128. The most striking neurological complication of von Economo encephalitis (encephalitis lethargica), a type of encephalitis that occurred in epidemic proportions along with viral influenza between 1917 and 1928, was which of the following?
a. Blindness
b. Hearing loss
c. Paraplegia
d. Parkinsonism
e. Incontinence
129. A 37-year-old woman is noted to have lymphadenopathy on routine physical examination. Following an extensive evaluation, she is diagnosed with sarcoid. She has been entirely normal neurologically. Which cranial nerve (CN) is most likely to be injured in this patient?
a. II
b. III
c. V
d. VII
e. VIII
130. A 17-year-old girl presents initially with fever and progressive weakness. An extensive neurological evaluation including electromyography (EMG)/nerve conduction studies (NCS) suggests a motor neuron disease. The motor neuron disease most certainly traced to a virus is which of the following?
a. Poliomyelitis
b. Subacute sclerosing panencephalitis (SSPE)
c. Progressive multifocal leukoencephalopathy (PML)
d. Subacute human immunodeficiency virus (HIV) encephalomyelitis
e. Kuru
131. A 35-year-old woman who has received a liver transplant develops meningeal signs and fever. Cerebrospinal fluid (CSF) testing with India ink stain reveals a fungal infection. Which of the following is the cause of this patient’s fungal meningitis?
a. Aspergillus
b. Candida
c. Mucor
d. Cryptococcus
e. Rhizopus
132. A 28-year-old man who has recently immigrated from Brazil presents with 3 months of fluctuating but slowly progressive bilateral lower extremity weakness, a little worse on the left side than on the right. After a complete evaluation, a parasite is diagnosed as the etiology. This organism’s ova usually damage the nervous system at the level of which of the following?
a. Cerebrum
b. Cerebellum
c. Basal ganglia
d. Spinal cord
e. Peripheral nerves
133. A 12-year-old boy has left body weakness. A brain magnetic resonance imaging (MRI) scan reveals a polycystic lesion. The parasitic brain lesion most likely to have a large cyst containing numerous daughter cysts is that associated with which of the following?
a. Taenia solium
b. Schistosoma haematobium
c. Echinococcus granulosa
d. Diphyllobothrium latum
e. Schistosoma japonicum
134. An 82-year-old previously healthy woman with a recent upper respiratory infection presents with generalized weakness, headache, and blurry vision. For the past 2 weeks she has had upper respiratory symptoms that started with a sore throat, nasal congestion, and excessive coughing. She went to her primary care doctor 4 days ago and was diagnosed with sinusitis. She was given a prescription for an antibiotic and took it for 2 days, then stopped. She thereafter had chills, lightheadedness, vomiting, blurry vision, general achiness, and a headache that started abruptly and has not gotten better since. Except for blurry vision, she has not had any other visual symptoms. The blurry vision remains when she closes either eye. She also has eye tenderness with movement and mild photosensitivity. She has no drug allergies. Examination findings include temperature of 102.5°F (39.16°C), nuchal rigidity, and sleepiness. Which of the following is the next most appropriate action in this case?
a. Get a brain MRI, then perform a lumbar puncture.
b. Give the patient a prescription for oral azithromycin and let her go home.
c. Immediately give intravenous ceftriaxone plus ampicillin.
d. Immediately start intravenous acyclovir.
e. Obtain CSF and blood cultures and observe the patient until the results come back.
135. A 52-year-old generally healthy woman has had a gradual neurological deterioration over the past 6 to 8 months. It began with depression and a mild change in personality. Eventually she developed weakness and nonpurposeful movements of her left hand, as well as significant cognitive decline. All serologies were negative. MRI showed abnormal restricted diffusion in portions of the cortical gray matter and deep nuclei. Electroencephalography (EEG) had diffuse slowing and triphasic waves. Routine spinal fluid examination in this patient would be expected to show which of the following?
a. No abnormalities on routine studies
b. Elevated protein
c. More than 100 lymphocytes
d. More than 1000 red blood cells
e. Decreased glucose
136. A 17-year-old right-handed boy has had infectious meningitis eight times over the past 3 years. He has otherwise been generally healthy and developed normally. Recurrent meningitis often develops in persons with which of the following?
a. Otitis media
b. Epilepsy
c. Multiple sclerosis
d. Whipple disease
e. CSF leaks
137. An 82-year-old man with a history of pulmonary tuberculosis in 1947 presents with left body weakness and neglect. MRI shows a right frontal lesion, which is subsequently biopsied. The pathology suggests that the patient has recurrent tuberculosis. This mass lesion most likely consisted of which of the following?
a. Dysplastic central nervous system (CNS) tissue
b. Caseating granulomas
c. Heterotopias
d. Gram-positive bacteria
e. Mesial sclerosis
138. A 31-year-old homosexual man has had headache, sleepiness, and poor balance that have worsened over the past week. The patient is known to be HIV seropositive, but has done well in the past and has not seen a doctor in over 1 year. On examination, his responses are slow and he has some difficulty sustaining attention. He has a right hemiparesis with increased reflexes on the right. Routine cell counts and chemistries are normal. Which of the following is the most appropriate next step in management?
a. Head computed tomography (CT) with contrast
b. Noncontrast head CT
c. Perform a lumbar puncture
d. Start antiretroviral therapy
e. Start intravenous heparin
139. A 52-year-old woman with acquired immune deficiency syndrome (AIDS) presents to the emergency room with mild left hemiparesis and altered mental status. A CT scan reveals several rim-enhancing lesions with minimal mass effect. Which of the following is the best next step in management?
a. Get a cerebral angiogram.
b. Order a ventricular CSF aspiration.
c. Perform a lumbar puncture and include CSF for Epstein-Barr virus (EBV) PCR in tests ordered.
d. Stop all antiretroviral therapy.
e. Treat with intravenous acyclovir.
140. A 32-year-old intravenous drug abuser presents with more than 2 weeks of left body weakness. Brain CT scan reveals several ring-enhancing lesions, and an HIV test is positive. Serological, CSF, and MRI testing support the diagnosis of an obligate intracellular parasite. Which of the following is the best treatment for HIV associated with this opportunistic infection?
a. Intravenous acyclovir
b. Neurosurgical removal of the lesions
c. Oral fluconazole
d. Sulfadiazine and pyrimethamine
e. Thiabendazole
141. A 35-year-old woman has progressive numbness of the right arm and difficulty seeing objects in the right visual field. She is known to be HIV positive, but has not consistently taken medications in the past. On examination, she appears healthy, but has a right homonymous hemianopsia and decreased sensory perception in her right upper extremity and face. Her CD4 count is 75 cells per μL, and her MRI is consistent with a demyelinating lesion of the left parietooccipital area. CSF PCR for JC virus is positive. Which of the following is the most appropriate treatment in this case?
a. Amphotericin B
b. Cranial radiation
c. Highly active antiretroviral therapy (HAART)
d. Intravenous acyclovir
e. Intravenous ceftriaxone
142. A 72-year-old right-handed woman has 2 days of headache and fever, followed by worsening confusion. She is taken to the hospital after having a generalized seizure. A head CT is consistent with left temporal hemorrhage and swelling. Localization of encephalitis to the medial temporal or orbital frontal regions of the brain is most consistent with which of the following?
a. Treponema pallidum
b. Varicella zoster virus
c. Herpes simplex virus
d. Cryptococcus neoformans
e. Toxoplasma gondii
143. A 21-year-old college student was found walking around his dormitory naked. He is disoriented, inattentive, and shows poor comprehension. In the emergency room he is found to have a fever of 102°F (38.8°C). There are no apparent motor, sensory, or coordination abnormalities. The emergency room physician orders a brain MRI and then decides to perform a lumbar puncture. Neuroimaging of the brain before attempting a lumbar puncture is advisable in cases of acute encephalitis for which one of the following reasons?
a. The diagnosis may be evident on the basis of MRI alone.
b. Massive edema in the temporal lobe may make herniation imminent.
c. The CT picture may determine whether a brain biopsy should be obtained.
d. Shunting of the ventricles is usually indicated, and the imaging studies are needed to direct the placement of the shunt.
e. It may establish which pathology is responsible.
144. A 67-year-old man presents with headache, fever, disorientation, and seizures. CSF testing establishes that the patient has the most common form of acute encephalitis. The CSF changes late in the course of this disease typically include which of the following?
a. An increased number of lymphocytes
b. A glucose content of less than two-thirds the serum level
c. A protein content of less than 45 mg/dL
d. A normal opening pressure
e. A predominance of polymorphonuclear white blood cells (WBCs)
145. A 27-year-old man presents to his primary care doctor with a low-grade fever, headache, and neck stiffness, which have become more bothersome over the past 1-to-2 weeks. CSF and serological testing for Lyme disease is positive, and antibiotic treatment is initiated. The cranial neuropathy most commonly found with Lyme disease is that associated with damage to which CN?
a. III
b. V
c. VII
d. IX
e. XII
146. The pathologic specimen depicted here shows the only intracranial lesion found in this patient. This patient would be expected to exhibit which of the following symptoms?
a. Seizures
b. Gait ataxia
c. Hemiparesis
d. Visual loss
e. Hallucinations
147. A 13-year-old boy is brought into the emergency room lethargic with a stiff neck and fever. Despite aggressive therapy, the child dies. Postmortem evaluation reveals that the child had primary amebic meningoencephalitis. This condition is usually acquired through which of the following means?
a. Freshwater swimming
b. Eating contaminated meat
c. Eating calves’ brains
d. Anal intercourse
e. Animal bites
148. A 40-year-old man died from complications related to AIDS. Prior to his death, there had been a steady cognitive decline. Both HIV and cytomegalovirus infections in the brain characteristically produce which of the following?
a. Senile plaques
b. Intraneuronal amyloid
c. Intranuclear inclusions
d. Intracytoplasmic inclusions
e. Microglial nodules
149. Following several days of low-grade fever and mild neck and head pain, a 10-year-old boy develops bilateral face drooping and difficulty fully closing his eyes. Serum is positive for Borrelia burgdorferiIgM. CSF polymerase chain reaction (PCR) is also positive for this organism’s DNA. After B burgdorferi is introduced by the tick that carries it, the skin around the bite develops which of the following?
a. An exfoliative dermatitis
b. Purpura
c. Localized edema
d. Erythema chronicum migrans
e. Vesicular lesions
150. A 59-year-old right-handed woman has been clinically diagnosed with encephalitis. While CSF and MRI studies are pending, a medical student suggests ordering an EEG. Which of the following EEG findings is most associated with herpes encephalitis?
a. α Activity over the frontal regions
b. β Activity over the temporal regions
c. Three-per-second spike-and-wave discharges
d. Bilateral, periodic epileptiform discharges
e. Unilateral δ activity over the frontal region
151. A 9-year-old boy presents with bilateral CN VII deficits. Serum and CSF analysis suggests B burgdorferi is the etiology. Which of the following medications is the most appropriate treatment?
a. Streptomycin
b. Ceftriaxone
c. Gentamicin
d. Isoniazid
e. Rifampin
152. A 41-year-old homosexual man is brought to medical attention by his partner because of headache, sluggish mentation, and impaired ambulation worsening over the previous week. The patient is known to be HIV seropositive, but has done well in the past and has not sought regular medical attention. On examination, his responses are slow and he has some difficulty sustaining attention. He has a right hemiparesis with increased reflexes on the right. Routine cell counts and chemistries are normal. A contrast head CT reveals several ring-enhancing lesions. Eventually, surgical aspiration of one of the lesions reveals that they are abscesses. Abscesses in the brain most often develop from which of the following?
a. Hematogenous spread of infection
b. Penetrating head wounds
c. Superinfection of neoplastic foci
d. Dental trauma
e. Neurosurgical intervention
153. A 50-year-old woman presents to the emergency room with lethargy, fever, and moderately low blood pressure. She has a fever workup, is started on IV fluids and antibiotics, and is then admitted to the hospital. A diagnosis of bacterial endocarditis is made. On day 2 of her admission, she has developed a right upper extremity drift and her speech has decreased fluency. A head CT reveals a rim-enhancing lesion in the left frontal lobe. Which of the following is the most common site for formation of this type of lesion?
a. Putamen
b. Thalamus
c. Head of the caudate
d. Gray-white junction
e. Subthalamus
154. A 70-year-old man presents with right body (face and arm > leg) weakness, which he says has gradually developed over several months. Medical history includes hypertension, smoking, and the occasional use of prostitutes. RPR serological testing is positive. Which of the following consequences of this patient’s likely diagnosis may present a picture easily confused with brain tumor?
a. A reaction to penicillin treatment occurs.
b. An intracranial gumma forms.
c. Tabes dorsalis is the primary manifestation of the disease.
d. Meningovascular disease develops.
e. The patient is a newborn with congenital acquired disease.
155. A 9-year-old girl is playing in a wooded area of her backyard. She notices a furry animal in the brush. As it does not seem to fear her, she approaches to pet it. As soon as she touches the creature, it bites her and runs away. Her parents bring her to the emergency room for evaluation. The emergency room physician is extremely concerned that the patient may have been exposed to a deadly virus and orders immediate injections of immunoglobulin. From the brain, this pathogen establishes itself for transmission to another host by spreading to which of the following?
a. Intestines
b. Nasopharynx
c. Lungs
d. Bladder
e. Salivary glands
156. A 38-year-old man, who is immunocompromised because of HIV, presents with 1 month of worsening right headache, ear pain, and fever. He is determined to have malignant external otitis and osteomyelitis of the base of the skull. Culture of the lesion reveals a fungal etiology. What is the most likely causative organism?
a. Nocardia
b. Cryptococcus neoformans
c. Actinomyces
d. Aspergillus
e. Candida
157. A 55-year-old woman has progressive dementia over the past year. Within the past 3 months she has also developed dysarthria, myoclonus, intention tremor, and hyperreflexia. CSF VDRL is positive. This patient’s symptoms are being caused by which of the following?
a. A response to penicillin treatment
b. An autoimmune reaction
c. An acute meningoencephalitis
d. A chronic meningoencephalitis
e. A chronic rhombencephalitis
158. Which of the following is the most common cause of brain abscess in patients with AIDS?
a. Cryptococcus neoformans
b. Toxoplasma gondii
c. Tuberculosis
d. Cytomegalovirus
e. Herpes zoster
159. A 35-year-old woman is bitten by a small doglike wild animal while camping. The animal immediately runs away. Her skin is barely broken, and, besides feeling a little frightened, she says that she is fine. Despite this, her friend convinces her to be evaluated in the nearest emergency room. Which of the following viruses that typically invade the CNS by extending centripetally (ie, inward away from the periphery) along peripheral nerves is the woman most at risk for?
a. Mumps
b. Measles
c. Varicella zoster
d. Polio
e. Rabies
160. A 39-year-old man was treated 1 year ago for a brain abscess. He has largely recovered, but still has occasional word-finding difficulties. Which of the following is the most common symptom in patients with brain abscess?
a. Nausea and vomiting
b. Ataxia
c. Headache
d. Neck stiffness
e. Seizures
161. A 75-year-old left-handed woman presented to the emergency room with what at first was thought to be a stroke. History was significant for pneumonia 5 weeks ago. Following neuroimaging, the situation became less clear, and ultimately an enhancing brain lesion was aspirated via stereotaxic needle placement. Culture of the aspirate grew out bacteria. The most likely organism is which of the following?
a. Streptococcal
b. Staphylococcal
c. Bacteroides spp.
d. Proteus spp.
e. Pseudomonas spp.
162. A 52-year-old woman develops progressive dementia, tremors, gait ataxia, and myoclonic jerks over the course of 6 months. Her speech is slow and slurred, and hand movements are clumsy. No members of her immediate family have a history of degenerative neurological disease. MRI of the head reveals a subtle increase in T2 signal in the basal ganglia bilaterally. EEG reveals disorganized background activity with periodic sharp-wave discharges that occur repetitively at 1-second intervals and extend over both sides of the head. Arteriogram reveals no vascular abnormalities. The clinical picture is most consistent with which of the following?
a. Multi-infarct dementia
b. Tabes dorsalis
c. Friedreich disease (Friedreich ataxia)
d. Subarachnoid hemorrhage
e. Spongiform encephalopathy
163. A 91-year-old woman has 3 days of gradually worsening fever and headache. She then develops blurry vision and a stiff neck. Her granddaughter becomes concerned and brings her to the emergency room. MRI with contrast has an enhancement pattern suggesting rhombencephalitis. CSF shows a mild pleocytosis with no organisms. All blood and CSF cultures are negative. Which of the following medications is the best treatment for the organism likely responsible for the patient’s condition?
a. Penicillin G
b. Ampicillin plus gentamicin
c. Tetracycline
d. Ceftriaxone
e. Rifampin
164. A 51-year-old woman with an 8-month history of neurological decline dies after a severe bout of aspiration pneumonia. Autopsy of her brain reveals extensive loss of granule cells in the cerebellum and other changes most obvious in the cerebellar cortex. Fine vacuoles give the brain a spongiform appearance. No senile plaques are evident. The patient could have acquired this progressive disease through which of the following means?
a. Sexual intercourse
b. A blood transfusion
c. Consumption of raw fish
d. An upper respiratory infection
e. Growth hormone treatment
Questions 165 to 170
Select the condition that best fits each clinical scenario. Each lettered option may be used once, more than once, or not at all.
a. Subacute HIV encephalomyelitis (AIDS encephalopathy)
b. SSPE
c. PML
d. Rabies encephalitis
e. Guillain-Barré syndrome
f. Tabes dorsalis
g. Neurocysticercosis
h. Bartonella henselae encephalitis
i. HTLV-I infection
165. A 27-year-old man develops recurrent episodes of involuntary movements. He abused intravenous drugs for several years and has had several admissions for recurrent infections, including subacute bacterial endocarditis. His involuntary movements are largely restricted to the right side of his body and are associated with hoarseness and difficulty swallowing. The patient has lost 40 lb over the past 4 months. Examination reveals diffuse lymphadenopathy and right-sided hypertonia. His CSF is normal except for a slight increase in protein content. CT reveals a large area of decreased density on the left side of the cerebrum. EEG reveals diffuse slowing over the left side of the head. Biopsy of this lesion reveals oligodendrocytes with abnormally large nuclei that contain darkly staining inclusions. There is extensive demyelination, and there are giant astrocytes in the lesion. Over the course of 1 month, the man exhibits increasing ataxia. Within 2 months, he shows evidence of mild dementia and seizures. Within 3 months of presentation, his dementia is profound, and he has bladder and bowel incontinence. Over the course of a few days, he becomes obtunded and dies.
166. An 18-year-old man notices tingling about his ankles 2 weeks after an upper respiratory tract infection. Within 2 days, he has weakness in dorsiflexion of both feet, and within 1 week he develops problems with walking. He has no loss of bladder or bowel control. His weakness progresses rapidly over the ensuing week and necessitates his being placed on a ventilator to support his breathing. He is quadriplegic, but retains control of his eye movements. CSF studies reveal a protein content of greater than 1 g/dL with a normal white cell count. There are no red blood cells in the CSF.
167. Over the course of 6 months, a 50-year-old immigrant from Eastern Europe develops problems with bladder control, an unsteady gait, and pain in his legs. On examination, it is determined that he has absent deep tendon reflexes in his legs, markedly impaired vibration sense in his feet, and a positive Romberg sign. Despite his complaint of unsteady gait, he has no problems with rapid alternating movement of the feet, and no tremors are evident. He has normal leg strength. The pain in his legs is sharp, stabbing, and paroxysmal. His serum glucose and glycohemoglobin levels are normal.
168. A 10-year-old girl is referred to a physician because of rapidly deteriorating school performance. Over the course of a few weeks, the child has lost interest in her schoolwork, appeared apathetic at home, and had frequent temper tantrums with little provocation. A psychiatric evaluation reveals that, in addition to emotional lability, the child has substantial intellectual deficits that appear to be new. Within 1 month of this evaluation, the child has a generalized tonic-clonic seizure. A neurologist examining the child discovers chorioretinitis, ataxia, hyperactive reflexes, and bilateral Babinski signs. Her EEG exhibits periodic bursts of high-voltage slow waves followed by recurrent low-voltage stretches (burst suppression pattern). The CSF is remarkable for an increase in the γ-globulin fraction. The child becomes increasingly lethargic and obtunded over the ensuing 2 months. She remains in a coma for several months before dying.
169. A 37-year-old female navy officer presents with 3 days of confusion and seizures. Her colleagues report that she has been acting strangely for 3 days. This is followed by generalized status epilepticus. The woman has previously been well. She has traveled to the Caribbean several times annually, and she has a new pet cat. General examination discloses epitrochlear lymphadenopathy. Neurological examination shows the woman to be in status epilepticus. CSF is negative; MRI shows increased signal in the pulvinar bilaterally.
170. A 29-year-old immigrant from El Salvador is brought to the emergency room after a generalized seizure. After awakening, he relates that he has had two or three episodes of unexplained loss of consciousness in the past 2 years. He has otherwise been healthy. He served in the Salvadoran military for 3 years. His examination is normal. CT scan with contrast reveals two small hyperintense foci in the right frontal lobe, as well as a 1-cm cystic lesion with a nodular focus within it in the left frontal region. The cyst wall of the latter lesion enhances with contrast. The two right frontal lesions do not enhance.