抗LGI 1抗体脑炎临床特征分析  

Clinical characteristics of anti LGI 1 antibody encephalitis

作　　者：刘婷 史福平[1] 陈亚南 蔡耘[1] 关鸿志[2] 任海涛[2] 张杏红[1] 刘娜 王雅彤 邸卫英[1] LIU Ting;SHI Fuping;CHEN Yanan;CAI Yun;GUAN Hongzhi;REN Haitao;ZHANG Xinghong;LIU Na;WANG Yatong;DI Weiying(Department of Neurology,Affiliated Hospital of Hebei University,Baoding 071000,China)

机构地区：[1]河北大学附属医院神经内科,保定071000 [2]北京协和医院神经科

出　　处：《中国神经精神疾病杂志》2022年第8期456-462,共7页Chinese Journal of Nervous and Mental Diseases

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LGI 1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。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 Anti-LGI 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.

关 键 词：抗 LGI 1抗体 脑炎 临床特征 免疫治疗 癫痫 记忆力下降 睡眠障碍 精神症状 面臂肌张力障碍 头晕 

分 类 号：R742[医药卫生—神经病学与精神病学]