Creutzfeldt–Jakob disease (CJD) is a rapidly progressive, fatal and transmissible neurodegenerative disease associated with the accumulation of misfolded prion protein in the CNS. International CJD surveillance programmes have been active since the emergence, in the mid-1990s, of variant CJD (vCJD), a disease linked to bovine spongiform encephalopathy. Control measures have now successfully contained bovine spongiform encephalopathy and the incidence of vCJD has declined, leading to questions about the requirement for ongoing surveillance. However, several lines of evidence have raised concerns that further cases of vCJD could emerge as a result of prolonged incubation and/or secondary transmission. Emerging evidence from peripheral tissue distribution studies employing high-sensitivity assays suggests that all forms of human prion disease carry a theoretical risk of iatrogenic transmission. Finally, emerging diseases, such as chronic wasting disease and camel prion disease, pose further risks to public health. In this Review, we provide an up-to-date overview of the transmission of prion diseases in human populations and argue that CJD surveillance remains vital both from a public health perspective and to support essential research into disease pathophysiology, enhanced diagnostic tests and much-needed treatments.

克雅氏病 (CJD) 是一种快速进行性、致命性和传染性的神经退行性疾病，与中枢神经系统中错误折叠的朊病毒蛋白的积累有关。自 20 世纪 90 年代中期出现变异型克雅氏病 (vCJD) 以来，国际克雅氏病监测计划一直很活跃，变异型克雅氏病是一种与牛海绵状脑病相关的疾病。控制措施现已成功地遏制了牛海绵状脑病，vCJD 的发病率也有所下降，这引发了对持续监测需求的质疑。然而，一些证据引起了人们的担忧，即由于长时间的潜伏期和/或二次传播，可能会出现更多的 vCJD 病例。来自采用高灵敏度分析的外周组织分布研究的新证据表明，所有形式的人类朊病毒病在理论上都具有医源性传播的风险。最后，慢性消耗性疾病和骆驼朊病毒病等新出现的疾病对公众健康构成了进一步的风险。在这篇综述中，我们提供了人类人群中朊病毒病传播的最新概述，并认为克雅氏病监测从公共卫生的角度来看仍然至关重要，并支持对疾病病理生理学的基本研究、增强的诊断测试和许多-需要的治疗。