Variant Creutzfeldt-Jakob disease

Prion diseases have been the subject of intense scientific interest and debate for many years, but the advent of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jakob disease (vCJD) has resulted in extensive public awareness and concern about this group of diseases. Sporadic CJD (sCJD) and vCJD are rare conditions, but there is now convincing evidence that an animal prion disease, BSE, has been transmitted to humans, causing vCJD. This has raised concerns for public health in the United Kingdom and other countries, which are difficult to quantify accurately because of the many uncertainties concerning vCJD, such as the incubation period of BSE in humans and the possibility of iatrogenic case-to-case transmission of vCJD. As the numbers of cases of vCJD in the United Kingdom increases, scientific information on vCJD is accumulating. This article summarizes current information on the clinical, epidemiologic, and neuropathologic characteristics of vCJD.