| Summary: | The large-scale epidemic of bovine spongiform encephalopathy (BSE) in the United Kingdom (UK) created significant fears of a possible threat to public health. This threat was realized in 1996 when variant Creutzfeldt-Jakob disease (vCJD) was first recognized in the UK and was attributed to the oral transmission of BSE to humans. Although the incidence of vCJD is declining the extremely long incubation periods for which genetic effects are clearly important and the unknown prevalence of pre- or sub-clinically infected individuals remain matters of ongoing concern. Such healthy carriers pose a threat of iatrogenic transmission to others during medical and surgical procedures and there have been four cases of transmission of vCJD via blood transfusion. Before vCJD appeared, kuru and iatrogenic CJD provided our experience of acquired prion diseases caused by human-to-human transmission. Kuru reached epidemic proportions amongst the Fore and surrounding linguistic groups in Papua New Guinea, and was transmitted during endocannibalism (transumption) of dead family members at mortuary feasts, a practice that ended in the late 1950s. Study of kuru therefore became of renewed interest with the arrival of vCJD as it comprises not only the largest example of an epidemic human prion disease, but one that is nearly complete, offering a number of insights into key parameters of potential relevance to public health in the UK and elsewhere. The aim of this study is to explain the historical spread and the changing epidemiological patterns of kuru by analyzing factors that affect the transmission of kuru. Although other possible factors are considered, the analysis principally involves the dominant factors of mortuary practices and human genetics. The main thrust of the thesis is on the ethnographic study of mortuary practices, firstly for its primary data, and secondly for its relevance to the transmission of kuru. Though the genetic results of these studies have proven to be of exceptional interest in understanding genetic susceptibility to and selection pressure imposed by the kuru epidemic and have provided new insights into human history and evolution, they do not explain the spatiotemporal epidemiological changes. The mortuary rites and related behaviours constitute the principal outcomes of this thesis and can now satisfactorily explain the spread and changing epidemiological patterns of kuru.
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