Abstract

Kuru is a prion disease which became epidemic among the Fore and surrounding linguistic groups in Papua New Guinea, peaking in the late 1950s. It was transmitted during the transumption (endocannibalism) of dead family members at mortuary feasts. In this study, we aimed to explain the historical spread and the changing epidemiological patterns of kuru by analysing factors that affected its transmission. We also examined what cultural group principally determined a family's behaviour during mortuary rituals. Our investigations showed that differences in mortuary practices were responsible for the initial pattern of the spread of kuru and the ultimate shape of the epidemic, and for subsequent spatio-temporal differences in the epidemiology of kuru. Before transumption stopped altogether, the South Fore continued to eat the bodies of those who had died of kuru, whereas other linguistic groups, sooner or later, stopped doing so. The linguistic group was the primary cultural group that determined behaviour but at linguistic boundaries the neighbouring group's cultural practices were often adopted. The epidemiological changes were not explained by genetic differences, but genetic studies led to an understanding of genetic susceptibility to kuru and the selection pressure imposed by kuru, and provided new insights into human history and evolution.

Highlights

  • Surveillance of kuru ceased in September 2012 after 55 years of fieldwork first instigated by Carleton Gajdusek and Vincent Zigas in 1957 [1] and completed by a collaboration between the UK Medical Research Council (MRC) Prion Unit and the Papua New Guinea Institute of Medical Research

  • By examining previous studies about the cultural reasons for the traditional mortuary rites [4,5,6,7,8], the origin and spread of kuru by the anthropologists Robert Glasse and Shirley Lindenbaum [9,10], data on the epidemiology of the disease [2,11,12,13,14,15], recently published genetic data [16,17,18] and new ethnographic data on the traditional mortuary rites of the kuru-affected region [3,19,20], we were able to answer the two principal unresolved epidemiological questions. These questions are, firstly, why the epidemic spread the way it did from the village of Uwami in the Keiagana linguistic group near the edge of the kuru-affected region and, secondly, why there have been no cases of kuru north of a line across the centre of the kuru-affected region since 1985, when the last case to the south of the line was in 2009

  • We show the exposure index (EI) for the kuru-affected region on a map with the location of all the villages that had a history of kuru

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Summary

Introduction

By examining previous studies about the cultural reasons for the traditional mortuary rites [4,5,6,7,8], the origin and spread of kuru by the anthropologists Robert Glasse and Shirley Lindenbaum [9,10], data on the epidemiology of the disease [2,11,12,13,14,15], recently published genetic data [16,17,18] and new ethnographic data on the traditional mortuary rites of the kuru-affected region [3,19,20], we were able to answer the two principal unresolved epidemiological questions. History of endo- and exocannibalism in Papua New Guinea and the kuru-affected region

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