Kuru has been found only among the Fore tribe in Papua New Guinea and has been virtually eliminated since the cessation of the ritual handling and eating of the brains of deceased relatives. The disease is characterized by progressive problems with coordination which are typically followed by dementia.

Kuru and CJD should be thought of as two different spongiform encephalopathies. Kuru studies were instructive since they showed for the first time that a slowly progressive neurological disease of humans can be infectious, that is transmitted from one person to another. For this discovery Carleton Gajdusek was awarded the Nobel Prize. Recently, as the power of molecular biology has been applied to the spongiform encephalopathies, it has become clear that each disease (scrapie, BSE, and others in animals, and CJD, kuru and Gerstmann-Straussler-Scheinker disease in humans) is caused by a distinct variant prion.