G34 UK children with progressive neurological disease: differential diagnoses by age
- Resource Type
- Authors
- Christopher M Verity; PJ Maunder; Anne Marie Winstone; E Baker
- Source
- British academy of childhood disability and british paediatric neurology association.
- Subject
- Estimation
medicine.medical_specialty
business.industry
Family medicine
Epidemiology
Laboratory reports
medicine
Disease
Differential diagnosis
Medical diagnosis
business
Hospital records
Independent research
- Language
Aims To review the cases referred to this UK-wide study of children with progressive intellectual and neurological deterioration (PIND) and analyse the differential diagnoses in these children by age at presentation. Methods Children meeting the case definition for PIND were identified via the British Paediatric Surveillance Unit. Data were obtained by standard questionnaire, with additional information from hospital records and laboratory reports, allowing validation of diagnoses and estimation of the age of presentation to clinicians. Results Between April 1997 and October 2019, 4612 children had been notified to the study. 2072 were found not to meet the PIND case definition. 2009 had an underlying diagnosis to explain their deterioration; of these the numbers presenting by age were: Conclusion Since 1997 the PIND Study has provided unique epidemiological data about neurodegenerative diseases in UK children. It shows that children meeting the criteria for PIND tend to present early in life (over 80% before the age of 5 years) – not surprising because of the inherited basis for so many of these disorders. The study provides a differential diagnosis for PIND children by age and remains the only means of performing systematic surveillance of the neurodegenerative diseases that make up the differential diagnosis of vCJD. Acknowledgement Independent research funded by the Department of Health (DH) Policy Research Programme [PR-ST-1216-10001]. Views expressed are not necessarily those of DH.