克雅病（ CJD）是一种罕见的中枢神经系统退行性疾病，既有传染性又有遗传性，是快速进展性痴呆最常见的原因之一。目前CJD主要分为四型：散发型、遗传／家族型、医源型、变异型。由于其临床表现不典型，早期诊断较为困难，容易漏诊和误诊。本病致死率高，早期诊断对预后至关重要。该文就CJD的发病机制、诊断以及最新的治疗进展进行综述。“,”Creutzfeldt-Jakob disease(CJD) is a rare neurodegenerative disorder.It is both infectious and genetic, and is one of the most common causes of rapidly progressive dementia .Four types of CJD are known so far:sporadic,genetic or familial,iatrogenic and variant.It is hard to make an early diagnosis of CJD for its atypical symptoms,and misdiagnosis and missed diagnosis often occurs.The disease is lethal,and early diagnosis is essential for prognosis.Here reviews the pathogenesis,diagnosis and most recent therapy of CJD.