Variant Creutzfeldt-Jakob disease in UK children after 27 years of active prospective surveillance
Year: 2025
Type: article
Study design: Observational Study
Abstract: OBJECTIVE: To determine whether any children in the UK had variant Creutzfeldt-Jakob disease (vCJD). DESIGN: This 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. RESULTS: Between 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. CONCLUSIONS: This 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.
Source: Archives of Disease in Childhood
Institutions: Addenbrooke's Hospital, Western General Hospital
Language: English
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: hybrid
Award: G4076109980
OpenAlex ID: W4409089485