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Kuru

ORPHA:454745· ICD-10 A81.8

Definition

A rare acquired human prion disease characterized by rapidly progressive, fatal neurodegeneration, caused by the consumption of prion-containing tissue in endocannibalistic funeral rituals in Papua New Guinea until the late 1950s. After a decades-long asymptomatic period and a non-specific prodromal phase with headaches and arthralgia, the most prominent neurological feature is ataxia, in addition to other symptoms involving the cerebellum, brain stem, mid-brain, hypothalamus, and cerebral cortex, and emotional changes including inappropriate euphoria and compulsive laughter, or depression and apprehension. There have been no further description in the literature since 2004.

Prevalence
<1 / 1 000 000
Inheritance
Multigenic/multifactorial, Not applicable
Age of onset
All ages