WONOEP 评估:针对早发性癫痫的靶向疗法开发。

IF 6.6 1区 医学 Q1 CLINICAL NEUROLOGY Epilepsia Pub Date : 2024-11-19 DOI:10.1111/epi.18187
Pablo M Casillas-Espinosa, Jennifer C Wong, Wanda Grabon, Ana Gonzalez-Ramos, Massimo Mantegazza, Nihan Carcak Yilmaz, Manisha Patel, Kevin Staley, Raman Sankar, Terence J O'Brien, Özlem Akman, Ganna Balagura, Adam L Numis, Jeffrey L Noebels, Stéphanie Baulac, Stéphane Auvin, David C Henshall, Aristea S Galanopoulou
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引用次数: 0

摘要

早发性癫痫包括一组异质性疾病,其中一些会导致耐药性癫痫发作、发育迟缓、精神并发症和猝死。随着靶向基因组以及基因组和外显子组测序技术的广泛应用,在这些患者中发现不同致病基因的工作取得了进展。识别早发性癫痫遗传基础的能力不断提高,新编码变异占已发现遗传病因的大多数。尽管目前针对特定疾病和改变疾病的疗法仍然有限,但小分子、细胞疗法和其他形式的早发性癫痫基因疗法等新型精准医疗方法在研究人员、临床医生和护理人员中引起了热烈反响。在此,我们总结了癫痫神经生物学研讨会(WONOEP XVI,法国塔卢瓦,2022 年 7 月)期间关于早发性癫痫靶向治疗的新型治疗策略的演讲和讨论的主要结果。发言讨论了氯化物转运体抑制剂在新生儿癫痫发作中的应用、针对儿童失神癫痫的奥曲肽能信号转导、针对Dravet综合征的能量代谢,以及大麻素受体2型、可逆性乙酰胆碱酯酶抑制剂、细胞疗法和基于RNA的疗法在早发性癫痫中的作用。
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WONOEP appraisal: Targeted therapy development for early onset epilepsies.

The early onset epilepsies encompass a heterogeneous group of disorders, some of which result in drug-resistant seizures, developmental delay, psychiatric comorbidities, and sudden death. Advancement in the widespread use of targeted gene panels as well as genome and exome sequencing has facilitated the identification of different causative genes in a subset of these patients. The ability to recognize the genetic basis of early onset epilepsies continues to improve, with de novo coding variants accounting for most of the genetic etiologies identified. Although current disease-specific and disease-modifying therapies remain limited, novel precision medicine approaches, such as small molecules, cell therapy, and other forms of genetic therapies for early onset epilepsies, have created excitement among researchers, clinicians, and caregivers. Here, we summarize the main findings of presentations and discussions on novel therapeutic strategies for targeted treatment of early onset epilepsies that occurred during the Workshop on Neurobiology of Epilepsy (WONOEP XVI, Talloires, France, July 2022). The presentations discussed the use of chloride transporter inhibitors for neonatal seizures, targeting orexinergic signaling for childhood absence epilepsy, targeting energy metabolism in Dravet syndrome, and the role of cannabinoid receptor type 2, reversible acetylcholinesterase inhibitors, cell therapies, and RNA-based therapies in early life epilepsies.

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来源期刊
Epilepsia
Epilepsia 医学-临床神经学
CiteScore
10.90
自引率
10.70%
发文量
319
审稿时长
2-4 weeks
期刊介绍: Epilepsia is the leading, authoritative source for innovative clinical and basic science research for all aspects of epilepsy and seizures. In addition, Epilepsia publishes critical reviews, opinion pieces, and guidelines that foster understanding and aim to improve the diagnosis and treatment of people with seizures and epilepsy.
期刊最新文献
Structural connectivity changes in focal epilepsy: Beyond the epileptogenic zone. Efficacy and safety of perampanel in patients with seizures associated with Lennox-Gastaut syndrome: A randomized trial. Focal negative motor seizures: Multimodal evaluation. Automatic responsiveness testing in epilepsy with wearable technology: The ARTiE Watch. WONOEP appraisal: Targeted therapy development for early onset epilepsies.
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