Kuru (disease)
| Kuru | |
|---|---|
| A Fore child with advanced kuru. He is unable to walk or sit upright without assistance and is severely malnourished. | |
| Pronunciation |
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| Specialty | Neuropathology, infectious disease |
| Symptoms | Body tremors, random outbursts of laughter, emotional degradation, gradual loss of coordination |
| Complications | Infection and pneumonia during the terminal stage. |
| Usual onset | 10-50 years after initial exposure. |
| Duration | 11–14 month life expectancy after onset of symptoms[1] |
| Causes | Transmission of infected prion proteins |
| Risk factors | Cannibalism |
| Diagnostic method | Autopsy |
| Differential diagnosis | Creutzfeldt–Jakob disease |
| Prevention | Avoiding cannibalism |
| Treatment | Supportive care |
| Prognosis | Fatal |
| Frequency | Rare |
| Deaths | Approximately 2,700 as of 2005 |
Kuru is a rare, incurable, and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. It is a prion disease which leads to tremors and loss of coordination from neurodegeneration. The term kúru means "trembling" and comes from the Fore word kuria or guria ("to shake").[2][3] It is also known as "laughing sickness" due to abnormal bursts of laughter which occur.
It was spread among the Fore people via funerary cannibalism. Deceased family members were traditionally cooked and eaten, which was thought to help free the spirit of the dead.[4] Women and children usually ate the brain, where infectious prions were most concentrated, and therefore were more commonly affected.
The outbreak likely started when a villager developed sporadic Creutzfeldt–Jakob disease and died. When villagers ate the brain, they contracted the disease and then spread it to other villagers who ate their infected brains.[5]
While the Fore people stopped eating human meat in the early 1960s, when this was first speculated as the cause, the disease lingered due to kuru's long incubation period of anywhere from 10 to over 50 years.[6] Cases finally declined after half a century, from 200 deaths per year in 1957 to no deaths from at least 2010 onward, with the last known death in 2005 or 2009.[7][8][9][10]
- ^ "The epidemiology of kuru in the period 1987 to 1995", Department of Health (Australia), retrieved February 5, 2019
- ^ Hoskin, J.O.; Kiloh, L.G.; Cawte, J.E. (April 1969). "Epilepsy and guria: The shaking syndromes of New Guinea". Social Science & Medicine. 3 (1): 39–48. doi:10.1016/0037-7856(69)90037-7. PMID 5809623.
- ^ Scott, Graham (1978). The Fore Language of Papua New Guinea. Pacific Linguistics. pp. 2, 6. doi:10.15144/PL-B47. hdl:1885/146489. ISBN 978-0-85883-173-5.
- ^ Whitfield, Jerome T; Pako, Wandagi H; Collinge, John; Alpers, Michael P (27 November 2008). "Mortuary rites of the South Fore and kuru". Philosophical Transactions of the Royal Society B: Biological Sciences. 363 (1510): 3721–3724. doi:10.1098/rstb.2008.0074. PMC 2581657. PMID 18849288.
- ^ Bichell, Rae Ellen (September 6, 2016). "When People Ate People, A Strange Disease Emerged". NPR.org. Retrieved 2018-04-08.
- ^ "Kuru". MedlinePlus Medical Encyclopedia. Retrieved 2016-11-14.
- ^ Alpers, MP (2007). "A history of kuru". Papua and New Guinea Medical Journal. 50 (1–2): 10–9. PMID 19354007.
- ^ Rense, Sarah (September 7, 2016). "Here's What Happens to Your Body When You Eat Human Meat". Esquire.
- ^ Cite error: The named reference
Lifeofwas invoked but never defined (see the help page). - ^ Collinge, John; Whitfield, Jerome; McKintosh, Edward; Beck, John; Mead, Simon; Thomas, Dafydd J; Alpers, Michael P (June 2006). "Kuru in the 21st century—an acquired human prion disease with very long incubation periods". The Lancet. 367 (9528): 2068–2074. doi:10.1016/S0140-6736(06)68930-7. PMID 16798390. S2CID 11506094.