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Definition of Kuru

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Kuru: Kuru is a slowly progressive fatal disease of the brain caused by an infectious agent transmitted among  the South Fore people in Papua New Guinea.

Kuru is a form of subacute spongiform encephalopathy. It was once thought to be due to a slow virus, but it is now believed to be caused by a tiny protein particle called a prion. It appears to be similar to bovine spongiform encephalopathy (BSE or "mad cow disease") and Creutzfeldt-Jakob disease.

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The disease causes a gait disturbance, incoordination, trembling, and shivering. In time, difficulty in swallowing and feeding oneself leads to malnutrition. Death usually occurs  within several years of the onset of the disease. Kuru is also called trembling disease (kuru means trembling). In sheep and goats, a similar disease is called scrapie.

The discovery of kuru is one of the more interesting detective stories of 20th-century medicine. Dr. D. Carlton Gajdusek (1923-2008) first described the disease among the Fore people of New Guinea. It was known by them as kuru (meaning "trembling"). After years of living among the Fore people, Gajdusek came to the conclusion that the disease was transmitted in the ritualistic eating of the brains of the deceased, a Fore funeral custom. With the elimination of cannibalism, Kuru disappeared within a generation. In 1976 Gajdusek shared the Nobel Prize in Physiology or Medicine for "discoveries concerning new mechanisms for the origin and dissemination of infectious diseases."

 

WebMD Medical Reference

Reviewed by Richard Senelick, MD on January 30, 2013

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