我国Creutzfeldt-Jakob病的若干特殊性

Special characteristics of Creutzfeldt Jakob disease in China

目的通过本组资料，企图发现我国ＣｒｅｕｔｚｆｅｌｄｔＪａｋｏｂ病（ＣＪＤ）在临床与病理方面有不同于西方的若干特殊性。方法１５例经病理检查确诊的ＣＪＤ，６例进行免疫组化染色，７例进行鼠脑传递，５例进行ＰｒＰ基因检测。结果我国ＣＪＤ急性发病较多，急性发病者多表现抽搐或共济失调、病程短、脑萎缩不明显。结论我国ＣＪＤ确有不同于西方的若干特殊性，充分认识这些特殊性，对早期临床诊断，减少医源性传播有积极意义。

objective To investigate the differentiation in clinical aspects and pathological findings between the Chinese cases and the Western cases with Creutzfeldt Jakob disease (CJD). Methods Fifteen cases with CJD were confirmed with neuropathological examination. Six cases of them had immunohistological examinations. Seven cases were given animal transmisson. Five cases of them were gaven gene analysis. Results Compared with the Western cases of CJD, the following special characteristics could be found in Chinese cases: 1. the occurrence of acute onset was much more; 2. the initial symptoms of cases with acute onset usually were seizures and ataxia; 3. the durations of the disease were much shorter; and 4. brain atrophy was not so obvious. Conclusion There are some differences between the Chinese and the Western cases of CJD. It is important to understand them for ealy diagnosis and prevention of contamination.