Kuru (disease)

Not to be confused with Koro (disease).
Kuru
A Fore child with advanced kuru. He is unable to walk or sit upright without assistance and is severely malnourished.
Pronunciation
  • IPA: kuru
SpecialtyNeuropathology, infectious disease
SymptomsBody tremors, random outbursts of laughter, emotional degradation, gradual loss of coordination
ComplicationsInfection and pneumonia during the terminal stage.
Usual onsetApproximately 10-50 years after initial exposure.
Duration11–14 month life expectancy after onset of symptoms[1]
CausesTransmission of infected prion proteins
Risk factorsCannibalism
Diagnostic methodAutopsy
Differential diagnosisCreutzfeldt–Jakob disease
PreventionAvoiding practices of cannibalism
TreatmentSupportive care
PrognosisFatal
FrequencyRare
DeathsApproximately 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. Kuru is a form of 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 the "laughing sickness" due to the pathologic bursts of laughter which are a symptom of the infection.

It is now widely accepted that kuru was transmitted 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 consumed the brain, the organ in which infectious prions were most concentrated, thus allowing for transmission of kuru. The disease was therefore more prevalent among women and children.

The epidemic 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 consuming human meat in the early 1960s, when it was first speculated to be transmitted via endocannibalism, the disease lingered due to kuru's long incubation period of anywhere from 10 to over 50 years.[6] The epidemic finally declined sharply after half a century, from 200 deaths per year in 1957 to no deaths from at least 2010 onwards, but sources disagree on whether the last known kuru victim died in 2005 or 2009.[7][8][9][10]

  1. ^ "The epidemiology of kuru in the period 1987 to 1995", Department of Health (Australia), retrieved February 5, 2019
  2. ^ 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.
  3. ^ 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.
  4. ^ 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.
  5. ^ Bichell, Rae Ellen (September 6, 2016). "When People Ate People, A Strange Disease Emerged". NPR.org. Retrieved 2018-04-08.
  6. ^ "Kuru". MedlinePlus Medical Encyclopedia. Retrieved 2016-11-14.
  7. ^ Alpers, MP (2007). "A history of kuru". Papua and New Guinea Medical Journal. 50 (1–2): 10–9. PMID 19354007.
  8. ^ Rense, Sarah (September 7, 2016). "Here's What Happens to Your Body When You Eat Human Meat". Esquire.
  9. ^ Cite error: The named reference Lifeof was invoked but never defined (see the help page).
  10. ^ 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.