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朗格汉斯细胞组织细胞增生症:当前治疗方法、新疗法和未来方向。

Lennox-Gastaut Syndrome: Current Treatments, Novel Therapeutics, and Future Directions.

机构信息

Section of Child Neurology, Children's Hospital Colorado, Anschutz School of Medicine, University of Colorado, Aurora, CO, USA.

出版信息

Neurotherapeutics. 2023 Sep;20(5):1255-1262. doi: 10.1007/s13311-023-01397-x. Epub 2023 Jun 23.

Abstract

Lennox-Gastaut syndrome is a severe drug-resistant developmental and epileptic encephalopathy with slow spike and wave on EEG (DEE-SSW) composing about 1-2% of epilepsy patients. Seizures in DEE-SSW are caused by a variety of etiologies, and there is a large unmet treatment need as seizures are usually treatment-resistant and individuals are often unable to function independently. The updated definition by the International League Against Epilepsy has established formal diagnostic criteria allowing for more uniform diagnosis. This article provides a review of typical medication management and treatment strategies, including new and developing surgical approaches. Future directions in treatment include expanding genetic testing with the potential for gene therapy and continuously improving surgical options with the goal to prevent progression to DEE-SSW.

摘要

Lennox-Gastaut 综合征是一种严重的药物难治性发育性和癫痫性脑病,脑电图上表现为慢棘慢波(DEE-SSW),约占癫痫患者的 1-2%。DEE-SSW 的发作由多种病因引起,治疗需求巨大,但由于发作通常对治疗有抵抗性,且个体通常无法独立生活,因此往往难以满足。国际抗癫痫联盟的最新定义确立了正式的诊断标准,从而实现了更统一的诊断。本文综述了典型的药物管理和治疗策略,包括新出现和发展中的手术方法。未来的治疗方向包括扩大基因检测,可能包括基因治疗,以及不断改进手术选择,目标是防止进展为 DEE-SSW。

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