CAJ | 학술논문

目的总结早期肌阵挛脑病的临床及脑电图特征、治疗、预后.方法收集2008年9月至2012年1月中南大学湘雅医院儿科诊治的3例早期肌阵挛脑病患儿的临床和脑电图资料,进行分析和随访,总结其临床和脑电图特点.结果 3例早期肌阵挛脑病患儿,其中男2例,女1例.起病年龄为生后第2至23天,平均为13 d.3例患儿起病均有游走性、局灶性肌阵挛发作,其中2例伴频繁部分运动性发作.1例在生后3个月开始出现强直痉挛发作.3例发作间期脑电图均有暴发-抑制波形,1例呈双侧不对称暴发-抑制波形,暴发段时间短,抑制段时间长.抗癫(癎)药物治疗疗效差.1例于生后1岁5个月死亡,其余两例有频繁部分运动性发作和明显精神运动发育异常.结论早期肌阵挛脑病起病时肌阵挛发作是特征性发作类型,频繁部分性发作可在肌阵挛发作后很快出现,生后3～5个月可伴发强直痉挛发作.暴发-抑制波形脑电图是发作间期特征性异常.本病治疗效果差,预后差,是婴儿早期起病的难治性癫(癎)性脑病.
목적 총결조기기진련뇌병적림상급뇌전도특정、치료、예후.방법 수집2008년9월지2012년1월중남대학상아의원인과진치적3례조기기진련뇌병환인적림상화뇌전도자료,진행분석화수방,총결기림상화뇌전도특점.결과 3례조기기진련뇌병환인,기중남2례,녀1례.기병년령위생후제2지23천,평균위13 d.3례환인기병균유유주성、국조성기진련발작,기중2례반빈번부분운동성발작.1례재생후3개월개시출현강직경련발작.3례발작간기뇌전도균유폭발-억제파형,1례정쌍측불대칭폭발-억제파형,폭발단시간단,억제단시간장.항전(간)약물치료료효차.1례우생후1세5개월사망,기여량례유빈번부분운동성발작화명현정신운동발육이상.결론 조기기진련뇌병기병시기진련발작시특정성발작류형,빈번부분성발작가재기진련발작후흔쾌출현,생후3～5개월가반발강직경련발작.폭발-억제파형뇌전도시발작간기특정성이상.본병치료효과차,예후차,시영인조기기병적난치성전(간)성뇌병.
Objective To investigate the clinical and electroencephalographic (EEG) 1characteristics,therapeutic response and long-term prognosis of early myoclonic encephalopathy.Method The clinical and EEG data of three patients with early myoclonic encephalopathy were analyzed.These patients were admitted to our hospital between September 2008 and January 2012.The patients were followed up for therapeutic response and long-term prognosis.Result The age of onset was from 2 to 23 days after birth.All patients had the onset of erratic or fragmentary myoclonus.Two patients had frequent simple focal seizures.One patient had tonic spasms when he was 3 months old.The EEG characteristic of all patients was repetitive suppression-burst pattern.The suppression-burst pattern was characterized by paroxysmal short bursts and long periods of suppression.The EEG paroxysms of one patient was asynchronous over both hemispheres.There is no effective therapy for early myoclonic encephalopathy.A patient died before two years of age.Two patients had severe partial epilepsy and showed very severe retardation.Conclusion Early myoclonic encephalopathy usually starts in the first month of life.Erratic myoclonus appears first.Myoclonus is the principal features of early myoclonic encephalopathy.Frequent focal seizures occur shortly after erratic myoclonus.Tonic epileptic spasms may develop within 3-5 months.The suppression-burst pattern is EEG characteristic.There is no effective therapy for early myoclonic encephalopathy and the prognosis is poor.