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The Merck Manual--Second Home Edition logo
 
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Chapter 90. Prion Diseases
Topics: Introduction | Creutzfeldt-Jakob Disease | Fatal Familial Insomnia | Gerstmann-Sträussler-Scheinker Disease | Kuru
 
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Kuru

Kuru is a prion disease that causes rapid deterioration of mental function and that used to occur in the Fore natives of the New Guinea highlands.

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 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. 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 after about 3 to 24 months of illness, usually as a result of pneumonia or infection resulting from bedsores (pressure sores).

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