Walsh & Hoyt: Gerstmann-Straussler-Scheinker (GSS) Disease

GerstmannStrausslerScheinker disease was the first inherited human prion disease described. As emphasized by by subsequent cases reported in the literature, GSS disease occurs in patients from approximately 2570 years of age, with a mean age of 48 years. It occurs in men and women with equal frequency. The duration of GSS disease varies from 2 to 10 years, with a mean duration of 5 years. The disease is characterized primarily by ataxia, dysarthria, myoclonus, and late-onset dementia, although a variant of the disease exists in which the initial manifestation is a slowly progressive spastic paraparesis. A peripheral neuropathy occurs in some patients.