抗富含亮氨酸胶质瘤失活蛋白 1抗体相关自身免疫性脑炎 1例并文献复习

目的 探讨、总结抗富含亮氨酸胶质瘤失活蛋白1(LGl1)抗体相关自身免疫性脑炎的临床表现、辅助检查、诊断思路和免疫治疗方法等,以期能不断丰富对自身免疫性脑炎的认识.方法 回顾分析2016年4月南京鼓楼医院集团安庆市石化医院1例抗LGI1抗体相关自身免疫性脑炎的诊治体会,并进行文献复习.结果 LGI1抗体相关自身免疫性脑炎主要表现为认知功能障碍、精神行为异常、癫痫发作等,其中面-臂肌张力障碍发作(FBDS)是该病特征性发作症状,血和脑脊液抗LGI1抗体均阳性,激素冲击治疗预后良好.结论 以认知功能障碍、精神行为异常并有特征性FBDS者,要考虑抗LGI1抗体相关自身免疫性脑炎,治疗上激素冲击治疗可能优于静脉注射免疫球蛋白(IVIg).

LGll antibody-related limbic encephalitis: a case report and literature review

Objective To explore and summarize the clinical manifestations, auxiliary examination, diagnostic ideas and immunotherapy methods of anti-leucine-rich glioma inactivated 1 (LGI1) encephalitis, in order to enrich the understanding of autoimmune encephalitis.Methods A retrospective analysis of diagnosis and treatment about one anti-LGI1 antibody-related autoimmune encephalitis admitted to Anqing Shihua Hospital of Nanjing Drum Tower Hospital Group in April 2016 was conducted, meanwhiles the literatures were reviewed.Results Anti-LGI1 encephalitis mainly manifested as cognitive dysfunction, epileptic seizures ,abnormal mentalbehavior and so on. Especially, the "faciobrachial dystonic seizure (FBDS)" is a characteristic episode of the disease, the anti-LGI1 antibodies in CSF and serum are positive, and the prognosis of corticosteroids pulse therapy is good.Conclusion The patients with cognitive dysfunction, abnormal mental behavior and FBDS should be considered as anti-LGI1 antibody-related encephalitis. Treatment with corticosteroids pulse therapy may be superior to intravenous therapy with immune globulin.