Jang Yoonhyuk, Kim Dong Wook, Yang Kwang Ik, Byun Jung Ick, Seo Jong Geun, No Young Joo, Kang Kyung Wook, Kim Daeyoung, Kim Keun Tae, Cho Yong Won, Lee Soon Tae
Department of Neurology, Seoul National University Hospital, Seoul, Korea.
Department of Neurology, Konkuk University School of Medicine, Seoul, Korea.
J Clin Neurol. 2020 Oct;16(4):519-529. doi: 10.3988/jcn.2020.16.4.519.
Autoimmune epilepsy is a newly emerging area of epilepsy. The concept of "autoimmune" as an etiology has recently been revisited thanks to advances in autoimmune encephalitis and precision medicine with immunotherapies. Autoimmune epilepsy presents with specific clinical manifestations, and various diagnostic approaches including cerebrospinal fluid analysis, neuroimaging, and autoantibody tests are essential for its differential diagnosis. The diagnosis is often indeterminate despite performing a thorough evaluation, and therefore empirical immunotherapy may be applied according to the judgment of the clinician. Autoimmune epilepsy often manifests as new-onset refractory status epilepticus (NORSE). A patient classified as NORSE should receive empirical immunotherapy as soon as possible. On the other hand, a morecautious, stepwise approach is recommended for autoimmune epilepsy that presents with episodic events. The type of autoimmune epilepsy is also an important factor to consider when choosing from among various immunotherapy options. Clinicians should additionally take the characteristics of antiepileptic drugs into account when using them as an adjuvant therapy. This expert opinion discusses the diagnostic and treatment approaches for autoimmune epilepsy from a practical point of view.
自身免疫性癫痫是癫痫领域中一个新兴的领域。由于自身免疫性脑炎和免疫治疗精准医学的进展，“自身免疫”作为病因的概念最近得到了重新审视。自身免疫性癫痫具有特定的临床表现，包括脑脊液分析、神经影像学和自身抗体检测在内的各种诊断方法对其鉴别诊断至关重要。尽管进行了全面评估，诊断往往仍不明确，因此可根据临床医生的判断应用经验性免疫治疗。自身免疫性癫痫常表现为新发难治性癫痫持续状态（NORSE）。被归类为NORSE的患者应尽快接受经验性免疫治疗。另一方面，对于表现为发作性事件的自身免疫性癫痫，建议采用更谨慎、逐步的方法。在从各种免疫治疗方案中进行选择时，自身免疫性癫痫的类型也是一个需要考虑的重要因素。临床医生在将抗癫痫药物用作辅助治疗时，还应考虑其特性。本专家意见从实际角度讨论了自身免疫性癫痫的诊断和治疗方法。
