摘要
朊病毒病是一组人和动物的神经退行性疾病，包括羊瘙痒病、牛海绵状脑病（即疯牛病，BSE）和人克-雅病（Creutzfeldt—Jakob disease，CJD）。这类疾病的发病机制与一种被称为“朊蛋白”的细胞表面糖蛋白有关。人朊病毒病可分为散发性、遗传性和获得性。对于临床表现为迅速进展的痴呆患者，或痴呆伴其他神经精神症状的患者应考虑此类疾病。
Prion diseases are a diverse group of human and animal neuro-degenerative disorders;examples inclode sheep scrapie,bovine spongiform encephalopathy（BSE）and human Creutzfeldt-Jakob disease（CJI）,The pathogenesis of these conditions involves a cell surface glycoprotein termed the‘prion protein’.Human prion diseases may be cassified as sporadie,inherited or acquired.Doctors should consider them in patients presenting with rapidly progressive dementias and in those with dementia and additional neurologieal signs or psychiatrie symptoms.
