Department of Neurology, NYU Langone School of Medicine, New York, NY.
Epilepsy Curr. 2013 Mar;13(2):62-8. doi: 10.5698/1535-7597-13.2.62.
Systemic autoimmune disorders affect multiple organ systems. Brain involvement commonly causes seizures, which may be the presenting symptom. Systemic lupus erythematosus, Sjorgren's syndrome, Wegener's granulomatosis, sarcoidsosis, celiac disease, Crohn's disease, Behcet's, and Hashimoto's encephalopathy are reviewed. Mechanisms underlying CNS pathology in systemic autoimmune disorders-and specifically factors predisposing these patients-are discussed, including vascular disease (e.g., prothrombotic state, anticardiolipin antibody, emboli, vasculitis), antineuronal antibodies, immune complexes, cytokines, metabolic disorders, infection, and therapy. Diagnostic and therapeutic strategies must be individualized for both the disorder and the patient. Systemic autoimmune disorders affect multiple organ systems and frequently involve the central and peripheral nervous systems. Seizures are among the most common neurological manifestation and occasionally can be the presenting symptom. There are many causes of seizures in systemic autoimmune disorders (Table 1), and the first clinical challenge is to determine not only the cause but also the significance of seizures. In some cases, they are clues to metabolic or infectious disorders or medication toxicity; in other cases, seizures herald a life-threatening progression of the underlying illness.
系统性自身免疫性疾病影响多个器官系统。脑受累通常会导致癫痫发作，这可能是其首发症状。本文回顾了系统性红斑狼疮、干燥综合征、韦格纳肉芽肿病、结节病、乳糜泻、克罗恩病、贝赫切特病和桥本脑病。讨论了系统性自身免疫性疾病中中枢神经系统病理的机制，以及易导致这些患者发生癫痫的因素，包括血管疾病（如高凝状态、抗心磷脂抗体、栓子、血管炎）、抗神经元抗体、免疫复合物、细胞因子、代谢紊乱、感染和治疗。诊断和治疗策略必须针对疾病和患者进行个体化。系统性自身免疫性疾病影响多个器官系统，常累及中枢和外周神经系统。癫痫发作是最常见的神经系统表现之一，偶尔也可能是首发症状。在系统性自身免疫性疾病中，癫痫发作有许多原因（表 1），临床面临的首要挑战不仅是确定病因，还要确定癫痫发作的意义。在某些情况下，癫痫发作是代谢或感染性疾病或药物毒性的线索；在其他情况下，癫痫发作预示着潜在疾病的致命进展。
