Extensive information on the epidemiology of Creutzfeldt-Jakob Disease
(CJD) has accumulated since the original transmission of CJD to prima
tes in 1968. One aim of this research was to discover the mechanism of
natural transmission of CJD but the epidemiological evidence virtuall
y precludes case to case transmission as a causative mechanism, except
in rare iatrogenic cases, and has provided little evidence to suggest
an environmental 'source of infection'. An understanding of the few p
ositive epidemiological findings such as the high incidence in Slovaki
a has depended on major advances in molecular biology rather than on e
pidemiological evidence. The occurrence of Bovine Spongiform Encephalo
pathy (BSE) has however reinforced the importance of having establishe
d the epidemiological characteristics of CJD and this information is a
n important background on which to evaluate the findings from basic sc
ientific research.