Epidemiology of absence epilepsy. II. Typical absences in children with encephalopathies

A population-based study of absence epilepsy in Swedish children, aged 0-15 years, comprised cases selected on the basis of EEG criteria. Absence epilepsy was found in 119 of the 134 children with 3 Hz spike-and-wave discharges, and 12 of these 119 (10.1%) had typical absences in addition to other generalized seizures and slow irregular spike-and-wave activity on the EEG. The mean annual incidence of this type of absence epilepsy was 0.7/100,000. The median age at onset of absences was 6 years. Eight of the 12 patients had neurological abnormalities and/or severe mental retardation. The patients constitute a heterogeneous group of encephalopathies. They may have a genetic predisposition for absence epilepsy, causing it to appear during the course of a more severe, encephalopathy related, type of epilepsy.