ArticleArchives of disease in childhood2025
Variant Creutzfeldt-Jakob disease in UK children after 27 years of active prospective surveillance.
Article in Archives of disease in childhood, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 1 paper.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- Epidemiology of progressive intellectual and neurological deterioration in UK children.Developmental medicine and child neurology · 2026Article
Corrections and comments
- Erratum issuedCorrection:2025
Authors and funding
5 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo determine whether any children in the UK had variant Creutzfeldt-Jakob disease (vCJD).
designThis active prospective epidemiological study used the British Paediatric Surveillance Unit, asking UK paediatricians to notify all childhood cases of progressive intellectual and neurological deterioration (PIND), a group that would include all cases of vCJD. Clinical data were obtained by questionnaire or via a site visit. An independent expert group classified the cases. If vCJD was suspected, referral to the National Creutzfeldt-Jakob Disease Research and Surveillance Unit was recommended.
resultsBetween May 1997 and April 2024 (27 years), 5222 children were notified. There were four groups. (1) 2540 were 'not cases'-they did not meet the case definition or there were notification errors. (2) 2367 had a known underlying diagnosis other than vCJD; the group contained more than 220 different diseases. (3) 309 had no diagnosis to explain their deterioration; there was evidence that none of these cases had vCJD. (4) There were six cases of vCJD: two males and four females. They developed symptoms between 1998 and 2000, aged 12-15 years, and the last two died in 2003. Their clinical features were similar to those of adults. Four were classified as definite vCJD and two as probable vCJD.
conclusionsThis study has provided unique data about neurodegenerative diseases in UK children. There is no reliable vCJD screening test; so for 27 years, the PIND study has provided reassurance that childhood vCJD cases were not missed. New vCJD cases with the methionine/valine genotype could appear.
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