Blepharoclonus and parkinsonism associated with aqueductal stenosis

A 28-year-old man with a history of congenital hydrocephalus due to aqueductal stenosis shunted at 45 days of age is presented. At age 4 years the valve had to be removed because of septicemia. Twenty-three years later he developed parkinsonian signs and abnormal, involuntary rhythmic contractions of the eyelids. The latter were elicited on gentle eye closure. Parkinsonism promptly improved after ventriculoperitoneal shunting, but blepharoclonus persisted unchanged.