245. A 19-year-old, left-handed woman has had several weeks of nausea, vomiting, and 8 lb of weight gain. She has also noticed the recent onset of an involuntary movement disorder that involves relatively rapid and fluid, but not rhythmic, limb and trunk movements. Which of the following is the most likely diagnosis?
a. Chorea gravidarum
b. Huntington chorea
c. Alzheimer disease
d. Multiple sclerosis
e. Amyotrophic lateral sclerosis
246. The influenza epidemic of 1918 to 1926 was associated with von Economo encephalitis and left many persons with a syndrome indistinguishable from which of the following?
a. Sydenham chorea
b. Alzheimer disease
c. Multiple sclerosis
d. Amyotrophic lateral sclerosis
e. Parkinson disease
247. A 43-year-old man has a father who died from Huntington disease. The son was tested and found to have the gene for Huntington disease. Which of the following is true regarding the offspring of those with Huntington disease?
a. Half the offspring are at risk only if the affected parent is male.
b. Half the offspring are at risk only if the affected parent is female.
c. Half the offspring are at risk if either parent is symptomatic for the disease before the age of 30.
d. Half the offspring are at risk for the disease.
e. One of four children is at risk for the disease.
248. A 42-year-old woman has a strong family history of neurological disease, dementia, and early death. Her father died at age 55 and she has been told that she has the defective gene, making her own fate inevitable should she live long enough. Within the past year, her personality has subtly changed toward increased irritability and she has begun to develop nonpurposeful slow, rhythmic movements of her hands and face. A magnetic resonance imaging (MRI) indicates atrophy in the head of the caudate nucleus. This MRI finding would be expected to affect the shape of which of the following?
a. Cerebellum
b. Lateral ventricle
c. Third ventricle
d. Lenticular nuclei
e. Temporal lobe
249. A 40-year-old man had undergone genetic testing several years ago for an autosomal dominant condition, which had afflicted members of his maternal family for several generations. The testing revealed he has the defective gene, and he now believes he is showing signs of the disease, including nonpurposeful movement of the extremities that are socially awkward and make daily activities more difficult. If this patient were to be exposed to L-dopa, which of the following would most likely be evoked?
a. Generalized seizures
b. Partial seizures
c. Intention tremor
d. Scanning speech
e. Writhing and jerking movements of the limbs
250. A 26-year-old heroin addict has been using a street version of artificial heroin. The drug actually contains 1-methyl-4-phenyl-1,2,3, 6-tetrahydropyridine (MPTP). The neurological syndrome for which he is at risk is clinically indistinguishable from which of the following?
a. Huntington disease
b. Friedreich disease
c. Sydenham chorea
d. Parkinson disease
e. Amyotrophic lateral sclerosis
251. A 61-year-old, right-handed man presents with involuntary twitches of his left hand. He first noticed between 6 months and 1 year ago that when he is at rest, his left hand shakes. He can stop the shaking by looking at his hand and concentrating. The shaking does not impair his activities in any way. He has no trouble holding a glass of water. There is no tremor in his right hand, and his lower extremities are not affected. He has had no trouble walking, and there have been no falls. There have been no behavioral or language changes. On examination, a tremor of the left hand is evident when the man is distracted. His handwriting is mildly tremulous. He has bilateral cogwheel rigidity with contralateral activation, which is worse on the left. His rapid alternating movements are bradykinetic on the left. Which of the following neurological structures is most likely dysfunctional?
a. Cerebral cortex
b. Peripheral nerves
c. Cerebral white matter
d. Brainstem nuclei
e. Cerebellum
252. A 67-year-old woman first began having a tremor 12 years ago. Within the subsequent 2 years, she clearly had developed what appeared to be Parkinson disease. The condition has now progressed to the point where, despite aggressive medical therapy, she cannot carry out her basic daily activities. High-frequency stimulation of which of the following brain structures is most likely to improve her symptoms?
a. Globus pallidus, medulla, parietal lobe
b. Globus pallidus, subthalamic nucleus, thalamus
c. Hippocampus, medulla, thalamus
d. Medulla, occipital lobe, subthalamic nucleus
e. Parietal lobe, temporal lobe, thalamus
253. A 78-year-old woman is referred to a neurologist for evaluation of a tremor. She says that it is not very bothersome to her, but others have noticed it. It primarily involves the right hand and apparently has been slowly worsening over the past 12-to-18 months. Examination reveals a resting tremor of the right upper extremity, accompanied by mild rigidity and slowness of rapid alternating movements. Which of the following medications is the best choice to treat the symptoms of this disease?
a. Alteplase
b. Carbidopa-levodopa
c. Glatiramer
d. Interferon β-1A
e. Sertraline
254. The pathophysiology of Parkinson disease is thought to involve dysfunction of the substantia nigra, including loss of neurons. Postmortem study of the substantia nigra of a patient with Parkinson disease is likely to exhibit which of the following?
a. Intranuclear inclusion bodies
b. Intranuclear and intracytoplasmic inclusion bodies
c. Intracytoplasmic inclusion bodies
d. Neurofibrillary tangles
e. Amyloid plaques
255. A 48-year-old female psychiatric patient has parkinsonism secondary to long-term neuroleptic use. Which of the following medications might minimize her parkinsonism?
a. Trihexyphenidyl
b. Haloperidol
c. Methamphetamine
d. Thioridazine
e. L-Dopa
256. A 70-year-old woman has 1 year of worsening gait, right-hand tremor, and rigidity. She is diagnosed with Parkinson disease and improves dramatically with treatment. If her disease progresses, the decrement in speech that would be expected would result in which of the following?
a. Progressively inaudible speech
b. Fluent aphasia
c. Nonfluent aphasia
d. Word salad
e. Neologisms
257. Even though the physiologic deficiency in Parkinson disease is of dopamine, L-dopa rather than dopamine is given to patients for which of the following reasons?
a. L-Dopa induces less nausea and vomiting than dopamine.
b. Dopamine is readily metabolized in the gastrointestinal tract to ineffective compounds.
c. L-Dopa is more readily absorbed in the gastrointestinal tract than is dopamine.
d. Dopamine cannot cross the blood–brain barrier and therefore has no therapeutic effect in the central nervous system (CNS).
e. L-Dopa is more effective at dopamine receptors than is dopamine itself.
258. A 25-year-old man has had motor tics since age 13. They seem to be getting worse, and now he also has involuntary obscene vocalizations. He may have largely normal behavior while being treated with which of the following?
a. L-Dopa
b. Trihexyphenidyl
c. Phenytoin
d. Carbamazepine
e. Haloperidol
259. A 72-year-old man presented 2 years ago with asymmetric rigidity, bradykinesia, and tremor. Since that time, disease progression appears to have been minimal. He is being treated with carbidopa and L-dopa. The role of carbidopa in this case is which of the following?
a. It has anticholinergic activity.
b. It has dopaminergic activity.
c. It is an antihistaminic.
d. It is an antiemetic.
e. It is a dopa decarboxylase inhibitor.
260. After several years of successful antiparkinsonian treatment, a patient abruptly develops acute episodes of profound bradykinesia and rigidity. Remission of these signs occurs as abruptly as the onset. Which of the following is the most likely etiology?
a. Acute dystonia
b. Absence attacks
c. On-off phenomenon
d. Complex partial seizures
e. Drug toxicity
Questions 261 to 264
For each clinical scenario, select the most likely condition. Each lettered option may be used once, more than once, or not at all.
a. Meige syndrome
b. Dopa-responsive dystonia
c. Parkinson disease
d. Olivopontocerebellar atrophy
e. Tardive dyskinesia
f. Spasmodic torticollis
g. Whipple disease
h. Hemifacial spasm
i. Essential tremor
261. A 53-year-old woman is unable to stop blinking forcefully and has frequent grimacing movements of the face. At times, she protrudes her tongue against her will. She has never taken any medications.
262. A 42-year-old woman has a long history of twisting movements of her head to the left. These are painful and have resulted over the years in muscular hypertrophy affecting the sternocleidomastoid and trapezius muscles. There is no family history. The remainder of her examination is normal.
263. A 40-year-old literary agent has had worsening tremor of the hands. This has been present for 2 years, but has increasingly impaired her work ability because she is frequently required to take her clients to lunch, and she is embarrassed by her inability to eat and drink normally. A glass of wine with the meal typically helps somewhat. On examination, there is a mild head tremor, but no rest tremor of the hands. When she holds a pen by the tip at arm’s length, however, a coarse tremor is readily apparent. Examination is otherwise normal.
264. A 64-year-old man has noticed dragging of the right leg and tremor and stiffness of the right hand. On examination, he has a tremor of the right hand, which disappears when he reaches to grab a pen. Movements are slower on the right than the left. He has cogwheel rigidity of the right arm.
Questions 265 to 269
For each clinical scenario, select the most likely diagnosis. Each lettered option may be used once, more than once, or not at all.
a. Hepatolenticular degeneration
b. Hyperparathyroidism
c. Central pontine myelinolysis
d. Akinetic mutism
e. MPTP poisoning
f. Locked-in syndrome
g. Postencephalitic parkinsonism
h. Neuroleptic effect
i. Essential tremor
j. Vegetative state
k. Hypermagnesemia
l. Rhombencephalitis
265. A 34-year-old man develops progressive depression and memory impairment over the course of 6 months. His initial neurological evaluation reveals a metabolic acidosis associated with his dementia. His liver is firm, and his spleen appears to be slightly enlarged. He has tremor and rigidity in his arms and walks with relatively little swing in his arms. His blink is substantially reduced, which gives him the appearance of staring. An MRI of the brain reveals some atrophy of the putamen and globus pallidus. His cerebrospinal fluid (CSF) is normal. His electroencephalography (EEG) is unremarkable.
266. A 19-year-old woman develops auditory hallucinations and persecutory delusions over the course of 3 days. She is hospitalized and started on haloperidol, 2 mg three times daily. Within 1 week of treatment, she develops stooped posture and a shuffling gait. Her head is slightly tremulous, and her movements are generally slowed. Her medication is changed to a very low dose of thioridazine, and trihexyphenidyl is added. Over the next 2 weeks, she became much more animated and reports no recurrence of her hallucinations.
267. A 65-year-old man develops slurred speech, difficulty swallowing, and labored breathing over the course of 30 minutes. When he arrives at the emergency room, he requires ventilatory assistance. His arms and legs are flaccid, and he exhibits no voluntary movements in any of his limbs. He is able to blink his eyes when instructed and appears to have completely intact comprehension of spoken and written language. An MRI reveals extensive infarction of the ventral pons. The basilar artery is not visible on magnetic resonance angiography (MRA).
268. A 72-year-old man requires bypass surgery to alleviate myocardial ischemia. During surgery, he has a massive myocardial infarct and protracted asystole. Resuscitative measures succeed in reestablishing a normal sinus rhythm, but postoperatively the patient remains unconscious after 48 hours. Over the ensuing weeks, the patient’s level of consciousness improves slightly. He appears awake at times, but does not interact in meaningful ways with visitors. He breathes independently and even swallows food when it is placed in his mouth, but he remains mute. With painful stimuli, he exhibits semipurposeful withdrawal of his limbs. His clinical status remains unchanged for several more months.
269. A 62-year-old man exhibits excessive sleepiness, slowing of movements, mild depression, and proximal muscle weakness. His proximal limb muscles are obviously atrophied. Although his blood count is normal, routine screening of serum chemistries reveals an elevated calcium level. He also has an elevated serum creatinine with reduced creatinine clearance. The patient has had abdominal discomfort intermittently for several months and has been told that his episodes of joint swelling were due to pseudogout.