抗LGI1抗体相关边缘性脑炎17例临床分析

摘要

目的 探讨抗LGI1抗体相关边缘性脑炎患者的临床特点.方法 回顾性分析17例抗LGI1抗体相关边缘性脑炎患者的临床表现、病程、影像学、治疗方案及预后.结果 本研究17例患者男14例,女3例,年龄中位数61岁.临床表现认知功能障碍14例(82％),面-臂肌张力障碍(FBDS)11例(65％),部分性发作10例(59％),强直-阵挛发作8例(47％),精神行为异常9例(53％).17例患者均行血清LGI1抗体检测,16例阳性,1例阴性(脑脊液抗体检测阳性)16例患者行脑脊液LGI1抗体检测,14例阳性.17例患者均给予一线治疗,2例患者复发,随访时间>1年,部分患者遗留轻度近记忆力障碍,无患者死亡.结论 抗LGI1抗体相关边缘性脑炎是一种自身免疫性脑炎,中老年男性患者多见,单向病程为主,可以复发.临床常以FBDS、部分性发作为首发表现,继而出现认知功能障碍,对免疫治疗敏感,预后较好.%Objective The objective of this study was to study LGI1 limbic encephalitis (LE). Methods We performed a retrospective analysis on the clinical features,laboratory findings,imaging profiles and treatment outcomes of 17 patients with LGI1. Results The study included 14 male and 3 female cases. The median age was 61 years old. The clinical manifestations includes 14 cases with cognitive dysfunction, 11 cases with faciobrachial dystonic seizures (FBDS), 10 cases with focal seizures, 8 cases with generalizedtonic-clonic seizure and 9 cases with mental and behavioral disorder. Among 17 examined patients, there were 16 patients with positive and 1 with negative serum LGI1 antibody (but whose CSF LGI1 antibody was positive). Among 16 examined patients, there were 14 patients with positive cerebrospinal fluid LGI1 antibody. All patients had good responses to the first-line treatment and 2 patients experienced recurrence. During more than one-year follow-up, the recurrence rate was 33％ and no patient died. Conclusion LGI1 LE is an autoimmune encephalitis, which is mainly unidirectional progression and can relapse. FBDS and focal seizures usually are first symptoms, followed by cognitive dysfunction. Patients are responded to immunotherapy well and have good prognosis.