Until recently, chronic wasting disease of cervids, the only wildlife prion disease, was believed to be geographically concentrated to Colorado and Wyoming within the United States. However, increased surveillance has unveiled several additional pockets of CWD-infected deer and elk in 12 additional states and 2 Canadian provinces. Deer and elk with CWD have extensive aggregates of PrP Sc not only in the central nervous system, but also in peripheral lymphoid tissues, skeletal muscle, and other organs, perhaps influencing prion shedding. Indeed, CWD is transmitted efficiently among animals by horizontal routes, although the mechanism of spread is unknown. Genetic polymorphisms in the Prnp gene may affect CWD susceptibility, particularly at codon 225 (S/F) in deer and codon 132 (M/L) in elk. Since CWD infects free-ranging animals and is efficiently spread, disease management will be a challenge.
A chronicle of CWDA prion disease of free-ranging wildlife, chronic wasting disease (CWD) affects mule deer (Odocoileus hemionus), white-tailed deer (O. virginianus), Rocky Mountain elk (Cervus elaphus nelsoni) [1], and moose (Alces alces shirasi) [2], all members of the family Cervidae. CWD was first noted in 1967 within a research facility in Fort Collins, Colorado where captive mule deer used for nutrition research were reported with a body wasting syndrome [2]. After more than a decade of uncertainty about the etiology of CWD, pathologists Elizabeth Williams and Stewart Young recognized the brain lesions as those of a transmissible spongiform encephalopathy (TSE) in 1978, and CWD was subsequently demonstrated as a prion disease not only by the classic neuronal perikaryonic vacuoles [3], but also by the accumulation of aggregated prion protein [4] (Fig. 1) as well as prion infectivity in the brain [5]. In the late 70s and early 80s, CWD was detected in two zoological collections, in Wyoming and in Canada [6]. Beginning in 1981, cases of CWD were discovered in wild deer and elk on the eastern slope of the Rocky Mountains and extending out on the plains following river valleys within Colorado and Wyoming [7,8]. By 1996, CWD was first detected in Canada's farmed elk, and soon thereafter in the US elk industry, although it may have occurred in this industry far earlier.More recently, CWD-infected ranched elk have been discovered in several other US states and in South Korea [9] [10] raising international awareness and concern regarding CWD. The origin of CWD remains an enigma.*Corresponding author e-mail: E-mail: csigurd@lamar.colostate.edu. Publisher's Disclaimer: This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final citable form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the jou...