225. A 75-year-old woman with suspected normal-pressure hydrocephalus undergoes lumbar puncture. Forty milliliters of fluid are removed. Three hours later, she is able to walk unassisted and turns well. Spinal fluid would be expected to show which of the following?
a. No abnormalities
b. Elevated protein
c. Low protein
d. Atypical lymphocytes
e. Low glucose
226. A physician believes that her patient has Alzheimer disease. Which of the following is most characteristic of the brain in patients with Alzheimer disease?
a. Neuronal loss in the cerebral cortex
b. Demyelination in the cerebral cortex
c. Posterior column degeneration
d. Neuronal loss in the cerebellar cortex
e. Pigmentary degeneration in the hippocampus
227. An 80-year-old man has had a gradual memory decline over the past 10 years. A reversible cause of dementia cannot be found, and positron emission tomography scan supports the diagnosis of Alzheimer disease. In the dementia associated with Alzheimer disease, the electroencephalography (EEG) will usually show which of the following?
a. Spike-and-wave discharges
b. Periodic frontal lobe discharges
c. Focal slowing
d. Generalized background slowing
e. An isoelectric record
228. A 55-year-old man has a steep decline in his cognitive abilities over a 3-month period. Initial testing is nondiagnostic. He continues to progress and develops myoclonus and a left hemiparesis. Eventually, he dies of an aspiration about 8 months after the onset of symptoms. In the diseases that cause dementia, myoclonus is usually most evident in which of the following?
a. Alzheimer disease
b. Creutzfeldt-Jakob disease
c. Parkinson disease
d. Huntington disease
e. Pick disease
229. A 29-year-old mentally retarded woman living in an institution has had a subacute to chronic decline in memory. Testing for reversible causes of dementia is nondiagnostic. The brain of the adult with trisomy 21 (Down syndrome) exhibits many of the histopathologic features of which of the following?
a. Tay-Sachs disease
b. Friedreich disease
c. Pick disease
d. Parkinson disease
e. Alzheimer disease
230. An 80-year-old man has a history of 2 years of progressive gait disturbance and incontinence, which had been attributed to old age and prostatism. Within the past 3 months, he has been forgetful, confused, and withdrawn. His gait is short-stepped, and he turns very slowly, almost toppling over. He has a 30-year history of head trauma. His computed tomography (CT) scan is shown below. Which of the following is the most likely diagnosis?
a. Alzheimer disease
b. Creutzfeldt-Jakob disease
c. Progressive multifocal leukoencephalopathy
d. Normal-pressure hydrocephalus
e. Chiari malformation
231. An 82-year-old man has 6 months of worsening memory loss. His family is concerned, and he is taken to a physician. After an extensive evaluation and neuropsychological testing, he is diagnosed with dementia. Which of the following is the most common cause of dementia in the general population?
a. Epilepsy
b. Vascular disease
c. Alzheimer disease
d. Parkinson disease
e. Head trauma
232. A patient undergoes ventriculoperitoneal shunt placement for hydrocephalus. He is discharged 2 days later, his gait and cognition much improved. The following morning, his wife finds him lying in bed, very confused and complaining of a headache. He is unable to walk. The surgeon who performed the procedure is concerned that these new symptoms are owing to which of the following?
a. Chemical meningitis
b. Subdural hematoma
c. Epidural hematoma
d. Seizures
e. Bacterial ventriculitis
233. A 67-year-old man has a history of progressive memory loss for 2 years. His examination is otherwise normal. A diagnosis of Alzheimer disease is made. Which of the following medications may result in some cognitive improvement?
a. Donepezil
b. L-Dopa
c. Risperidone
d. Prednisone
e. Vitamin B12
234. Language testing is most likely to uncover which of the following deficits in a patient with Alzheimer disease?
a. No abnormalities
b. Mutism
c. Conduction aphasia
d. Transcortical sensory aphasia
e. Transcortical global aphasia
Questions 235 to 240
For each clinical scenario, choose the most likely diagnosis. Each lettered option may be used once, more than once, or not at all.
a. Transient global amnesia (TGA)
b. Normal-pressure hydrocephalus
c. Alzheimer disease
d. Parkinson disease
e. Creutzfeldt-Jakob disease
f. Vitamin B12 deficiency
g. Hypothyroidism
h. Huntington disease
i. Rett syndrome
j. Multi-infarct dementia
k. General paresis
l. Temporal lobe epilepsy
235. A 73-year-old man steps out of the shower on a Saturday evening and is unable to remember that he and his wife have tickets to a play. He asks her repeatedly, “Where are we going?” He appears bewildered, but is alert, knows his own name, speaks fluently, and has no motor deficits. He has no history of memory disturbance and after 8 hours returns to normal.
236. A 50-year-old woman began having double vision and blurry vision 3 months ago and has since had diminishing interaction with her family, a paucity of thought and expression, and unsteadiness of gait. Her whole body appears to jump in the presence of a loud noise. A magnetic resonance imaging (MRI) scan and routine cerebrospinal fluid (CSF) examination are unremarkable.
237. A 2-year-old girl developed normally until the past year. She has since become unable to speak or otherwise communicate with her parents, sits in a chair, and makes nearly continuous wringing movements with her hands. She also has episodes of breath holding alternating with hyperventilation.
238. A 17-year-old girl develops mild dementia, tremor, and rigidity. Her father died in his fourth decade of life of a progressive dementing illness associated with jerking (choreiform) limb movements. On exposure to L-dopa, she becomes acutely agitated and has jerking limb movements.
239. A 62-year-old man has had 2 years of progressive memory loss and inappropriate behavior. He has been delusional. More recently, he has developed tremors, myoclonus, dysarthria, and unsteadiness of gait. The CSF shows a lymphocytic pleocytosis, protein of 150, and positive VDRL.
240. A 44-year-old woman from Africa presents with inattentiveness, poor concentration, and lethargy. She has paranoid delusions. There is mild proximal weakness and ataxia. On general examination, she has edema, coarse and pale skin, and macroglossia. On reflex examination, she has delayed relaxation of the ankle reflexes.
Questions 241 to 243
For each patient, select the likely organism that caused the disease. Each lettered option may be used once, more than once, or not at all.
a. HTLV-I
b. Tropheryma whippeli
c. Treponema pallidum
d. JC virus
e. Prion protein
f. Cytomegalovirus
g. Herpes simplex virus
h. Taenia solium
241. A 54-year-old woman presents with 6 months of progressive memory loss. She has limited vertical eye movements, and on examination, she has rhythmic, synchronous grimacing and eye closure movements (oculomasticatory myorhythmia). Jejunal biopsy reveals periodic acid–Schiff (PAS)–positive cells.
242. A 35-year-old intravenous drug abuser presents with inability to control his left hand. He reports that at times he will button his shirt with his right hand, only to find that his left hand is unbuttoning the shirt against his control. He has a history of thrush. He is alert and oriented. MRI shows an increased T2 signal affecting the subcortical white matter of the right parietal lobe without enhancement.
243. A previously healthy 24-year-old man presents with 3 days of headaches and fever, followed by hallucinations, speech disturbance, and lethargy. He has a mild right hemiparesis. Spinal fluid is bloody, and MRI shows abnormal signal, with enhancement, in the left anterior temporal lobe.
244. A 78-year-old retired electrical engineer has had a progressive cognitive decline over the past 10-to-15 years. His wife reports that every 6-to-8 months she will notice another significant decrease in his functioning. It is now at the point where he is belligerent and has little short-term memory. There is a history of hypertension and cardiac stenting after a myocardial infarction at age 65. Examination findings include poor attention and memory, mild left hemiparesis (face, arm, and leg), and brisk reflexes throughout with upgoing toes. Which of the following is most likely to prevent further deterioration in this patient?
a. Deep brain stimulation of subthalamic nucleus
b. Control of hypertension
c. Ventriculoperitoneal shunt
d. Carbidopa/levodopa
e. Psychotherapy