抗富亮氨酸胶质瘤失活蛋白1抗体相关脑炎临床特征分析

doi:10.3969/j.issn.1672-6731.2023.10.012

摘要/Abstract

摘要：

目的: 总结抗富亮氨酸胶质瘤失活蛋白1（LGI1）抗体相关脑炎临床特点。方法与结果: 江苏省淮安市第三人民医院2018年3月至2022年10月诊断与治疗13例抗LGI1抗体相关脑炎患者，首发症状分别为局灶性癫痫发作6例、精神症状3例、近事记忆减退2例、面-臂肌张力障碍发作2例；10例血清抗LGI1抗体阳性，2例脑脊液抗LGI1抗体阳性；4例伴低钠血症；9例MRI异常，表现为海马FLAIR成像高信号3例、腔隙性梗死4例、皮质及皮质下轻度萎缩1例、额叶皮质下白质缺血性改变1例；10例脑电图异常，表现为局灶性痫样放电5例，慢波增多5例；12例行免疫治疗，6例同时行抗癫痫发作治疗；10例预后良好，3例预后不良。结论: 抗LGI1抗体相关脑炎临床主要表现为癫痫发作、精神障碍、近事记忆减退，面-臂肌张力障碍发作为其特征性表现，可伴低钠血症，应尽早启动免疫治疗。

关键词: 脑炎, 神经系统自身免疫疾病, 张力障碍, 低钠血症, 免疫疗法

Abstract:

Objective: To summarize the clinical features of anti-leucine-rich-glioma-inactivation 1 (LGI1) antibody associated encephalitis. Methods and Results: A total of 13 patients with anti - LGI1 antibody associated encephalitis were admitted to Huai'an No. 3 People's Hospital from March 2018 to October 2022. The first symptoms were focal seizure in 6 cases, psychiatric symptom in 3 cases, recent memory loss in 2 cases and faciobrachial dystonic seizures (FBDS) in 2 cases. Serum anti-LGI1 antibody was positive in 10 cases, cerebrospinal fluid (CSF) anti-LGI1 antibody was positive in 2 cases. And 4 cases had hyponatremia. MRI showed abnormal findings in 9 cases, including 3 cases of hippocampal FLAIR hyperintensity, 4 cases of lacunar infarction, one case of cortical and subcortical mild cerebral atrophy, and one case of subcortical white matter ischemic changes. EEG abnormalities were observed in 10 cases, including focal epileptoid discharge in 5 cases and slow wave increase in 5 cases. Twelve cases received immunotherapy and 6 cases received antiepileptic seizure medicine. The prognosis was good in 10 cases and poor in 3 cases. Conclusions: The main clinical manifestations of anti - LGI1 antibody associated encephalitis include seizures, mental disorders and recent memory loss, FBDS as its characteristic manifestations, may be accompanied by hyponatremia, immunotherapy should be started as soon as possible.

Key words: Encephalitis, Autoimmune diseases of the nervous system, Dystonic disorders, Hyponateramia, Immunotherapy

潘合跃, 仲玲, 李艳, 王守勇, 史向松, 徐建洋. 抗富亮氨酸胶质瘤失活蛋白1抗体相关脑炎临床特征分析[J]. 中国现代神经疾病杂志, 2023, 23(10): 940-947.

He-yue PAN, Ling ZHONG, Yan LI, Shou-yong WANG, Xiang-song SHI, Jian-yang XU. Clinical characteristics of anti- leucine - rich glioma - inactivated 1 antibody associated encephalitis[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2023, 23(10): 940-947.

http://journal11.magtechjournal.com/cjcnn/CN/Y2023/V23/I10/940

图/表 5

表 1 13例抗LGI1抗体相关脑炎患者临床资料

Table 1. Clinical data of 13 patients with anti-LGI1 antibody associated encephalitis

序号	性别	年龄（岁）	首发症状	实验室检查	MRI特征	脑电图特征
1	男性	68	癫痫发作	血清抗LGI1抗体1∶300	腔隙性梗死	右侧额区、前中颞区尖慢复合波
2	男性	74	精神症状	血清抗LGI1抗体1∶30	腔隙性梗死	正常
3	女性	2	癫痫发作	血清抗LGI1抗体1∶30，血清钠130.48 mmol/L	正常	右侧额区尖波
4	女性	10	癫痫发作	血清抗LGI1抗体1∶10	正常	右侧中颞区尖波
5	女性	44	精神症状	血清抗LGI1抗体1∶10	正常	正常
6	男性	67	癫痫发作	血清抗LGI1抗体1∶32	左侧海马FLAIR成像高信号	慢波增多
7	男性	63	FBDS	血清抗LGI1抗体1∶100，血清FT₃ 2.96 pmol/L	右侧海马FLAIR成像高信号	慢波增多
8	女性	74	近事记忆减退	脑脊液抗LGI1抗体1∶3.20，血清钠126.88 mmol/L	皮质及皮质下轻度萎缩	慢波增多
9	男性	62	近事记忆减退	脑脊液抗LGI1抗体1∶32，血清钠130.47 mmol/L	额叶皮质下白质缺血性改变	慢波增多
10	女性	49	癫痫发作	血清抗LGI1抗体1∶10，血清FT₃ 6.58 pmol/L、T₃ 3.36 nmol/L	正常	正常
11	男性	72	癫痫发作	血清抗LGI1抗体1∶10	腔隙性梗死	右侧前颞区尖慢复合波
12	男性	69	FBDS	正常	腔隙性梗死	双侧前中颞区尖慢复合波
13	男性	60	精神症状	血清抗LGI1抗体1∶32，血清钠126.93 mmol/L	双侧海马FLAIR成像高信号	慢波增多

图 1 例13血清抗LGI1抗体阳性CBA法× 400 1a 首次入院时血清抗LGI1抗体滴度为1∶32 1b 再次入院时血清抗LGI1抗体滴度为1∶100 1c 再次出院后随访3个月血清抗LGI1抗体滴度为1∶32

Figure 1 Serum anti-LGI1 antibody was positive in Case 13 CBA × 400 Serum anti - LGI1 antibody at first admission was 1∶32 (Panel 1a). Serum anti- LGI1 antibody at second admission was 1 ∶ 100 (Panel 1b). Serum anti - LGI1 antibody at secondary discharge and follow - up again for 3 months was 1∶ 32 (Panel 1c).

图 2 头部MRI检查所见 2a 例6横断面T₂-FLAIR成像显示左侧海马高信号（箭头所示） 2b 例13横断面T₂-FLAIR成像显示双侧海马高信号（箭头所示）

Figure 2 Head MRI findings Axial T₂ - FLAIR showed hyperintensity in the left hippocampus of Case 6 (arrow indicates, Panel 2a). Axial T₂ - FLAIR showed hyperintensity in the bilateral hippocampus of Case 13 (arrows indicate, Panel 2b).

图 3 例1视频脑电图显示右侧额区、前中颞区尖慢复合波（箭头所示）

Figure 3 The VEEG of Case 1 showed sharp and slow wave complex in the right frontal area and the anterior middle temporal area (arrows indicate).

图 4 例13于清醒状态下临床监测到右侧面部、上肢、下肢FBDS，同步视频脑电图显示肌电伪迹掩盖脑电信号，爆发约3 s后减弱

Figure 4 FBDS occurred on the right face, upper limb and lower limb during waking state of Case 13, and the VEEG showed myoelectric artifacts masked EEG signals, and weakened after about 3 s.

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