Department of Neurology, National Reference Center for CJD Surveillance, University Medical Centre Göttingen, 37075 Göttingen, Germany.
Network Center for Biomedical Research in Neurodegenerative Diseases (CIBERNED), L'Hospitalet de Llobregat, 08908 Llobregat, Spain.
Biomolecules. 2020 Feb 12;10(2):290. doi: 10.3390/biom10020290.
Human prion diseases are classified into sporadic, genetic, and acquired forms. Within this last group, iatrogenic Creutzfeldt-Jakob disease (iCJD) is caused by human-to-human transmission through surgical and medical procedures. After reaching an incidence peak in the 1990s, it is believed that the iCJD historical period is probably coming to an end, thanks to lessons learnt from past infection sources that promoted new prion prevention and decontamination protocols. At this point, we sought to characterise the biomarker profile of iCJD and compare it to that of sporadic CJD (sCJD) for determining the value of available diagnostic tools in promptly recognising iCJD cases. To that end, we collected 23 iCJD samples from seven national CJD surveillance centres and analysed the electroencephalogram and neuroimaging data together with a panel of seven CSF biomarkers: 14-3-3, total tau, phosphorylated/total tau ratio, alpha-synuclein, neurofilament light, YKL-40, and real-time quaking induced conversion of prion protein. Using the cut-off values established for sCJD, we found the sensitivities of these biomarkers for iCJD to be similar to those described for sCJD. Given the limited relevant information on this issue to date, the present study validates the use of current sCJD biomarkers for the diagnosis of future iCJD cases.
人类朊病毒病分为散发性、遗传性和获得性三种形式。在最后一组中，医源性克雅氏病（iCJD）是由通过手术和医疗程序进行人与人之间的传播引起的。在 20 世纪 90 年代达到发病高峰后，人们认为 iCJD 的历史时期可能即将结束，这要归功于从过去的感染源中吸取的教训，这些教训促进了新的朊病毒预防和去污协议的制定。在这一点上，我们试图描述 iCJD 的生物标志物特征，并将其与散发性克雅氏病（sCJD）进行比较，以确定现有诊断工具在及时识别 iCJD 病例方面的价值。为此，我们从七个国家的 CJD 监测中心收集了 23 例 iCJD 样本，并分析了脑电图和神经影像学数据以及一组七种 CSF 生物标志物：14-3-3、总 tau、磷酸化/总 tau 比值、α-突触核蛋白、神经丝轻链、YKL-40 和实时震颤诱导的朊病毒蛋白转化。使用为 sCJD 确定的截止值，我们发现这些生物标志物对 iCJD 的敏感性与为 sCJD 描述的敏感性相似。鉴于迄今为止关于该问题的相关信息有限，本研究验证了当前用于诊断未来 iCJD 病例的 sCJD 生物标志物的使用。
