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克雅病(Creutzfeldt-Jakob disease,CJD)是一种由朊蛋白感染引起的进展性疾病,表现为快速进展的痴呆、共济失调、肌阵挛和精神症状等,好发于50～70岁人群,感染途径包括受损皮肤黏膜接触含朊蛋白的血液或分泌物,进食患病动物制品,注射含病原体的生物制品或使用病原体污染的手术器械等,人群普遍易感,感染后潜伏期为4～30年。根据病因,临床上将CJD分为散发性(sCJD)、遗传性(fCJD)、医源性(iCJD)及变异型(vCJD)。85%以上病例为散发性,发病率极低[2]。本文近期诊断1例临床很可能的CJD,结合文献复习予以报道。
Creutzfeldt-Jakob disease (CJD) is a progressive disease caused by prion protein infection, manifested as rapid progression of dementia, ataxia, myoclonus and psychiatric symptoms, which occur in 50 to 70 years old The population, the route of infection including impaired skin and mucosal contact with prion protein-containing blood or secretions, the consumption of diseased animal products, the injection of pathogens containing biological products or the use of pathogens contaminated surgical instruments, etc., the general population susceptibility to post-infection incubation period of 4 ~ 30 years. According to the etiology, CJD is divided into sporadic (sCJD), hereditary (fCJD), iCJD and variant (vCJD) clinically. More than 85% of cases were sporadic and the incidence was very low [2]. This article recently diagnosed one case of clinical CJD, combined with literature review to be reported.