Abstract
Aims: To identify any UK children with variant Creutzfeldt-Jakob disease (vCJD) and obtain information about the causes of progressive intellectual and neurological deterioration (PIND) and the geographical distribution of cases.
Methods: The PIND Study uses the monthly surveillance card that is sent to all UK paediatricians by the British Paediatric Surveillance Unit. Case details are obtained from the reporting paediatricians by telephone interview, site visit, or self completion of a questionnaire. A paediatric neurology expert group then classifies the anonymised cases. The Communicable Disease Surveillance Centre (CDSC) provides mapping support.
Results: After five years and five months of surveillance, 1400 children had been reported. In the UK the majority of PIND cases had a confirmed diagnosis (comprising 99 different conditions); 505 "no cases" and 97 "outstanding" cases were excluded. A total of 798 PIND cases were included as follows: 577 with a confirmed underlying diagnosis; six with definite or probable vCJD, 51 who had undiagnosed PIND but were not thought to have vCJD, and 164 cases who were still under investigation. In some districts there were unexpectedly high numbers of PIND cases with a heterogeneous mixture of underlying diagnoses. In the five districts with the largest numbers of resident cases the majority not only came from a particular ethnic group but also had high reported rates of consanguinity.
Conclusions: In districts with large numbers of PIND cases there are major resource implications. These children and their families have complex problems and they need access to diagnostic facilities and appropriate service provision.
Full Text
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Figure 1 .
Total PIND cases by study category. "No cases" (n = 505) and "outstanding" cases (n = 97) were excluded from the analysis and are shown exploded. The remaining 798 cases were analysed according to the PHLS health district of residence as described in the text.
Figure 2 .
Map of the UK showing PHLS districts. The five highlighted districts are those in which the largest numbers of PIND cases resided.
Figure 3 .
The five most frequently diagnosed causes of PIND in the UK.
Figure 4 .
The causes of PIND diagnosed in Bradford.
Figure 5 .
All UK PIND cases; ethnicity.
Figure 6 .
Bradford PIND cases; ethnicity.
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
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