The epidemiology of variant Creutzfeldt-Jakob disease

Smith, PG; Cousens, SN; Huillard d'Aignaux, JN; Ward, HJT; Will, RG; (2004) The epidemiology of variant Creutzfeldt-Jakob disease. In: Harris, DA, (ed.) Mad Cow Disease and Related Spongiform Encephalopathies. Springer-Verlag Berlin, Berlin, pp. 161-191. ISBN 1286-4579 DOI:

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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.

Item Type: Book Section
Keywords: BOVINE SPONGIFORM ENCEPHALOPATHY, INCUBATION PERIOD, PRION PROTEIN, RISK-FACTORS, GEOGRAPHICAL-DISTRIBUTION, BLOOD-TRANSFUSION, UNITED-KINGDOM, CJD EPIDEMIC, UK, BSE, bovine spongiform encephalopathy, incubation period, prion protein, risk-factors, geographical-distribution, blood-transfusion, united-kingdom, cjd epidemic, uk, bse
Faculty and Department: Faculty of Epidemiology and Population Health > Dept of Infectious Disease Epidemiology
Research Centre: Centre for Global Non-Communicable Diseases (NCDs)
Web of Science ID: 221885700007


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