Gerstmann-Sträussler-Scheinker syndrome (redirected from Gerstmann-straussler-scheinker disease)

Gerstmann-Sträussler-Scheinker syndrome

[gerst′män shtrois′lershīn′ker]

a group of rare prion diseases, inherited as an autosomal-dominant trait but linked to different mutations of the prion protein gene. All forms of the syndrome have the common characteristics of cognitive and motor disturbances and the presence of numerous amyloid plaques in the brain. Three forms have been recognized: the ataxic form, which is accompanied by progressive cerebellar ataxia and dementia; the telencephalic form, which is accompanied by dysarthria, dementia, rigidity, tremor, and hyperreflexia; and Gerstmann-Sträussler-Scheinker syndrome with neurofibrillary tangles, in which there is progressive short-term memory loss and clumsiness. Death occurs in 1 to 5 years.