论文部分内容阅读
目的探讨国人Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病（ＣＪＤ）的临床、病理及免疫组化、ＰｒＰ基因、１４－３－３蛋白及实验鼠传递结果。方法 统计２４例ＣＪＤ患者的临床资料，进行脑组织病理检查。其中１０例脑切片作ＰｒＰ免疫组化染色，１０例进行ＰｒＰ基因表达，５例脑脊液行１４－３－３蛋白检测，７例进行实验鼠传递。结果（１）２４例ＣＪＤ中散发１９例，可能为医源性３例，家族性１例，与Ａｌｚｈｅｉｍｅｒ病并存１例；（２）国人ＣＪＤ急性、亚急性发病高达９６％，急性发病者病程短，脑萎缩不明显；（３）脑组织石蜡切片以ＰｒＰ抗血清为第一抗体免疫组化染色，均呈突触型阳性；（４）１４－３－３蛋白表达对ＣＪＤ的临床诊断有特异性；（５）活检脑组织对实验鼠传递成功。结论 国人ＣＪＤ发病过程和临床表现有若干特殊性，通过１４－３－３蛋白表达，可早期确诊ＣＪＤ，其对早期发现ＣＪＤ、减少医源性传播有重要意义。
Objective To investigate the clinical, pathological and immunohistochemical characteristics of Creutzfeldt-Jakob disease (CJD) in Chinese and the results of PrP gene, 14-3-3 protein and experimental mice. Methods The clinical data of 24 CJD patients were collected for brain histopathological examination. PrP immunohistochemical staining was performed on 10 cases of brain slices, 10 cases of PrP gene expression, 5 cases of cerebrospinal fluid 14-3-3 protein detection, and 7 cases of experimental mice. Results (1) Of the 24 patients with CJD, 19 cases were emaciated, which may be iatrogenic in 3 cases and familial in 1 case, with Alzheimer’s disease coexisting in 1 case. (2) The acute and subacute morbidity of CJD is 96% Short, and atrophy of brain was not obvious. (3) The paraffin sections of brain tissue were stained with PrP antiserum as the first antibody and all showed synapse positive; (4) The clinical diagnosis of CJD Specificity; (5) Biopsy brain tissue was successfully delivered to mice. Conclusion The pathogenesis and clinical manifestations of CJD in Chinese people have some particularities. CJD can be diagnosed early by the expression of 14-3-3 protein, which is of great importance for early detection of CJD and reduction of iatrogenic transmission.