NEUROLOGY 1996;46:1287
© 1996 American Academy of Neurology
Risk factors for Creutzfeldt-Jakob diseaseA reanalysis of case-control studies
D. P.W.M. Wientjens, MD,
Z. Davanipour, DVM, PhD,
A. Hofman, MD, PhD,
K. Kondo, MD,
W. B. Matthews, DM, FRCP,
R. G. Will, MD, FRCP and
C. M. van Duijn, PhD
Department of Epidemiology & Biostatistics, Erasmus University Medical School, Rotterdam, The Netherlands (Dr. Wientjens) (Dr. Hofman) (Dr. van Duijn)
Department of Neurology, Erasmus University Medical School, Rotterdam, The Netherlands (Dr. Wientjens)
University of Southern California, Los Angeles, CA (Dr. Davanipour)
Department of Public Health, Hokkaido University, Sapporo, Japan (Dr. Kondo)
University Department of Clinical Neurology, Radcliffe Infirmary, Oxford, UK (Dr. Matthews)
Department of Clinical Neurosciences, Western General Hospital, Edinburgh, UK. (Dr. Will)
Address correspondence and reprint requests to Dr van Duijn, Department of Epidemiology & Biostatistics, Erasmus University Medical School, PO BOX 1738, 3000 DR Rotterdam, The Netherlands.
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.
Supported by the Netherlands Institute for Health Sciences (NIHES), the Slow Virus Infection Research Group Japan, and the Japan Ministry of Health and Welfare.
Received August 21, 1995. Accepted in final form November 1, 1995.
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