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To review the evidence for risk factors of Creutzfeldt-Jakob disease (CJD), we pooled and reanalyzed the raw data of three case-control studies. The pooled data set comprised 178 patients and 333 control subjects. The strength of association between CJD and putative risk factors was assessed by computing the odds ratio as estimate of the relative risk. The risk of CJD was statistically significantly increased for subjects with a family history of CJD (odds ratio = 19.1; 95% CI 1.1 to 348.0). Further, there was a significant association between the risk of CJD and a history of psychotic disease (odds ratio = 9.9; 95% CI 1.1 to 86.1). Although not significantly increased, there was an elevated risk of CJD for subjects with a family history of dementia, a history of poliomyelitis, subjects employed as health professionals, and subjects ever exposed to cows and sheep. No association could be shown with organ meat consumption, including brain. The negative results of this reanalysis reassures the absence of a common risk factor in all CJD patients. However, the ongoing epidemiologic surveillance of CJD in several European countries may provide more evidence to exclude any environmental exposure early in childhood.

Original publication

DOI

10.1212/wnl.46.5.1287

Type

Journal article

Journal

Neurology

Publication Date

05/1996

Volume

46

Pages

1287 - 1291

Addresses

Department of Epidemiology & Biostatistics, Erasmus University Medical School, Rotterdam, The Netherlands.

Keywords

Animals, Cattle, Sheep, Humans, Creutzfeldt-Jakob Syndrome, Odds Ratio, Risk Assessment, Risk Factors, Case-Control Studies, Environmental Exposure, Reference Values, Meat, MEDLINE, United States, Japan, Europe