Gerstmann-Straussler-Scheinker Disease


Disease, Gerstmann-Straussler

Diseases, Gerstmann-Straussler

Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type

Gerstmann Straussler Disease

Gerstmann Straussler Inherited Spongiform Encephalopathy

Gerstmann Straussler Scheinker Disease

Gerstmann Straussler Scheinker Syndrome

Gerstmann Straussler Syndrome

Gerstmann-Straussler Disease

Gerstmann-Straussler Diseases

Gerstmann-Straussler Inherited Spongiform Encephalopathy

Gerstmann-Straussler Syndrome

Gerstmann-Straussler-Scheinker Syndrome

Inherited Spongiform Encephalopathy, Gerstmann Straussler

Inherited Spongiform Encephalopathy, Gerstmann-Straussler

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)