Ostendorf Adam P, Ng Yu-Tze
Department of Pediatrics, Neurology Section, Nationwide Children's Hospital, The Ohio State University, Columbus, OH.
Department of Pediatrics, Baylor College of Medicine, The Children's Hospital of San Antonio, San Antonio, TX, USA.
Neuropsychiatr Dis Treat. 2017 Apr 20;13:1131-1140. doi: 10.2147/NDT.S115996. eCollection 2017.
Lennox-Gastaut syndrome is a severe, childhood-onset electroclinical syndrome comprised of multiple seizure types, intellectual and behavioral disturbances and characteristic findings on electroencephalogram of slow spike and wave complexes and paroxysmal fast frequency activity. Profound morbidity often accompanies a common and severe seizure type, the drop attack. Seizures often remain refractory, or initial treatment efficacy fades. Few individuals are seizure free despite the development of multiple generations of antiseizure medications over decades and high-level evidence on several choices. Approved medications such as lamotrigine, topiramate, rufinamide, felbamate and clobazam have demonstrated efficacy in reducing seizure burden. Cannabidiol has emerged as a promising investigational therapy with vast social interest yet lacks a standard, approved formulation. Palliative surgical procedures, such as vagal nerve stimulation and corpus callosotomy may provide reduction in total seizures and drop attacks. Emerging evidence suggests that complete callosotomy provides greater improvement in seizures without additional side effects. Etiologies such as dysplasia or hypothalamic hamartoma may be amenable for focal resection and thus offer potential to reverse this devastating epileptic encephalopathy.
伦诺克斯-加斯托综合征是一种严重的儿童期起病的电临床综合征，由多种发作类型、智力和行为障碍以及脑电图上慢棘慢复合波和阵发性快频率活动的特征性表现组成。严重的发病率常伴随着一种常见且严重的发作类型——跌倒发作。癫痫发作往往难以控制，或者初始治疗效果逐渐消失。尽管数十年来开发了多代抗癫痫药物，并且有几种选择的高级别证据，但很少有人能无癫痫发作。已批准的药物，如拉莫三嗪、托吡酯、卢非酰胺、非氨酯和氯巴占，已证明在减轻癫痫发作负担方面有效。大麻二酚已成为一种有前景的研究性疗法，引起了广泛的社会关注，但缺乏标准的、已批准的制剂。姑息性手术，如迷走神经刺激术和胼胝体切开术，可能会减少癫痫发作总数和跌倒发作。新出现的证据表明，完全胼胝体切开术能在不产生额外副作用的情况下，使癫痫发作得到更大改善。发育异常或下丘脑错构瘤等病因可能适合进行病灶切除，从而有可能逆转这种毁灭性的癫痫性脑病。
