| Gerstmann-Straussler-Scheinker syndrome | <syndrome> A familial spongiform encephalopathy. Transgenic mice with a mutant form of the PrP gene from patients with this syndrome develop degenerative brain disease that is similar, but not identical, to that caused by scrapie. It is a more chronic cerebellar form of spongiform encephalopathy, producing a neurodegenerative condition that has morphological similarities to Creutzfeldt-Jakob syndrome. However, in this syndrome there is slower progression, signs of spinocerebellar ataxia, and the spongiosis is less pronounced. Prion proteins and amyloid plaques are found in the brain of patients with the syndrome. A germline mutation of the prion protein has been demonstrated. (12 Jul 2000) |
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Synonyms : Gerstmann-Straussler Inherited Spongiform Encephalopathy, Inherited Spongiform Encephalopathy, Gerstmann-Straussler, Gerstmann Straussler Inherited Spongiform Encephalopathy, Gerstmann Straussler Scheinker Disease, Gerstmann Straussler Syndrome
| Gerstmann-Sträussler s. |
a group of rare prion diseases, of autosomal dominant inheritance but linked to different mutations of the prion protein gene, having the common characteristics of cognitive and motor disturbances and the presence of multicentric amyloid plaques in the brain. In the ataxic form, there are progressive cerebellar ataxia and dementia; in the telencephalic form, there are dysarthria, dementia, rigidity, tremor, and hyperreflexia; in GSS with neurofibrillary tangles, there are progressive short-term memory loss and clumsiness. Death occurs in 1 to 5 years.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| Gerstmann-Sträussler syndrome |
Gerstmann-Strässler-Scheinker syndrome.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| Gerstmann-Sträussler-Scheinker syndrome |
see under syndrome.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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제품명 |
판매사 |
보험코드 | 성분/함량 | 구분/보험급여 |
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