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The epidemiology of progressive intellectual and neurological deterioration in childhood
  1. Christopher Verity1,*,
  2. Anne Marie Winstone1,
  3. Lesley Stellitano1,
  4. Robert Will2,
  5. Angus Nicoll3
  1. 1 PIND Surveillance Group, Addenbrookes Hospital, United Kingdom;
  2. 2 National Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, United Kingdom;
  3. 3 European Centre for Disease Prevention and Control, Sweden
  1. Correspondence to: Chris Verity, The Child Development Centre, Box 107, Addenbrooke's Hospital, Hills Road, Cambridge, CB2 2QQ, United Kingdom; christopher.verity{at}


Objective: To study the epidemiology of diseases that cause progressive intellectual and neurological deterioration (PIND) in United Kingdom (UK) children

Design: Since May 1997 we have performed active surveillance to search for vCJD among the many diseases that cause neurological deterioration in children, using the monthly surveillance card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. We obtain clinical details from reporting paediatricians by questionnaire or site visit and an Expert Group then independently classifies the cases.

Results: After 12 years 2636 patients less than 16 years old with suspected PIND had been reported of whom 1114 had a confirmed diagnosis to explain their deterioration: in these children there were 147 different diseases. These were the six commonest diagnostic groups: leukoencephalopathies (183 cases), neuronal ceroid lipofuscinoses (141 cases), mitochondrial diseases (122 cases), mucopolysaccharidoses (102 cases), gangliosidoses (100 cases) and peroxisomal disorders (69 cases). Relatively large numbers of PIND children were reported from parts of the UK where there are high rates of consanquinity. Only 6 children with vCJD (4 definite, 2 probable) had been identified.

Conclusions: Although our study does not ascertain all UK cases it provides a novel insight into the epidemiology of the neurodegenerative diseases that cause PIND in children. It is reassuring that in general these children are carefully investigated and that active surveillance has found only 6 children with vCJD. However there is concern that more childhood vCJD cases may appear, possibly with a different genotype from those identified so far.

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