Degenerative Disease of the Central Nervous System in New Guinea: The Endemic Occurrence of Kuru in the Native Population
Abstract
DURING the past two years' service as medical officer of the Kainantu Sub-District, Eastern Highlands of the Australian Trust Territory of New Guinea, one of us (V.Z.) has had the opportunity of observing in the inhabitants of the mountain-dissected highlands some 30 to 50 miles southeast of Kainantu (Fig. 1) an unusually high incidence of a syndrome similar to paralysis agitans affecting children and adults, predominantly females, in natives of the recently controlled Forei linguistic and cultural group and in their neighbors of the Keiagana, Kanite, Kimi, Usurufa and Auiyana linguistic groups. During the past three months an intensive clinicopathological . . .
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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 [27]
Kuru is a prion disease that affected the Fore linguistic group of the Eastern Highlands Province of Papua New Guinea and provides major experience with epidemic prion disease in humans.
“Kuru is a prion disease that had a dramatic impact on the Fore linguistic group of the Eastern Highlands Province of Papua New Guinea 1 and that provides our major experience with epidemic prion disease in humans.”
From A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure (2009) · cites this paper as [1]
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Pathogenesis of prion diseases: current status and future outlook
Aguzzi, A., & Heikenwalder, M. (2006) · Nature Reviews Microbiology · doi:10.1038/nrmicro1492 · Cited by 207 · see citing works
A Novel Protective Prion Protein Variant that Colocalizes with Kuru Exposure
Mead, S., Whitfield, J., Poulter, M., Shah, P., Uphill, J., Campbell, T., Al-Dujaily, H., Hummerich, H., Beck, J., Mein, C. A., et al. (2009) · New England Journal of Medicine · doi:10.1056/nejmoa0809716 · Cited by 206 · see citing works
Aguzzi, A., & Zhu, C. (2017) · Journal of Clinical Investigation · doi:10.1172/jci90605 · Cited by 122 · see citing works
Prions and protein-folding diseases: Review: Prions and protein-folding diseases
Norrby, E. (2011) · Journal of Internal Medicine · doi:10.1111/j.1365-2796.2011.02387.x · Cited by 75 · see citing works
Kuru, the First Human Prion Disease
Liberski, P. P., Gajos, A., Sikorska, B., & Lindenbaum, S. (2019) · Viruses · doi:10.3390/v11030232 · Cited by 69 · see citing works
Phenotypic variability in human prion diseases
Ironside, J. W., Ritchie, D. L., & Head, M. W. (2005) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2005.00697.x · Cited by 58 · see citing works
Liberski, P. P., Sikorska, B., Lindenbaum, S., Goldfarb, L. G., McLean, C., Hainfellner, J. A., & Brown, P. (2012) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e3182444efd · Cited by 50 · see citing works
“Kuru and Cannibalism When kuru was first reported as occurring in certain families and hamlets, confined to the Fore and adjacent people with whom they intermarried, and showing a predilection for young children and adult women (27), a genetic or hormonal explanation seemed to provide a key.”
BackgroundResult
- Authors
- Gajdusek, D. C., & Zigas, V.
- Affiliations
- March of Dimes (US)
- Venue
- New England Journal of Medicine
- Publisher
- Massachusetts Medical Society
- Published
- 1957-11-14
- Volume
- 257
- Issue
- 20
- Pages
- 974-978
- ISSN
- 0028-4793 (ISSN-L)
- 1533-4406
- OpenAlex
- W2035004702
- Cited by
- 572
- References
- 0
- Citations by year
- 2026: 3 · 2025: 4 · 2024: 8 · 2023: 7 · 2022: 7 · 2021: 9 · 2020: 14 · 2019: 12 · 2018: 6 · 2017: 16 · 2016: 14 · 2015: 9 · 2014: 15 · 2013: 12 · 2012: 13
- FWCI
- 34.10
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- New England Journal of Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Prion Diseases and Protein Misfolding
- Health Sciences › Health Professions › General Health Professions › Indigenous Health and Education
- Keywords
- parkinsonism, Parkinson's disease, Kimi, clinicopathological correlation
- MeSH terms
- Central Nervous System, Humans, Kuru, Nervous System Diseases, New Guinea
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