An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)

Descriptor ID	D016098
MeSH Number(s)	C10.228.228.800.350 C10.574.500.425 C10.574.843.400 C16.320.400.350
Concept/Terms	Gerstmann-Straussler-Scheinker Disease Gerstmann-Straussler-Scheinker Disease Gerstmann Straussler Scheinker Disease Gerstmann-Straussler Inherited Spongiform Encephalopathy Gerstmann Straussler Inherited Spongiform Encephalopathy Gerstmann-Straussler Disease Disease, Gerstmann-Straussler Diseases, Gerstmann-Straussler Gerstmann Straussler Disease Gerstmann-Straussler Diseases Gerstmann-Straussler-Scheinker Syndrome Gerstmann Straussler Scheinker Syndrome Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type Gerstmann-Straussler Syndrome Gerstmann Straussler Syndrome Inherited Spongiform Encephalopathy, Gerstmann-Straussler Inherited Spongiform Encephalopathy, Gerstmann Straussler

Below are MeSH descriptors whose meaning is more general than "Gerstmann-Straussler-Scheinker Disease".

Below are MeSH descriptors whose meaning is related to "Gerstmann-Straussler-Scheinker Disease".

Below are MeSH descriptors whose meaning is more specific than "Gerstmann-Straussler-Scheinker Disease".