促皮质激素治疗大田原综合征的疗效观察.docVIP

促皮质激素治疗大田原综合征的疗效观察 【摘要】nbsp; 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。 【关键词】nbsp; 促肾上腺皮质激素 大田原综合征 视频脑电图 抑制图形 nbsp;nbsp; Evaluation to efficacy of adrenocorticotropic hormone on Ohtahara syndrome nbsp;nbsp;nbsp; CHEN Yan1， CHEN Li1，WU Tao2，XIAO Zhitian1，HU Yan1，LIAO Jianxiang1，CHEN Weiyan1（1.Shenzhen Children’s Hospital of Gungdong Province,Guangdong Shenzhennbsp; 518026,China;2.Zhongshan Boai Hospital of Guangdong Province,Guangdong Zhongshannbsp; 528403，China） nbsp;nbsp;nbsp; ［Abstract］ Objective： To evaluate the efficacy of adrenocorticotropic hormone on Ohtahara syndrome. Methods:To analyze retrospectively clinical and videoEEG characteristics before and after adrenocorticorticotropic 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EE