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Washington University Experience | PRION DISEASES | Prion Diseases | 15A0 Case 15 History
Case 15 History ---- The patient was a 60 year old man who was evaluated in July of 1983 for dementia and pill rolling tremor present for 9 months with progressive difficulty with fine coordinated movements, and frequent falls. He frequently became lost while driving and occasionally got lost in his own home, confusing his home with a hospital. He forgot telephone numbers and names of his family members. There was no improvement with Artane, Sinemet and Bromocryptine. He lost 40 lbs and deteriorated rapidly over the next six months. An evaluation for an occult malignancy was negative. During the month prior to admission. he was unable to feed or dress himself, was incontinent of urine and had a marked loss of memory. Blood pressure was 110/70 and pulse 84 with no orthostatic changes. He appeared alert but was oriented to person only. Speech was slow and monotonous but with no evidence of aphasia. He was aware of his memory impairment and said "I'm losing my marbles". Eye movements were full and conjugate, and he had a mask like facies. The motor examination revealed a coarse, non-rhythmic tremor that was accentuated with action. There was no myoclonus. Strength was good and fine movements were deliberate and slow. The tendon reflexes were 2+ and symmetric and the toes down going. Grasp and snout reflexes were elicited. His gait was slow, hesitant and slightly wide based. An exhaustive workup revealed mild dilatation of the lateral, third and fourth ventricles compatible with atrophy. An awake EEG contained diffuse theta and delta slow activity with left temporal sharp waves. A chloral hydrate induced sleep EEG revealed no significant abnormalities. No improvement in his condition followed withdrawal of Sinemet, Bromocryptine and Artane. Because of the possibility of depression in addition to the dementia, he underwent a course of right unilateral electroconvulsive treatment without improvement. Brain biopsy was declined. His dementia progressed and he became confused, disoriented, agitated and experienced visual and auditory hallucinations. Major tranquilizers had a calming effect. He was transferred to the Veterans Administration Hospital for nursing care. He continued to deteriorate to a vegetative state. He died in December 1985, a bit more than two years of symptoms.
