Background Paediatric neurological diseases individually are rare; however, collectively affect thousands of children and have life-long impacts. The incidence of many of these is not readily available, and yet essential for improving clinical care, advocacy and health service planning.
Aims To obtain/examine national population-based data, in a timely manner, on acute flaccid paralysis (AFP), progressive intellectual and neurological deterioration (PIND), acquired demyelinating syndromes of the central nervous system (ADS), congenital myotonic dystrophy (CMD) and paediatric myasthenia (PM).
Methods Studies were conducted through the Canadian Paediatric Surveillance Program, a network of >2,500 paediatricians, reporting cases monthly according to preset protocols. Confidentiality is mandatory; studies receive ethical approval.
Results The AFP study, with 657 cases in 15 years, affirms that Canada is free of wild-type poliovirus. The PIND study demonstrated several genetically defined neurodegenerative disorders, and only one case of iatrogenic Creutzfeldt-Jakob disease. A yearly incidence of 0.9 per 100,000 was estimated to affect Canadian children during the ADS study, with optic neuritis being the most common presentation. Awareness of multiple sclerosis as a possible outcome of ADS increased remarkedly. Of 38 confirmed CMD cases in six years, 61% were index cases for families. In year one of surveillance, 33 cases of PM were confirmed; almost half not having elevated titers of acetylcholine receptor antibodies, and 21% having other co-existing or familial immune disorders.
Conclusion Active national surveillance has more reliably characterized several rare neurological disorders and their associated burdens, supporting and informing the development of medical and public health interventions.