中国克-亚病临床特点研究

Some distinguishing features of Cruetzfeldt Jakob disease in China

目的 探索中国人种克 亚病 (CJD)与其他国家的差异。方法 收集在中国境内发病的中国人CJD病例 41例 ,重点报告分析北京宣武医院的 6例 ,按CJD的经典诊断标准和程序分为病理确诊、临床诊断和高度疑诊三类 ,提供临床表现、影像学、组织病理和免疫组化等资料比较分析。结果中国CJD的发病和临床与其他国家比较有许多共性 ,但中国的病例病程较短 ,癫痫发作 (5 9% )和去皮质状态 (76 % )比较常见 ,脑萎缩表现比较少见。结论 中国的CJD与其他国家比较存在一定程度的差异 ,这些差异的出现可能与遗传和环境因素等有关。

Objective To find out the differences of Cruetzfeldt Jakob disease (CJD)between patients in China and other countries Methods 41 cases CJD patients of Han Nationality Chinese were collected The cases were defined as definite, probable (clinical diagnosis) and possible CJD according to the program and criteria of classical diagnosis Clinical manifestation, neuroimage information, histopathological and immunocytochemical data were collected Results The clinic characteristics of the present series were mostly similar to those reported in other countries, but the age of onset was younger and the duration of disease was shorter Signs and symptoms such as epileptic onset (seizures) (59%) and decerebration (76%) were more common in Chinese patients Brain atrophy was less frequentlly found in Chinese patients Conclusion There was some distinguishing features between CJD patients in China and other countries Genetic and environmental factors may be considered as the causes of the differences