Variant Creutzfeldt-Jakob disease in France and the United Kingdom: Evidence for the same agent strain

Ann Neurol. 2009 Mar;65(3):249-56. doi: 10.1002/ana.21583.


Objective: Variant Creutzfeldt-Jakob disease (vCJD) was first reported in the United Kingdom in 1996. Since then, the majority of cases have been observed in the United Kingdom where there was a major epidemic of bovine spongiform encephalopathy. France was the second country affected. To address the hypothesis of the involvement of a common strain of agent, we have compared clinical, neuropathological, and biochemical data on vCJD patients from both countries.

Methods: In France and the United Kingdom, epidemiological and clinical data were obtained from analysis of medical records and direct interview of the family of the patients using the same standardized questionnaire in both countries. When brain material was available, we performed with similar methods a comparative study of brain lesions and PrP(res) glycoform ratios in both vCJD populations.

Results: Clinical data, genetic background, neuropathological finding, and biochemical findings in the 185 patients observed in France (n = 23) and the United Kingdom (n = 162) were similar except for age at death. Currently, blood transfusion is a risk factor identified only in the United Kingdom.

Interpretation: The close similarity between the cases of vCJD in France and the United Kingdom supports the hypothesis that a common strain of infectious agent is involved in both countries. The 5-year delay in the peak that we observed in France compared with the United Kingdom fits well with the increase in the importation of beef products to France from the United Kingdom between 1985 and 1995.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Brain / pathology
  • Child
  • Creutzfeldt-Jakob Syndrome / epidemiology*
  • Creutzfeldt-Jakob Syndrome / genetics*
  • Creutzfeldt-Jakob Syndrome / pathology
  • Female
  • France / epidemiology
  • Humans
  • Male
  • Medical Records / statistics & numerical data
  • Middle Aged
  • PrPSc Proteins / metabolism
  • Retrospective Studies
  • Risk Factors
  • Surveys and Questionnaires
  • Transfusion Reaction
  • United Kingdom / epidemiology
  • Young Adult


  • PrPSc Proteins