Kuru

Definition

Kuru is a disease of the nervous system.

Causes, incidence, and risk factors

Kuru is an extremely rare disease. It is caused by an infectious protein (prion) found in contaminated human brain tissue.

Kuru is found almost exclusively among people from New Guinea, who practiced a form of cannibalism in which the brains of dead people were eaten as part of a funeral ritual.

Kuru causes neurodegenerative changes similar to Creutzfeldt-Jakob disease. Similar diseases appear in cows as bovine spongiform encephalopathy (BSE), also called mad cow disease.

The main risk factor for kuru is eating human brain tissue, which can contain the infectious particles.

Symptoms

Symptoms of kuru include:

• Coordination problems, eventually becoming severe
• Difficulty walking (cerebellar ataxia)
• Swallowing difficulty
• Tremors and muscle jerks (myoclonus)

Difficulty swallowing and the inability to feed oneself lead to malnutrition or starvation.

It can take up to 30 years or even longer to develop symptoms (incubation period).

Signs and tests

A neurological exam may show changes in coordination and walking ability.

Treatment

There is no known treatment for kuru.

Expectations (prognosis)

Death usually occurs within 1 year after the first sign of symptoms.

Calling your health care provider

Call your health care provider if you have any walking, swallowing, or coordination problems. Kuru is extremely rare. Your doctor will rule out other neurological diseases.

References

Zeidler M. Prion diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: chap 442.