Epilepsy in children with tuberous sclerosis complex: Chance of remission and response to antiepileptic drugs
Epilepsy in children with tuberous sclerosis complex: Chance of remission and response to antiepileptic drugs
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DOI：
10.1111/epi.13050
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发表时间：
2015-08-01
期刊：
影响因子：
5.6
通讯作者：
de Wit, Marie-Claire Y.
中科院分区：
文献类型：
--
作者：
Overwater, Iris E.;Bindels-de Heus, Karen;de Wit, Marie-Claire Y.
目的描述儿童结节性硬化症（TSC）癫痫的治疗和结局。方法纳入1988年至2014年在ENCORE TSC专家中心治疗的71例TSC和癫痫儿童。从我们的临床数据库中提取患者特征和抗癫痫治疗的持续时间和有效性。癫痫发作后复发的治疗反应，和几个病人的characteristics.ResultsMedian年龄为9.4岁（范围0.9-18.0）时，列入之间的相关性。71例患儿中有55例（77%）发作后1个月以上无发作，21例（30%）发作后24个月以上无发作。癫痫发作的缓解与较高的智商有关，癫痫发作缓解与癫痫发作时的年龄之间存在趋势。共使用了19种抗癫痫药物（AEDs）。丙戊酸、氨己烯酸、左乙拉西坦和卡马西平的使用频率最高。非药物治疗（生酮饮食，癫痫手术和迷走神经刺激）使用了13次。癫痫手术是最有效的，五个孩子中有四个没有癫痫。作为第一次和第二次治疗处方的AED最有效。丙戊酸最常作为第一次和第二次治疗处方，其次是氨己烯酸。31名儿童患有婴儿痉挛，其中18名儿童（58%）在此之前出现局灶性癫痫发作。29名儿童（94%）使用了氨己烯酸，15名儿童（48%）首次接受治疗。Vigabatrin是更有效的比其他AEDs处方时，作为第一treatment.SignificanceWe表明，虽然77%的儿童癫痫由于TSC达到癫痫发作缓解，通常在他们的第一或第二AED，这是持续至少24个月，只有38%。几乎一半的24个月缓解后癫痫复发。我们的研究结果支持氨己烯酸作为首选药物，并显示需要更好的治疗方案，为这些儿童。
ObjectiveTo describe treatment and outcome of epilepsy in children with tuberous sclerosis complex (TSC).MethodsSeventy-one children with TSC and epilepsy treated at the ENCORE TSC Expertise Center between 1988 and 2014 were included. Patient characteristics and duration and effectiveness of antiepileptic treatments were extracted from our clinical database. Correlations were made between recurrence of seizures after response to treatment, and several patient characteristics.ResultsMedian age at time of inclusion was 9.4years (range 0.9-18.0). Seizure history showed that 55 children (77%) of 71 became seizure-free for longer than 1month, and 21 (30%) of 71 for longer than 24months. Remission of seizures was associated with higher IQ, and a trend was observed between seizure remission and age at onset of seizures. A total of 19 antiepileptic drugs (AEDs) were used. Valproic acid, vigabatrin, levetiracetam, and carbamazepine were used most frequently. Nonpharmacologic therapies (ketogenic diet, epilepsy surgery, and vagus nerve stimulation) were used 13 times. Epilepsy surgery was most effective, with four of five children becoming seizure-free. AEDs prescribed as first and second treatment were most effective. Valproic acid was prescribed most frequently as first and second treatment, followed by vigabatrin. Thirty-one children had infantile spasms, preceded by focal seizures in 18 children (58%). Vigabatrin was used by 29 children (94%), and was first treatment in 15 (48%). Vigabatrin was more effective than other AEDs when prescribed as first treatment.SignificanceWe showed that, although 77% of children with epilepsy due to TSC reached seizure remission, usually after their first or second AED, this was sustained for at least 24months in only 38%. Almost half of those with 24 months of remission later had relapse of seizures. Our results support vigabatrin as first choice drug, and show the need for better treatment options for these children.