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Current Pharmacologic Strategies for Treatment of Intractable Epilepsy in Children.
International Neurourology Journal ( IF 2.3 ) Pub Date : 2021-05-31 , DOI: 10.5213/inj.2142166.083
Ja Un Moon , Kyung-Ok Cho

Epileptic encephalopathy (EE) is a devastating pediatric disease that features medically resistant seizures, which can contribute to global developmental delays. Despite technological advancements in genetics, the neurobiological mechanisms of EEs are not fully understood, leaving few therapeutic options for affected patients. In this review, we introduce the most common EEs in pediatrics (i.e., Ohtahara syndrome, Dravet syndrome, and Lennox-Gastaut syndrome) and their molecular mechanisms that cause excitation/inhibition imbalances. We then discuss some of the essential molecules that are frequently dysregulated in EEs. Specifically, we explore voltage-gated ion channels, synaptic transmission-related proteins, and ligand-gated ion channels in association with the pathophysiology of Ohtahara syndrome, Dravet syndrome, and Lennox-Gastaut syndrome. Finally, we review currently available antiepileptic drugs used to treat seizures in patients with EEs. Since these patients often fail to achieve seizure relief even with the combination therapy, further extensive research efforts to explore the involved molecular mechanisms will be required to develop new drugs for patients with intractable epilepsy.

中文翻译:

目前治疗儿童顽固性癫痫的药理学策略。

癫痫性脑病 (EE) 是一种破坏性的儿科疾病,其特征是癫痫发作具有抗药性,可导致全球发育迟缓。尽管遗传学技术取得了进步,但尚未完全了解 EE 的神经生物学机制,因此受影响的患者几乎没有治疗选择。在这篇综述中,我们介绍了儿科中最常见的 EE(即 Ohtahara 综合征、Dravet 综合征和 Lennox-Gastaut 综合征)及其导致兴奋/抑制失衡的分子机制。然后我们讨论了一些在 EE 中经常失调的基本分子。具体而言,我们探索了与 Ohtahara 综合征、Dravet 综合征和 Lennox-Gastaut 综合征的病理生理学相关的电压门控离子通道、突触传递相关蛋白和配体门控离子通道。最后,我们回顾了目前可用于治疗 EE 患者癫痫发作的抗癫痫药物。由于这些患者即使联合治疗也常常无法缓解癫痫发作,因此需要进一步广泛的研究工作来探索所涉及的分子机制,为难治性癫痫患者开发新药。
更新日期:2021-06-02
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