Gerstmann-Straussler-Scheinker Disease D016098

Related MeSH Hierarchy (5)

Diseases [C] » Nervous System Diseases [C10] » Central Nervous System Diseases » Central Nervous System Infections » Prion Diseases » Gerstmann-Straussler-Scheinker Disease

Diseases [C] » Infections [C01] » Central Nervous System Infections » Prion Diseases » Gerstmann-Straussler-Scheinker Disease

Diseases [C] » Nervous System Diseases [C10] » Neurodegenerative Diseases » Heredodegenerative Disorders, Nervous System » Gerstmann-Straussler-Scheinker Disease

Diseases [C] » Nervous System Diseases [C10] » Neurodegenerative Diseases » Prion Diseases » Gerstmann-Straussler-Scheinker Disease

Diseases [C] » Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16] » Genetic Diseases, Inborn » Heredodegenerative Disorders, Nervous System » Gerstmann-Straussler-Scheinker Disease

Description

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)   MeSH

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