Spencer S. Eccles Health Sciences Library, University of Utah
Abstract
We present a case of Gerstmann-Straussler-Scheinker syndrome (GSS) presenting with vertical-gaze palsy who was initially thought to have spinocerebellar ataxia versus atypical parkinsonism. The few prior reports of eye movement abnormalities in GSS highlight early SWJs, supranuclear vertical gaze palsy, impaired vertical pursuits/OKN, and cerebellar oculomotor abnormalities (1,2). Our case emphasizes that in patients with progressive ataxia and supranuclear gaze palsy, a diagnosis of prion disease should be considered