Childhood absence epilepsy

Childhood absence epilepsy (CAE), formerly known as pyknolepsy, is an idiopathic generalized epilepsy syndrome that begins in childhood, typically between the ages of 4 and 10, with a peak onset between 5 and 7 years. It is characterized by frequent absence seizures — brief episodes of impaired awareness that start and end suddenly, often accompanied by subtle automatisms such as eyelid fluttering or lip smacking. Seizures usually last less than 30 seconds and may occur dozens or even hundreds of times per day. Children with CAE are otherwise developmentally normal, and the electroencephalogram (EEG) shows characteristic generalized 3 Hz spike-and-wave discharges. The syndrome is genetically complex, with seizures believed to arise from thalamocortical network dysfunction. Prognosis is generally favorable, with many children achieving seizure remission during adolescence. Ethosuximide is the preferred first-line treatment.