促皮质激素治疗大田原综合征的疗效观察  

Evaluation to efficacy of adrenocorticotropic hormone on Ohtahara syndrome

作　　者：陈彦[1] 陈黎[1] 吴涛[2] 肖志田[1] 胡雁[1] 廖建湘[1] 陈伟燕[1] 

机构地区：[1]广东省深圳市儿童医院,广东深圳518026 [2]广东省中山市博爱医院,广东中山528403

出　　处：《儿科药学杂志》2008年第4期14-16,共3页Journal of Pediatric Pharmacy

摘　　要：目的:通过分析大田原综合征(OS)用促皮质激素(ACTH)治疗前后临床和视频脑电图变化特点,评价ACTH治疗OS的疗效。方法:收集深圳市儿童医院癫痫中心1999年5月至2006年12月住院的12例OS患儿的临床相关资料,回顾性分析其用ACTH治疗前后视频脑电图变化特点。结果:用药前12例患儿发作间期脑电图均见暴发-抑制现象出现,临床可见痉挛发作或强直痉挛发作及(或)部分性发作,并可见同期特征性发作图形。ACTH治疗2周后,2例患儿发作无明显改变;10例(83%)临床痉挛发作或强直痉挛发作改善,或痉挛停止,其中3例发作幅度减轻,发作频率减少,3例患儿视频脑电图可见家长不易注意到的微小临床发作,另外有1例患儿未见强直性痉挛发作,但仍有部分性发作存在,3例(25%)患儿完全未见临床发作。发作间期背景脑电图有一定改善,无发作病例脑电图暴发-抑制现象消失。结论:大田原综合征用ACTH短期效果较好,可以作为一种治疗选择。用ACTH后视频脑电图监测是判断其疗效的重要方法。Objective： To evaluate the efficacy of adrenocorticotropic hormone on Ohtahara syndrome. Methods： To analyze retrospectively clinical and video-EEG characteristics before and after adrenoconicorticotropic hormone treatment in 12 patients with Ohtahara syndrome. Results： The interictal period EEG demonstrated a burst-suppression pattern during waking and sleeping states in OS, axial spasms and the partial seizures the interictal EEG were recorded. After two-week treatment with ACTH, there was no any effect in two cases, 10 cases （83%） got better in their spasms, among them three cases still had minor seizures, three cases had sutble seizures only captured by video-EEG but not recognized by parents, one case only left partial seizures. The left three cases （25%） became seizure （spasms） free. The EEG background improved in all cases. Burst-suppression pattern resolved in spasm free patients. Conclusions： With help of video-EEG monitoring we find clinical and EEG status getting better in OS patients treated with ACTH. ACTH might be a choice of OS treatment. Video-EEG might have important role in efficacy evaluation of OS treatment.

关 键 词：促肾上腺皮质激素 大田原综合征 视频脑电图 暴发-抑制图形 

分 类 号：R748[医药卫生—神经病学与精神病学]