Gerstmann-Straussler-Scheinker Disease
"Gerstmann-Straussler-Scheinker Disease" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
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)
| Descriptor ID |
D016098
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| MeSH Number(s) |
C10.228.228.800.350 C10.574.500.425 C10.574.843.400 C16.320.400.350
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| Concept/Terms |
Gerstmann-Straussler-Scheinker Disease- Gerstmann-Straussler-Scheinker Disease
- Gerstmann Straussler Scheinker Disease
- Gerstmann-Straussler Inherited Spongiform Encephalopathy
- Gerstmann Straussler Inherited Spongiform Encephalopathy
- Gerstmann-Straussler Disease
- Disease, Gerstmann-Straussler
- Diseases, Gerstmann-Straussler
- Gerstmann Straussler Disease
- Gerstmann-Straussler Diseases
- Gerstmann-Straussler-Scheinker Syndrome
- Gerstmann Straussler Scheinker Syndrome
- Encephalopathy, Subacute Spongiform, Gerstmann-Straussler Type
- Gerstmann-Straussler Syndrome
- Gerstmann Straussler Syndrome
- Inherited Spongiform Encephalopathy, Gerstmann-Straussler
- Inherited Spongiform Encephalopathy, Gerstmann Straussler
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Below are MeSH descriptors whose meaning is more general than "Gerstmann-Straussler-Scheinker Disease".
Below are MeSH descriptors whose meaning is more specific than "Gerstmann-Straussler-Scheinker Disease".
This graph shows the total number of publications written about "Gerstmann-Straussler-Scheinker Disease" by people in this website by year, and whether "Gerstmann-Straussler-Scheinker Disease" was a major or minor topic of these publications.
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| Year | Major Topic | Minor Topic | Total |
|---|
| 1995 | 1 | 0 | 1 |
| 1996 | 1 | 0 | 1 |
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Below are the most recent publications written about "Gerstmann-Straussler-Scheinker Disease" by people in Profiles.
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Liberski PP, Yanagihara R, Brown P, Kordek R, Kloszewska I, Bratosiewicz J, Gajdusek DC. Microwave treatment enhances the immunostaining of amyloid deposits in both the transmissible and non-transmissible brain amyloidoses. Neurodegeneration. 1996 Mar; 5(1):95-9.
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Biernat W, Liberski PP, Guiroy DC, Yanagihara R, Gajdusek DC. Proliferating cell nuclear antigen immunohistochemistry in astrocytes in experimental Creutzfeldt-Jakob disease and in human kuru, Creutzfeldt-Jakob disease and Gerstmann-Str?ussler-Scheinker syndrome. Neurodegeneration. 1995 Jun; 4(2):195-201.
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Guiroy DC, Yanagihara R, Gajdusek DC. Localization of amyloidogenic proteins and sulfated glycosaminoglycans in nontransmissible and transmissible cerebral amyloidoses. Acta Neuropathol. 1991; 82(2):87-92.