颅脑眼手术后的Creutzfeldt-Jakob病
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《中国神经精神疾病杂志》
2000年第4期
医源性Creutzfeldt-Jakob病|免疫组化|PrP基因,关键词：
林世和 赵节绪 江新梅 宋晓南 王为民 林世和 赵节绪 江新梅 宋晓南(白求恩医科大学第一临床医学院130021)；王为民(兰州医学院第二附属医院) 中国神经精神疾病杂志 2000 0 26 4
关键词：医源性Creutzfeldt-Jakob病；免疫组化；PrP基因
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【摘要】 目的 探讨医源性Creutzfeldt-Jakob病(CJD)的发病与手术间隔时间、临床表现、基因表达、异常朊蛋白(prion protein, PrP)沉积和实验动物传递。方法 颅脑眼手术后1～4年发生肌阵挛、痴呆并经脑活检证实的3例CJD病人，2例行PrP免疫组化染色，1例行PrP基因检测和实验动物传递。结果 2例脑内发现了异常PrP沉积，1例PrP基因密码子129为甲硫氨酸，缬氨酸杂合子型，实验鼠传递成功。结论 颅脑手术后CJD，其潜伏期明显短于眼部手术，PrP沉积均为突触型，即使129 M/V多态性的脑组织匀浆，通过昆明鼠传递也能获得成功。医疗实践中宜警惕本组疾病，防止医源性传播。
Craniocular postoperative Creutzfeldt-Jakob disease.
Lin Shihe Wang Weimin Zhao Jiexu Jiang Xinmei Song Xiaonan
( Department of Neurology, First Clinical Hospital, Bethunn Medical University, 1 Xinmin Street,Changchun, 130021. Tel:0431-5653532-2763 )
【 Abstract 】 Objective To study the relationshipamang iatrogenic Creutzfeldt-Jakob disease (CJD) and interval of operation, clinical manifestation, gene expression, abnormal PrP deposition and experimental animal transmission. Methods Three cases of CJD patients confirmed by biopsy with symptoms of myoclonus and dementia in one to four years after craniocular operation were studied. Two of the biopsies were treated with PrP immunohistochemistrystainning. Another was investigated for the presence of PrP gene and experimental animal transmission. Results Abnormal PrP deposition was found in two cases of brains. One case was codon 129 M/V. Experimental mice transmission of CJD was successful. Conclusions The interval between cranial operation and CJD was shorter than ocular operations. PrP deposition was all synapse type. Transmision to animal was successful in Kunming mice even selecting the brain tissue of 129 M/V polymorphism. In clinical practice, CJD should attract more attention to prevent iatrogenic spreading.
【 Key words 】 Iatrogenic Creutzfeldt-Jakob disease Immunohistochemistry PrP gene ▲
Creutzfeldt-Jakob病(CJD)与AIDS被认为是21世纪初的世界性两大顽疾 ......