Citation, DOI & article data
Kuru is a human prion disease that occurred in parts of Papua New Guinea. It is an acquired disease, transmitted from cultural practices of mortuary feasts. Kuru was common before the 1960s with the last death reported in 2009. Unlike sporadic Creutzfeldt–Jakob disease, cerebellar ataxia is a more prominent component than dementia. It is a universally fatal condition.
Kuru is now virtually an extinct condition, and formal epidemiological monitoring in Papua New Guinea was stopped in 2012, a remarkable 55 years of almost continual fieldwork monitoring the affected regions 8-10. In total 2700 cases of kuru were documented, and no-one born after 1959 has been affected by the condition 8-10.
Since 1996 only 14 new cases have been recorded, and the last recorded death from kuru was in 2009; moreover there had been only two other deaths from it in the period 2002-2006 10. There are no recorded recoveries from the condition 8-10.
Since kuru was prevalent before the advent of CT/MRI and new cases have been rare since 1980s, the neuroimaging findings of kuru have never been published 7.
History and etymology
Kuru is the word used by the Foré peoples of the Eastern Highland province of Papua New Guinea to describe the "shivering tremors" characteristic of the disease 3.
The disease was first described by Vincent Zigas (1920-1983), a Lithuanian physician working in Papua New Guinea, and Daniel Carleton Gajdusek (1923-2008), an American virologist working at the Walter and Eliza Hall Institute, Melbourne, Australia, in 1957 4-6.
- 1. Wadsworth J, Joiner S, Linehan J, Asante E, Brandner S, Collinge J. The Origin of the Prion Agent of Kuru: Molecular and Biological Strain Typing. Phil Trans R Soc B. 2008;363(1510):3747-53. doi:10.1098/rstb.2008.0069
- 2. Mathews J. The Changing Face of Kuru: A Personal Perspective. Phil Trans R Soc B. 2008;363(1510):3679-84. doi:10.1098/rstb.2008.0085
- 3. Asher D & Gregori L. Human Transmissible Spongiform Encephalopathies: Historic View. Human Prion Diseases. 2018;:1-17. doi:10.1016/b978-0-444-63945-5.00001-5
- 4. Zigas V & Gajdusek D. Kuru: Clinical Study of a New Syndrome Resembling Paralysis Agitans in Natives of the Eastern Highlands of Australian New Guinea. Medical Journal of Australia. 1957;2(21):745-54. doi:10.5694/j.1326-5377.1957.tb60287.x
- 5. Goudsmit J. Daniel Carleton Gajdusek (1923–2008). Nature. 2009;457(7228):394. doi:10.1038/457394a
- 6. Gajdusek D. Vincent Zigas1920-1983. Neurology. 1983;33(9):1199. doi:10.1212/wnl.33.9.1199
- 7. Macfarlane R, Wroe S, Collinge J, Yousry T, Jäger H. Neuroimaging Findings in Human Prion Disease. J Neurol Neurosurg Psychiatry. 2007;78(7):664-70. doi:10.1136/jnnp.2006.094821
- 8. Pedersen N & Smith E. Prion Diseases: Epidemiology in Man. APMIS. 2002;110(1):14-22. doi:10.1034/j.1600-0463.2002.100103.x
- 9. Alpers M. Review. The Epidemiology of Kuru: Monitoring the Epidemic from Its Peak to Its End. Philos Trans R Soc Lond B Biol Sci. 2008;363(1510):3707-13. doi:10.1098/rstb.2008.0071
- 10. Whitfield J, Pako W, Collinge J, Alpers M. Cultural Factors That Affected the Spatial and Temporal Epidemiology of Kuru. R Soc Open Sci. 2017;4(1):160789. doi:10.1098/rsos.160789