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By Pierluigi Gambetti, MD, Professor of Pathology, Case Western Reserve University

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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 of people with kuru. However, a PrP gene variation that protects against prion disease has been identified in people in the Papua population who have not contracted kuru (1). Autopsy can show typical PrPSc-containing plaques, with the greatest density in the cerebellum.

Death usually occurs within 2 yr after symptoms begin; cause of death is usually pneumonia or infection due to pressure sores.

There is only supportive treatment for kuru.

General reference

  • 1. Asante EA, Smidak M, Grimshaw A, et al: A naturally occurring variant of the human prion protein completely prevents prion disease. Nature 522 (7557):478–481, 2015. doi: 10.1038/nature14510.