Abstract

BackgroundThe 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.MethodsUsing 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.ResultsThe 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%.ConclusionsThis 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.

Highlights

  • The strikingly young age of new variant Creutzfeldt-Jacob disease cases remains unexplained

  • Owing to the lack of data concerning age dependent dietary exposure to bovine material in food in the UK, mathematical models used in the study of new variant Creutzfeldt-Jacob disease have postulated an age risk function for contamination where the influence of age dependent dietary exposure to contaminated material could not be separated from intrinsic age related susceptibility [1,2,3]

  • The estimates for dietary exposure show that it peaked during adolescence and decreased with age afterwards, a pattern which is consistent with the finding that most variant Creutzfeldt-Jacob disease (vCJD) cases are young and were at most teenagers during the years when the bovine spongiform encephalopathy (BSE) epidemic was at its maximum

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Summary

Introduction

The strikingly young age of new variant Creutzfeldt-Jacob disease (vCJD) cases remains unexplained. We modelled exposure/susceptibility by a plateau during the first 15 years of age followed by an exponential decrease afterwards [2] Using this approach we estimated the duration of the incubation period for vCJD to circa 15 years; and predicted that the epidemic had likely peaked in 2001–2002 which is consistent with the observed data [2,3]. The estimates for dietary exposure show that it peaked during adolescence and decreased with age afterwards, a pattern which is consistent with the finding that most vCJD cases are young and were at most teenagers during the years when the bovine spongiform encephalopathy (BSE) epidemic was at its maximum

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