摘要：

目的 探讨抗富亮氨酸胶质瘤失活基因 1（LGI1）抗体相关边缘性脑炎临床特点。方法与结果 2016 年 6 月至 2017 年 10 月共诊断与治疗 7 例抗 LGI1 抗体相关边缘性脑炎病例，平均发病年龄为（48.29 ± 15.09）岁。呈急性（4例）或亚急性（3例）发病，以癫 发作和记忆障碍为主要表现，可伴有面-臂肌张力障碍发作（5 例）、精神行为异常或性格改变（4 例），或合并难治性低钠血症（2 例）、胸腺瘤（1 例）；脑脊液（6 例）或血清（7 例）抗 LGI1 抗体呈阳性；头部 MRI 检查单侧或双侧颞叶内侧异常信号（6 例），脑电图呈连续棘慢复合波和慢波（1 例）。大剂量糖皮质激素序贯治疗（6 例）有效。7 例患者中 2 例失访，余 5 例遗留远期记忆障碍（3 例）或近期与远期记忆障碍并存（2 例），其中 1 例出院 6 个月后复发。结论 依据特异性临床表现如面-臂肌张力障碍发作、记忆障碍等，结合影像学及脑脊液检查结果可明确诊断；免疫抑制剂可有效改善临床症状与预后。

关键词: 边缘叶脑炎, 肿瘤抑制蛋白质类, 抗体, 免疫疗法, 癫痫, 记忆障碍

Abstract:

Objective To investigate the clinical characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-associated encephalitis. Methods and Results From June 2016 to October 2017, a total of 7 patients with anti-LGI1 antibody-associated limbic encephalitis were diagnosed and treated, with an average age at onset (48.29 ± 15.09) years. Patients presented acute (4 cases) or subacute onset (3 cases), with seizures and memory dysfunction as the main manifestations. It may be accompanied by faciobrachial dystonic seizures (FBDS, 5 cases), mental and behavioral abnormalities or personality changes (4 cases), or even combined with intractable hyponatremia (2 cases) or thymoma (one case). Serum anti-LGI1 antibody tests showed positive results in 7 cases, and cerebrospinal fluid (CSF) anti-LGI1 antibody tests showed positive results in 6 cases. MRI showed unilateral or bilateral medial temporal lobe abnormal signals (6 cases), and EEG showed continuous spike-slow waves or slow waves (one case). High-dose glucocorticoid sequential therapy was effective in 6 cases. During the follow-up period, 2 cases were lost, and the other 5 cases presented long-term memory disorder (3 cases) or long-term and short-term memory disorders (2 cases). Among them, one case relapsed 6 months after discharge. Conclusions According to specific clinical manifestations of patients (such as onset of FBDS, memory disorders, etc.), combined with imaging and CSF examination results, this disease can be clearly diagnosed. Immunosuppressive agents can effectively improve the clinical symptoms and prognosis.

Key words: Limbic encephalitis, Tumor suppressor proteins, Antibodies, Immunotherapy, Epilepsy, Memory disorders