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NEUROLOGY 2005;64:1586-1591
© 2005 American Academy of Neurology

Mortality from Creutzfeldt–Jakob disease and related disorders in Europe, Australia, and Canada

A. Ladogana, MD, M. Puopolo, DStat, E. A. Croes, MD, PhD, H. Budka, MD, C. Jarius, MD, S. Collins, MD, FRACP, G. M. Klug, BSc(Hons), T. Sutcliffe, BA, A. Giulivi, MD, FRCP, A. Alperovitch, MD, N. Delasnerie-Laupretre, MD, J. -P. Brandel, MD, S. Poser, MD, H. Kretzschmar, MD, FRCPath, I. Rietveld, MD, E. Mitrova, MD, J. de Pedro Cuesta, MD, PhD, P. Martinez-Martin, MD, PhD, M. Glatzel, MD, A. Aguzzi, MD, PhD, R. Knight, FRCP(Ed), H. Ward, MRCP, FFPH, M. Pocchiari, MD, C. M. van Duijn, PhD, R. G. Will, MD, FRCP and I. Zerr, MD

From the Istituto Superiore di Sanità(Drs. Ladogana, Puopolo, and Pocchiari), Department of Cell Biology and Neurosciences–ISS, Rome, Italy; Department of Epidemiology and Biostatistics (Drs. Croes, Rietveld, and van Duijn), Erasmus Medical College, Rotterdam, the Netherlands; Austrian Reference Centre for Human Prion Diseases (OERPE) and Institute of Neurology (Drs. Budka and Jarius), Vienna, Austria; Australian National CJD Registry (Dr. Collins and G.M. Klug), Department of Pathology, University of Melbourne, Victoria, Australia; Blood Safety Surveillance and Health Care Acquired Infections Division (Dr. Giulivi and T. Sutcliffe), Centre for Infectious Disease Prevention and Control, Ottawa, Ontario, Canada; U.360 INSERM (Drs. Alperovitch, Delasnerie-Laupretre, and Brandel), Hopital de la Salpetriere, Paris, France; Department of Neurology (Drs. Poser and Zerr), Georg-August-Universität Göttingen, Germany; Institute of Neuropathology (Dr. Kretzschmar), University of Munich, Germany; Institute of Preventative and Clinical Medicine (Dr. Mitrova), National Reference Centre of Slow Virus Neuroinfections, Bratislava, Slovakia; Instituto de Salud Carlos III (Drs. de Pedro Cuesta and Martinez-Martin), Departamento de Epidemiologia Aplicada, Centro Nacional de Epidemiologia, Madrid, Spain; Swiss National Reference Centre for Prion Diseases (Drs. Glatzel and Aguzzi), University Hospital of Zurich, Switzerland; and National CJD Surveillance Unit (Drs. Knight, Ward, and Will), Western General Hospital, Edinburgh, UK.

Address correspondence and reprint requests to Dr Will, National CJD Surveillance Unit, Western General Hospital, Edinburgh EH4 2XU, UK; e-mail: r.g.will{at}ed.ac.uk

Background: An international study of the epidemiologic characteristics of Creutzfeldt–Jakob disease (CJD) was established in 1993 and included national registries in France, Germany, Italy, the Netherlands, Slovakia, and the United Kingdom. In 1997, the study was extended to Australia, Austria, Canada, Spain, and Switzerland.

Methods: Data were pooled from all participating countries for the years 1993 to 2002 and included deaths from definite or probable CJD of all etiologic subtypes.

Results: Four thousand four hundred forty-one cases were available for analysis and included 3,720 cases of sporadic CJD, 455 genetic cases, 138 iatrogenic cases, and 128 variant cases. The overall annual mortality rate between 1999 and 2002 was 1.67 per million for all cases and 1.39 per million for sporadic CJD. Mortality rates were similar in all countries. There was heterogeneity in the distribution of cases by etiologic subtype with an excess of genetic cases in Italy and Slovakia, of iatrogenic cases in France and the UK, and of variant CJD in the UK.

Conclusions: This study has established overall epidemiologic characteristics for Creutzfeldt–Jakob disease (CJD) of all types in a multinational population–based study. Intercountry comparisons did not suggest any relative change in the characteristics of sporadic CJD in the United Kingdom, and the evidence in this study does not suggest the occurrence of a novel form of human bovine spongiform encephalopathy infection other than variant CJD. However, this remains a possibility, and countries currently unaffected by variant CJD may yet have cases.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 10 issue to find the title link for this article.

Funded through an EU Concerted Action (BIOMED2 contract no. BMH4-CT97-2216). The Australian National CJD Registry is funded by the Commonwealth Department of Health and Ageing. The Canadian Surveillance System is funded by Health Canada. The German Surveillance System is funded by the Federal Ministry of Health (BMG, 325-4471-02/15). The surveillance of CJD in Italy is funded by the Ministry of Health and the Istituto Superiore di Sanitá. CJD surveillance in the Netherlands is funded by the Dutch Ministry of Health, Welfare, and Sports. The Slovak Surveillance System is funded by the Slovak Ministry of Health. The Swiss Reference Center for Prion Diseases is funded by the Swiss Federal Office of Public Health. The UK CJD Surveillance System is funded by the Department of Health and the Scottish Executive Health Department. Also supported by the Kanton of Zurich and by grants from the European Union.

Received February 27, 2004. Accepted in final form January 17, 2005.


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