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Central nervous system
Central nervous system

Kuru
Definition:
Kuru is a neurodegenerative disease caused by a prion (infectious protein) which is transmitted to humans via ingestion of contaminated human brain tissue.

Causes, incidence, and risk factors:

Kuru is a rare prion disease, originally described among New Guineans. Kuru causes neurodegenerative changes similar to another prion disease, Creutzfeldt-Jakob disease, whose distribution is worldwide. In addition, similar prion diseases appear in sheep as scrapie, mink as mink encephalopathy, and in cows as bovine spongiform encephalopathy (BSE). The main risk factor in the development of kuru has been the practice of cannibalism, which results in contamination with infected brain tissue.

Kuru may begin with a gait disturbance (cerebellar ataxia) and increasing incoordination. Incoordination and unsteady gait eventually leads to severe disability. Tremors and muscle jerks are often seen in the later stages of the disease. Difficulty in swallowing and inability to feed oneself lead to malnutrition or starvation.

The incubation period (time to the development of symptoms) for this disease can be up to 30 years or longer. Death, however, usually occurs within a year after the onset of symptoms.
Symptoms:
Signs and tests:
Neurologic evaluation may show characteristic changes in coordination and gait in someone with an appropriate exposure history.
Treatment:
No treatment is currently available for kuru or any of the prion diseases.
Expectations (prognosis):
Kuru is universally fatal.
Calling your health care provider:
Call your health care provider if you have any gait disturbance, swallowing difficulty, or incoordination. Although kuru is extremely rare (less than 10 patients per year), other severe neurologic disorders can cause these symptoms and should be evaluated.
Prevention:
The incidence of kuru diminished considerably with the discontinuation of cannibalism and ritualistic practices which expose humans to infectious brain tissue.
Review Date: 8/9/2001
Reviewed By: Rocio Hurtado, M.D., Infectious Diseases Division, Massachusetts General Hospital and Brigham and Women's Hospital, Boston, MA. Review provided by VeriMed Healthcare Network.
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