摘要： Creutzfeldt-Jakob 病是由朊蛋白感染所导致的中枢神经系统变性疾病，病程短、进展迅速，病死率高达100% 。本文通过文献复习，探讨Creutzfeldt-Jakob 病的发病机制、组织病理学特点、临床表现、脑电图改变、影像学特点及治疗进展。对中老年快速进展性痴呆及青年以精神症状发病并伴多系统受累的疑似Creutzfeldt-Jakob 病患者，应尽早行脑电图、MRI 及脑脊液14-3-3 蛋白检测，有助于早期诊断。

关键词: 克-亚综合征, 朊病毒, 综述

Abstract: Creutzfeldt-Jakob disease (CJD) is a degenerative central nervous system (CNS) disease caused by infection of prion protein (PrP), with clinical features including short course, rapid development and 100% mortality. This article aims to discuss the pathogenesis, histopathological features, clinical manifestations, electroencephalogram (EEG) findings, imaging data and treatment progress of this disease based on literature review. Cerebrospinal fluid 14-3-3 protein detection, EEG and MRI are three important methods to make an early diagnosis on patients with suspected CJD, such as elderly patients with rapidly progressive dementia (RPD) and young patients with mental symptoms involving multiple systems (MS).

Key words: Creutzfeldt-Jakob syndrome, Prions, Review