Dietary Risk Factors for Sporadic Creutzfeldt-Jakob Disease: A Confirmatory Case-Control Study

This study's primary purpose was to determine whether earlier findings suggesting an association between sporadic Creutzfeldt-Jakob disease (sCJD), a transmissible spongiform encephalopathy of humans and specific dietary components could be replicated. The a priori hypotheses were that consumption of (i) foods likely to contain organ tissue and (ii) raw/rare meat are associated with increased sCJD risk.

Population-based case-control study.

Department of Neurology, School of Medicine, Loma Linda University, Loma Linda, CA, USA; 4 years.

An 11-state case-control study of pathologically confirmed, definite sCJD cases, matched controls, and a sample of control-surrogates was conducted. Ninety-six percent (106/110) of the case data was obtained in 1991-1993, prior to variant CJD publicity.

Using control self-responses, consumption of hot dogs, sausage, pepperoni, kielbasa, "other" canned meat, poultry liver, any stomach/intestine, beef stomach/intestine, any organ tissue, and beef organ tissue was individually associated with increased sCJD risk; odds ratios (OR) ranged from 2.4 to 7.2 (0.003 <p<0.025). Rare/raw meat consumption was associated with sCJD (OR=2.0; p<0.05). Greater consumption of hot dogs, bologna, salami, sausage, pepperoni and kielbasa was associated with significantly higher risk. The OR for gizzard consumption was 7.6, p<0.04. Bologna, salami, any liver, beef liver and pork stomach/intestine were marginally associated with sCJD: ORs ranged from 1.7 to 3.7; 0.05 <p< 0.10. Brain consumption was not associated with an elevated risk. Analyses using control-surrogate data indicate that use of the control self-responses did not bias the results away from the null hypothesis.

The a priori hypotheses were supported. Consumption of various meat products may be one method of transmission of the infectious agent for sCJD.