비전형 결신 발작으로 발현한 사춘기 발생형의 Lennox Gastaut 증후군 1례
A Case of Juvenile Onset Lennox-Gastaut Syndrome Presenting as Atypical Absence
하병립, 천상명, 김상호,
소속 상세정보
하병립 ( Ha Byoung-Lip )
동아대학교 의과대학 신경과학교실
천상명 ( Cheon Sang-Myung )
동아대학교 의과대학 신경과학교실
김상호 ( Kim Sang-Ho )
동아대학교 의과대학 신경과학교실
KMID : 0877220020060020147
Abstract
Atypical absence is less understood than typical absence. Several conditions that produce atypical absence are known including Lennox-Gastaut syndrome, myoclonic astatic epilpsy and epileptic encephalopathy with continuous spike and waves in slow wave sleep. A 17-year-old girl with mental retardation had developed frequent loss of consciousness and occasional falling attack with traumatic facial injury for 2 years. The interictal EEG showed 2 Hz slow spike-and-wave complex with maximum over right frontotemporal area and the brain MRI was normal. Carbamazepin was prescribed initially but the drug seemed to worsen the seizures. Long term video-EEG monitoring showed very frequent atypical absence seizures consisting of sudden hypotonia of head and oral automatism with or without secondary generalization. Generalized 2 to 2.5 Hz slow spike-and-wave complexes with duration of 10 to 40 seconds were seen during ictal period. About 10% to 20% of the non REM sleep was occupied with generalized slow spike-and-wave complex and/or polyspikes or polyspikes-and-wave complex with duration of within 1 second. Valprorate monotherapy had failed, then lamotrigin was added. In spite of polytherapy, the seizure was intractable. We think this intractable atypical absence might be associated with juvenile onset Lennox-Gastaut syndrome.
키워드
Lennox-Gastaut Syndrome; atypical absence
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