- Concept UI
- M0024593
- 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)
- Terms
-
Gerstmann-Straussler-Scheinker Disease
Preferred Term
Term UI
T047840
Date12/11/1989
LexicalTag
EPO
ThesaurusID
-
Gerstmann-Straussler Inherited Spongiform Encephalopathy
Term UI
T368339
Date11/05/1999
LexicalTag
EPO
ThesaurusID
NLM (2000)
-
Gerstmann-Straussler Syndrome
Term UI
T047839
Date01/01/1999
LexicalTag
EPO
ThesaurusID
NLM (1991)
-
Gerstmann-Straussler-Scheinker Syndrome
Term UI
T751207
Date05/14/2009
LexicalTag
EPO
ThesaurusID
-
Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type
Term UI
T751208
Date05/14/2009
LexicalTag
EPO
ThesaurusID
-
Gerstmann-Straussler Disease
Term UI
T811555
Date11/15/2011
LexicalTag
NON
ThesaurusID
OMIM (2013)
-
Inherited Spongiform Encephalopathy, Gerstmann-Straussler
Term UI
T368340
Date11/05/1999
LexicalTag
EPO
ThesaurusID
NLM (2000)