Aicardi Syndrome: A Longitudinal Clinical and Electroencephalographic Study

Thirty-two EEGs from six cases of Aicardi's syndrome were reviewed. A characteristic EEG pattern was found in all cases. This consists of multifocal epileptiform abnormalities occurring on a burst-suppression pattern showing complete asynchrony between the two hemispheres. This pattern has been described so far only in Aicardi's syndrome. These characteristic EEG features are more readily found early in the course of the disease and occur less frequently six months after from the onset of symptoms, at which time they are often replaced by multiple epileptic foci on a severely disorganized background. The EEG sleep pattern was profoundly altered in all stages of the disease. The EEG is considered a helpful tool in the diagnosis of Aicardi's syndrome.