Progressive Intellectual and Neurological Deterioration (PIND)
Surveillance of Progressive Intellectual and Neurological Deterioration (PIND) in Children (including Creutzfeldt-Jakob Disease) through the British Paediatric Surveillance Unit began in May 1997 with the main aim of determining whether a child developed variant Creutzfeldt-Jakob disease (vCJD). As the clinical presentation of vCJD was not typical of classical CJD, the aim was to detect suspected cases by looking at a broader group of conditions.
Lead investigator
Dr Chris Verity
Contact: annemarie.winstone@nhs.net
About the study
Active prospective surveillance of UK children with progressive intellectual and neurological deterioration (PIND) commenced in May 1997. The primary objective was to determine whether any child had developed variant Creutzfeldt-Jakob disease (vCJD). vCJD had appeared in patients as young as 12 years of age and could occur in younger children. Although no paediatric cases have appeared since 2001, vCJD has not gone away. As the clinical presentation of vCJD is not typical of classical CJD and may differ in children, the aim was to detect suspected cases by examining a broader range of conditions. This group needed to be large enough to include all possible cases of CJD, hence the need to perform surveillance for all children with PIND.
Duration
May 1997 – December 2023
Funding
Department of Health
Approval
Approved by Addenbrooke’s NHS Trust and the PHE
Published papers
Verity CM, Maunder PJ, Winstone AM, Pal S. Epidemiology of progressive intellectual and neurological deterioration in UK children. Dev Med Child Neurol. 2025; 00: 1–11. https://doi.org/10.1111/dmcn.70008
Verity CM, Nicoll A, Will RG, Devereux G, Stellitano L. Variant Creutzfeldt-Jakob disease in UK children: a national surveillance study. Lancet. 2000 Oct 7;356(9237):1224-7. doi: 10.1016/s0140-6736(00)02785-9. PMID: 11072940.
Support Groups
Batten Disease Family Association
Creutzfeldt-Jakob Disease Support Network
The Society for Mucopolysaccharide Diseases