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kuru

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kuru

Organic brain disease first reported in the eastern highlands of Papua New Guinea in the early 1950s. It is a degenerative condition similar to Creutzfeldt-Jakob disease. Victims suffered rapid physical and mental deterioration, culminating in paralysis, coma, and death.

In 1965 US researchers succeeded in transferring kuru to chimpanzees by means of contaminated human tissue. It thus became the first degenerative disease of the human central nervous system shown to be caused by a transmissible agent (see transmissible spongiform encephalopathy). The disease declined in incidence with a change in burial practices - the cause was ritual cannibalism when people at the brains of their deceased relatives - and had virtually disappeared by the late 1970s. However, in 2000, 11 new cases of kuru came to light. The new cases indicate that the incubation period for the disease can be much longer than previously thought.


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Outbreaks of prion disease include an epidemic of kuru among the cannibalistic Fore tribe of the New Guinea highlands (9) and an epizootic of BSE in the United Kingdom, caused by feeding to cattle protein supplements derived from prion-infected cattle offal (10).
Scientists have discovered that the condition, called fatal familial insomnia, arises from a genetic mutation that creates prions, the same kind of misshapen proteins that cause scrapie, mad cow disease, kuru, and Creutzfeldt-Jakob disease.
It is a prion disease requiring a protein component (prion) for transmissibility, similar to kuru, bovine spongiform encephalopathy (mad cow disease), and scrapie.
 
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