抗富亮氨酸胶质瘤失活蛋白1抗体相关脑炎临床分析

doi:10.3969/j.issn.1672-6731.2020.09.009

摘要/Abstract

摘要：

目的探讨抗富亮氨酸胶质瘤失活蛋白1（LGI1）抗体相关脑炎的临床特点。方法与结果 2016年9月至2018年4月共诊治6例抗LGI1抗体相关脑炎患者，临床表现为认知功能障碍（6例）、癫发作（4例）、精神行为异常（3例）、睡眠障碍（5例）、自主神经功能障碍（3例），病程中可伴有低钠血症（4例）。脑电图异常较为常见（4例）。头部MRI显示炎症性病变主要累及海马、颞叶内侧（3例）。血清和脑脊液LGI1抗体检测（间接免疫荧光法）双阳性者4例，单纯血清或脑脊液LGI1抗体阳性者分别为6和4例。采用激素和（或）免疫球蛋白治疗，随访6~24个月无一例死亡；其中3例遗留记忆力下降、1例面-臂肌张力障碍发作、2例低钠血症、1例睡眠障碍。。结论抗LGI1抗体相关脑炎以认知功能障碍、癫发作、睡眠障碍、低钠血症为临床特征，对免疫治疗有效，预后相对良好。

关键词: 脑炎, 免疫系统疾病, 认知障碍, 睡眠障碍, 免疫疗法

Abstract:

Objective To explore the clinical characteristics of anti-leucine-rich glioma-inactivated 1 (LGI1) antibody-related encephalitis. Methods and Results Clinical data of 6 patients with anti-LGI1 antibody-related encephalitis from September 2016 to April 2018 were analyzed retrospectively. The clinical manifestations of 6 cases were lognition disorders in 6 cases, seizures in 4 cases, behavioral changes in 3 cases, sleep disorders in 5 cases and autonomic nervous system abnormality in 3 cases. Hyponatremia (4 cases) could be presented. Abnormal EEG (4 cases) was often seen. Brain MRI showed abnormal signals in 3 cases, mainly involved hippocampus and medial temporal lobe. Indirect immunofluorescence assay (IIF) was used to detect anti-LGI1 antibody. Four cases were positive in both serum and cerebrospinal fluid, while 6 cases only in serum and 4 cases only in cerebrospinal fluid. The follow-up results of 6 to 24 months after intravenous hormone or immunoglobulin therapies showed no one died; the sequelae were memory deficit in 3 cases, faciobrachial dystonic seizures in one case, hyponatremia in 2 cases, sleep disorders in one case. Conclusions Anti-LGI1 antibody-related encephalitis is often characterized by cognitive impairment, seizures, sleep disorders and hyponatremia. Immunotherapy is effective, which has good prognosis comparatively.

Key words: Encephalitis, Immune system diseases, Cognition disorders, Sleep disorders, Immunotherapy

赵清青, 戴淑娟, 艾明达, 陈玲, 艾青龙. 抗富亮氨酸胶质瘤失活蛋白1抗体相关脑炎临床分析[J]. 中国现代神经疾病杂志, 2020, 20(9): 806-812.

ZHAO Qing-qing, DAI Shu-juan, AI Ming-da, CHEN Ling, AI Qing-long. Clinical analysis of anti-leucine-rich glioma-inactivated 1 antibody-related encephalitis[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2020, 20(9): 806-812.

http://journal11.magtechjournal.com/cjcnn/CN/Y2020/V20/I9/806

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2 抗富亮氨酸胶质瘤失活蛋白1抗体相关脑炎临床分析

Clinical analysis of anti-leucine-rich glioma-inactivated 1 antibody-related encephalitis

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2 抗富亮氨酸胶质瘤失活蛋白1抗体相关脑炎临床分析

Clinical analysis of anti-leucine-rich glioma-inactivated 1 antibody-related encephalitis

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