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Home Eurosurveillance Weekly Release  2002: Volume 6/ Issue 29 Article 11
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Eurosurveillance, Volume 6, Issue 29, 18 July 2002

Citation style for this article: Molesworth AM, Cousens S, Ward H. Rising incidence of Creutzfeldt-Jakob disease in Switzerland. Euro Surveill. 2002;6(29):pii=1921. Available online:

Rising incidence of Creutzfeldt-Jakob disease in Switzerland

Last week, the Lancet reported on a recent rise in the incidence of Creutzfeldt-Jakob disease (CJD) in Switzerland over a 15 month period. From 1996, the annual number of diagnoses of CJD has been between 8 and 11, corresponding to an annual incidence of about one per million. In 2001 the number of confirmed cases rose to 19, corresponding to an incidence of 2.7 per million, and in the first quarter of 2002, seven cases were identified, which extrapolates to an annual incidence of 3.9 per million this year (1 and figure).

Figure. Incidence of CJD in Switzerland per 1 000 000 population, 1996-2002* (* based on data from January-March 2002).

020718 swiss cjd.gif (4662 bytes)

CJD is a predominantly sporadic disorder but can also occur as a dominantly inherited or infective condition. Only one of the 26 most recent confirmed cases was identified as carrying a disease related mutation of the PRNP gene, none had identifiable iatrogenic exposure, and none resembled variant CJD. Thus 25 of the 26 cases appear to be sporadic cases. Sporadic CJD is distributed worldwide with a reported incidence of about one in a million per year. Raised awareness of the disease in recent years could account for an increase in reported cases of CJD, although neither an increase in the average age of patients nor more frequent recognition of CJD amongst residents of nursing homes (where dementing illness is prevalent and misdiagnosis might be expected) were seen in the Swiss cases. Moreover, improved ascertainment as an explanation for the observed increase would imply levels of under-reporting in countries other than Switzerland, which appear implausible. The authors of the Lancet report suggest that the rise in cases might be due to some form of unidentified iatrogenic transmission or to exposure to a zoonotic source of infection, though cases do not resemble variant Creutzfeldt-Jakob disease (vCJD). The ongoing surveillance of CJD in Switzerland and the rest of Europe is essential to monitor the situation to see if this rise is sustained in Switzerland, and if a similar rise occurs in other countries (see

References :

1. Glatzel M, Rogivue C, Ghani A, Streffer JR, Amsler L, Aguzzi A. Incidence of Creutzfeldt-Jakob disease in Switzerland. Lancet 2002; 360: 139-141. (

Reported by Anna Molesworth ( Public Health Laboratory Service Communicable Disease Surveillance Centre; Simon Cousens, London School of Hygiene and Tropical Medicine, London, England; and Hester Ward, National CJD Surveillance Unit, Edinburgh, Scotland.

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