|
Gerstmann–Sträussler–Scheinker syndrome (GSS) is a very rare, usually familial, fatal neurodegenerative disease that affects patients from 20 to 60 years in age. This extremely rare disease is classified as a transmissible spongiform encephalopathy (TSE). The exact incidence of GSS is unknown but is estimated to be between 1 and 10 per 100 million. Familial cases are associated with autosomal-dominant inheritance. Gerstmann-Straussler-Scheinker Disease (GSS) is an extremely rare neurogenetic brain disorder. It is always inherited and is found in only a few families all over the world (NINDS ¶ 1). The trait is an autosomal-dominant trait, caused by a gene mutation. It is also in a group of hereditary prion protein diseases or also known as TSEs. Many symptoms are associated with GSS, such as progressive ataxia, pyramidal signs, and even adult-onset dementia; they progress more as the disease progresses (Farlow, et al., Nov. 1989). ==Eponym== It is named after Josef Gerstmann, Ernst Sträussler and Ilya Scheinker. 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「Gerstmann–Sträussler–Scheinker syndrome」の詳細全文を読む スポンサード リンク
|