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Disease Gerstmann-Straussler-Scheinker Disease

Name Gerstmann-Straussler-Scheinker Disease
Synonyms AMYLOIDOSIS, CEREBRAL, WITH SPONGIFORM ENCEPHALOPATHY | CEREBELLAR ATAXIA, PROGRESSIVE DEMENTIA, AND AMYLOID DEPOSITS IN CNS | Disease, Gerstmann-Straussler | Diseases, Gerstmann-Straussler | Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type | Gerstmann Straussler Disease | Gerstmann-Straussler Disease | Gerstmann-Straussler Diseases | Gerstmann Straussler Inherited Spongiform Encephalopathy | Gerstmann-Straussler Inherited Spongiform Encephalopathy | Gerstmann Straussler Scheinker Disease | GERSTMANN-STRAUSSLER-SCHEINKER DISEASE | Gerstmann Straussler Scheinker Syndrome | Gerstmann-Straussler-Scheinker Syndrome | Gerstmann Straussler Syndrome | Gerstmann-Straussler Syndrome | GSD | GSS | Inherited Spongiform Encephalopathy, Gerstmann Straussler | Inherited Spongiform Encephalopathy, Gerstmann-Straussler | PRION DEMENTIA CEREBRAL AMYLOID ANGIOPATHY, PRNP-RELATED, INCLUDED
Definition 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)
Categories Genetic disease (inborn) | Nervous system disease
MeSH® ID D016098
OMIM® ID 137440
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1. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Central Nervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Central Nervous System Infections Has associated chemicals Has associated genes Prion Diseases Has associated chemicals Has associated genes Gerstmann-Straussler-Scheinker Disease Has associated chemicals Has associated genes
2. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Neurodegenerative Diseases Has associated chemicals Has associated genes Has associated exposure references Heredodegenerative Disorders, Nervous System Has associated chemicals Has associated genes Gerstmann-Straussler-Scheinker Disease Has associated chemicals Has associated genes
3. DiseasesNervous System Diseases Has associated chemicals Has associated genes Has associated exposure references Neurodegenerative Diseases Has associated chemicals Has associated genes Has associated exposure references Prion Diseases Has associated chemicals Has associated genes Gerstmann-Straussler-Scheinker Disease Has associated chemicals Has associated genes
4. DiseasesCongenital, Hereditary, and Neonatal Diseases and Abnormalities Has associated chemicals Has associated genes Has associated exposure references Genetic Diseases, Inborn Has associated chemicals Has associated genes Has associated exposure references Heredodegenerative Disorders, Nervous System Has associated chemicals Has associated genes Gerstmann-Straussler-Scheinker Disease Has associated chemicals Has associated genes

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None.