5Neurology
1.
A boy is found to have patches of retinal pigmentation after he is brought to clinical attention because of several episodes of seizures. He subsequently is diagnosed with Aicardi syndrome following an MRI of the brain and cervical spine. What abnormality was seen on imaging to prompt the diagnosis of Aicardi syndrome?
A. Absence of the septum pellucidum
B. Dandy-Walker malformation
C. Fusion of the cervical vertebrae
D. Agenesis of the corpus callosum
E. Chiari type 3 malformation
2.
A positive Romberg sign indicates a disturbance of what structure(s)?
A. Posterior columns
B. Cerebellar vermis
C. Lateral geniculate body
D. Cerebellar hemisphere
E. Corticospinal tract
3.
A 7-year-old boy presents to the emergency department after suffering a first-time seizure at home. His mother says that he had been febrile recently and seemingly slightly confused. On exam, the child is lethargic without focal deficits. Mild papilledema is seen on funduscopic examination. Routine laboratory studies are unremarkable, but a lumbar puncture reveals cerebrospinal fluid with leukocytosis and an elevated protein content. Multiple cultures are sent to the lab. An MRI with FLAIR sequence reveals an abnormality within the left temporal lobe. Electroencephalography demonstrates periodic lateralizing epileptiform discharges from the left temporal lobe. What are the most likely diagnosis and next step in the patient’s management?
A. Varicella zoster encephalitis; begin intravenous acyclovir
B. Varicella zoster encephalitis; await culture results before initiating treatment
C. Herpes simplex encephalitis; begin intravenous acyclovir
D. Herpes simplex encephalitis; await culture results before initiating treatment
4.
Apert syndrome is distinguished from Crouzon syndrome as individuals with Apert, and not Crouzon, syndrome tend to have:
A. Absence of a cleft palate
B. Normal intelligence
C. Mental retardation
D. Complex syndactyly
E. Craniosynostosis
5.
Damage to what neural structure(s) is most associated with prosopagnosia?
A. Mid-fusiform gyrus and inferior occipital gyrus
B. Bilateral amygdalae
C. Mesial temporal lobe
D. Calcarine sulcus and dominant angular gyrus
E. Superior parietal lobule and supramarginal gyrus
6.
A 62-year-old man presents with masked facies without tremor. His family members report that over the past year, the patient had multiple falls, dysarthria, and dysphagia. They report he has had some personality changes. On exam, he has a hyperactive jaw jerk, axial dystonia, and down-gaze paresis. You suspect the patient has what condition?
A. Parkinson disease
B. Olivopontocerebellar degeneration
C. Progressive supranuclear palsy
D. Multiple system atrophy
7.
Bilateral periodic lateralizing epileptiform discharges (PLEDs) that are not reactive to painful stimulation are suggestive of what condition?
A. Subacute sclerosing panencephalitis
B. Creutzfeldt-Jakob disease
C. Herpes simplex encephalitis
D. Hepatic encephalopathy
8.
Transcranial Doppler can detect changes in flow velocities in the cerebral arteries. Vasospasm is differentiated from a hyperemic state by:
A. A mean flow velocity greater than 120 cm/s, indicating vasospasm
B. Measuring the middle cerebral to internal carotid artery velocity ratio
C. Checking for flow symmetry on the contralateral side
D. Measuring the velocity/vessel diameter ratio
E. Determining the direction of flow
9.
What involuntary movement disorder typically persists during sleep?
A. Hemifacial spasm
B. Blepharospasm
C. Athetosis
D. Facial myokymia
10.
The Cantu system for concussion grading uses what three variables to determine severity?
A. Loss of consciousness, transient confusion, and symptom duration
B. Loss of consciousness, posttraumatic amnesia, and neurologic deficits
C. Eye opening, motor response, and verbal response
D. Loss of consciousness, posttraumatic amnesia, and symptom duration
11.
A 49-year-old man presents with left elbow pain. His symptoms have not improved with rest, ice, splinting, and analgesics. He has noticed progressive weakness in extension of his thumb and fingers without weakness of his wrist or arm. There is no sensory loss. What is the suspected diagnosis?
A. Radial nerve neuropathy in the upper arm
B. Tennis elbow
C. Posterior interosseous neuropathy
D. C8 radiculopathy
E. Radial tunnel syndrome
12.
A 28-year-old woman presents to the emergency room with a severe orbital headache. What findings would support a diagnosis of spontaneous internal carotid artery dissection?
A. Complete Horner syndrome with hemisensory loss, dysphagia, ataxia, and vertigo
B. Complete Horner syndrome with shoulder and arm pain
C. Complete Horner syndrome with neck pain and delayed contralateral weakness and sensory loss
D. Incomplete Horner syndrome with neck pain and delayed contralateral weakness and sensory loss
E. Mydriasis with contralateral weakness and sensory loss
13.
A 15-year-old boy presents with significant discharge from the left ear with severe headache, facial pain, and diplopia. On exam, he is noted to have a left-sided lateral gaze palsy. What is the patient’s suspected diagnosis?
A. Venous sinus thrombosis
B. Tolosa-Hunt syndrome
C. Raeder paratrigeminal neuralgia
D. Gradenigo syndrome
E. Ramsay-Hunt syndrome
14.
A patient has a cerebellar lesion causing symptoms including a wide and staggering gait with little impairment of arm or hand movement. What is the likely location of such a lesion?
A. Vestibulocerebellum
B. Cerebellar tonsils
C. Cerebrocerebellum
D. Spinocerebellum
E. Dentate nucleus
15.
What is the Wartenberg sign?
A. Wasting of the interossei muscles in the hand
B. Grasping a sheet of paper resulting in extension of the proximal phalanx and flexion of the distal phalanx
C. Hyperextension at the metacarpophalangeal joints and flexion at the interphalangeal joints in digits four, five, and partially three
D. Abduction of fifth digit due to weakness of the third palmar interosseous muscle
E. Sensory loss over the hypothenar eminence, fifth finger, and ulnar half of the fourth finger on the palmar aspect only
16.
A 53-year-old man with a heavy smoking history comes to the office complaining of right upper extremity pain and weakness. Physical examination reveals a small right pupil and right eye ptosis. What disease process likely explains this finding?
A. Perineural spread of metastatic disease
B. Tumor compression of the stellate ganglion
C. Cerebral metastatic tumor compressing the hypothalamus
D. Spinal metastatic disease
17.
An 8-year-old girl is brought to the emergency room by her grandmother after 1 week of fever and lethargy and a 2-day history of progressive rightsided weakness. A head CT is normal, and an MRI with FLAIR sequence of the brain and spine demonstrates a subtle abnormality within the subcortical white matter. Cerebrospinal fluid studies show an elevated white blood cell and protein count. Further studies are pending. What is the most likely diagnosis?
A. Juvenile-onset multiple sclerosis
B. Progressive multifocal leukoencephalopathy
C. Adrenoleukodystrophy
D. Acute disseminated encephalomyelitis
18.
Fill in the blanks.
Korsakoff psychosis results from deficiency of ______________ (nutrient), is ______________ (reversible/irreversible), and affects the ______________ (anatomic structure).
A. Folate; irreversible; medial dorsal thalamic nuclei
B. Folate; reversible; mammillary bodies
C. Thiamine; irreversible; medial dorsal thalamic nuclei
D. Thiamine; reversible; mammillary bodies
E. Thiamine; irreversible; cerebellum
19.
The nurse calls you to evaluate a recently admitted patient with Guillain-Barré syndrome. The patient initially had only lower extremity weakness but now has a weak quality of voice and is taking shallow, rapid breaths. What is the next most appropriate step in this patient’s management?
A. Initiate plasmapheresis.
B. Give intravenous dexamethasone.
C. Intubate the patient.
D. Give intravenous immunoglobulin.
20.
A 72-year-old woman was admitted to the hospital for severe vertigo of 1 day’s duration. She subsequently developed nausea, vomiting, dysphagia, hoarseness, and loss of left facial pain sensation. The patient is alert and oriented to time, space, and person. She has no motor deficits but does have ptosis on the left and clumsiness of the left arm. The patient also has decreased proprioception in the left foot and loss of pain and temperature sensation on the right side. The patient’s MRI demonstrates an ischemic stroke. What is the most likely localization of the stroke?
A. Right lateral medulla
B. Left lateral medulla
C. Right medial medulla
D. Left medial medulla
E. Right and left lateral medulla
21.
An 81-year-old man with a history of hip replacement 3 weeks ago complicated by a mild myocardial infarction is brought to the hospital with an acute-onset middle cerebral artery stroke. The patient has been maintained on warfarin, and the INR is 2.3. The platelet count is 120,000. In this patient, what is an absolute contraindication to thrombolysis?
A. Age over 80 years
B. Hip replacement within 3 weeks of presentation
C. Recent history of myocardial infarction
D. INR of 2.3
E. Platelets count of 120,000
22.
A 3-year-old boy who was previously healthy develops a red, scaly rash over his face, neck, hand, and legs. He then starts having emotional liability and episodic cerebellar ataxia, and eventually experiences developmental delay. After being worked up, he is treated with high doses of nicotinamide. What is the patient’s diagnosis?
A. Hartnup disease
B. Phenylketonuria
C. Homocystinuria
D. G6PD deficiency
E. Tetrahydrobiopterin deficiency
23.
A 48-year-old woman presents with a sudden onset of blurred vision and pain on movement of her right eye. She reports that 2 years ago, she had loss of sensation in the hands that progressed over a couple of weeks to motor involvement limiting her ability to write with the left hand. At the time, she was afraid to seek medical attention. What is the most likely diagnosis?
A. Multiple sclerosis
B. Guillain-Barré syndrome
C. Transient ischemic attack
D. Myasthenia gravis
E. Amyotrophic lateral sclerosis
24.
A 30-year-old man with a history of homocystinuria develops a headache followed by hemiparesis and focal epilepsy. He is brought to the emergency room for evaluation. What is the most sensitive imaging modality to detect this patient disease?
A. Noncontrast CT scan
B. T2-weighted MRI
C. Angiogram
D. Whole body PET CT
E. Perfusion-weighted imaging
25.
When examining a man, you notice that he has difficulty maintaining balance when his feet are together and his eyes are closed. He also struggles to rub accurately the heel of one foot along the shin of the opposite leg. What system is most likely malfunctioning?
A. Vision
B. Proprioception
C. Vestibular system
D. Cerebellum
26.
A retired boxer with a history of more than 20 bouts comes to your office for evaluation of “abnormal behavior” reported by the family. On exam, the patient has a resting tremor, decreased coordination, poor attention span, no focal weakness, and subtle paranoia upon questioning. You suspect chronic traumatic encephalopathy. What would you expect to find on pathological examination of his cerebral tissue?
A. Normal cerebral architecture
B. Loss of pigmentation in the substantia nigra
C. Caudate atrophy
D. Deposition of β-amyloid plaques
27.
What is the most effective mean of aborting a cluster headache?
A. Inhaled 100% O2
B. Sublingual nitroglycerin
C. Ergotamine
D. Methysergide
E. Intravenous nonsteroidal anti-inflammatory agents
28.
