晚发型癫痫性痉挛临床与脑电图特征分析

探讨晚发型癫痫性痉挛患儿的临床和脑电图（EEG）特征、治疗反应及预后。方法　对北京大学第一医院儿科2000年6月至2007年8月59例晚发型癫痫性痉挛患儿的临床和EEG资料进行回顾性研究，其中37例有随访，随访时间13～90个月，末次随访年龄为2岁6个月至12岁2个月（中位数60个月）。结果　59例患儿癫痫起病年龄为12～98个月，中位年龄22个月。以痉挛发作起病36例（61.0%），以其他发作类型起病23例（39.0%）。17例（28.8%）在病程中有部分性发作，22例（37.3%）合并其他全面性发作形式，20例（33.9%）病程中仅有痉挛发作。EEG 14例（23.7%）为典型高度失律；2例（3.4%）为一侧性高度失律；43例（72.9%）无典型的高度失律。总计22例（37.3％）表现为局灶性或一侧性临床、影像学和（或）EEG异常。44例（74.6%）为症状性癫痫，围生期脑损伤、中枢神经系统感染、脑结构异常为主要病因。14例（23.7%）符合晚发型West综合征，7例（11.9%）符合Lennox-Gastaut综合征。促肾上腺皮质激素（ACTH）治疗后近期无发作率32.0%，有效率58.0%，复发率为61.5%。随访的37例患儿中，在抗癫痫药物治疗下至末次随访时46%维持临床无发作。80%以上患儿在治疗随访过程中精神运动发育落后或倒退，16%为重度落后。统计学分析显示，癫痫性痉挛病因、癫痫发作类型、痉挛发作形式、ACTH疗效与长期发作控制率及精神运动发育转归均无显著相关性。结论　晚发型癫痫性痉挛并非仅见于West综合征，可由诸多严重的脑部疾病引起，常合并其他部分性或全面性发作，EEG多数无典型高度失律，治疗困难，预后不良，ACTH仍为本病首选治疗方法。

Clinical and electroencephalogram characteristics of children with late-onset epileptic spasms.

To analyze the clinical and electroencephalogram （EEG） characteristics， therapeutic response and long-term prognosis of the children with late-onset epileptic spasms. Methods　The clinical data， EEG features and response to different treatment protocols in59 children with late-onset epileptic spasms were retrospectively reviewed. Thirty-seven of them were followed up for 13～90 months and the age at last contacting ranged from 2 years 6 months to 12 years 2 months （median 60 months）. Results　The onset age of epileptic seizures ranged from 12 to 98 months （median 22 months）. The first seizure type was epileptic spasms in 36 cases （60.0%） and other seizure type in 23 children （39.0%）. Epileptic spasms were the only seizure type during entire period in 20 patients （33.9%）. There were also partial seizures in 17 cases （28.8%） and other generalized seizures in 22 patients （37.3%） during the course of disease. The EEG appeared classic hypsarrhythmia in 14（23.7%）， unilateral hypsarrhythmia in 2 （33.4%） and no hypsarrhythmia in 43 patients（72.9%）. Twenty-two patients （37.3%） presented with focal or unilateral clinical image and/or EEG abnormal. Forty-four patients were symptomatic epilepsies and the predominant diseases included perinatal brain injury， central nervous system infection and brain malformation. Fourteen patients （23.7%） met the criteria of late-onset West syndrome， 7 patients （11.9%） were diagnosed with Lennox-Gastaut syndrome. Fifty patients were responsible to ACTH therapy， including seizure-free in 32% of them. But recurrent rate was 61.5%. In 37 patients who were followed up， 46% maintained seizure free under taking the anti-epileptic drugs at the last follow up. Psychomotor development delay was observed in over 80% of patients who were followed up，16% of them had severe  egression. Multivariate regression with Cox analysis showed that etiologies， seizure types， phenotypes of epileptic spasms， and response to ACTH therapy all had no correlation with the long-term outcome of the seizure or psychomotor development. Conclusion　Epileptic spasms not only occur in West syndrome or infantile spasms， but also in the children beyond infantile period. Late-onset epileptic spasms may be caused by varied severe encephalopathy and it is usually accompanied with partial or other generalized seizures. Most cases do not present typical hypsarrhythmia on EEG. Similar to infantile spasms，most late-onset epileptic spasms are drug resistant and had a very poor outcome. ACTH therapy is still the first line therapeutic drug for such a catastrophic disease.