The patient is a right-handed man who had been a bank executive. He claimed to have been in good health until September 1985, when at the age of 39 he developed a fever to 104°F and was hospitalized. During the hospitalization he was said to have been very lethargic and having problems with calculations and memory. He developed abnormal involuntary movements of his right arm at that time, which were described as "masturbatory," as well as total body slowness and slurred speech.Laboratory tests were significant, with a platelet count of 122,000, a cerebrospinal fluid (CSF) protein level of 71 mg/dl, and elevated liver function tests. An electroencephalogram (EEG) was normal. Computed tomography (CT) scan of the brain showed bilateral areas of decreased density adjacent to the thalami, with periventricular extension of the low density areas to the posterior horns of the lateral ventricle (Fig. 1). A brain scan was normal, and he was treated with Acyclovir for presumptive herpes encephalitis. Over the next 10 days, his level of consciousness improved although he did not return to his baseline mental status, and the abnormal movements of his right arm, although decreased, never fully disappeared.In March 1986 the same movements began to increase in amplitude and in June 1986 the same movements appeared in the left hand. The hand movements interfered with his ability to eat, shave, or dress himself, and he was completely unable to write. In November 1986 he noted a mild tremor of his head and in the beginning of January 1987 he noted a tremor in the right foot when he lifted it off the ground. Other new symptoms were postural instability with a tendency to retropulse, progressively stooped posture when he walked, slurred speech, a blank facial expression, and nocturnal drooling. He also had difficulties with short term memory. Toward the end of January, the tremor in the right foot increased and he developed a tremor in the left foot. Trials of trihexyphenidyl, haloperidol, propranolol, levodopa, amantadine, and primidone were unsuccessful.Case report and videotape were submitted by Heidi Shale and Stanley Fahn.
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