Sudden unexpected death in epilepsy in childhood.

Sudden unexpected death in epilepsy (SUDEP) is the most feared complication of a seizure disorder. It has been less studied in childhood, probably because the incidence of sudden death is higher in adults than in children. SUDEP occurs more commonly in children where there is an underlying neuropathological disorder and a high rate of seizure. The literature reports rates of SUDEP in childhood between 1.1 and 4.3/10,000 patient years. Children with what has been called idiopathic epilepsy have an incidence of sudden death that is similar to the general population. Many children have a witnessed seizure before death, but in other witnessed deaths no seizure was identified. Cardiac arrhythmias and central apnea have been proposed as mechanisms of sudden death in SUDEP. At autopsy, apart from the underlying neuropathological disorder, there may be evidence of seizure activity in the form of bite marks and a voided bladder, though often the autopsy will prove negative. These autopsies remain challenging for the pathologist because acute findings are often absent. Before a diagnosis of SUDEP is made, a full knowledge of the history and scene information is required, as well as autopsy and ancillary investigation information.