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 changing face of kuru: a personal perspective

The epidemic of kuru is now known to have been transmitted among the Fore by ritual consumption of infected organs from deceased relatives. As cannibalism was suppressed by government patrol officers during the 1950s, most transmission had ceased by 1957, when the kuru research programme first commenced. As predicted in the 1960s, the epidemic has waned, with progressive ageing of kuru-affected cohorts over the years to 2007. The few cases seen in the twenty-first century, with the longest incubation periods, were almost certainly exposed as children prior to 1960. Although the research programme had almost no role in bringing the kuru epidemic to an end, it did provide important knowledge that was to help the wider world in controlling the later epidemics of iatrogenic and variant Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.     

Review. The origin of the prion agent of kuru: molecular and biological strain typing

Kuru is an acquired human prion disease that primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. The central clinical feature of kuru is progressive cerebellar ataxia and, in sharp contrast to most cases of sporadic Creutzfeldt-Jakob disease (CJD), dementia is a less prominent and usually late clinical feature. In this regard, kuru is more similar to variant CJD, which also has similar prodromal symptoms of sensory disturbance and joint pains in the legs and psychiatric and behavioural changes. Since a significant part of the clinicopathological diversity seen in human prion disease is likely to relate to the propagation of distinct human prion strains, we have compared the transmission properties of kuru prions with those isolated from patients with sporadic, iatrogenic and variant CJD in both transgenic and wild-type mice. These data have established that kuru prions have prion strain properties equivalent to those of classical (sporadic and iatrogenic) CJD prions but distinct from variant CJD prions. Here, we review these findings and discuss how peripheral routes of infection and other factors may be critical modifiers of the kuru phenotype.     

Review. The neuropathology of kuru and variant Creutzfeldt-Jakob disease

A comparison of the pathological profiles of two spongiform encephalopathies with a similar presumptive route of infection was performed. Archival kuru and recent variant Creutzfeldt-Jakob disease (vCJD) cases reveal distinct lesional differences, particularly with respect to prion protein, suggesting that the strain of agent is important in determining the phenotype. Genotype analysis of the polymorphism on codon 129 reveals (in conjunction with updated information from more kuru cases) that all three genotypes (VV, MV and MM (where M is methionine and V is valine)) are detected in kuru with some preference for MM homozygosity. The presence of valine does not therefore appear to determine peripheral selection of PrPCJD. vCJD remains restricted to date to MM homozygosity on codon 129. It remains to be determined whether this genotype is dictating a shorter incubation period.     

Review. Lessons of kuru research: background to recent studies with some personal reflections

The widespread exposure of the UK population to bovine spongiform encephalopathy prions, and the potential consequences for public health, led to a renewed interest in kuru, the principal example of epidemic human prion disease. Kuru research in Papua New Guinea was expanded to study the range of incubation periods possible in human prion infection, to investigate maternal and other possible natural routes of transmission, to characterize genetic susceptibility and resistance factors and to gain insights into the peripheral pathogenesis of orally acquired prion disease in humans. Although now essentially over, the kuru epidemic continues to provide important lessons.     

Genetic susceptibility, evolution and the kuru epidemic

The acquired prion disease kuru was restricted to the Fore and neighbouring linguistic groups of the Papua New Guinea highlands and largely affected children and adult women. Oral history documents the onset of the epidemic in the early twentieth century, followed by a peak in the mid-twentieth century and subsequently a well-documented decline in frequency. In the context of these strong associations (gender, region and time), we have considered the genetic factors associated with susceptibility and resistance to kuru. Heterozygosity at codon 129 of the human prion protein gene (PRNP) is known to confer relative resistance to both sporadic and acquired prion diseases. In kuru, heterozygosity is associated with older patients and longer incubation times. Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are predominantly PRNP codon 129 heterozygotes and this group show marked Hardy-Weinberg disequilibrium. The deviation from Hardy-Weinberg equilibrium is most marked in elderly women, but is also significant in a slightly younger cohort of men, consistent with their exposure to kuru as boys. Young Fore and the elderly from populations with no history of kuru show Hardy-Weinberg equilibrium. An increasing cline in 129V allele frequency centres on the kuru region, consistent with the effect of selection in elevating the frequency of resistant genotypes in the exposed population. The genetic data are thus strikingly correlated with exposure. Considering the strong coding sequence conservation of primate prion protein genes, the number of global coding polymorphisms in man is surprising. By intronic resequencing in a European population, we have shown that haplotype diversity at PRNP comprises two major and divergent clades associated with 129M and 129V. Kuru may have imposed the strongest episode of recent human balancing selection, which may not have been an isolated episode in human history.     

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.     

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.     

Understanding racism in physical (biological) anthropology

The mainstream of American physical anthropology began as racist and eugenical science that defended slavery, restricted “non-Nordic” immigration, and justified Jim Crow segregation. After World War II, the field became more anti-racial than anti-racist. It has continued as a study of natural influences on human variation and thus continues to evade the social histories of inequitable biological variation. Also reflecting its occupancy of white space, biological anthropology continues to deny its own racist history and marginalizes the contributions of Blacks. Critical disciplinary history and a shift toward biocultural studies might begin an anti-racist human biology.     

Central and peripheral pathology of kuru: pathological analysis of a recent case and comparison with other forms of human prion disease

While the neuropathology of kuru is well defined, there are few data concerning the distribution of disease-related prion protein in peripheral tissues. Here we report the investigation of brain and peripheral tissues from a kuru patient who died in 2003. Neuropathological findings were compared with those seen in classical (sporadic and iatrogenic) Creutzfeldt-Jakob disease (CJD) and variant CJD (vCJD). The neuropathological findings of the kuru patient showed all the stereotypical changes that define kuru, with the occurrence of prominent PrP plaques throughout the brain. Lymphoreticular tissue showed no evidence of prion colonization, suggesting that the peripheral pathogenesis of kuru is similar to that seen in classical CJD rather than vCJD. These findings now strongly suggest that the characteristic peripheral pathogenesis of vCJD is determined by prion strain type alone rather than route of infection.     

Not just in the past: Racist and sexist biases still permeate biology,anthropology, medicine,and education

In the past decades, it has been increasingly recognized that some areas of science, such as anthropology, have been plagued by racist, Western-centric, and/or sexist biases. Unfortunately, an acculturation process to racism and sexism has been occurring for generations leading to systemic inequities that will take a long time to disappear. Here, we highlight the existence of current examples of racism, Western-centrism and sexism within: (1) the most popular anatomical atlases used in biological, anthropological and medical education; (2) prominent natural history museums and World Heritage Sites; (3) biological and anthropological scientific research publications; and (4) popular culture and influential children's books and educational materials concerning human biology and evolution.     

Good anthropology in dark times: Critical appraisal and ethnographic application

‘Dark Anthropology’ and its complementary ‘Anthropology of the Good’ have become influential and debated notions in anthropology in recent years. I here parse distinctive features of these emphases, address their relation to theory and to ethnography, and consider the stakes involved in concretely applying their conceptual designations. I discuss the general shift in anthropology from grand theory to key concept, and the topical delimitation of theory that results. In larger purview, Dark Anthropology and the Anthropology of the Good both have long theoretical genealogies as well as practical contexts of political and social understanding, including vis‐à‐vis recent events in the U.S. and elsewhere. I suggest that considering the relationship between politico‐economically structured inequality and attempts to assert positive meaning and purpose is the most productive way to ethnographically apply their alternative conceptualisations. This brings to greater focus the thorny question of whose understanding of inequality or suffering, or of moral value and positive wellbeing, is being articulated—the sentiments of the people studied, or the concepts of the analyst? It seems vital to examine both analytic and indigenous views of dark times, and of the good, to refine our understanding of both, that is, in order to consider our complementary conceptualisations in relation to both sides of the emic/etic coin. This refines our understanding of local sensibilities and also of the appropriate limit points of our own conceptual associations.     

Redescription of Polydactylus sexfilis (Valenciennes in Cuvier and Valenciennes, 1831), a senior synonym of P. kuru (Bleeker, 1853) with designation of a lectotype (Perciformes: Polynemidae)

Polydactylus kuru (Bleeker, 1853), originally described from Jakarta, Java, Indonesia, has been regarded as a valid species. However, examination of two syntypes of Polynemus kuru revealed their close similarity to three syntypes of Polynemus sexfilis Valenciennes in Cuvier and Valenciennes, 1831, in the synonym of which the former is herein included. Polydactylus sexfilis, which is widely distributed in the Indo-Pacific region, is redescribed on the basis of a newly-designated lectotype and two paralectotypes, and a wide range of non-type material. The species is characterized by six pectoral filaments, 15 or 16 pectoral fin rays, 61–67 pored lateral line scales, 8–10 scales above the lateral line, 12–14 below, 11–14 and 15–18 upper and lower series gill rakers, respectively (27–31 total), teeth present on vomer and a long second dorsal fin ray (mean 26% [range 21–30%] of standard length). Received: July 7, 2000 / Revised: August 29, 2000 / Accepted: September 30, 2000     

A natural anthropology of art

This article discusses the relevance of recent developments in neurobiology for the study of art worldwide. It is argued that both anthropologists and art historians can gain a better understanding of species-specific art and art practices over time and space by broadening their vision and approaches, building on the fundamental and exciting findings concerning art in neurobiology.     

A history of forensic anthropology

Forensic anthropology represents a dynamic and rapidly evolving complex discipline within anthropology and forensic science. Academic roots extend back to early European anatomists but development coalesced in the Americas through high-profile court testimony, assemblage of documented collections and focused research. Formation of the anthropology section of the American Academy of Forensic Sciences in 1972, the American Board of Forensic Anthropology in 1977/1978 and other organizational advances provided important stimuli for progress. While early pioneers concentrated on analysis of skeletonized human remains, applications today have expanded to include complex methods of search and recovery, the biomechanics of trauma interpretation, isotopic analysis related to diet and region of origin, age estimation of the living and issues related to humanitarian and human rights investigations.     

A history of physical anthropology and the development of evolutionary thought in Canada

Canada is a large country geographically, but relatively small in terms of population. As a result, our major problem has always been communication. It is expensive and time consuming to get together to discuss problems. For example, a scholar in Newfoundland would find Prague to be as far away as a visit to a university in her own country (British Columbia). Then too, communication is often confounded by language because we are a bilingual country, but relatively few people speak both languages. Understandably, then, it is very difficult to speak of a national identity on such a topic as human evolution. Rather, we find that individuals have influenced the field either through their research or through their teaching. Academic development has been strongly influenced in the past by developments in the United States and Europe, particularly the United Kingdom. Nevertheless, we will attempt to trace the development of evolutionary throught, but not without a preliminary apology for oversights to our colleagues. The task is difficult, and we will surely overlook some names. We hope that our attempt will still be accurate in the wholistic sense. Regardless of the foregoing problems, we do see a broad historical development and the beginning of a Canadian perspective. This is more clearly seen if we trace the development of evolutionary thought within the context of the history of physical anthropology in Canada.     

Epilogue — Demography and anthropology

As a conclusion, this paper reviews briefly the content of the volume. The wealth of demographic data has not been adequately exploited in anthropology; this is why this publication is valuable in showing attempts to apply demographic data in a variety of anthropological problems. This symposium has explored many interesting points which we recall here. Yet it has also opened up a whole range of further questions on the material presented as well as in this broad field. Several directions of research could be developed, for instance, testing among human populations, over long periods, the ecological thoughts of ecosystems evolving as a cascade of instabilities, rather than a succession of equilibrium states. Let us also recall the pervasive nature of demographic facts in topics such as the energy cycle or the genetic structure and evolution of human populations.