title: The epidemiology of variant Creutzfeldt-Jakob disease.
abstract: Variant Creutzfeldt-Jakob disease (vCJD) was identified as a new disease in 1996. It was linked to infection with the bovine spongiform encephalopathy (BSE) agent although the epidemiological evidence for this was not strong, but later strain typing studies confirmed the association. The disease has affected predominantly young adults whose dietary and other characteristics are unexceptional compared to control groups, other than that all patients to date have been methoinine homozygous at codon 129 of the prion protein gene and the incidence has been about two times higher in the North of the UK. The number of cases in the 7 years after first identification of the disease has been considerably lower than initially feared, given the likely widespread exposure of the UK population to the BSE agent through contaminated beef products. Predictions of the possible future course of the epidemic have many associated uncertainties, but current mathematical models suggest that more than a few thousand cases is unlikely. Such modelling is limited by the absence of a test for infection with the vCJD agent. The development of a test that could be used on easily accessible tissue to detect infection early in the incubation period would not only advance understanding of the epidemiology of infection with the agent but would also aid the implementation of control measures to prevent potential iatrogenic spread.
authors.affiliation: Department of Infectious and Tropical Diseases, London School of Hygiene and Tropical Medicine, Keppel Street, London WC1E 7HT, UK. p.smith@lshtm.ac.uk
authors.forename: P G
authors.initials: PG
authors.lastname: Smith
authors.forename: S N
authors.initials: SN
authors.lastname: Cousens
authors.forename: J N
authors.initials: JN
authors.lastname: d' Huillard Aignaux
authors.forename: H J T
authors.initials: HJ
authors.lastname: Ward
authors.forename: R G
authors.initials: RG
authors.lastname: Will
mesh_terms: D000367:Age Factors; D000818:Animals; D002417:Cattle; D007562:Creutzfeldt-Jakob Syndrome / Q000453:epidemiology* / Q000635:transmission; D016643:Encephalopathy, Bovine Spongiform / Q000453:epidemiology* / Q000635:transmission; D005506:Food Contamination; D020022:Genetic Predisposition to Disease; D006801:Humans; D015994:Incidence; D008460:Meat; D012307:Risk Factors