Chronic wasting disease: an evolving prion disease of cervids

Chronic wasting disease (CWD) is a relatively new and burgeoning prion epidemic of deer, elk, reindeer, and moose, which are members of the cervid family. While the disease was first described in captive deer, its subsequent discovery in various species of free-ranging animals makes it the only currently recognized prion disorder of both wild and farmed animals. In addition to its expanding range of host species, CWD continues to spread from North America to new geographic areas, including South Korea, and most recently Norway, marking the first time this disease was detected in Europe. Its unparalleled efficiency of contagious transmission, combined with high densities of deer in certain areas, complicates strategies for controlling CWD, raising concerns about its potential for spread to new species. Because there is a high prevalence of CWD in deer and elk, which are commonly hunted and consumed by humans, and since prions from cattle with bovine spongiform encephalopathy have been transmitted to humans causing variant Creutzfeldt-Jakob disease, the possibility of zoonotic transmission of CWD is particularly concerning. Here we review the clinical and pathologic features of CWD and its disturbing epidemiology, and discuss features that affect its transmission, including genetic susceptibility, pathogenesis, and agent strain variability. Finally, we discuss evidence that speaks to the potential for zoonotic transmission of this emerging disease.