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


CONCLUSIONS
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.
Brandel
Jean-Philippe
JP
Institut National de la Santé et de la Recherche Médicale, Equipe Avenir Human Prion Diseases, Paris, F-75013, France. jean-philippe.brandel@psl.aphp.fr
Heath
Craig A
CA
Head
Mark W
MW
Levavasseur
Etienne
E
Knight
Richard
R
Laplanche
Jean-Louis
JL
Langeveld
Jan Pm
JP
Ironside
James W
JW
Hauw
Jean-Jacques
JJ
Mackenzie
Jan
J
Alpérovitch
Annick
A
Will
Robert G
RG
Haïk
Stéphane
S