Objective To report on our study of progressive intellectual and neurological deterioration (PIND), carried out via the British Paediatric Surveillance Unit (BPSU) to detect UK children with vCJD.

Methods Our surveillance method was specially designed for the complex nature of the problem. An Expert Group of paediatric neurologists reviews and validates the clinical information obtained by telephone interview, questionnaire or hospital visit.

Results Between April 1997 and February 2008 UK paediatricians notified 2391 children thought to meet the criteria for PIND. Among them were 6 with probable or definite variant Creutzfeldt-Jakob Disease (vCJD). 1012 PIND children had other confirmed diagnoses: in these children there were 115 known neurodegenerative conditions, providing unique epidemiological data that illustrate the complexity of classifying children with PIND.

Conclusions We have identified only six children with vCJD. In all UK age groups the total number of vCJD cases is 166. The concern is that more cases will appear because four adult cases have been associated with blood transfusion. Also all vCJD cases have had a genotype found in just 37% of the UK general population and the other genotypes may present after a longer incubation period.

Only two cases of variant CJD in children less than 16 years of age have been recognised outside the UK. The unique methodology we describe could provide a useful model for surveillance in other countries outside the UK.

Acknowledgements: The study is funded by the UK Department of Health. Many thanks to the BPSU and the Expert Group members.