Yıldız Edibe Pembegül, Hızlı Zeynep, Bektaş Gonca, Ulak-Özkan Melis, Tatlı Burak, Aydınlı Nur, Çalışkan Mine, Özmen Meral
Departments of Pediatric Neurology, Istanbul University Istanbul Faculty of Medicine, İstanbul, Turkey.
Departments of Pediatrics, Istanbul University Istanbul Faculty of Medicine, İstanbul, Turkey.
Turk J Pediatr. 2018;60(3):238-243. doi: 10.24953/turkjped.2018.03.002.
Yıldız EP, Hızlı Z, Bektaş G, Ulak-Özkan M, Tatlı B, Aydınlı N, Çalışkan M, Özmen M. Efficacy of rufinamide in childhood refractory epilepsy. Turk J Pediatr 2018; 60: 238-243. Rufinamide has been used as a new antiepileptic drug in the treatment of drug-resistant epilepsy, in recent years. The objective of this study was to evaluate the reliability of rufinamide and its impact on seizure frequency in patients diagnosed with drug-resistant epilepsy, where seizures could not be controlled with classical antiepileptic drugs. We retrospectively reviewed the data of epileptic patients who were followed up between January 2004 and December 2014 in the Pediatric Neurology Department. Patients who were diagnosed with drug resistant epilepsy and treated with rufinamide were evaluated. Decrease in seizure frequency and drug side effects were assessed as parameters. A total of 38 patients (14 girls, 24 boys) with a mean age of 8.5 (range, 3.5-17) years were included in the study. The mean follow-up duration was 25.5 (23-29.5) months, while the mean maximal dose of rufinamide was 32.5 (28-42) mg/kg/day. Response to treatment was assessed by the reduction in frequency of seizures. The decrease was < 50% (essentially unresponsive to treatment) in 20 patients and 50‑99% in 8 patients. Ten patients (26.3%) remained seizure-free. The response rate for tonic seizures was 50%. In drop/attacks seizures, this ratio was found as 73%, which was quite high. Patients with myoclonic and tonic-clonic seizures did not significantly benefit from rufinamide. The rate of patients with Lennox-Gestaut syndrome (LGS) who responded very well (reduction in seizure frequency > 50%) was 55.5%. In the LGS group, patients with drop/attacks showed the best response to treatment. Rufinamide was not effective in two patients diagnosed with Dravet syndrome. Rufinamide can be safely used in pediatric patients who use multiple antiepileptic drugs and are unresponsive to the treatment. It was seen to be effective especially in patients diagnosed with LGS and drop/attacks types of seizures.
于尔迪兹·E·P、赫兹利·Z、贝伊塔什·G、乌拉克-厄兹坎·M、塔特利·B、艾登利·N、恰尔什坎·M、厄兹门·M。鲁非酰胺治疗儿童难治性癫痫的疗效。《土耳其儿科学杂志》2018年；60: 238 - 243。近年来，鲁非酰胺已作为一种新型抗癫痫药物用于治疗耐药性癫痫。本研究的目的是评估鲁非酰胺的可靠性及其对诊断为耐药性癫痫患者癫痫发作频率的影响，这些患者使用“经典”抗癫痫药物无法控制癫痫发作。我们回顾性分析了2004年1月至2014年12月在儿科神经科随访的癫痫患者的数据。对诊断为“耐药性癫痫”并接受鲁非酰胺治疗的患者进行了评估。将癫痫发作频率的降低和药物副作用作为参数进行评估。共有38例患者（14例女孩，24例男孩）纳入研究，平均年龄8.5岁（范围3.5 - 17岁）。平均随访时间为25.5个月（23 - 29.5个月），而鲁非酰胺的平均最大剂量为32.5毫克/千克/天（28 - 42毫克/千克/天）。通过癫痫发作频率的降低来评估治疗反应。20例患者癫痫发作频率降低<50%（基本对治疗无反应），8例患者降低50 - 99%。10例患者（26.3%）癫痫发作停止。强直发作的反应率为50%。在跌倒/发作性癫痫发作中，该比例为73%，相当高。肌阵挛和强直 - 阵挛发作的患者未从鲁非酰胺中显著获益。伦诺克斯 - 加斯东综合征（LGS）患者中反应良好（癫痫发作频率降低>50%）的比例为55.5%。在LGS组中，跌倒/发作性癫痫发作的患者对治疗反应最佳。鲁非酰胺对两名诊断为德雷维特综合征的患者无效。鲁非酰胺可安全用于使用多种抗癫痫药物且对治疗无反应的儿科患者。尤其在诊断为LGS和跌倒/发作性癫痫发作类型的患者中可见其有效。
