CREUTZFELDT-JAKOB DISEASE: A CASE-CONTROL STUDY

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Abstract
From the reports of 69 pathologically verified cases of Creutz-feldt-Jakob disease (CJD) 38 patients were selected for a case-control comparison using a detailed questionnaire. Nearest relatives (usually a spouse) served as the source of information and as a control, and they then selected a friend of the patient of the same age and sex as the patient. The patients were classified into 3 clinical-pathologic types. Comparisons collectively, by matched pairs and by patient types, yielded no striking correlations for a broad range of possible etiologic factors. However, the following observations were made: 1) Those patients with the classic and more slowly progressive form of the disease, designated here as type III, are on the average 10 years or more younger than the type I and II patients. 2) The methods of ascertainment employed yielded a population of CJD cases with a geographic distribution in temperate and northern climates. 3) Clinical onset of CJD occurred more frequently in the fall months especially for type II patients. 4) Upper respiratory infections were recalled in about one-third of the patients for the year prior to onset. Most of these were severe illnesses and were generally referred to as the “flu.” In three type II patients with an ataxic form of the disease a “flu-like” illness immediately preceded the onset of CJD. 5) In light of the experience with the other spongiform encephalopathies concerning possible oral routes of transmission, it was of interest that one-third of patients and controls ate brains. The practice was more frequent among patients and the patients had a greater preference for hog brains.

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