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a progressive disease of the central nervous system marked by increasing lack of coordination and advancing to paralysis and death within a year of the appearance of symptoms; thought to have been transmitted by cannibalistic consumption of diseased brain tissue since the disease virtually disappeared when cannibalism was abandoned

A prion disease found exclusively among the Fore liguistic group natives of the highlands of NEW GUINEA. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773)