Abstract:
Lennox-Gastaut syndrome (LGS) is a severe, chronic, complex form of early childhood-onset epilepsy characterized by multiple seizure types, generalized slow (≤2.5 Hz) spike-and-wave activity and other electroencephalography abnormalities, and cognitive impairment. A key treatment goal is early seizure control, and several anti-seizure medications (ASMs) are available. Due to the low success rate in achieving seizure control with monotherapy and an absence of efficacy data supporting any particular combination of ASMs for treating LGS, a rational approach to selection of appropriate polytherapy should be applied to maximize benefit to patients. Such \"rational polytherapy\" involves consideration of factors including safety (including boxed warnings), potential drug-drug interactions, and complementary mechanisms of action. Based on the authors\' clinical experience, rufinamide offers a well-considered first adjunctive therapy for LGS, particularly in combination with clobazam and other newer agents for LGS, and may be particularly useful for reducing the frequency of tonic-atonic seizures associated with LGS.
摘要:
Lennox-Gastaut综合征(LGS)是一种严重的,慢性,以多种癫痫发作类型为特征的儿童早期发作癫痫的复杂形式,广义慢(≤2.5Hz)尖峰波活动和其他脑电图异常,和认知障碍。一个关键的治疗目标是早期控制癫痫发作,和几种抗癫痫药物(ASM)是可用的。由于单药治疗实现癫痫发作控制的成功率较低,并且缺乏支持任何特定ASM组合治疗LGS的疗效数据,应采用合理的方法选择适当的综合疗法,以使患者受益最大化。这种“合理的综合疗法”涉及考虑包括安全性(包括盒装警告)在内的因素,潜在的药物-药物相互作用,和互补的行动机制。根据作者的临床经验,rufinamide为LGS提供了经过深思熟虑的第一辅助治疗,特别是与Clobazam和其他较新的LGS代理商结合使用,并且可能对降低与LGS相关的强直-失超性癫痫发作的频率特别有用。