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The epidemiology of variant Creutzfeldt-Jakob disease
P G Smith1, S N Cousens, J N d' Huillard Aignaux
1Department of Infectious and Tropical Diseases, London School of Hygiene and Tropical Medicine, Keppel Street, London WC1E 7HT, UK. p.smith@lshtm.ac.uk
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.
Insights
Variant Creutzfeldt-Jakob disease (vCJD) emerged in 1996, linked to bovine spongiform encephalopathy (BSE). Mathematical models predict few future cases, but a diagnostic test is needed to understand spread and prevent iatrogenic transmission.
Area of Science:
- Neurology
- Epidemiology
- Prion Diseases
Background:
- Variant Creutzfeldt-Jakob disease (vCJD) was identified in 1996.
- Epidemiological and strain typing studies confirmed its link to the bovine spongiform encephalopathy (BSE) agent.
- vCJD predominantly affects young adults, with all cases to date being methionine homozygous at codon 129 of the prion protein gene.
Purpose of the Study:
- To analyze the epidemiological characteristics of vCJD.
- To assess the incidence and potential future course of the vCJD epidemic.
- To highlight the need for a diagnostic test for vCJD infection.
Main Methods:
- Review of epidemiological data and patient characteristics.
- Analysis of vCJD incidence in the UK.
- Mathematical modeling to predict future cases.
- Strain typing studies to confirm the BSE-vCJD association.
Main Results:
- The incidence of vCJD has been lower than initially feared.
- Mathematical models suggest a limited number of future cases.
- A significant association between vCJD and methionine homozygosity at codon 129 of the prion protein gene was observed.
- Incidence was approximately two times higher in the North of the UK.
Conclusions:
- The vCJD epidemic has been smaller than initially predicted.
- Mathematical modeling indicates a low likelihood of widespread future cases.
- The absence of a diagnostic test hinders understanding of vCJD epidemiology and control of iatrogenic spread.
- Development of a diagnostic test is crucial for advancing research and implementing control measures.
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