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A Clinical Study on Lennox-Gastaut Syndrome

Journal of the Korean Pediatric Society 1998;41(5):663-668.
Published online May 15, 1998.
A Clinical Study on Lennox-Gastaut Syndrome
Yong Hun Choi, Gyo Dang Koo, Yeong Ho Rah, Sa Jun Chung
Department of Pediatrics, College of Medicine, Kyunghee University, Seoul, Korea
Lennox-Gastaut 증후군의 임상적 고찰
최용훈, 구교당, 나영호, 정사준
경희대학교 의과대학 소아과학교실
Correspondence: 
Yong Hun Choi, Email: 1
Abstract
Purpose
: Lennox-Gastaut syndrome is an intractable epilepsy which is comprised of several types of etiology and generalized seizures. It also combines with mental retardation. The present study was made to ascertain the clinical study on Lennox-Gastaut syndrome in Korea.
Methods
: From January, 1985 to December, 1995, 31 children, diagnosed with Lennox-Gastaut syndrome at Kyung Hee University Hospital, were analyzed retrospectively.
Results
: The ratio of male to female was 2.4:1. With regard to etiology, idiopathic form was found in 7 cases(22.6%) and symptomatic form in 24 cases(77.4%). Transition from West syndrome to Lennox-Gastaut syndrome was observed in 14 cases(45.2%). The most common type of seizure was tonic seizure which was seen in 19 cases(61.3%) and atypical absence, myoclonic seizure and atonic seizure were also observed. The most common clinical signs and symptoms were mental retardation, but others such as progressive loss of psychomotor function and attention deficit were also frequently associated with this syndrome. The EEG usually had abnormal background activity, slow spike and wave complexes and often showed multifocal abnormalities. In spite of intractable epilepsy, 18 cases(58.1%) were controlled with complex anticonvulsant therapy.
Conclusion
: Lennox-Gastaut syndrome manifested itself in children aged 1-6 years and had polyetiology. Most tonic seizures and mental retardation were observed. After complex anticonvulsant therapy with vigabatrin, complete remission was induced in many cases.
Key Words: Lennox-Gastaut syndrome, Childhood epilepsy, Electroencephalogram


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