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From: TSS ()
Date: May 10, 2005 at 12:11 pm PST

-------- Original Message --------
Date: Mon, 9 May 2005 17:06:28 -0500
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

##################### Bovine Spongiform Encephalopathy #####################

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

Mortality from CreutzfeldtJakob 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 Sanitŕ(Drs. Ladogana, Puopolo, and
Pocchiari), Department of Cell Biology and NeurosciencesISS, 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;

Background: An international study of the epidemiologic characteristics
of CreutzfeldtJakob 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 CreutzfeldtJakob disease (CJD) of all types in a
multinational populationbased 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
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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