Kuru (disease)

Kuru is a rare, incurable, and invariably fatal neurodegenerative disorder that was primarily found among the Fore people of Papua New Guinea. It is believed to be caused by the transmission of abnormally folded proteins known as prions, and is most known for its association with ritualistic cannibalism.

Cerebellum of kuru victim

Etiology

Kuru belongs to a class of diseases called transmissible spongiform encephalopathies (TSEs), also known as prion diseases. These are caused by misfolded proteins called prions, which can induce normal proteins in the brain to also misfold, leading to brain damage.

Kuru is thought to have spread among the Fore people through a practice of mortuary cannibalism, wherein family members consumed the bodies of the deceased to return the "life force" of the deceased to the hamlet. This practice was stopped in the 1950s, and as a result, Kuru has largely disappeared.

Symptoms

The disease has an incubation period of several years, even up to several decades. Symptoms of Kuru include muscle twitching and loss of coordination, leading to difficulty walking. This is followed by more severe neurological and motor dysfunctions such as dementia, emotional instability, depression, tremors, and eventually an inability to move or eat.

Diagnosis

There are no definitive tests for Kuru in living patients. It is primarily diagnosed based on symptoms and the patient's medical and cultural history. The only conclusive test is a postmortem examination of brain tissue.

Treatment

There is no cure or treatment for Kuru. Care is supportive and aimed at relieving symptoms and maintaining patient comfort.

Prognosis

Kuru is invariably fatal, typically within one year of the onset of symptoms. In the final stages of the disease, patients often cannot move or speak, and they require full nursing care.

See Also

• Prion
• Transmissible spongiform encephalopathies
• Creutzfeldt-Jakob disease

References

• Collinge, J., Whitfield, J., McKintosh, E., Beck, J., Mead, S., Thomas, D. J., & Alpers, M. P. (2006). Kuru in the 21st century—an acquired human prion disease with very long incubation periods. The Lancet, 367(9528), 2068-2074.
• Lindenbaum, S. (1979). Kuru sorcery: Disease and danger in the New Guinea highlands. Palgrave Macmillan.
• Prusiner, S. B. (1995). The prion diseases. Scientific American, 272(1), 48-57.

External Links

• Kuru: the dynamics of a prion disease

Kuru (disease) Resources
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NIH genetic and rare disease info

• NIH info on Kuru (disease)

Kuru (disease) is a rare disease.