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

Authors

  • Jean-Philippe Brandel MD,

    Corresponding author
    1. Institut National de la Santé et de la Recherche Médicale, Equipe Avenir Human Prion Diseases, Paris, F-75013, France
    2. Institut National de la Santé et de la Recherche Médicale, U 708, Paris, F-75013, France
    3. Assistance Publique-Hôpitaux de Paris, Hôpital de la Salpêtrière, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Paris, F-75013, France
    • Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Groupe Hospitalier Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75651 Paris Cedex 13 France
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  • Craig A. Heath MD,

    1. Department of Neurology, Ninewells Hospital, Dundee, DD1 9S7, UK
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  • Mark W. Head PhD,

    1. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH42XU, UK
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  • Etienne Levavasseur PhD,

    1. Institut National de la Santé et de la Recherche Médicale, U 708, Paris, F-75013, France
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  • Richard Knight MD,

    1. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH42XU, UK
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  • Jean-Louis Laplanche PharmD, PhD,

    1. Assistance Publique-Hôpitaux de Paris, Hôpital Lariboisière, Service de Biochimie et de Biologie Moléculaire, Paris, F-75010, France
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  • Jan PM. Langeveld PhD,

    1. Central Veterinary Institute of Wageningen UR (CVI), NL-8200 AB, Lelystad, The Netherlands
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  • James W. Ironside MD,

    1. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH42XU, UK
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  • Jean-Jacques Hauw MD,

    1. Institut National de la Santé et de la Recherche Médicale, U 708, Paris, F-75013, France
    2. Assistance Publique-Hôpitaux de Paris, Hôpital de la Salpêtrière, Laboratoire Raymond Escourolle, Paris, F-75013, France
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  • Jan Mackenzie,

    1. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH42XU, UK
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  • Annick Alpérovitch MD,

    1. Institut National de la Santé et de la Recherche Médicale, Equipe Avenir Human Prion Diseases, Paris, F-75013, France
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  • Robert G. Will MD,

    1. National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, EH42XU, UK
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  • Stéphane Haïk MD, PhD

    1. Institut National de la Santé et de la Recherche Médicale, U 708, Paris, F-75013, France
    2. Assistance Publique-Hôpitaux de Paris, Hôpital de la Salpêtrière, Cellule Nationale de Référence des Maladies de Creutzfeldt-Jakob, Paris, F-75013, France
    3. Assistance Publique-Hôpitaux de Paris, Hôpital de la Salpêtrière, Laboratoire Raymond Escourolle, Paris, F-75013, France
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  • Potential conflict of interest: Nothing to report.

Abstract

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 PrPres 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. Ann Neurol 2009;65:249–256

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