目的:分析早发性癫痫脑病伴暴发-抑制(EOEE-BS)脑电图患儿的病因，总结临床特点，为治疗选择及预后评估提供线索。方法:收集首都医科大学附属北京儿童医院神经科2016年9月至2019年4月收治的EOEE-BS患儿，回顾性分析其临床资料。结果:入组30例，男11例、女19例。24例(80%)检测到致病性/可能致病性基因变异，分别为n KCNQ2 8例、n SCN2A 5例、n STXBP1 5例，n CDKL5、KCNT1、KCNQ3、HUWE1、MTHFR、NOTCH3各1例，新生变异18例、遗传性变异6例；9例存在脑发育畸形，为多微小脑回伴或不伴巨脑，其中4例合并上述钾或钠离子通道基因变异；病因不明2例。起病年龄2.5 h～4个月，中位年龄4 d。以局灶性起源的强直/强直-阵挛发作起病25例，其中14例合并或转型为痉挛发作；以痉挛发作起病5例。脑电图监测到暴发-抑制的年龄为3 d～9月余，于1～10月龄转变为高度失律或多灶性痫样放电。应用1～6种抗癫痫药物，13例应用钠离子通道阻滞剂患儿中11例有效，7例联合生酮饮食均有效。25例随访6个月～3年，13例于1月余～1.5岁抽搐缓解，但均有不同程度发育落后。n 结论:EOEE-BS中超过半数患儿由单基因变异致病，起病早，发作类型及脑电图可随年龄增长而转变。预后与病因有关，离子通道基因变异者应用离子通道阻滞剂抗癫痫疗效较好。“,”Objective:To analyze the etiology of early-onset epileptic encephalopathy with burst-suppression (EOEE-BS) electroencephalogram, and summarize its clinical features, provide clues for treatment selection and prognosis evaluation.Methods:EOEE-BS patients who were admitted to the Department of Neurology, Beijing Children′s Hospital Affiliated to Capital Medical University from September 2016 to April 2019 were enrolled, so as to retrospectively analyze clinical resources.Results:Thirty children were enrolled, with 11 males and 19 females.Pathoge-nic/likely pathogenic gene variations were detected in 24 cases (80%), with n KCNQ2 in 8 cases, n SCN2A in 5 cases, n STXBP1 in 5 cases, and n CDKL5, KCNT1, KCNQ3, HUWE1, MTHFR and n NOTCH3 all in 1 case.Among them, 18 cases suffered from de novo variations, and 6 cases experienced hereditary variations.Nine cases had brain malformation that was polymicrogyria with or without megalencephaly, and 4 cases were combined with potassium or sodium channel gene variation.Two cases displayed unknown etiology.The onset age was from 2.5 hours to 4 months, and the median age was 4 days.Twenty-five cases were onset with focal onset tonic/tonic-clonic seizures, among which 14 cases were evolved into or combined with spasms during the follow-up, while 5 cases were onset with epileptic spasms.BS was monitored by electroencephalogram (EEG) aged from 3 days to 9 months, and evolved to hypsarrhythmia or multiple epileptiform discharges at 1-10 months old.Patients were treated with 1-6 antiepileptic drugs, 13 cases were treated with sodium channel blocker, and 11 cases were effective.Ketogenic diet was effective for all 7 cases.Twenty-five patients were followed-up between 6 months and 3 years, and 13 cases were seizure-free aged from 1 month to 1.5 years old.Howe-ver, they all had psychomotor retardation in various degrees.n Conclusions:More than half of EOEE-BS is caused by single gene variation.Seizure type and EEG would change with age.Its prognosis is associated with etiology.Ion channel blocker may have effect on patients with ion channel gene variation.