Kuru (disease)

Kuru is a disease which affects the brain. It is endemic among the Fore tribe of Papua New Guinea and is universally fatal. It is characterized by headaches, joint pains and shaking of the limbs. It is believed to be caused by prions and is related to Creutzfeldt-Jakob disease.^[1] It is best known for the epidemic that occurred in Papua New Guinea in the middle of the twentieth century.^[2] The word kuru means "trembling with fear" in the language of the Fore people, those most commonly afflicted with the disease.^[3] Trembling is a symptom of dying brain tissue, and as such is present in almost all patients with transmissible spongiform encephalopathy.

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History

Kuru was first noted in native peoples in the early part of the 1900s. It was in the 1950s that the full extent of the disease was realized, after it had reached large infection rates in the South Fore of the Okapa Subdistrict.^[4]

The disease was researched by Daniel Carleton Gajdusek who was awarded (with Baruch S. Blumberg) the Nobel Prize in Physiology or Medicine in 1976 for his work on it.

Transmission

The disease spread easily in the Fore people due to their cannibalistic practices. The dysmorphism evident in the infection rates -- it was more prevalent in women and children -- is due to the fact that while the men of the village ate the flesh of the deceased, the women and children ate the brain, where the disease particles are concentrated.^[3]

References

1. ^ Collinge, J; Whitfield J, McKintosh E et al. (Jun 2006). "Kuru in the 21st century — an acquired human prion disease with very long incubation periods". Lancet 367 (9528): 2068-2074.
2. ^ http://www.ninds.nih.gov/disorders/kuru/kuru.htm
3. ^ ^{a b} http://www.emedicine.com/med/topic1248.htm
4. ^ [1]