| 单词 | Gerstmann Straussler Scheinker Disease |
| 释义 | Gerstmann Straussler Scheinker Disease《英文msh词典》Gerstmann Straussler Scheinker Disease [入口词] Gerstmann Straussler Scheinker Disease [主题词] Gerstmann-Straussler-Scheinker Disease [英文释义] An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA,spastic paraparesis,extrapyramidal signs,and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis,and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75) |
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