Bakpa Ochuko D, Reuber Markus, Irani Sarosh R
Academic Neurology Unit, Royal Hallamshire Hospital, University of Sheffield, Sheffield S10 2JF, UK.
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford OX3 9DS, UK.
Seizure. 2016 Oct;41:26-41. doi: 10.1016/j.seizure.2016.07.002. Epub 2016 Jul 15.
The growing recognition of epilepsies and encephalopathies associated with autoantibodies against surface neuronal proteins (LGI1, NMDAR, CASPR2, GABABR, and AMPAR) means that epileptologists are increasingly asking questions about mechanisms of antibody-mediated epileptogenesis, and about the use of immunotherapies. This review summarizes clinical and paraclinical observations related to autoimmune epilepsies, examines the current evidence for the effectiveness of immunotherapy, and makes epilepsy-specific recommendations for future research.
Systematic literature search with summary and review of the identified publications. Studies describing the clinical characteristics of autoantibody-associated epilepsies and treatments are detailed in tables.
Literature describing the clinical manifestations and treatment of autoimmune epilepsies associated with neuronal cell-surface autoantibodies (NSAbs) is largely limited to retrospective case series. We systematically summarize the features of particular interest to epileptologists dividing patients into those with acute or subacute encephalopathies associated with epilepsy, and those with chronic epilepsy without encephalopathy. Available observational studies suggest that immunotherapies are effective in some clinical circumstances but outcome data collection methods require greater standardization.
The clinical experience captured suggests that clusters of clinical features associate well with specific NSAbs. Intensive and early immunotherapy is indicated when patients present with autoantibody-associated encephalopathies. It remains unclear how patients with chronic epilepsy and the same autoantibodies should be assessed and treated. Tables in this paper provide a comprehensive resource for systematic descriptions of both clinical features and treatments, and highlight limitations of current studies.
对与针对神经元表面蛋白（LGI1、NMDAR、CASPR2、GABABR和AMPAR）的自身抗体相关的癫痫和脑病的认识不断增加，这意味着癫痫学家越来越多地询问抗体介导的癫痫发生机制以及免疫疗法的使用情况。本综述总结了与自身免疫性癫痫相关的临床和临床旁观察结果，审查了免疫疗法有效性的现有证据，并对未来研究提出了针对癫痫的建议。
对已识别的出版物进行系统的文献检索、总结和综述。描述自身抗体相关癫痫临床特征和治疗的研究详细列于表格中。
描述与神经元细胞表面自身抗体（NSAbs）相关的自身免疫性癫痫临床表现和治疗的文献主要限于回顾性病例系列。我们将患者分为与癫痫相关的急性或亚急性脑病患者以及无脑病的慢性癫痫患者，系统地总结了癫痫学家特别感兴趣的特征。现有观察性研究表明，免疫疗法在某些临床情况下有效，但结果数据收集方法需要更大程度的标准化。
所获得的临床经验表明，临床特征群与特定的NSAbs密切相关。当患者出现自身抗体相关脑病时，应进行强化和早期免疫治疗。对于患有慢性癫痫且具有相同自身抗体的患者应如何评估和治疗仍不清楚。本文中的表格为临床特征和治疗的系统描述提供了全面的资源，并突出了当前研究的局限性。
