Validation and utilization of amended diagnostic criteria in Creutzfeldt-Jakob Disease surveillance

Objective: To validate an amended protocol for clinical diagnosis of sporadic Creutzfeldt-Jakob Disease (sCJD) including Real-Time Quaking-induced Conversion (RT-QuIC) and to observe its utilization in CJD surveillance. Methods: In the framework of a prospective epidemiological study, all neuropathological confirmed sCJD cases that received CSF RT-QuIC analysis during diagnostic work up (n=65) and a control group of non- CJD cases (n=118) were selected to investigate the accuracy of an amended diagnostic protocol. The patients had been referred to the German National Reference Center for Transmissible Spongiform Encephalopathies. The influence of the amended protocol on incidence figures was evaluated in the context of three years of surveillance activity (screened cases using 14-3-3 test: n=18.789, highly suspicious cases of CJD: n=704). Annual incidences were calculated using current criteria and the amended protocol. Results: The amended protocol showed a sensitivity of 97% and a spe...

研究目标：验证纳入实时震颤诱导转化试验（Real-Time Quaking-induced Conversion, RT-QuIC）的修订版散发性克雅氏病（sporadic Creutzfeldt-Jakob Disease, sCJD）临床诊断方案，并观察该方案在克雅氏病监测中的应用情况。研究方法：本研究为一项前瞻性流行病学研究，选取在诊断流程中接受脑脊液实时震颤诱导转化试验（Real-Time Quaking-induced Conversion, RT-QuIC）分析、且经神经病理学确诊的65例散发性克雅氏病（sporadic Creutzfeldt-Jakob Disease, sCJD）病例，以及118例非克雅氏病病例作为对照组，以评估修订版诊断方案的诊断准确性。所有受试患者均转诊至德国国家传染性海绵状脑病参考中心。本研究结合三年的监测工作数据（其中采用14-3-3检测进行筛查的病例共18789例，高度疑似克雅氏病的病例共704例），评估修订版方案对发病统计数据的影响，并分别采用现行诊断标准与修订版方案计算年发病率。研究结果：修订版诊断方案的灵敏度达97%，特异度……