【摘要】 目的探讨23例大田原综合征的临床与脑电图特点。方法回顾性分析23例大田原综合征临床与脑电图资料。结果23例患儿主要临床表现为强直痉挛性发作,脑电图均为暴发抑制型脑电图,21例患儿精神运动发育迟滞,15例患儿4~6月龄时转变为婴儿痉挛症,脑电图表现为高度失律,2例患儿在1.5~3岁时转变为Lennox-Gastaut综合征,脑电图发作间歇期可见2Hz慢棘慢波综合。结论大田原综合征大部分病因明确,除依据典型发作形式和精神运动发育障碍诊断外,其特异性脑电图改变也是确诊该病的主要依据。更多还原

【Abstract】 Objective To investigate the clinical and electroencephalogram (EEG) features of Ohtahara syndrome. MethodsThe clinical data of 23 cases of Ohtahara syndrome, including electroencephalogram (EEG) results, were retrospectively analyzed. Results The 23 patients mainly presented with tonic spasm and EEGs of all the cases showed "burst suppression" pattern. Twenty-one cases developed mental and physical retardation. Fifteen patients evolved into West syndrome at 4-6 months old with typical hypsarrhythmia in their EEGs. Two cases of Ohtahara transformed into Lennox-Gastaut syndrome at the age of 1.5-3 years and their EEGs revealed spike and slow wave complex at the intermittent period. Conclusion Most cases in this series have definite etiological factors. The specific EEG changes help diagnosis of Ohtahara syndrome as well as typical seizure onset and mental and physical disorder.更多还原