Department of Neurology, New York University School of Medicine, New York, NY, USA.
Department of Neurology, Erasmus University Medical Center, Rotterdam, The Netherlands.
Brain. 2021 Mar 3;144(2):372-390. doi: 10.1093/brain/awaa362.
Systemic autoimmune disorders occur more frequently in patients with epilepsy than in the general population, suggesting shared disease mechanisms. The risk of epilepsy is elevated across the spectrum of systemic autoimmune disorders but is highest in systemic lupus erythematosus and type 1 diabetes mellitus. Vascular and metabolic factors are the most important mediators between systemic autoimmune disorders and epilepsy. Systemic immune dysfunction can also affect neuronal excitability, not only through innate immune activation and blood-brain barrier dysfunction in most epilepsies but also adaptive immunity in autoimmune encephalitis. The presence of systemic autoimmune disorders in subjects with acute seizures warrants evaluation for infectious, vascular, toxic and metabolic causes of acute symptomatic seizures, but clinical signs of autoimmune encephalitis should not be missed. Immunosuppressive medications may have antiseizure properties and trigger certain drug interactions with antiseizure treatments. A better understanding of mechanisms underlying the co-existence of epilepsy and systemic autoimmune disorders is needed to guide new antiseizure and anti-epileptogenic treatments. This review aims to summarize the epidemiological evidence for systemic autoimmune disorders as comorbidities of epilepsy, explore potential immune and non-immune mechanisms, and provide practical implications on diagnostic and therapeutic approach to epilepsy in those with comorbid systemic autoimmune disorders.
系统性自身免疫性疾病在癫痫患者中的发病率高于普通人群，提示存在共同的发病机制。系统性自身免疫性疾病谱中癫痫的风险增加，但在系统性红斑狼疮和 1 型糖尿病中最高。血管和代谢因素是系统性自身免疫性疾病与癫痫之间最重要的中介。系统性免疫功能障碍还可以通过先天免疫激活和大多数癫痫中的血脑屏障功能障碍以及自身免疫性脑炎中的适应性免疫来影响神经元兴奋性。急性发作的患者存在系统性自身免疫性疾病，需要评估急性症状性发作的感染、血管、毒性和代谢原因，但不应忽视自身免疫性脑炎的临床症状。免疫抑制药物可能具有抗癫痫特性，并与抗癫痫治疗发生某些药物相互作用。需要更好地了解癫痫与系统性自身免疫性疾病共存的机制，以指导新的抗癫痫和抗癫痫发作治疗。本综述旨在总结系统性自身免疫性疾病作为癫痫合并症的流行病学证据，探讨潜在的免疫和非免疫机制，并就合并系统性自身免疫性疾病的癫痫患者的诊断和治疗方法提供实用建议。
