Open Access Highly Accessed Research article

Human transmissible spongiform encephalopathies in eleven countries: diagnostic pattern across time, 1993–2002

Jesús de Pedro-Cuesta1*, Markus Glatzel2, Javier Almazán1, Katharina Stoeck3, Vittorio Mellina4, Maria Puopolo4, Maurizio Pocchiari4, Inga Zerr5, Hans A Kretszchmar6, Jean-Philippe Brandel7, Nicole Delasnerie-Lauprêtre7, Annick Alpérovitch7, Cornelia Van Duijn8, Pascual Sanchez-Juan8, Steven Collins9, Victoria Lewis9, Gerard H Jansen10, Michael B Coulthart10, Ellen Gelpi11, Herbert Budka11 and Eva Mitrova12

Author Affiliations

1 Instituto de Salud Carlos III, Centro Nacional de Epidemiologia, Departamento de Epidemiologia Aplicada, Calle Sinesio Delgado 6, 28029, Madrid, Spain

2 Institute of Neuropathology, University Medical Center Hamburg-Eppendorf, Martinistraße 52, D-20246 Hamburg, Germany

3 Institute of Neuropathology, University Hospital Zurich, Switzerland

4 Registry of Creutzfeldt-Jakob disease, -Department of Cell. Biology and Neurosciences, Istituto Superiore di Sanita, Viale Regina Elena 299, 00161 Rome, Italy

5 Department of Neurology, Georg-August-Universität Göttingen, Robert-Koch Strasse 40, 37075 Gottingen, Germany

6 Department. of Neuropathology, Ludwig-Maximilian University, Munich, Germany

7 U.708 INSERM, Hopital de la Salpetriere, 75651 Paris, Cedex 13, France

8 Department of Epidemiology and Biostatistics, Erasmus MC, PO Box 1738, 3000 DR Rotterdam, The Netherlands

9 Australian National Creutzfeldt-Jakob disease Registry, Department of Pathology, The University of Melbourne, Parkville, Australia

10 CJD Surveillance System, Division of Host Genetics and Prion Diseases, Public Health Agency of Canada, LCDC Building, AL 0601E2, Tunney's Pasture, Ottawa, Ontario, K1A 0L2, Canada

11 Institute of Neurology, Medical University of Vienna, and Austrian Reference Centre for Human Prion Diseases, AKH 4J, A-1097 Vienna, Austria

12 Research base of Slovak Medical University, Bratislava, Slovakia

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BMC Public Health 2006, 6:278  doi:10.1186/1471-2458-6-278

Published: 10 November 2006



The objective of this study was to describe the diagnostic panorama of human transmissible spongiform encephalopathies across 11 countries.


From data collected for surveillance purposes, we describe annual proportions of deaths due to different human transmissible spongiform encephalopathies in eleven EUROCJD-consortium countries over the period 1993–2002, as well as variations in the use of diagnostic tests. Using logistic models we quantified international differences and changes across time.


In general, pre-mortem use of diagnostic investigations increased with time. International differences in pathological confirmation of sporadic Creutzfeldt-Jakob disease, stable over time, were evident. Compared to their counterparts, some countries displayed remarkable patterns, such as: 1) the high proportion, increasing with time, of variant Creutzfeldt-Jakob disease in the United Kingdom, (OR 607.99 95%CI 84.72–4363.40), and France (OR 18.35, 95%CI 2.20–152.83); 2) high, decreasing proportions of iatrogenic Creutzfeldt-Jakob disease in France, (OR 5.81 95%CI 4.09–8.24), and the United Kingdom, (OR 1.54 95%CI 1.03–2.30); and, 3) high and stable ratios of genetic forms in Slovakia (OR 21.82 95%CI 12.42–38.33) and Italy (OR 2.12 95%CI 1.69–2.68).


Considerable international variation in aetiological subtypes of human transmissible spongiform encephalopathies was evident over the observation period. With the exception of variant Creutzfeldt-Jakob disease and iatrogenic Creutzfeldt-Jakob disease in France and the United Kingdom, these differences persisted across time.