{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T03:11:24Z","timestamp":1792293084356,"version":"4.4.0"},"reference-count":73,"publisher":"The Royal Society","issue":"1510","license":[{"start":{"date-parts":[[2008,11,27]],"date-time":"2008-11-27T00:00:00Z","timestamp":1227744000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/royalsociety.org\/journals\/ethics-policies\/data-sharing-mining\/"}],"content-domain":{"domain":["royalsocietypublishing.org"],"crossmark-restriction":true},"short-container-title":["Phil. Trans. R. Soc. B"],"published-print":{"date-parts":[[2008,11,27]]},"abstract":"<jats:p>\n                    Kuru is so far the principal human epidemic prion disease. While its incidence has steadily declined since the cessation of its route of transmission, endocannibalism, in Papua New Guinea in the 1950s, the arrival of variant Creutzfeldt\u2013Jakob disease (vCJD), also thought to be transmitted by dietary prion exposure, has given kuru a new global relevance. We investigated all suspected cases of kuru from July 1996 to June 2004 and identified 11 kuru patients. There were four females and seven males, with an age range of 46\u201363 years at the onset of disease, in marked contrast to the age and sex distribution when kuru was first investigated 50 years ago. We obtained detailed histories of residence and exposure to mortuary feasts and performed serial neurological examination and genetic studies where possible. All patients were born a significant period before the mortuary practice of transumption ceased and their estimated incubation periods in some cases exceeded 50 years. The principal clinical features of kuru in the studied patients showed the same progressive cerebellar syndrome that had been previously described. Two patients showed marked cognitive impairment well before preterminal stages, in contrast to earlier clinical descriptions. In these patients, the mean clinical duration of 17 months was longer than the overall average in kuru but similar to that previously reported for the same age group, and this may relate to the effects of both patient age and\n                    <jats:italic>PRNP<\/jats:italic>\n                    codon 129 genotype. Importantly, no evidence for lymphoreticular colonization with prions, seen uniformly in vCJD, was observed in a patient with kuru at tonsil biopsy.\n                  <\/jats:p>","DOI":"10.1098\/rstb.2008.0068","type":"journal-article","created":{"date-parts":[[2008,10,14]],"date-time":"2008-10-14T01:52:55Z","timestamp":1223949175000},"page":"3725-3739","update-policy":"https:\/\/doi.org\/10.1098\/crossmark-policy","source":"Crossref","is-referenced-by-count":73,"title":["A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea"],"prefix":"10.1098","volume":"363","author":[{"given":"John","family":"Collinge","sequence":"first","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jerome","family":"Whitfield","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"},{"name":"Papua New Guinea Institute of Medical ResearchPO Box 60, Goroka, EHP 441, Papua New Guinea"},{"name":"Centre for International Health, ABCRC, Shenton Park Campus, Curtin UniversityGPO Box U1987, Perth, WA 6845, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edward","family":"McKintosh","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adam","family":"Frosh","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Mead","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew F","family":"Hill","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sebastian","family":"Brandner","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dafydd","family":"Thomas","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael P","family":"Alpers","sequence":"additional","affiliation":[{"name":"MRC Prion Unit and Department of Neurodegenerative Disease, UCL Institute of Neurology, The National Hospital for Neurology and NeurosurgeryQueen Square, London WC1N 3BG, UK"},{"name":"Papua New Guinea Institute of Medical ResearchPO Box 60, Goroka, EHP 441, Papua New Guinea"},{"name":"Centre for International Health, ABCRC, Shenton Park Campus, Curtin UniversityGPO Box U1987, Perth, WA 6845, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"175","published-online":{"date-parts":[[2008,11,27]]},"reference":[{"key":"e_1_3_2_2_1","first-page":"65","volume-title":"NINDB Monograph No. 2","author":"Alpers M","year":"1964"},{"key":"e_1_3_2_3_1","unstructured":"Alpers M Kuru: a clinical study. 1964b Adelaide Australia:Department of Medicine University of Adelaide."},{"key":"e_1_3_2_4_1","unstructured":"Alpers M Kuru: age and duration studies. 1964c Adelaide Australia:Department of Medicine University of Adelaide."},{"key":"e_1_3_2_5_1","first-page":"234","volume-title":"The central nervous system, some experimental models of neurological diseases","author":"Alpers M","year":"1968"},{"key":"e_1_3_2_6_1","first-page":"67","volume-title":"Slow transmissible diseases of the nervous system, vol. 1, Clinical, epidemiological, genetic and pathological aspects of the spongiform encephalopathies","author":"Alpers M","year":"1979"},{"key":"e_1_3_2_7_1","first-page":"451","volume-title":"Prions: novel infectious pathogens causing scrapie and Creutzfeldt\u2013Jakob disease","author":"Alpers M","year":"1987"},{"key":"e_1_3_2_8_1","unstructured":"Alpers M. 1999 Burnet oration to the joint meeting of the Australasian Society for Infectious Diseases and the Australasian College of Tropical Medicine Palm Cove Cairns."},{"key":"e_1_3_2_9_1","first-page":"391","article-title":"The epidemiology of kuru in the period 1987 to 1995","volume":"29","author":"Alpers M","year":"2005","journal-title":"Commun. 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M. 1962 The spread of kuru among the Fore. Department of Public Health Papua New Guinea 6 pp."},{"key":"e_1_3_2_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.2164-0947.1967.tb02298.x"},{"key":"e_1_3_2_41_1","doi-asserted-by":"publisher","DOI":"10.1038\/2151043a0"},{"key":"e_1_3_2_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(59)92081-1"},{"key":"e_1_3_2_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tim.2003.10.007"},{"key":"e_1_3_2_44_1","doi-asserted-by":"publisher","DOI":"10.1038\/38925"},{"key":"e_1_3_2_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(97)24002-X"},{"key":"e_1_3_2_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(98)12075-5"},{"key":"e_1_3_2_47_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.97.18.10248"},{"key":"e_1_3_2_48_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(98)24035-9"},{"key":"e_1_3_2_49_1","doi-asserted-by":"publisher","DOI":"10.1136\/jcp.2003.012278"},{"key":"e_1_3_2_50_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/91.1.53"},{"key":"e_1_3_2_51_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.73.5.597"},{"key":"e_1_3_2_52_1","first-page":"799","article-title":"Pathology of kuru","volume":"8","author":"Klatzo I","year":"1959","journal-title":"Lab Invest"},{"key":"e_1_3_2_53_1","doi-asserted-by":"publisher","DOI":"10.1159\/000110837"},{"key":"e_1_3_2_54_1","doi-asserted-by":"publisher","DOI":"10.1086\/317935"},{"key":"e_1_3_2_55_1","unstructured":"Lindenbaum S Kuru sorcery: disease and danger in the New Guinea highlands. 1979 Palo Alto CA:Mayfield."},{"key":"e_1_3_2_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)15486-X"},{"key":"e_1_3_2_57_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.101130398"},{"key":"e_1_3_2_58_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10048-002-0133-9"},{"key":"e_1_3_2_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(65)91959-8"},{"key":"e_1_3_2_60_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(68)90482-0"},{"key":"e_1_3_2_61_1","doi-asserted-by":"publisher","DOI":"10.1086\/324710"},{"key":"e_1_3_2_62_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1083320"},{"key":"e_1_3_2_63_1","doi-asserted-by":"publisher","DOI":"10.1038\/352340a0"},{"key":"e_1_3_2_64_1","first-page":"249","volume-title":"NINDB Monograph 2","author":"Pattison I.H","year":"1965"},{"key":"e_1_3_2_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(04)16811-6"},{"key":"e_1_3_2_66_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.6801762"},{"key":"e_1_3_2_67_1","doi-asserted-by":"publisher","DOI":"10.1111\/imj.1959.8.1.8"},{"key":"e_1_3_2_68_1","doi-asserted-by":"publisher","DOI":"10.1006\/geno.2000.6320"},{"key":"e_1_3_2_69_1","doi-asserted-by":"publisher","DOI":"10.1128\/jvi.76.5.2510-2517.2002"},{"key":"e_1_3_2_70_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(01)05403-4"},{"key":"e_1_3_2_71_1","doi-asserted-by":"publisher","DOI":"10.1136\/vr.142.5.103"},{"key":"e_1_3_2_72_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(06)69835-8"},{"key":"e_1_3_2_73_1","doi-asserted-by":"publisher","DOI":"10.5694\/j.1326-5377.1957.tb60287.x"},{"key":"e_1_3_2_74_1","first-page":"1","article-title":"Kuru: clinical, pathological and epidemiological study of a recently discovered acute progressive degenerative disease of the central nervous system reaching \u2018epidemic\u2019 proportions among natives of the Eastern Highlands of New Guinea","volume":"3","author":"Zigas V","year":"1959","journal-title":"Papua New Guinea Med. 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