MSD Manual

Please confirm that you are a health care professional

Loading

Kuru

By

Pierluigi Gambetti

, MD, Case Western Reserve University

Last full review/revision Jul 2020| Content last modified Jul 2020
Click here for Patient Education

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 years.

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

Cerebrospinal fluid (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 years 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.

Click here for Patient Education
NOTE: This is the Professional Version. CONSUMERS: Click here for the Consumer Version
Professionals also read

Also of Interest

Videos

View All
Overview of Epilepsy
Video
Overview of Epilepsy
3D Models
View All
Vertebra and Spinal Nerve Roots
3D Model
Vertebra and Spinal Nerve Roots

SOCIAL MEDIA

TOP