Sudden unexpected death in epilepsy: Who are the children at risk?

The Canadian Paediatric Surveillance Program (CPSP) is a joint project of the Canadian Paediatric Society and the Public Health Agency of Canada, which undertakes the surveillance of rare diseases and conditions in children and youth. For more information, visit our website at www.cpsp.cps.ca. CASE 1 A developmentally appropriate 14-year-old girl presented with a history of two generalized tonic-clonic seizures. Further history was positive for myoclonic jerks, most often in the morning. An electroencephalogram demonstrated generalized spike and slow wave discharges, and a diagnosis of juvenile myoclonic epilepsy was established. She was initially treated with valproic acid, which resulted in unwanted side effects and was changed to lamotrigine. During the period of medication changes, she experienced another generalized tonic-clonic seizure. Over the next two years, she had two additional generalized tonic-clonic seizures. With questioning, these seizures could be attributed to forgotten medication doses. She was an excellent student and excelled in athletics. Three years after her initial diagnosis, at 17 years of age, she was found one morning, dead in her bed in the supine position. There was evidence that a seizure had occurred, including tongue lacerations and disruption of the bed sheets. Autopsy did not reveal the cause of death. As expected, the family was profoundly distraught, heightened by their anger at never having been told of the risk of death associated with epilepsy. In follow-up discussions with her parents, they expressed concerns about their daughter’s compliance with anticonvulsant medications.