Gerstmann-Strдussler syndrome

Gerstmann-Strдussler syndrome
Gerstmann-Strдussler-Scheinker syndrome (GSS) a group of rare prion diseases, of autosomal dominant inheritance but linked to different mutations of the prion protein gene, having the common characteristics of cognitive and motor disturbances and the presence of multicentric amyloid plaques in the brain. In the ataxic form, there are progressive cerebellar ataxia and dementia; in the telencephalic form, there are dysarthria, dementia, rigidity, tremor, and hyperreflexia; in GSS with neurofibrillary tangles, there are progressive short-term memory loss and clumsiness. Death occurs in 1 to 5 years.

Gerstmann-Strдussler syndrome; immunostaining of a section through the cerebellum with antibodies to prion protein demonstrates an amyloid plaque (arrow) in the molecular layer.

Medical dictionary. 2011.

Look at other dictionaries:

  • Gerstmann-Strдussler syndrome — Gerst·mann Strдus·sler syndrome (gerstґmahn shtroisґlər) [J. Gerstmann; E. Strдussler, Austrian physician, 20th century] Gerstmann Strдssler Scheinker syndrome …   Medical dictionary

  • Gerstmann-Strдussler-Scheinker syndrome — Gerst·mann Strдus·sler Schein·ker syndrome (gerstґmahn shtroisґlər shīnґkər) [J. Gerstmann; E. Strдussler; I. Scheinker, Austrian physician, 20th century] see under syndrome …   Medical dictionary

  • prion disease — any of a group of fatal, transmissible neurodegenerative diseases caused by abnormalities of prion protein metabolism, which may result from mutations in the prion protein gene or from infection with pathogenic isoforms of the protein (see prion) …   Medical dictionary

  • GSS — gamete shedding substance; General Social Survey; genome survey sequence; Gerstmann Strassler Scheinker [disease]; granulomatous slack skin; glutathione synthetase * * * Gerstmann Strдussler Scheinker syndrome …   Medical dictionary

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