rheumatoid
arthritis), polyarteritis nodosa, Wegeners granulomatosis, HIV
infection, and isolated peripheral nervous system vasculitis. The
systemic findings in this case suggest connective tissue disease or systemic necrotizing vasculitis.
The effects of amyloidosis on the peripheral nervous system typically involve the autonomic
nerves and may be associated with bilateral carpal tunnel syndrome. Guillain-Barré syndrome
causes acute symmetric limb or bulbar weakness often preceded by sensory symptoms, but would
not cause multiple asymmetric mononeuropathies, as is the case in this patient.

Neurology:Question 54
The correct answer is D
Educational Objectives
Recognize juvenile myoclonic epilepsy.
The patient had myoclonic jerks that led to a generalized tonic-clonic seizure. This is a typical
presentation of juvenile myoclonic epilepsy, an idiopathic epilepsy syndrome. Affected patients
have a normal neurologic examination and normal development. Unlike other idiopathic
epilepsies, remission is rare even after years without a seizure, so lifelong treatment is necessary.
Patients are sensitive to sleep deprivation, alcohol, and stress.
Unlike juvenile myoclonic epilepsy, which is a primary, generalized epilepsy, partial seizures
originate from a focal part of the brain, and their clinical presentation depends on their
neuroanatomic location. Absence seizures mainly affect children. They manifest with sudden
onset of staring, with or without eye blinking or lip smacking. Patients with syncope often have
several myoclonic jerks while unconscious, but they recover quickly once blood pressure is
reestablished. Nonepileptic events (pseudoseizures) vary in presentation, but they are often
associated with moaning, crying, and arrhythmic, on-and-off shaking of the body.

Neurology:Question 55
The correct answer is B
Educational Objectives
Assess effect of immunomodulatory therapy on pregnancy.
Interferon beta is teratogenic in animals; spontaneous abortion is a risk. This patient should be
advised to stop interferon beta treatment immediately and see her obstetrician for evaluation and
monitoring of fetal development. Women taking disease-modifying treatment for multiple
sclerosis should be advised to practice effective contraception and to stop interferon or glatiramer
acetate several months before attempting to conceive. There have, however, been successful
pregnancies with early exposure to interferon beta.
Pregnancy has a short-term effect on the course of multiple sclerosis. Typically, the frequency of
relapse decreases significantly in the third trimester, with a rebound increase in the first 6 months
post partum. No studies have demonstrated a long-term effect of pregnancy on disability
progression. First-degree relatives of a person with multiple sclerosis have a risk of disease 20 to
50 times greater than that of the general population. However, the transmission of increased
susceptibility is believed to be multigenic; no simple one-gene pattern, such as autosomal-
recessive transmission, is observed. There is no medical indication for therapeutic abortion in this

Neurology:Question 56
The correct answer is D
Educational Objectives
Diagnose hemicrania continua.
Hemicrania continua is a primary headache disorder characterized by chronic, daily unilateral
headache with some autonomic phenomena. Indomethacin is effective in managing the disorder;
other preventive agents are usually ineffective. Cluster headaches last 1 to 2 hours and
predominantly occur in men. Chronic tension-type headaches are usually bilateral and do not
have associated autonomic phenomena. Chronic migraine is typically preceded by years of
episodic migraine.

Neurology:Question 57
The correct answer is B
Educational Objectives
Assess iron levels in patients with restless legs syndrome.
This patients description is typical of restless legs syndrome. Because restless legs syndrome may
occasionally be a manifestation of iron deficiency, it is appropriate to check iron levels. If
deficiency is identified, iron supplementation may resolve symptoms. Otherwise, restless legs
syndrome usually responds to a dopamine agonist medication (such as pergolide, pramipexole, or
ropinirole) or gabapentin. Carbidopa/levodopa is effective against restless legs syndrome, but it is
second-line therapy because of an associated rebound phenomenon („augmentation‟).
Typically, obtaining the serum ferritin level is an adequate work-up if the clinical picture fits with
restless legs syndrome, the neurologic examination is normal, and there are no major medical
problems. Hence, magnetic resonance imaging of the brain is unnecessary. Serum ceruloplasmin
or urine porphyrin levels are not relevant to this clinical presentation. Nerve conduction studies
are not helpful in the diagnosis of restless legs syndrome, and this patient has no findings on
examination to suggest neuropathy.

Neurology:Question 58
The correct answer is A
Educational Objectives
Recognize Creutzfeldt-Jakob disease and identify the appropriate confirmatory test.
This patient has signs of a subacute dementing illness. The pace of the illness, normal imaging
study, and presence of myoclonus suggest Creutzfeldt-Jakob disease. Treatable inflammatory
conditions, such as granulomatous angiitis of the nervous system, are unlikely but remain in the
differential diagnosis, and brain biopsy might be required to exclude them. Before considering
brain biopsy, a positive diagnosis of Creutzfeldt-Jakob disease can be made if there are periodic
sharp waves on the electroencephalogram or if 14-3-3 protein is present in the cerebrospinal fluid.
Although the electroencephalogram in this case might evolve over time and become diagnostic,
the pace of disease progression makes waiting inadvisable.
Functional imaging can localize dysfunctional tissue, but not characterize it. The presenilin test is
indicated only when familial Alzheimer-profile dementia is suspected.

Neurology:Question 59
The correct answer is C
Educational Objectives
Recognize the association of specific tumors with paraneoplastic neurologic disease.
This patient has paraneoplastic cerebellar degeneration. Paraneoplastic neurologic disorders are
rare complications of cancer. In this patient, a structural lesion of the posterior fossa, such as a
tumor or infarct, has been appropriately excluded by imaging.
In each of the paraneoplastic syndromes, the systemic tumor cell is believed to express an
“onconeural antigen” that produces an immune response in the patient. This onconeural antigen
shares similarities to antigens normally expressed by specific neural tissue. The host immune
response (cell mediated and humoral) then attacks both the tumor and the specific neural tissue
that share antigenic similarity. This theory is strengthened by the fact that patients‟ tumors often
share antigenic similarities with neural tissue and by the frequent observation that patients with
paraneoplastic neurologic disease often have limited or no metastatic disease and small primary
tumors. The latter observation suggests that the immune response to tumor may be particularly
strong in these patients.
The presence in this patient‟s serum of the anti-Yo antibodies is
very specific for paraneoplastic cerebellar degeneration in the
setting of ovarian, uterine, fallopian tube, or breast cancer. Anti-Hu
(anti-neuronal antibody type I) is the antibody associated with
small cell lung cancer and paraneoplastic neurologic disease.
Paraneoplastic neurologic disorders are very rare in non-Hodgkin‟s lymphoma or melanoma, and
there is no serologic antibody specifically associated with either tumor.

Neurology:Question 60
The correct answer is C
Educational Objectives
Recognize frontal lobe seizures.
The patient most likely has simple partial seizures of frontal lobe origin. The clinical presentation
of partial seizures depends on their neuroanatomic location. In this case, the seizures originate in
Broca‟s language area and spread to the primary motor area. Frontal seizures are brief and usually
without any aura or postictal confusion. The recent onset of seizures should lead to brain imaging
as soon as possible to rule out a space-occupying lesion. Transient ischemic attack, hemiplegic
migraine, and hypoglycemia can cause focal neurologic deficits, but would not cause twitching of
the face and arm. Anxiety attacks last longer (minutes) and are associated with other symptoms,
such as shortness of breath and fear.

Neurology:Question 61
The correct answer is B
Educational Objectives
Diagnose aneurysmal subarachnoid hemorrhage.
This young patient has sudden onset of severe, debilitating headache without prior history of such
events. It is a classic first, worst‟ headache scenario that suggests subarachnoid hemorrhage.
Further work-up is needed to evaluate for subarachnoid blood. Computed tomography (CT) is
sensitive for detecting acute hemorrhage, but its ability to detect subarachnoid hemorrhage is not
absolute, and after 3 days, 25% of affected patients have normal CT scans. When subarachnoid
hemorrhage is clinically suspected despite a normal CT scan, lumbar puncture is imperative to
rule it out. In general, magnetic resonance imaging is inferior to CT for detecting subarachnoid
blood. Arteriography is not warranted acutely until the presence of subarachnoid hemorrhage has
been established. Abortive treatment for headache should be given only after subarachnoid
hemorrhage has been ruled out.

Neurology:Question 62
The correct answer is D
Educational Objectives
Treat acute migraine.
This patient has migraine. An oral medication will have limited efficacy, because her headaches
are accompanied by early nausea and vomiting. Sumatriptan is the only 5HT1 B,D agonist
available in a parenteral form and is highly effective. Nasal sumatriptan is another reasonable
choice, but it is generally less effective. Subcutaneous or intramuscular dihydroergotamine could
also be considered for acute treatment, but intravenous administration is not appropriate in the
home setting.

Neurology:Question 63
The correct answer is B
Educational Objectives
Diagnose mild cognitive impairment.
This patients abnormal finding is limited to memory, and he has no impairment of interpersonal,
occupational, or daily living activities. Therefore, his condition should be classified as mild
cognitive impairment, not dementia. Follow-up evaluation in 1 year is appropriate, as many
affected patients may have pre-demential Alzheimers disease. The conversion rate from mild
cognitive impairment to mild dementia is 10% to 15% per year.
Although there may be an autosomal-dominant form of Alzheimers disease in this patients
family, affected members had disease onset when they were 20 years younger than this patient.
Thus, if the patient has Alzheimers disease, it is more likely to be a sporadic than familial case,
and genetic testing for presenilin-1 is not likely to be helpful. The specificity of cerebrospinal
fluid findings and functional imaging studies (such as positron emission tomography/single
photon emission computed tomography [PET/SPECT]) for early dementia has not been
determined, so it is unlikely that these tests would be helpful. Diffusion-weighted magnetic
resonance imaging is sensitive for acute ischemic stroke and has no role in evaluating
degenerative disease.

Neurology:Question 64
The correct answer is C
Educational Objectives
Treat clinically isolated demyelinating syndromes.
This woman has bilateral internuclear ophthalmoplegia, a classic presentation of multiple
sclerosis. Because there is no history of neurologic problems, this is a clinically isolated or
monosymptomatic presentation of demyelination. In this case, magnetic resonance imaging
(MRI) shows multiple hyperintense lesions adjacent to the lateral ventricle, which is typical of
multiple sclerosis. Treatment with interferon beta decreases the incidence of additional attacks in
patients with monosymptomatic demyelination (including optic neuritis and myelopathy) who
have multiple “silent” lesions that are typical of demyelination on brain MRI.
Methotrexate and cyclophosphamide may slow secondary progressive multiple sclerosis, but
would not be considered for treatment of a first attack. The patient‟s presentation does not suggest
cerebrovascular disease, so aspirin and clopidogrel are not appropriate.

Neurology:Question 65
The correct answer is B
Educational Objectives
Use botulinum toxin to treat adult-onset focal dystonia.
The patient has cervical dystonia (spasmodic torticollis), an idiopathic focal dystonia of
adulthood. Although its origins are presumed to be within the brain, imaging studies of the brain
are negative, as are studies of the cervical spine. Oral medications for cervical dystonia, including
muscle relaxants (diazepam, cyclobenzaprine), are mildly effective at best. Analgesics may be
necessary to relieve pain from muscle contraction. The most consistently effective medical
treatment is botulinum toxin injected into appropriate neck muscles, sometimes under
electromyographic guidance. Within a few days, the injected muscles weaken, tending to release
the spasm. Benefits persist for 2 to 4 months, and reinjection every 3 or more months maintains
the benefits. Excessive weakness of the injected muscles is rarely a problem.
Although carbidopa/levodopa may improve the dystonia of Parkinson‟s disease, it is ineffective
in treating primary idiopathic focal dystonias, such as cervical dystonia. Psychiatry referral may
improve stress management, but is unlikely to have a major impact on the motor manifestations
of cervical dystonia.

Neurology:Question 66
The correct answer is A
Educational Objectives
Recognize complex partial seizures of temporal lobe origin.
This is a typical description of complex partial seizure of temporal lobe origin. The symptoms of
complex partial seizures vary, but they are stereotypical for each patient. Patients may have a
warning (aura) prior to the seizure. The most common warning is a rising epigastric sensation;
other auras may include affective (such as fear), cognitive (such as déjà vu), and sensory
symptoms (such as olfactory hallucinations). Complex partial seizures typically last less than 3
minutes. During that time the patient appears awake, but loses contact with the environment and
does not respond normally to instructions or questions. Patients usually stare and remain
motionless, or engage in repetitive, semi-purposeful behavior (automatisms), such as gesturing,
chewing, lip smacking, repeating words or phrases, walking, running, or undressing. Patients do
not recall these behaviors. After the seizure the patient is confused or sleepy for minutes to hours.
Absence seizures occur primarily in children, are not preceded by aura, and are not followed by
postictal confusion. They last 5 to 10 seconds and occur primarily in clusters. Nonepileptic events
(pseudoseizures) are characteristically variable (not stereotypical) in presentation and last longer.
Migraine can be associated with neurologic symptoms, including confusion, but there is usually a
history of headache or other obvious symptom (for example, visual phenomena). Transient global
amnesia is a syndrome of acute memory loss of unknown etiology; it can last for minutes to
hours. There is no alteration or loss of consciousness during transient global amnesia.

Neurology:Question 67
The correct answer is B
Educational Objectives
Recognize the clinical presentation of Lambert-Eaton myasthenic syndrome.
This patient has Lambert-Eaton myasthenic syndrome (LEMS), which in 60% of cases occurs in
patients harboring small cell lung cancer. LEMS is a disorder of nicotinic and muscarinic
cholinergic synapses resulting in impaired acetylcholine release, with muscle weakness and
autonomic dysfunction. Affected patients produce antibody to the voltage-gated calcium channel
(VGCC), thus impairing calcium influx into the nerve terminal and reducing acetylcholine
release. The circulating anti-VGCC antibody is detectable in some but not all patients. Spinal
cord compression presents with spine and radicular pain and evidence of myelopathy (signs of
upper motor neuron weakness, spasticity, Babinski signs, and sensory loss typically with a
defined level). Polymyositis generally produces muscle pain and weakness without symptoms of
autonomic nervous system dysfunction. Polyneuropathy is a possibility, although it usually would
produce distal more than proximal weakness; the weakness would not improve with repeated
effort. Autonomic dysfunction is less commonly associated with polyneu ropathy.

Neurology:Question 68
The correct answer is C
Educational Objectives
Treat acute migraine in the emergency department.
This patient needs parenteral treatment for her prolonged, severe migraine headache that is
accompanied by repeated vomiting. The neuroleptic agent chlorpromazine is effective for such a
situation. Although dihydroergotamine may be useful when administered intravenously during a
severe migraine attack, it should not be used in this situation because the patient has already used
rizatriptan within the past 24 hours and both medications have vasoconstrictive effects.
Dihydroergotamine may cause nausea and vomiting, so when it can be used intravenously, an
antiemetic such as metoclopramide must be administered first. Oral codeine (or any oral agent) is
a poor choice since the patient is nauseated and vomiting. Finally, sumatriptan should not be used
because the patient has already used rizatriptan within the past 24 hours.

Neurology:Question 69
The correct answer is A
Educational Objectives
Prescribe cholesterol-lowering therapy for secondary prevention of stroke.
Among patients with known coronary artery disease, statin drugs decrease the risk of ischemic
stroke as well as myocardial infarction. The evidence is strongest for pravastatin, 40 mg/d, and
simvastatin, 20 to 40 mg/d. Previous studies of lipid-lowering therapy other than statins did not
show a decrease in stroke risk (and showed lesser decreases in cholesterol levels). Observational
studies suggest that a low-density lipoprotein (LDL) level below 70 mg/dL may increase the risk
of hemorrhagic stroke. Patients with diabetes or a history of stroke, coronary artery disease, or
peripheral artery disease should take a statin, if at all possible, to achieve a target LDL value
between 70 and 100 mg/dL. LDL cholesterol levels decrease in the first 24 to 48 hours after an
ischemic event and do not return to baseline for several weeks. It is likely that this patients LDL
value of 110 mg/dL in the hospital is lower than his pre-event value, further emphasizing his need
for lipid-lowering therapy.

Neurology:Question 70
The correct answer is B
Educational Objectives
Recognize treatment options for medically intractable epilepsy.
This patient has a seizure disorder of right temporal lobe origin. Epilepsy is considered medically
intractable when adequate trials of three different antiepileptic drug regimens have not controlled
seizures. Excision of the epileptogenic focus is now widely accepted for medically intractable
seizure disorders, particularly those of temporal lobe origin.
Vagal nerve stimulation is less efficacious than surgery for medically intractable epilepsy, but
should be considered if the patient is not a candidate for temporal lobectomy. Adding a third or
fourth antiepileptic drug would probably cause more side effects without significantly improving
seizure control. Positron emission tomography is not necessary, because there is enough clinical,
electroencephalographic, and radiographic evidence that the patients seizures originate from the
right temporal lobe. Repeat magnetic resonance imaging of the brain is not necessary, since
mesial temporal sclerosis does not usually progress, and there is no correlation between the
degree of sclerosis and seizure frequency.

Neurology:Question 71
The correct answer is A
Educational Objectives
Recognize hypertensive hemorrhage.
This patient has an intracranial cerebral hemorrhage in the left basal ganglia. She has
                                               basal ganglia are a
electroencephalographic evidence of chronic hypertension. The
classic site for primary hypertensive hemorrhage; other common
sites are the thalamus/internal capsule, pons, and cerebell urn.
The evidence of chronic hypertension and the location of this patient‟s hernorrhage make
arteriovenous malformation unlikely. Aneurysmal rupture presents with bleeding in the
subarachnoid space around the brain, not within the brain parenchyrna. Amyloid angiopathy
causes so-called “lobar‟ hemorrhage, which occurs in the parenchyma in locations closer to the

Neurology:Question 72
The correct answer is D
Educational Objectives
Use immunomodulatory therapy in multiple sclerosis.
This patient has clinically definite relapsing-remitting multiple sclerosis. The current consensus
of the National Multiple Sclerosis Society is that all patients with definite relapsing multiple
sclerosis be considered for treatment with either a form of interferon beta or glatiramer acetate.
These immunomodulatory agents decrease the frequency of relapse and the rate of accumulation
of new lesions on magnetic resonance imaging.
High-dose intravenous methylprednisolone (for example, 500 to 1000 mg daily for 3 to 5 days) is
standard treatment for a significant acute exacerbation of multiple sclerosis. Periodic pulses of
high-dose corticosteroids are also given to some patients with progressive disease. However, this
treatment is not indicated for patients who are stable between attacks. Chronic oral prednisone is
not used in multiple sclerosis, as there is no indication that its benefits outweigh its toxicity.
Azathioprine was used to treat relapsing multiple sclerosis in the past, but since the development
of interferon, it is used only as an investigational therapy for progressive multiple sclerosis.

Neurology:Question 73
The correct answer is D
Educational Objectives
Diagnose idiopathic intracranial hypertension.
Idiopathic intracranial hypertension most commonly occurs in obese young women and is usually
manifested by an unremitting headache; there may be associated visual obscurations. The
neurologic examination is typically normal except for papilledema and enlarged blind spots. Its
most serious long-term complication is vision loss. The elevated cerebrospinal fluid opening
pressure could be due to hydrocephalus, but this diagnosis is excluded by the normal findings on
magnetic resonance imaging of the brain. There is no spinal fluid pleocytosis, so meningitis is
unlikely in this patient. Chronic migraine is not associated with elevated cerebrospinal fluid
pressure and papilledema. The patient is not using medication at a frequency that would lead to
medication overuse (analgesic rebound) headache, nor is medication overuse headache associated
with evidence of increased intracranial pressure.

Neurology:Question 74
The correct answer is B
Educational Objectives
Recognize status epilepticus.
This patient‟s clinical presentation is consistent with partial status epilepticus. The patient has
been having complex partial seizures of right frontal lobe origin without recovery of baseline
mental status between seizures. The seizure in the hospital was probably a partial seizure with
secondary generalization due to her stroke. She requires urgent neurologic consultation and
urgent electroencephalography to confirm partial status epilepticus. The patient‟s neurologic
examination is consistent with the previous history of right hemispheric stroke. The worsening of
the left-sided weakness is more likely due to the frequent seizures (Todd‟s paralysis) than to
stroke in evolution. Phenytoin toxicity can worsen a previous neurologic deficit, but is unlikely to
cause more seizures. Creutzfeldt-Jakob disease is a subacute, degenerative disorder characterized
by rapidly progressive dementia, ataxia, and myoclonic jerks; it is unlikely in this patient.

Neurology:Question 75
The correct answer is A
Educational Objectives
Recognize medication-induced cognitive dysfunction.
The patient‟s altered level of consciousness and asterixis suggest a toxic/metabolic etiology of the
impairment. The patient is on several medications that might impair cognition. Nortriptyline has
anticholinergic properties, and is especially likely to cause impairment in patients with latent
cholinergic deficiency (for example, patients with early dementia or Parkinson‟s disease). The
sedative-hypnotic lorazepam and digoxin may also contribute to cognitive impairment.
Dementia with Lewy bodies is also associated with fluctuation of cognitive status and visual
hallucinations, but is less likely given the medication history, absence of parkinsonism, and
presence of asterixis. Vascular dementia, particularly that due to small-vessel cerebrovascular
disease, is unlikely given the absence of a stepwise course and absence of stroke on computed
tomography. Depression may cause chronic cognitive impairment (pseudodementia), but not
asterixis and an altered level of consciousness.

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