Other terms
Gerstmann Straussler Scheinker Disease,Inherited Spongiform Encephalopathy, Gerstmann-Straussler,Inherited Spongiform Encephalopathy, Gerstmann Straussler,Gerstmann-Straussler Syndrome,Gerstmann Straussler Syndrome,Gerstmann-Straussler-Scheinker Syndrome,Gerstmann Straussler Scheinker Syndrome,Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type,Gerstmann-Straussler Inherited Spongiform Encephalopathy,Gerstmann Straussler Inherited Spongiform Encephalopathy
Description
Gerstmann-Straussler-Scheinker Disease: 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)
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