Abnormal eye movements in Gerstmann-Sträussler-Scheinker disease.

Gerstmann-Sträussler-Scheinker disease is a familial disorder of progressive ataxia and dementia in adulthood with extrapyramidal signs in some families. Neuro-ophthalmic examinations and eye movement recordings were performed in members of a large Indiana kindred. Five affected members and 11 members at risk were studied. Eye movements were recorded with videotape, electro-oculography, and/or magnetic scleral search coil. All affected members had abnormal eye movements characteristic of extrapyramidal diseases and cerebellar disorders. Nine members at risk had normal eye movements, but two others had slightly abnormal eye movements. Neuro-ophthalmic examination and eye movement recordings might be helpful in detecting early signs of Gerstmann-Sträussler-Scheinker disease in persons at risk.