【摘要】 目的 研究抗富亮氨酸胶质瘤失活蛋白1(leucine-rich glioma-inactivated 1,LGI 1)抗体脑炎的临床特征。方法 回顾性分析抗LGI 1抗体脑炎患者的临床表现、抗体检测、影像学检查、脑电图、治疗及预后。结果 11例患者年龄中位数63(51,66)，男女比为7:4。临床表现癫痫发作6例，记忆力下降7例，睡眠障碍5例，精神行为异常6例，面臂肌张力障碍发作（faciobrachial dystonic seizures,FBDS）4例，阵发性头晕3例。11例患者血清抗LGI1抗体阳性，7例血清和脑脊液抗LGI 1抗体均阳性。头颅MRI中2例基底节区异常信号，3例海马异常信号。脑电图异常者3例，2例慢波，1例棘波及慢波。低钠血症8例，11例患者均未发现肿瘤。11例均给予免疫球蛋白（intravenous immunoglobulin,IVIg）和/或激素治疗，7例联合吗替麦考酚酯治疗。入院时改良Rankin量表（modified Rankin Scale,mRS）评分2～3分，经免疫治疗后6～12个月随访，mRS评分0～1分，无复发，总体预后良好。结论 抗LGI 1抗体脑炎中老年男性多见，临床特征以癫痫发作、FBDS、记忆力下降、低钠血症为主要症状，血清抗LGI 1抗体较脑脊液更具有临床诊断意义，头颅MR可累及海马、基底节区，经免疫治疗效果良好。更多还原

【Abstract】 Objective To summarize the clinical characteristics of 11 patients with anti-LGI 1 antibody encephalitis.Methods The clinical manifestations,antibody detection,imaging findings,electroencephalogram,treatment and prognosis of 11 patients with anti LGI-1 antibody encephalitis were retrospectively analyzed.Results The average age of the 11 patients was 59.9 years and the ratio of male to female was 7:4.The clinical manifestations included epileptic seizures(54.5%),faciobrachial dystonic seizures(FBDS,36.4%),memory deficits(63.6%),sleep disorders(45.5%),Paroxysmal dizziness(27.3%),mental and behavioral(54.5%) and hyponatremia(72.7%).The serum anti-LGI 1 antibody was positive in 11 patients and both serum and CSF anti-LGI 1 antibody were positive in 7 patients.MRI scans which were performed in all patients,showed basal ganglia lesions in 2 cases,hippocampus lesions in 3 cases.The abnormal electroencephalogram was found in 3 cases including slow wave in 2 cases and spinous and slow wave in one case.No tumor was found in any patient.Eleven cases were treated with intravenous immunoglobulin and/or steroids and 7 patients were treated with Mycophenolate mofetil and antiepileptic drugs.The mRS score at admission was 2～3 and 0～1 after immunotherapy.There was no recurrence in 11 patients and the prognosis was good.Conclusion AntiLGI 1 antibody encephalitis is common in middle-aged and old male.The clinical manifestations include epileptic seizures,memory deficits,hyponatremia.Serum anti-LGI 1 antibody has a higher clinical diagnostic value than CSF anti-LGI 1 antibody.Anti-LGI 1 antibody encephalitis can involve the hippocampus and basal ganglia and the response to immunotherapy is good.更多还原