ArticleArchives of disease in childhood2019
Surveillance for variant CJD: should more children with neurodegenerative diseases have autopsies?
Article in Archives of disease in childhood, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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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
4 citing papers in PubMed, 9 citations in OpenAlex.
- Epidemiology of progressive intellectual and neurological deterioration in UK children.Developmental medicine and child neurology · 2026Article
- Variant Creutzfeldt-Jakob disease in UK children after 27 years of active prospective surveillance.Archives of disease in childhood · 2025Article
- Differential diagnosis of progressive intellectual and neurological deterioration in children.Developmental medicine and child neurology · 2021Article
- A Scoping Review of Inborn Errors of Metabolism Causing Progressive Intellectual and Neurologic Deterioration (PIND).Frontiers in neurology · 2019Article
Corrections and comments
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Authors and funding
15 authors at 11 institutions in 1 country.
Funding
Abstract
objectivesTo report investigations performed in children with progressive neurodegenerative diseases reported to this UK study.
designSince 1997 paediatric surveillance for variant Creutzfeldt-Jakob disease (vCJD) has been performed by identifying children aged less than 16 years with progressive intellectual and neurological deterioration (PIND) and searching for vCJD among them.
settingThe PIND Study obtains case details from paediatricians who notify via the British Paediatric Surveillance Unit.
participantsBetween May 1997 and October 2017, a total of 2050 cases meeting PIND criteria had been notified and investigated.
resultsSix children had vCJD. 1819 children had other diagnoses, made in 12 cases by antemortem brain biopsy and in 15 by postmortem investigations. 225 children were undiagnosed: only 3 had antemortem brain biopsies and only 14 of the 108 who died were known to have had autopsies; postmortem neuropathological studies were carried out in just 10% (11/108) and only two had prion protein staining of brain tissue. Of the undiagnosed cases 43% were known to come from Asian British families.
conclusionsMost of the notified children had a diagnosis other than vCJD to explain their neurological deterioration. None of the undiagnosed cases had the clinical phenotype of vCJD but brain tissue was rarely studied to exclude vCJD. Clinical surveillance via the PIND Study remains the only practical means of searching for vCJD in UK children.
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