Abstract
Kuru, the first human transmissible spongiform encephalopathy was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, I briefly summarize the history of this seminal discovery along its epidemiology, clinical picture, neuropathology and molecular genetics. The discovery of kuru opened new windows into the realms of human medicine and was instrumental in the later transmission of Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease as well as the relevance that bovine spongiform encephalopathy had for transmission to humans. The transmission of kuru was one of the greatest contributions to biomedical sciences of the 20th century.
Highlights
Kuru is a disease that will forever be linked with the name of D
It was the first human prion disease transmitted to chimpanzees and subsequently classified as a transmissible spongiform encephalopathy (TSE), or slow unconventional virus disease
Proved that kuru is not merely an exotic disease caused by cannibalism on a remote island, but it represents a novel class of diseases caused by a novel class of pathogens
Summary
Kuru is a disease that will forever be linked with the name of D. Carleton Gajdusek [1,2,3,4,5,6,7,8,9,10,11] It was the first human prion disease transmitted to chimpanzees and subsequently classified as a transmissible spongiform encephalopathy (TSE), or slow unconventional virus disease. It was first reported in Western medicine in 1957 by Gajdusek and Vincent Zigas (Figure 1) [12,13]. South Fore moved temporarily to live with the Yar people and settled there for about a decade, they still had kuru cases. Enough, when kuru first appeared, it was considered poetically by Foré as similar to “the swaying of casuarinas tree” and kuru was labeled cassowary disease to stress the similarity between cassowary quills and “waving casuarinas fronds”
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