Kuru-fifty years later.

Kuru, the first human neurodegenerative disease classified as a transmissible spongiform encephalopathy, prion disease or, in the past, slow unconventional virus disease, was first reported to Western medicine in 1957 by D. Carleton Gajdusek and Vincent Zigas. Thus, this year marks the 50th anniversary of kuru discovery, highlighted by the symposium The end of kuru: 50 years of research into an extraordinary disease organized by John Collinge and Michael Alpers at the Royal Society, London, November 11-12, 2007. In this review, we summarize some data on the epidemiology, neuropathology and clinical picture of kuru.

Early perceptions of an epidemic.

This article surveys some descriptions of the Fore people made on early contact in the 1950s by patrol officers, social anthropologists and medical doctors. Sorcery accusations and cannibalism initially impressed these outside observers, though gradually they came to realize that a strange and fatal condition called kuru was a major affliction of the Fore, especially women and children. Fore attributed kuru to sorcery, anthropologists speculated on psychosomatic causes and medical officers began to wonder if it was a mysterious encephalitis.

Review. Kuru and its contribution to medicine.

The solution of kuru led us to the solution of Creutzfeldt-Jakob disease and to the elucidation, in humans and other species, of previously unknown mechanisms of infection. These require very close three-dimensional matching, which determines infectious nucleant or prion activity. Evidence for nucleation processes is found widely in the organic and inorganic worlds and in the interactions between them: in the formation of amyloid fibrils; in the biochemistry of silicon; in cave formations deep in the Earth; and in outer space. Kuru in its location in Papua New Guinea has also led to an understanding of the cultural achievements of the Palaeo-Melanesians, with deep roots in human history.

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

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.

Review. Understanding kuru: the contribution of anthropology and medicine.

To understand kuru and solve the problems of its cause and transmission required the integration of knowledge from both anthropological and medical research. Anthropological studies elucidated the origin and spread of kuru, the local mortuary practices of endocannibalism, the social effects of kuru, the life of women and child-rearing practices, the kinship system of the Fore and their willingness to incorporate outsiders into it, the myths, folklore and history of the Fore and their neighbours, sorcery as a powerful social phenomenon and way of explaining the causation of disease, and concepts of the treatment of disease. Many scientists from different disciplines, government officers and others have contributed to this chapter of medical history but it is the Fore people who have contributed the most, through their suffering, their cooperative and reliable witness to kuru, and their participation, in various ways, in the research process itself.

Mortuary rites of the South Fore and kuru.

This paper is part of a wider study to explain the historical spread and changing epidemiological patterns of kuru by analysing factors that affect the transmission of kuru. Part of the study has been to look at the mortuary feasts that were the means of transmission of the kuru agent. This paper shows the complexity of Fore eschatology, and the variations and contradictions of human behaviour in relation to mortuary rites and the transmission of kuru. It also confirms that oral ingestion was the primary route of inoculation though some cases of parenteral inoculation may have occurred. The exclusion of alternative routes of transmission is of importance owing to the dietary exposure of the UK and other populations to bovine spongiform encephalopathy prions.