ArticlePhilosophical transactions of the Royal Society of London. Series B, Biological sciences2008
A clinical study of kuru patients with long incubation periods at the end of the epidemic in Papua New Guinea.
Article in Philosophical transactions of the Royal Society of London. Series B, Biological sciences, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 36 papers, 1 of them a synthesis that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
36 citing papers in PubMed, 1 synthesis or guideline pooled it, 105 citations in OpenAlex.
- Interventions to reduce the risk of surgically transmitted Creutzfeldt-Jakob disease: a cost-effective modelling review.Health technology assessment (Winchester, England) · 2020Pooled it
- Safety and efficacy of quinacrine in human prion disease (PRION-1 study): a patient-preference trial.The Lancet. Neurology · 2009Trial
- Review
- Seed amplification and neurodegeneration marker trajectories in individuals at risk of prion disease.Brain : a journal of neurology · 2023Article
- Infections and immunity: associations with obesity and related metabolic disorders.Journal of pathology and translational medicine · 2023Review
- The importance of ongoing international surveillance for Creutzfeldt-Jakob disease.Nature reviews. Neurology · 2021Review
- Prion Diseases: A Unique Transmissible Agent or a Model for Neurodegenerative Diseases?Biomolecules · 2021Review
- A Review of Infectious Diseases Associated with Religious and Nonreligious Rituals.Interdisciplinary perspectives on infectious diseases · 2021Review
- Understanding and exploiting interactions between cellular proteostasis pathways and infectious prion proteins for therapeutic benefit.Open biology · 2020Article
- Invited Review: The role of prion-like mechanisms in neurodegenerative diseases.Neuropathology and applied neurobiology · 2020Review
- Prevalence in Britain of abnormal prion protein in human appendices before and after exposure to the cattle BSE epizootic.Acta neuropathologica · 2020Article
- Kuru, the First Human Prion Disease.Viruses · 2019Review
- Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein.Journal of the neurological sciences · 2018Article
- UK Iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches.Acta neuropathologica · 2017Article
- Viral Infections and Obesity.Current obesity reports · 2017Review
- Sporadic and Infectious Human Prion Diseases.Cold Spring Harbor perspectives in medicine · 2017Review
- Prion Diagnosis: Application of Real-Time Quaking-Induced Conversion.BioMed research international · 2017Review
- Vertebrate food products as a potential source of prion-like α-synuclein.NPJ Parkinson's disease · 2017Review
- Proteopathic Strains and the Heterogeneity of Neurodegenerative Diseases.Annual review of genetics · 2016Review
- Article
Corrections and comments
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Authors and funding
9 authors at 4 institutions in 3 countries.
Funding
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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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.