First documented in the early 1950s, Kuru is a rare and incurable disease that was common to the Fore people of Papua New Guinea. It was the first chronic degenerative disorder in humans to be shown to be transmissible and paved the way for the discovery of prions, an entirely new type of disease vector. This review aims to provide a short overview of the history, demographics, causes, symptoms, and neuropathology of kuru and how it has influenced research into other neurological disorders.
Kuru is a rare disease, and virtually all cases of the disease have been documented in the tribes inhabiting a subregion of Papua New Guinea. At that, less than 3000 cases were ever documented (Alpers, 2007). In 1957, there were 200 deaths due to kuru reported in the Fore population, and by 2005 only 1 death due to kuru was reported in the population, after which no more kuru-related deaths were reported (Alpers, 2007). Kuru first came under study in 1957 - when Charles Pfarr reported that it had become an epidemic in the Fore people - by a virologist named Daniel Carleton Gajdusek (Alpers, 2007). Gajdusek spent two years in Papua New Guinea examining the disease, after which he performed experiments on chimpanzees (Gajdusek, 1957). In 1960, shortly after his reports about the fact that cannibalism was the most likely cause of the disease, the practice was banned, but research into the disease continued (Alpers, 2007). In his experiments, Gajdusek demonstrated that kuru was transmissible and could cross the human-primate species barrier which he attributed to some unknown disease factor that came from the brain matter of infected humans (Gajdusek, 1966). Prions as the root cause of kuru came later, catalysed by E. J. Field’s work comparing kuru to scrapie and multiple sclerosis and his observation that molecular aggregation was critical to the infection process (Field, 1967).
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