The epidemiology of kuru: monitoring the epidemic from its peak to its end

Abstract

Kuru is a fatal transmissible spongiform encephalopathy restricted to the Fore people and their neighbours in a remote region of the Eastern Highlands of Papua New Guinea. When first investigated in 1957 it was found to be present in epidemic proportions, with approximately 1000 deaths in the first 5 years, 1957–1961. The changing epidemiological patterns and other significant findings such as the transmissibility of kuru are described in their historical progression. Monitoring the progress of the epidemic has been carried out by epidemiological surveillance in the field for 50 years. From its peak, the number of deaths from kuru declined to 2 in the last 5 years, indicating that the epidemic is approaching its end. The mode of transmission of the prion agent of kuru was the local mortuary practice of transumption. The prohibition of this practice in the 1950s led to the decline in the epidemic, which has been prolonged into the present century by incubation periods that may exceed 50 years. Currently, the epidemiological surveillance is being maintained and further studies on human genetics and the past mortuary practices are being conducted in the kuru-affected region and in communities beyond it.

Human prion diseases: surgical lessons learned from iatrogenic prion transmission

“…Remarkably, the kuru-affected people of Papua New Guinea were unique in their practice of “endocannabalism.” As Alpers describes, the people of the Fore region believed the “mortuary practice of consumption of the dead and incorporation of the body of the dead person into the bodies of living relatives, thus [helped] to free the spirit of the dead.” 4 In fact, the whole body of the deceased kin was eaten by female relatives and children of both sexes. Adult males, which included boys above age 7, rarely took part in this practice.…”

Infectious prion diseases in humans: Cannibalism, iatrogenicity and zoonoses

“…At present, a lim-157 ited number of prion strains has been isolated from human dis-158 eases (Haik and Brandel, 2011). In sCJD, a molecular classification ,000 people at that time (Alpers, 2008). The first remembered 176 case, before the region came under Australian administrative con-177 trol, was around 1920 (Mead et al, 2003).…”

Genome wide association studies and prion disease

“…Estimates of incubation times from acquired prion diseases that involve human-human transmission are around 12 years (for kuru, or iatrogenic CJD). 7,8 Based on animal experiments one expects the incubation times involving transmission between species to be prolonged compared with intraspecies transmission. 9 As human-human transmission incubation times can be over 50 years, 10 it would be incautious to conclude that vCJD is a completed epidemic.…”