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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. The last reported patient died in 2005 with an incubation period extending over four decades.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http: //www.orphadata.org. Data version October 2023.

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