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Gerstmann-Straussler-Scheinker Disease
Hierarchy: | ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease 32 coordinate concepts∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease 32 coordinate concepts∟ ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease 32 coordinate concepts∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease 32 coordinate concepts∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease 32 coordinate conceptsX |
Broader terms: | ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease ∟ ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease ∟ ∟ ∟ ∟ Gerstmann-Straussler-Scheinker Disease |
Annotation: | a prion dis: do not confuse with GERSTMANN SYNDROME, a type of agnosia
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History note: | Slow Virus Diseases (1988-1990)X |
historyNote*: | 2000(1991); use SLOW VIRUS DISEASES 1988-1990; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE was GERSTMANN-STRAUSSLER-SCHEINDER DISEASE see GERSTMANN-STRAUSSLER SYNDROME 1991
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publicMeSHNote*: | 2000; see GERSTMANN-STRAUSSLER SYNDROME 1991-1999; see SLOW VIRUS DISEASES 1988-1990; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE was GERSTMANN-STRAUSSLER-SCHEINDER DISEASE see GERSTMANN-STRAUSSLER SYNDROME 1991
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Scope note: | 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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activeMeSHYear*: | |
dateCreated*: | 1990-06-06X |
dateEstablished*: | 1991-01-01X |
dateRevised*: | 2001-07-25X |
recordAuthorizer*: | sjnX |
recordMaintainer*: | nnsX |
recordOriginator*: | AGSX |
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Labels and equivalent concepts: | Gerstmann-Straussler-Scheinkerin oireyhtymä (fi) XGerstmann-Strausslerin syndrooma (fi, replaced) Gerstmann-Strausslerin oireyhtymä (fi, replaced) Gerstmann-Straussler-Scheinkerin syndrooma (fi, replaced) Gerstmann-Straussler-Scheinkers sjukdom (sv) Gerstmann-Straussler Syndrome (en, replaced) Inherited Spongiform Encephalopathy, Gerstmann-Straussler (en, replaced) Gerstmann-Straussler Inherited Spongiform Encephalopathy (en, replaced) Syndroma Gerstmann-Straussler (la-FI, replaced) Syndroma Gerstmann-Straussler-Scheinker (la-FI, replaced) |
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