Shirley Lindenbaum

Kuru: genes, cannibals and neuropathology.

Abstract Kuru was the first human transmissible spongiform encephalopathy (TSE) or prion disease identified, occurring in the Fore linguistic group of Papua New Guinea. Kuru was a uniformly fatal cerebellar ataxic syndrome, usually followed by choreiform and athetoid movements. Kuru imposed a strong balancing selection on the Fore population, with individuals homozygous for the 129 Met allele of the gene (PRNP) encoding for prion protein (PrP) being the most susceptible. The decline in the incidence of kuru in the Fore has been attributed to the exhaustion of the susceptible genotype and ultimately by discontinuation of exposure via cannibalism. Neuropathologically, kuru-affected brains were characterized by widespread degeneration ofneurons, astroglial and microglial proliferation, and the presence of amyloid plaques. These early findings have been confirmed and extended by recent immunohistochemical studies for the detection of the TSE-specific PrP (PrPTSE). Confocal laser microscopy also showed theconcentration of glial fibrillary acidic protein–positive astrocytic processes at the plaque periphery. The fine structure of plaques corresponds to that described earlier by light microscopy. The successful experimental transmission of kuru led to the awareness of its similarityto Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease and formed a background against which the recent epidemics of iatrogenic and variant Creutzfeldt-Jakob disease could be studied.

Anthropological Methods Used in Kuru Research

This historical account of the methods used by anthropologists studying kuru from 1961 to 2010 illustrates the identity of anthropology as both a humanist and natural science. To understand and analyze complex historical processes anthropologists employ both interpretive and explanatory research methods. This chapter documents the emergence of medical anthropology as a subfield in anthropology, changes that have taken place in the collaborative relations between anthropology and medicine, and importance of the political context in Papua New Guinea, all of which have had an impact on the research methods of anthropologists and medical investigators. Fore forms of health care have also changed as local therapists adopt some aspects of biomedicine while retaining a belief that sorcerers cause illness and death, a theory that supports their own methods of investigation.