A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea

Collinge, John, Whitfield, Jerome, McKintosh, Edward, Frosh, Adam, Mead, Simon, Hill, Andrew F ORCID: 0000-0001-5581-2354, Brandner, Sebastian, Thomas, Dafydd and Alpers, Michael P (2008) A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea. Philosophical Transactions of the Royal Society B: Biological Sciences, 363 (1510). pp. 3725-3739. ISSN 0962-8436


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-Jakob 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 46-63 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 PRNP 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. © 2008 The Royal Society.

Dimensions Badge

Altmetric Badge

Item type Article
URI https://vuir.vu.edu.au/id/eprint/45316
DOI 10.1098/rstb.2008.0068
Official URL https://royalsocietypublishing.org/doi/10.1098/rst...
Subjects Current > FOR (2020) Classification > 3207 Medical microbiology
Current > Division/Research > Chancellery
Keywords Papua New Guinea, epidemic prion disease, prion, variant Creutzfeldt–Jakob disease, vCJD, genetics, neurological studies
Citations in Scopus 62 - View on Scopus
Download/View statistics View download statistics for this item

Search Google Scholar

Repository staff login