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Gerstmann-Straussler-Scheinker Disease MeSH Descriptor Data 2022


MeSH Heading
Gerstmann-Straussler-Scheinker Disease
Tree Number(s)
C01.207.800.350
C10.228.228.800.350
C10.574.500.425
C10.574.843.400
C16.320.400.350
Unique ID
D016098
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D016098
Annotation
a prion dis: do not confuse with GERSTMANN SYNDROME, a type of agnosia
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)
Entry Version
GERSTMANN STRAUSSLER SCHEINKER DIS
Entry Term(s)
Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type
Gerstmann-Straussler Disease
Gerstmann-Straussler Inherited Spongiform Encephalopathy
Gerstmann-Straussler Syndrome
Gerstmann-Straussler-Scheinker Syndrome
Inherited Spongiform Encephalopathy, Gerstmann-Straussler
Previous Indexing
Slow Virus Diseases (1988-1990)
Public MeSH Note
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
History Note
2000(1991); use SLOW VIRUS DISEASES 1988-1990; GERSTMANN-STRAUSSLER-SCHEINKER DISEASE was GERSTMANN-STRAUSSLER-SCHEINDER DISEASE see GERSTMANN-STRAUSSLER SYNDROME 1991
Date Established
1991/01/01
Date of Entry
1990/06/06
Revision Date
2019/06/17
Gerstmann-Straussler-Scheinker Disease Preferred
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