A peculiar grouping of Creutzfeldt-Jacob disease (CJD) and of other organic presenile dementia (OPD) cases in a small, prevalently rural area in Czechoslovakia to the east of 19 degrees 35' E and to the north of 48 degrees 15' N was studied. Between August, 1972 and November, 1976, three histologically proven CJD cases, bearing no sporadic of familial patterns, were observed. The distance between their dwellings was 13--17 km. In addition, seven cases of OPD were detected in the same area, two of them being suspected as possible CJD according to clinical evidence.

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