与自身免疫性疾病相关的癫痫发作-目前的治疗方法

IF 2 4区 医学 Q3 CLINICAL NEUROLOGY
Seminars in Neurology Pub Date : 2025-04-01 Epub Date: 2025-03-12 DOI:10.1055/a-2525-3511
Lisa Gillinder, Jeffrey W Britton
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引用次数: 0

摘要

自身免疫相关的癫痫发作和癫痫在临床实践中得到越来越多的认识,可在急性脑炎的情况下出现,但在某些情况下可能出现慢性局灶性癫痫。这些情况通常对抗癫痫治疗有抵抗性,但可能对及时的免疫治疗有明确的反应。早期诊断和治疗对于减少神经损伤和优化预后至关重要。治疗以共识意见为指导,因为目前缺乏明确的试验。最初的治疗方法通常包括一线药物,如皮质类固醇、静脉注射免疫球蛋白(IVIg)或血浆置换,二线药物如利妥昔单抗或环磷酰胺。维持治疗被认为可以预防复发,复发发生率高达35%。复发管理需要仔细区分脑后癫痫,在没有活动性炎症的情况下,免疫治疗没有反应。本文综述了自身免疫相关癫痫的治疗策略,包括急性症状性发作和癫痫。我们讨论了基于潜在发病机制的预期结果,包括针对特定神经元表面/突触抗原和细胞内表位的自身抗体介导的病例,以及缺乏明确生物标志物的病例。本文概述了针对抗lgi1、抗nmdar、抗gaba - br和抗gad65脑肽等疾病的具体方法,强调基于病理生理学和临床背景的量身定制的免疫治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Seizures Associated with Autoimmune Disorders - Current Treatment Approaches.

Autoimmune-associated seizures and epilepsy are increasingly recognized in clinical practice and can arise in the setting of acute encephalitis but in some cases may present with chronic focal epilepsy. These conditions are usually resistant to antiseizure therapy but may respond definitively to timely immunotherapy. Early diagnosis and treatment are critical to minimize neural injury and optimize outcomes. Treatment is guided by consensus opinion because definitive trials are currently lacking. The initial management approach usually involves first-line agents such as corticosteroids, intravenous immunoglobulin (IVIg), or plasma exchange, with second-line agents like rituximab or cyclophosphamide. Maintenance therapy is considered to prevent relapses, which occur in up to 35% of patients. Relapse management requires careful differentiation from postencephalitic epilepsy, which in the absence of active inflammation does not respond to immunotherapy. This review discusses treatment strategies for autoimmune-associated seizure disorders, including acute symptomatic seizures and epilepsy. We discuss expected outcomes on the basis of the underlying pathogenesis including cases mediated by autoantibodies targeting specific neuronal surface/synaptic antigens, and intracellular epitopes, and for cases lacking defined biomarkers. Specific approaches are outlined for disorders such as anti-LGI1, anti-NMDAR, anti-GABA-BR, and anti-GAD65 encephalitides, emphasizing tailored immunotherapy based on pathophysiology and clinical context.

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来源期刊
Seminars in Neurology
Seminars in Neurology 医学-临床神经学
CiteScore
4.60
自引率
3.70%
发文量
65
审稿时长
6-12 weeks
期刊介绍: Seminars in Neurology is a review journal on current trends in the evaluation, diagnosis, and treatment of neurological diseases. Areas of coverage include multiple sclerosis, central nervous system infections, muscular dystrophy, neuro-immunology, spinal disorders, strokes, epilepsy, motor neuron diseases, movement disorders, higher cortical function, neuro-genetics and neuro-ophthamology. Each issue is presented under the direction of an expert guest editor, and invited contributors focus on a single, high-interest clinical topic. Up-to-the-minute coverage of the latest information in the field makes this journal an invaluable resource for neurologists and residents.
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