Abnormal eye movements in Gerstmann-Straussler-Scheinker disease
R. D. Yee, M. R. Farlow, D. A. Suzuki, K. F. Betelak and B. Ghetti
Department of Ophthalmology, Indiana University School of Medicine, Indianapolis 46202-5124.
Gerstmann-Straussler-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-Straussler-Scheinker disease in
persons at risk.
