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

Topic mentions per year

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1976-2017
0102019762017

Related topics

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33 relations
Aggressive behaviorApraxiasAtaxia, TruncalAutosomal dominant inheritance
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Broader (2)

Prion DiseasesSlow Virus Diseases

Related mentions per year

Related mentions per year

1936-2019
19401960198020002020
Gerstmann-Straussler-Scheinker Disease
Muscle Rigidity
Parkinsonian Disorders
Nervous system structure
Psychotic Disorders
Brain

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2003
2003
Structural properties of Gerstmann-Straussler-Scheinker disease amyloid protein.
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
A new PRNP mutation (G131V) associated with Gerstmann-Sträussler-Scheinker disease.
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
Phenotypic variability of Gerstmann-Sträussler-Scheinker disease is associated with prion protein heterogeneity.
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
Different patterns of truncated prion protein fragments correlate with distinct phenotypes in P102L Gerstmann-Sträussler-Scheinker disease.
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
Proteinase-K-resistant prion protein isoforms in Gerstmann-Sträussler-Scheinker disease (Indiana kindred).
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
Beta PP participates in PrP-amyloid plaques of Gerstmann-Sträussler-Scheinker disease, Indiana kindred.
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
Mutant prion proteins in Gerstmann-Sträussler-Scheinker disease with neurofibrillary tangles
Two families with Gerstmann-Sträussler-Scheinker disease (GSS) are atypical in possessing neocortical neurofibrillary tangles… (More)
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1992
1992
Linkage of the Indiana kindred of Gerstmann-Sträussler-Scheinker disease to the prion protein gene
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
Prion protein preamyloid and amyloid deposits in Gerstmann-Sträussler-Scheinker disease, Indiana kindred.
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 in amyloid plaques of Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker disease.
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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