目的探讨儿童晚发型癫痫性痉挛的临床特点及脑电图(EEG)改变。方法回顾分析总结2010年6月-2015年8月就诊于南方医科大学附属中山市博爱医院儿科的13例临床诊断晚发型癫痫性痉挛患儿的临床资料、治疗、随访及结果。结果 13例入组患儿,其中男9例,女4例,起病年龄为1岁3个月~5岁7个月,病程1年3个月~4年8个月。7例有明确的病因(53.8%),其中2例明确病毒性脑炎,3例娩出时窒息、缺氧,1例新生儿期败血症、急性呼吸窘迫综合征,1例诊断甲基丙二酸血症伴高同型半胱氨酸血症;余6例未明确病因。痉挛发作仍为主要的发作类型,7例合并部分性起源发作,发作以清醒期及醒睡期为著。但易与其他的发作类型并存。发作期EEG为广泛性中-高波幅慢波、尖慢波阵发,复合或不复合低波幅快波,有时伴阵发后电压衰减数秒,成串或孤立性出现。同步双侧三角肌肌电监测可见双侧或单侧同步肌电爆发1~2s。间歇期EEG为多灶和广泛性放电,仍以尖(棘)慢波连续发放为主。治疗:所有患儿均行促肾上腺皮质激素(Adrenocorticotropic hormone,ACTH)或甲基强的松龙免疫调控治疗,3例行生酮饮食治疗。同时根据病情选择丙戊酸、托吡酯、氯硝西泮、拉莫三嗪、左乙拉西坦等抗广谱抗癫痫药物(AEDs),13例患儿均采取联合用药方式。预后:13例患儿运用ACTH或甲基强的松龙免疫调控疗程结束后发作减少或控制,随访3~12个月出现临床发作控制不良;3例生酮饮食治疗(现已持续1年3个月~2年5个月),其中1例发作控制、智力改善明显;另2例发作次数减少,智力水平明显改善。结论围产期因素与某些后天获得性脑损伤是儿童晚发型癫痫性痉挛最为常见的致病病因。发作以痉挛发作为主,与其他发作形式并存。EEG无典型高度失律表现,同步肌电监测可见双侧或单侧同步肌电爆发。对各种AEDs治疗效果欠佳,绝大多数发展为难治性癫痫。生酮饮食治疗或许是一个相对好的选择。 Objective To investigate the clinical features of children with late-onset epileptic spasm and changes of electroencephalogram (EEG). Methods The clinical data, treatment, follow-up and results of 13 cases of clinically diagnosed late-onset epileptic spasm admitted to Department of Pediatrics, Zhongshan Bo’ai Hospital affiliated to Southern Medical University from June 2010 to August 2015 were retrospectively analyzed. Results Thirteen patients were enrolled in the study, including 9 males and 4 females. The onset ages ranged from 1 year and 3 months to 5 years and 7 months. The course of disease ranged from 1 year and 3 months to 4 years and 8 months. Seven patients had a definite etiology (53.8%), of which two had clear viral encephalitis, three had asphyxiation during delivery, one had hypoxia, one had neonatal sepsis, acute respiratory distress syndrome and one had a diagnosis of methylmalonic acid Hyperlipidemia with hyperhomocysteinemia; more than 6 cases did not have a clear cause. Spasm seizure is still the main type of seizures, 7 patients with partial origins of seizures, seizures with awake and asymptomatic. But easy to co-exist with other types of attacks. Seizure EEG is a broad range of medium to high amplitude slow waves, sharp slow wave burst, complex or non-complex low-amplitude fast waves, sometimes accompanied by a few seconds after the array voltage decay, string or solitary. Synchronous bilateral deltoid EMG monitoring can be seen bilateral or unilateral synchronous EMG 1 ~ 2s. Intermittent EEG multi-focal and extensive discharge, still sharp (spine) slow wave continuous distribution based. Treatment: All children underwent adrenocorticotropin (ACTH) or methylprednisolone immunomodulatory therapy, 3 cases of ketogenic diet treatment. At the same time, according to the condition choose valproic acid, topiramate, clonazepam, lamotrigine, levetiracetam and other anti-epileptic drugs (AEDs), 13 cases of children taking combination therapy. Prognosis: 13 cases of children with ACTH or methylprednisolone immunosuppression after the end of seizures to reduce or control the seizure, followed up for 3 to 12 months of clinical onset of poor control; 3 cases of ketogenic diet (now for 1 year 3 Months to 2 years and 5 months), of which 1 case of seizure control, mental improvement significantly; the other 2 cases of seizures decreased, significantly improved mental status. Conclusion Perinatal and some acquired acquired brain injury are the most common etiological factors of late-onset epileptic spasm in children. Seizures to spasm-based attacks, and other forms of seizures co-exist. EEG without a typical high degree of loss of performance, synchronous EMG monitoring can be seen bilateral or unilateral EMG. Treatment of various AEDs ineffective, the vast majority of patients with refractory epilepsy. Ketogenic diet may be a relatively good choice.