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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… 
National Institutes of Health

Related topics

Related topics

33 relations
Aggressive behaviorApraxiasAtaxia, TruncalAutosomal dominant inheritance

Broader (2)

Prion DiseasesSlow Virus Diseases

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
2017
2017
Quantitative, functional MRI and neurophysiological markers in a case of Gerstmann-Sträussler-Scheinker syndrome.
Gerstmann-Sträussler-Scheinker syndrome (GSS) is an inherited autosomal dominant prion disease, caused by a codon 102 proline to… 
2016
2016
PiB PET in Gerstmann-Sträussler-Scheinker disease
Gerstmann-Sträussler-Scheinker Disease (GSS) is a familial neurodegenerative disorder characterized clinically by ataxia… 
2012
2012
A case report of Gorham–Stout syndrome remission
Abstract Gorham–Stout syndrome (GSS) is a rare disorder characterized by spontaneous bone resorption with rapid progression… 
2011
2011
Sialylated and O‐glycosidically linked glycans in prion protein deposits in a case of Gerstmann‐Sträussler‐Scheinker disease
Prion diseases are caused by an abnormal form of the prion protein (PrPSc). We identified, with lectins, post‐translational… 
2008
2008
臨床・画像的に，変異型Creutzfeldt-Jakob病との鑑別が問題となったGerstmann-Sträussler-Scheinker症候群の1例
症例は51歳の女性である．数年にわたる歩行障害・難治性下肢痛・小脳徴候の後，急速に進行する認知機能障害をみとめた．頭部MRIを施行したところ，拡散強調画像（DWI）・FLAIR画像にて皮質のリボン状高輝度をみとめた．Creutzfeldt-Jakob… 
2003
2003
Gerstmann-Straussler-Scheinker disease presenting as corticobasal degeneration.
1984
1984
[Subacute spongiform encephalopathy with Kuru-like plaques--comparison with Gerstmann-Sträussler-Scheinker disease].
1981
1981
Moyamoya disease in a patient with type I glycogenosis.
A 17-year-old boy with type I glycogen storage disease (GSD-I) and a stroke associated with moyamoya disease is described. This… 
1981
1981
[Morbus Gerstmann-Sträussler-Scheinker. The Sch. family-a report of three cases (author's transl)].
The clinical symptoms from three cases and histological findings from two patients suffering from morbus Gerstmann-Sträussler… 
Review
1976
Review
1976
Glycogen storage diseases.
Each of 12 types of glycogen storage disease (GSD O-XI) is delineated by clinical, biochemical and histologic features that allow…