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Aicardi Syndrome: A Longitudinal Clinical and Electroencephalographic Study
Author(s) -
Ohtsuka Yoko,
Oka Eiji,
Terasaki Tomoyuki,
Ohtahara Shunsuke
Publication year - 1993
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1157.1993.tb00439.x
Subject(s) - hypsarrhythmia , west syndrome , lennox–gastaut syndrome , electroencephalography , pediatrics , epileptic spasms , epilepsy , medicine , audiology , psychology , psychiatry
Summary: We report clinical and EEC follow‐up of 6 children with Aicardi syndrome. Age at seizure onset was <3 months in 5 patients and 4 months in 1 patient. All patients had spasms, and these continued at time of follow‐up in 5 patients. Five patients had seizures other than spasms which disappeared during early infancy. Bilateral independent bursts (BIBs) characteristic of Aicardi syndrome were noted in 4 patients. In 1, BIBs showed suppression‐burst patterns. BIBs converted to hypsarrhythmia or multifocal spikes with a tendency to BIBs during sleep. BIBs disappeared completely during both waking and sleeping states between the ages of 3 years 1 month and 4 years 9 months. After BIB disappearance, the EEG of 1 patient showed diffuse slow spike‐and‐wave complexes most of which appeared asynchronously. At onset, 1 patient had early‐infantile epileptic encephalopathy with suppression‐burst which evolved initially into West syndrome (WS) and then Lennox‐Gastaut syndrome (LGS). The other 3 children also had WS.