Gerstmann-Straussler-Scheinker Disease

Known as: Gerstmann Straussler Scheinker Syndrome, Gerstmann-Straussler-Scheinker Syndrome, Gerstmann-Straussler Inherited Spongiform Encephalopathy 
An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal… (More)
National Institutes of Health

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1976-2017
0102019762017

Papers overview

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2003
2003
Prion protein (PrP) amyloid formation is a central feature of genetic and acquired forms of prion disease such as Gerstmann-Str… (More)
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2001
2001
BACKGROUND Gerstmann-Sträussler-Scheinker disease is a rare form of prion disease. OBJECTIVE To determine the prion mutation in… (More)
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Highly Cited
1998
Highly Cited
1998
Gerstmann-Sträussler-Scheinker disease (GSS), a cerebello-pyramidal syndrome associated with dementia and caused by mutations in… (More)
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Highly Cited
1998
Highly Cited
1998
The clinicopathological phenotype of the Gerstmann-Sträussler-Scheinker disease (GSS) variant linked to the codon 102 mutation in… (More)
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1996
1996
Gerstmann-Sträussler-Scheinker (GSS) disease is a cerebral prion protein (PrP) amyloidosis associated with mutations in the PrP… (More)
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1993
1993
Gerstmann-Sträussler-Scheinker disease in the Indiana kindred is pathologically characterized by deposits of PrP-amyloid… (More)
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Highly Cited
1992
Highly Cited
1992
Two families with Gerstmann-Sträussler-Scheinker disease (GSS) are atypical in possessing neocortical neurofibrillary tangles… (More)
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1992
1992
The Indiana kindred variant of Gerstmann-Sträussler-Scheinker disease has amyloid plaques that contain prion protein (PrP), but… (More)
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1992
1992
Gerstmann-Sträussler-Scheinker disease (GSS) is a familial neurological disorder pathologically characterized by amyloid… (More)
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1984
1984
The presence of complements Clq, C4, C3, C3b, C3c and C3d, as well as amyloid P component, in the amyloid plaques of Creutzfeldt… (More)
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