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Kuru

by Pierluigi Gambetti, MD

Kuru is a rare prion brain disease endemic to Papua New Guinea and thought to be spread by ritual cannibalism.

Although ritual cannibalism ended in the 1950s, 11 new cases of kuru have been reported between 1996 and 2004, suggesting an incubation period that may exceed 50 yr.

Symptoms of kuru begin with tremors (resembling shivering) and ataxia. Movement disorders such as choreoathetosis, fasciculations, and myoclonus develop later, followed by dementia.

CSF testing does not appear to be useful. Few other test results have been reported. No diagnostic abnormalities have been identified in the PrP gene. Autopsy can show typical PrP Sc -reactive plaques, with the greatest density around the cerebellum.

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