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Brain, Spinal Cord, and Nerve Disorders
Prion Diseases
Kuru
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    Kuru

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    Kuru is a prion disease that causes rapid deterioration of mental function and loss of muscle coordination. This disease used to occur in the Fore natives of the New Guinea highlands and is related to ritual endocannibalism.

    Until the early 1960s, kuru was fairly common in New Guinea. Prions were probably acquired during a cannibalistic ritual accompanying the care of the dead and involving eating tissues of a dead relative as a sign of respect. Kuru probably started from the consumption of prion-contaminated tissues from a person affected by Creutzfeldt-Jakob disease. Kuru was more common among women and children because they were given the brain to eat. Many of these rituals have been abandoned, and kuru has been virtually eliminated.

    Symptoms include loss of muscle coordination and difficulty walking. The limbs become stiff, and muscles twitch. Abnormal involuntary movements, such as repetitive, slow writhing or rapid jerking of the limbs and body, may develop (kuru means shivering). Emotions may switch suddenly from sadness to happiness with sudden outbursts of laughter. People with kuru become demented and eventually placid, unable to speak, and unresponsive to their surroundings.

    Most people die about 3 to 24 months after symptoms appear, usually as a result of pneumonia or infection due to bedsores (pressure sores).

    Last full review/revision January 2007 by Pierluigi Gambetti, MD

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