Kuru

A slowly progressive fatal disease of the brain due to an infectious agent transmitted among people in Papua New Guinea by ritual cannibalism. Kuru is an infectious form of subacute spongiform encephalopathy. It was once thought to be due to a slow virus but 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. The disease causes a gait disturbance (cerebellar ataxia), incoordination, trembling and shivering. In time, difficulty in swallowing and feeding oneself leads to malnutrition. Death supervenes within several years of the onset of the disease. Kuru is also called trembling disease (kuru means trembling). The discovery of kuru is one of the more interesting detective stories of 20th-century medicine. Dr. D. Carlton Gajdusek (1923-) 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. In 1976 Gajdusek shared the Nobel Prize in Physiology or Medicine for "discoveries concerning new mechanisms for the origin and dissemination of infectious diseases."

* * *

A progressive, fatal form of spongiform encephalopathy endemic to Fore people in the highlands of New Guinea, initially attributed to a “slow virus” infection, but now known to be caused by prions. Transmission is believed to be effected by contamination and ingestion during ritual cannabalism. It is characterized by ataxia, tremors, lack of coordination and death; pathological lesions in the brain include neuronal loss, ostrocytosis and status spongiosus. See prion. [native dialect, to shiver from fear or cold]

* * *

ku·ru 'ku̇(ə)r-(.)ü n a rare progressive fatal spongiform encephalopathy that is caused by a prion, resembles Creutzfeldt-Jakob disease, and has occurred among tribespeople in eastern New Guinea who engaged in a form of ritual cannibalism called also laughing death, laughing sickness

* * *

(trembling disease)

n.

a disease that affects only members of the Fore tribe of New Guinea. It involves a progressive degeneration of the nerve cells of the central nervous system, particularly in the region of the brain that controls movement. Muscular control becomes defective and shiver-like tremors occur in the trunk, limbs, and head. Kuru affects mainly women and children and usually proves fatal within 9-12 months. It is thought to be caused by a prion and transmitted by cannibalism. See also spongiform encephalopathy.

* * *

ku·ru (kooґroo) [“shivering” in language of the Fore people of New Guinea] an infectious form of prion disease with a long incubation period, found only among the Fore and neighboring peoples of New Guinea and thought to be associated with ritual cannibalism. It is manifested by truncal and limb ataxia, a shivering-like tremor, and dysarthria and ends invariably in death; strabismus and extrapyramidal symptoms may also be found. Amyloid plaques are present in about two thirds of affected individuals.