kuru

kuru /ku·ru/ (koo´roo) an infectious form of prion disease with a long incubation period found only in New Guinea and thought to be associated with ritual cannibalism.

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ku·ru (kr)

n.

A progressive, fatal spongiform encephalopathy, probably caused by a slow-acting virus, that is endemic to certain peoples of New Guinea and may be transmitted through cannibalism.

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kuru

[ko̅o̅′ro̅o̅]

Etymology: New Guinea, trembling with fear

a slow, progressive, fatal infection of the central nervous system that was endemic to natives of the New Guinea highlands. The incubation period could be 30 or more years, but death usually occurred within months of the onset of symptoms. It was characterized by ataxia and decreased coordination progressing to paralysis, dementia, slurring of speech, and visual disturbances. Disease was transmitted by ritual cannibalism of brain tissue during funeral rites. No new cases have been recorded since cessation of the cannibalism. This disease is a model for prion diseases such as BSE and variant CJD.

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Kuru

A subacute spongiform encephalopathy, caused by a prion which, in its heyday, killed 90% of women in the once cannibalistic Fore tribe of New Guinea—women were most affected as they prepared and ate infected brains

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prion [pri´on]

any of several pathogenic, transmissible forms of the core of prion protein that cause a group of degenerative diseases of the nervous system known as prion diseases. Prions have a structure different from that of normal prion protein, lack detectable nucleic acid, and do not elicit an immune response.

prion disease any of a group of fatal degenerative diseases of the nervous system caused by abnormalities in the metabolism of prion protein. These diseases are unique in that they may be transmitted genetically as an autosomal dominant trait, or by infection with abnormal forms of the protein (prions). Inherited forms result from mutations in the gene that codes for prion protein; such mutations may also occur sporadically. Hereditary forms include some forms of Creutzfeldt-Jakob disease, Gerstmann-Sträussler syndrome, and fatal familial insomnia. Infectious forms of the disease result from ingestion of infected tissue or the introduction of infected tissue into the body (kuru and some forms of Creutzfeldt-Jakob disease). The latter has occasionally occurred during surgical procedures; it has also occurred as the result of injection of human growth hormone prepared from infected pituitary glands. Prion diseases also occur in animals. Called also transmissible neurodegenerative disease and subacute spongiform or transmissible spongiform encephalopathy.

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kuru

a chronic, progressive, uniformly fatal central nervous system prion disease of humans probably resembling the scrapie agent of sheep, and transmissible to nonhuman primates; seen only in the Fore and neighboring peoples of New Guinea. Believed to be transmitted by cannibalism.