Human Prion Disease Surveillance

Abstract

Human prion diseases are characterized by rapid fatal neurodegeneration caused by pathologic prion proteins that are transmissible under specific circumstances. Although the minority of human prion diseases are acquired through transmission, all prion diseases have the potential for transmission. The main goals of human prion disease surveillance are to reduce the public health burden by helping to recognize and prevent acquired prion diseases and to increase prion disease-related knowledge (e.g., identifying possible new prion diseases, new diagnostics, and treatments). Most international human prion disease surveillance programs were initially created in response to concerns about the zoonotic potential of an outbreak of a new cattle disease, bovine spongiform encephalopathy (BSE), which was identified in the 1980s and the discovery of its human form, variant Creutzfeldt-Jakob disease (CJD), first reported in 1996. Continued surveillance is warranted because of the continued public health threats of variant and iatrogenic CJD and concerns about the zoonotic potential of other animal prion diseases (e.g., chronic wasting disease and camel prion disease). These two animal prion diseases present many challenges to animal health and could potentially become a threat to human public health. The incidence of human prion disease has risen over time in most countries, prompting investigations of unrecognized methods of transmission. In this chapter, the authors will review known and potential transmissible causes of human prion diseases. Methods of human prion disease surveillance and the rationale for ongoing surveillance activities will be reviewed. Finally, the authors will offer recommendations for continued surveillance.