Dravet syndrome: Treatment options and management of prolonged seizures

Dravet syndrome: Treatment options and management of prolonged seizures
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DOI:
10.1111/epi.16334
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发表时间:
2019-12-01
期刊:
影响因子:
5.6
通讯作者:
Lagae, Lieven
Lagae, Lieven
中科院分区:
医学1区
文献类型:
--
作者:
Cross, J. Helen;Caraballo, Roberto H.;Lagae, Lieven

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随着时间的推移,通过对Dravet综合征的仔细描述,我们已经获得了最有可能改善癫痫发作的治疗经验,以及应该避免的治疗经验。丙戊酸钠、氯巴占、司替戊醇和托吡酯都是可能获益的药物,生酮饮食也是如此。溴化物可用于耐药病例。然而,同样重要的是概述了对长期癫痫发作的及时抢救治疗和避免促发剂。包括大麻二酚和芬氟拉明在内的较新药物已在临床试验中证明是有益的。我们提出了一个算法的管理,但认识到,新的代理人的定位尚未建立。
Over time, with careful delineation of Dravet syndrome, we have gained experience in treatments most likely to lead to improvement in seizures, as well as those that should be avoided. Sodium valproate, clobazam, stiripentol, and topiramate are all medications that may lead to benefit, as well as the ketogenic diet. Bromides may be utilized in resistant cases. However, equally important are outlining prompt rescue treatment for prolonged seizures and avoidance of precipitants. Newer agents including cannabidiol and fenfluramine have been demonstrated to be of benefit in clinical trials. We propose an algorithm for management, but appreciate that the positioning of newer agents is yet to be established.