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Epidemiological evidence of higher susceptibility to vCJD in the young
Pierre-Yves Boëlle1, Jean-Yves Cesbron, Alain-Jacques Valleron
1INSERM U444, Assistance Publique Hôpitaux de Paris, Université Pierre et Marie Curie, Paris, France. boelle@u444.jussieu.fr
Insights
Differential dietary exposure alone does not explain the young age of new variant Creutzfeldt-Jacob disease (vCJD) cases. Age-related susceptibility to infection is necessary to understand vCJD epidemiology.
Area of Science:
- Epidemiology
- Neurodegenerative Diseases
- Food Safety
Background:
- The young age of new variant Creutzfeldt-Jacob disease (vCJD) cases is not fully understood.
- While age-dependent susceptibility has been proposed, differential dietary exposure during the bovine spongiform encephalopathy (BSE) epidemic is another potential explanation.
Purpose of the Study:
- To investigate whether differential dietary exposure can explain the age distribution of vCJD cases.
- To assess the role of age-dependent susceptibility in vCJD epidemiology.
Main Methods:
- Utilized mathematical models incorporating dietary exposure estimates.
- Simulated vCJD case distribution based on exposure and susceptibility factors.
Main Results:
- Models without age-dependent susceptibility failed to replicate observed vCJD case distribution (predicted 48% over 40 vs. observed 10%).
- Models incorporating decreasing age-related susceptibility accurately reproduced the age characteristics of vCJD cases (predicted 12% over 40).
Conclusions:
- Differential dietary exposure alone is insufficient to explain the age pattern in vCJD cases.
- Age-related susceptibility is a crucial factor in understanding the epidemiology of vCJD.
Background:
The strikingly young age of new variant Creutzfeldt-Jacob disease (vCJD) cases remains unexplained. Age dependent susceptibility to infection has been put forward, but differential dietary exposure to contaminated food products in the UK population according to age and sex during the bovine spongiform encephalopathy (BSE) epidemic may provide a simpler explanation.
Methods:
Using recently published estimates of dietary exposure in mathematical models of the epidemiology of the new variant Creutzfeldt Jacob disease (vCJD), we examine whether the age characteristics of vCJD cases may be reproduced.
Results:
The susceptibility/exposure risk function has likely peaked in adolescents and was followed by a sharp decrease with age, evocative of the profile of exposure to bovine material consumption according to age. However, assuming that the risk of contamination was proportional to exposure, with no age dependent susceptibility, the model failed to reproduce the observed age characteristics of the vCJD cases: The predicted cumulated proportion of cases over 40 years was 48%, in strong disagreement with the observed 10%. Incorporating age dependent susceptibility led to a cumulated proportion of cases over 40 years old of 12%.
Conclusions:
This analysis provides evidence that differential dietary exposure alone fails to explain the pattern of age in vCJD cases. Decreasing age related susceptibility is required to reproduce the characteristics of the age distribution of vCJD cases.
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