克雅氏病(Creutzfeldt-Jakob disease, CJD)是一种在人类中最为常见的海绵状脑病，其致病因子为朊病毒。由于该病的致死率为100%，且具有传播风险，CJD的早期诊断对疾病防控具有重要意义。临床上一般依据患者的临床症状与体征以及相应的实验室检测来进行CJD的鉴别诊断。本文主要围绕现有的CJD实验室检测技术展开综述，包括朊病毒的直接与间接检测、疾病标志物的检测以及疾病相关基因PRNP的检测与分析等，旨在为进一步推广和规范CJD的实验室检测提供参考与技术依据。

Creutzfeldt-Jakob disease(CJD) is the most common prion disease in human beings. Due to the infectivity and 100% fatality rate of CJD, early diagnosis is critical for disease control and prevention. Diagnosis of CJD needs combined analysis of both clinical features and laboratory testing results. The current review mainly focuses on the laboratory testing technologies of CJD including direct and indirect tests of prion, detection of the disease markers, as well as the analysis of disease-related gene PRNP, aiming to provide technical references for the normalization and development of CJD laboratory testing.