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Kuru

Definition

Kuru is a disease of the nervous system.

Causes, incidence, and risk factors

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

Kuru is found among people from New Guinea who practiced a form of cannibalism in which they ate the brains of dead people as part of a funeral ritual. This practice stopped in 1960, but cases of kuru were reported for many years afterward because the disease has a long incubation period.

Kuru causes brain and nervous system 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:

  • Arm and leg pain
  • Coordination problems that become severe
  • Difficulty walking (cerebellar ataxia)
  • Headache
  • Swallowing difficulty
  • Tremors and muscle jerks (myoclonus)

Difficulty swallowing and beiong unable to feed oneself. This can lead to malnutrition or starvation.

The average time from exposure to symptoms (incubation period) is 10 to 13 years, but incubation periods of 30 years or even longer have been reported.

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

Bosque PJ, Tyler KL. Prions and prion diseases of the central nervous system (transmissible neurodegenerative diseases). In: Mandell GL, Bennett JE, Dolan R, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 7th ed. Orlando, FL:Saunders Elsevier;2009:chap 178.

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


Review Date: 12/7/2010
Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; Jatin M. Vyas, MD, PhD, Assistant Professor in Medicine, Harvard Medical School, Assistant in Medicine, Division of Infectious Disease, Department of Medicine, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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