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DIRECTIONS: Each item below contains a question or incomplete statement followed by suggested responses. Select the one best response to each question.

121. The most striking neurologic complication of von Economo's encephalitis (encephalitis lethargica), a type of encephalitis that occurred in epidemic proportions along with viral influenza between 1917 and 1928, was a. Blindness b. Hearing loss c. Paraplegia d. Parkinsonism e. Incontinence

Items 122-123

A 37-year-old woman is noted to have lymphadenopathy on routine physical exam. Following an extensive evaluation, she is diagnosed with sarcoid. She has been entirely normal neurologically.

122. The cranial nerve most likely to be injured in this patient is a. II

e. VIII

123. A 17-year-old female presents initially with fever and progressive weakness. An extensive neurological evaluation including EMG/NCS suggests a motor neuron disease. The motor neuron disease most certainly traced to a virus is a. Poliomyelitis b. Subacute sclerosing panencephalitis (SSPE)

c. Progressive multifocal leukoencephalopathy (PML)

d. Subacute HIV encephalomyelitis e. Kuru

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124. A 35-year-old woman who has received a liver transplant develops meningeal signs and fever. Cerebrospinal fluid testing reveals a fungal infection. The most common cause of fungal meningitis is a. Aspergillus b. Candida c. Mucor d. Cryptococcus e. Rhizopus

125. 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 than the right. After a complete evaluation, Schistosoma mansoni is diagnosed as the etiology. S. mansoni ova usually damage the nervous system at the level of the a. Cerebrum b. Cerebellum c. Basal ganglia d. Spinal cord e. Peripheral nerves

126. A 12-year-old boy has left body weakness. An MRI scan reveals a poly-cystic lesion. The parasitic brain lesion most likely to have a large cyst containing numerous daughter cysts is that associated with a. Taenia solium b. Schistosoma haematobium c. Taenia echinococcus d. Diphyllobothrium latum e. Schistosoma japonicum

Items 127-128

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. Other than 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. Exam findings include temperature of 102.5°F, nuchal rigidity, and sleepiness.

127. The next most appropriate action in this case is which of the following?

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 cerebrospinal fluid and blood cultures and observe the patient until the results come back

128. The next day the patient's spinal fluid cultures begin growing Listeria monocytogenes. The drug of choice in this case now is a. Penicillin G

b. Ampicillin plus gentamicin c. Tetracycline d. Ceftriaxone e. Rifampin

129. A 17-year-old right-handed boy has had infectious meningitis 8 times over the past 3 years. He has otherwise been generally healthy and developed normally. Recurrent meningitis often develops in persons with a. Otitis media b. Epilepsy c. Multiple sclerosis d. Whipple's disease e. Cerebrospinal fluid (CSF) leaks

130. An 82-year-old man with a history of pulmonary tuberculosis in 1947 presents with left body weakness and neglect. Imaging and subsequent biopsy reveal that recurrent tuberculosis was the cause. Mass lesions in the brain of the patient with tuberculosis may develop as a reaction to the tubercle bacillus and consist of a. Dysplastic central nervous system (CNS) tissue b. Caseating granulomas c. Heterotopias d. Colobomas e. Mesial sclerosis

Items 131-133

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.

131. Of the following, which is the most appropriate thing to do next?

a. Get a head CT with contrast b. Get a noncontrast head CT

c. Perform a lumbar puncture d. Start antiretroviral therapy e. Start intravenous heparin

132. A CT scan reveals several rim-enhancing lesions with minimal mass effect. An appropriate investigation at this point would be to a. Get a cerebral angiogram b. Order a ventricular cerebrospinal fluid (CSF) aspiration c. Perform a lumbar puncture and include cerebrospinal fluid for Epstein-Barr virus (EBV) PCR in tests ordered d. Stop all antiretroviral therapy e. Treat with intravenous acyclovir

133. The patient turns out to have CNS Toxoplasma gondii. Which of the following is the best treatment?

a. Intravenous acyclovir b. Neurosurgical removal of the lesions c. Oral fluconazole d. Sulfadiazine and pyrimethamine e. Thiabendazole

134. A 35-year-old female has progressive numbness of the right arm and difficulty seeing objects in the left visual field. She is known to be HIVpositive, 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 left upper extremity and face. Her CD4 count is 75 cells per ^L, and her MRI is consistent with a demylinat-ing 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

Items 135-138

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.

135. Localization of an encephalitis to the medial temporal or orbital frontal regions of the brain is most consistent with a. Treponema pallidum b. Varicella zoster virus c. Herpes simplex virus d. Cryptococcus neoformans e. Toxoplasma gondii

136. Neuroimaging of the brain before attempting a lumbar puncture is advisable in cases of acute encephalitis because a. The diagnosis may be evident on the basis of magnetic resonance imaging (MRI) alone b. Massive edema in the temporal lobe may make herniation imminent c. The computed tomography (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 what pathology is responsible

137. CSF testing establishes this case as being the commonest form of acute encephalitis. The CSF changes late in the course of this disease typically include 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

138. With this disease, EEG may exhibit a. a activity over the frontal regions b. P activity over the temporal regions c. Three-per-second spike-and-wave discharges d. Bilateral, periodic epileptiform discharges e. Unilateral S activity over the frontal region

Items 139-140

139. The pathologic specimen above shows the only intracranial lesion found in this patient. This patient would be expected to have exhibited which of the following symptoms?

a. Seizures b. Gait ataxia c. Hemiparesis d. Visual loss e. Hallucinations

140. If this patient had coincidental findings of oral moniliasis, diffuse lymphadenopathy, recent weight loss, and lymphopenia, the most probable cause of the lesion would be a. Ependymoma b. Medulloblastoma c. Granuloma d. Hemorrhage e. Infarction

141. 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 a. Freshwater swimming b. Eating contaminated meat c. Eating calves' brains d. Anal intercourse e. Animal bites

142. Both HIV and cytomegalovirus infections in the brain characteristically produce a. Senile plaques b. Intraneuronal amyloid c. Intranuclear inclusions d. Intracytoplasmic inclusions e. Microglial nodules

Items 143-145

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 burgdorferi IgM. CSF PCR is also positive for this organism's DNA.

143. After B. burgdorferi is introduced by the tick that carries it, the skin around the bite develops a. An exfoliative dermatitis b. Purpura c. Localized edema d. Erythema chronicum migrans e. Vesicular lesions

144. The cranial neuropathy most commonly found with Lyme disease is that associated with damage to cranial nerve a. III

145. The medication most appropriate in patients with CNS involvement by B. burgdorferi is a. Streptomycin b. Ceftriaxone c. Gentamicin d. Isoniazid e. Rifampin

Items 146-149

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 HIVseropositive, 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.

146. Abscesses in the brain most often develop from a. Hematogenous spread of infection b. Penetrating head wounds c. Superinfection of neoplastic foci d. Dental trauma e. Neurosurgical intervention

147. The most common site for abscess formation in the brain is the a. Putamen b. Thalamus c. Head of the caudate d. Gray-white junction e. Subthalamus

148. Most of the organisms found in brain abscesses are a. Streptococcal b. Staphylococcal c. Bacteroides spp.

d. Proteus spp.

e. Pseudomonas spp.

149. The most common cause of brain abscess in patients with AIDS is a. Cryptococcus neoformans b. Toxoplasma gondii c. Tuberculosis d. Cytomegalovirus e. Herpes zoster

150. The most common complaint in patients with brain abscess is a. Nausea and vomiting b. Ataxia c. Headache d. Neck stiffness e. Seizures

151. 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. The etiology is fungal. Fungal malignant external otitis and osteomyelitis of the base of the skull in HIV patients is most commonly caused by a. Nocardia b. Cryptococcus neoformans c. Actinomyces d. Aspergillus e. Candida

Items 152-153

A 55-year-old woman has a progressive dementia over the past year. Over the last 3 months she has also developed dysarthria, myoclonus, intention tremor, and hyperreflexia. CSF VDRL is positive.

152. 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

153. This patient's underlying disease may present a picture easily confused with brain tumor if a. A reaction to penicillin treatment occurs b. An intracranial gumma forms c. Tabes dorsalis is the primary manifestation of the disease d. Meningovascular syphilis develops e. The patient is a newborn with congenital syphilis

Items 154-156

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.

154. Which of the following viruses, which typically invades the CNS by extending centripetally (i.e., 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

155. From the brain, this virus establishes itself for transmission to another host by spreading to the a. Intestines b. Nasopharynx c. Lungs d. Bladder e. Salivary glands

156. The best therapy currently available for this disease is a. Supportive therapy b. Zidovudine c. Cytarabine d. Amantadine e. Ganciclovir

Items 157-159

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 neurologic disease. Magnetic resonance imaging (MRI) of the head reveals a subtle increase in signal in the basal ganglia bilaterally. EEG reveals disorganized background activity with periodic sharp-wave discharges that occur repetitively at 1-s intervals and extend over both sides of the head. Arteriogram reveals no vascular abnormalities.

157. The clinical picture is most consistent with a. Multi-infarct dementia b. Tabes dorsalis c. Friedreich's disease (Friedreich's ataxia)

d. Subarachnoid hemorrhage e. Spongiform encephalopathy

158. Routine spinal fluid examination in this patient would be expected to show a. No abnormalities on routine studies b. Elevated protein c. More than 100 lymphocytes d. More than 1000 red blood cells e. Decreased glucose

159. The patient 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 a. Sexual intercourse b. A blood transfusion c. Consumption of raw fish d. An upper respiratory infection e. Growth hormone treatment

DIRECTIONS: Each group of questions below consists of lettered options followed by a set of numbered items. For each numbered item, select the one lettered option with which it is most closely associated. Each lettered option may be used once, more than once, or not at all.

Items 160-165

Select the condition that best fits each clinical scenario.

a. Subacute HIV encephalomyelitis (AIDS encephalopathy)

b. Subacute sclerosing panencephalitis (SSPE)

c. Progressive multifocal leukoencephalopathy (PML)

d. Rabies encephalitis e. Guillain-Barre syndrome f. Tabes dorsalis g. Neurocysticercosis h. Bartonella henselae encephalitis i. HTLV-I infection

160. 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. Computed tomography 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. (SELECT 1 CONDITION)

161. 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. Cerebrospinal fluid 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 (SELECT 1 CONDITION)

162. 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. (SELECT 1 CONDITION)

163. 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 gamma globulin fraction. The child becomes increasingly lethargic and obtunded over the ensuing 2 months. She remains in a coma for several months before dying. (SELECT 1 CONDITION)

164. 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 exam discloses epitrochlear lym-phadenopathy. Neurologic exam shows the woman to be in status epilepti-cus. Cerebrospinal fluid is negative; MRI shows increased signal in the pulvinar bilaterally. (SELECT 1 CONDITION)

165. 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. Computed tomography 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. (SELECT 1 CONDITION)

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