The Epidemiology of Variant Creutzfeldt-Jakob Disease

  • P. G. Smith
  • S. N. Cousens
  • J. N. Huillard d’Aignaux
  • H. J. T. Ward
  • R. G. Will
Part of the Current Topics in Microbiology and Immunology book series (CT MICROBIOLOGY, volume 284)


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.


Bovine Spongiform Encephalopathy Transmissible Spongiform Encephalopathy Bovine Spongiform Encephalopathy Case Average Incubation Period Bovine Spongiform Encephalopathy Agent 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer-Verlag Berlin Heidelberg 2004

Authors and Affiliations

  • P. G. Smith
    • 1
  • S. N. Cousens
    • 1
  • J. N. Huillard d’Aignaux
    • 1
  • H. J. T. Ward
    • 2
  • R. G. Will
    • 2
  1. 1.Department of Infectious and Tropical DiseasesLondon School of Hygiene and Tropical MedicineLondonUK
  2. 2.National CJD Surveillance UnitWestern General HospitalEdinburghUK

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