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Cited by 110Prion Diseases and Protein MisfoldingAmyloidosis: Diagnosis, Treatment, OutcomesGenetic Neurodegenerative Diseases

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

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Collinge, John, Whitfield, Jerome, McKintosh, Edward, Frosh, Adam, Mead, Simon, Hill, Andrew F, Brandner, Sebastian, Thomas, Dafydd +1 more·2008·Philosophical Transactions of the Royal Society B: Biological Sciences
DOI 10.1098/rstb.2008.0068PMC PMC2581654
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Abstract

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.

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  • Kuru is a uniformly fatal and remarkably stereotyped cerebellar ataxic syndrome accompanied by tremor and choreiform and athetoid movements.

    “Kuru is a uniformly fatal and remarkably stereotyped cerebellar ataxic syndrome accompanied by tremor and choreiform and athetoid movements (27, 39, 57–61)”

    From Kuru (2012) · cites this paper as [59]

    The Introduction (page unavailable) describes progressive cerebellar ataxia, a well-characterized clinical progression, usually present tremor, and eventual death. It says athetoid and choreiform movements *may occur*, rather than presenting them as invariable accompaniments of kuru. (LLM, conf=0.85)

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References 71 · Cited by 110

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Authors
Collinge, J., Whitfield, J., McKintosh, E., Frosh, A., Mead, S., Hill, A. F., Brandner, S., Thomas, D., & Alpers, M. P.
Affiliations
  • MRC Prion Unit (GB)
  • Curtin University (AU)
Corresponding author
Collinge, John
ORCID
Venue
Philosophical Transactions of the Royal Society B: Biological Sciences
Publisher
The Royal Society
Published
2008-11-27
Volume
363
Issue
1510
Pages
3725-3739
ISSN
  • 0962-8436 (ISSN-L)
  • 1471-2970
DOI
10.1098/rstb.2008.0068
PMC
PMC2581654
OpenAlex
W2115682083
Cited by
110
References
71
Citations by year
2026: 1 · 2025: 1 · 2024: 2 · 2023: 25 · 2022: 1 · 2021: 9 · 2020: 5 · 2019: 6 · 2018: 1 · 2017: 6 · 2016: 8 · 2015: 8 · 2014: 3 · 2013: 6 · 2012: 3
FWCI
3.43
Type
journal article
Language
en
Open access
Licence
Locations
  • Philosophical Transactions of the Royal Society B Biological Sciences (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • Minerva Access (University of Melbourne) (submittedVersion, cc-by)
  • Victoria University Research Repository (Victoria University) (submittedVersion, cc-by)
  • UCL Discovery (University College London) (submittedVersion)
  • eSpace (Curtin University) (submittedVersion)
Funders
  • Wellcome Trust
  • Medical Research Council
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
  • Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
Keywords
kuru, prion disease, long incubation period, variant Creutzfeldt-Jakob disease, cerebellar syndrome, cognitive impairment, tonsil biopsy, clinical features, Papua New Guinea
MeSH terms
Prion Proteins, Disease Outbreaks, Female, Haplotypes, Humans, Kuru, Male, Middle Aged, Papua New Guinea, Population Surveillance, Prions, Palatine Tonsil, History, 20th Century, History, 21st Century

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