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By Pierluigi Gambetti, MD, 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. 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.

* This is the Professional Version. *