富亮氨酸胶质瘤失活1蛋白抗体相关边缘叶脑炎5例临床分析

doi:10.6040/j.issn.1671-7554.0.2018.1405

摘要/Abstract

摘要： 目的探讨富亮氨酸胶质瘤失活1蛋白(LGI1)抗体相关边缘叶脑炎的临床特点、辅助检查及治疗随访结果。方法回顾性分析2017年7月至2018年5月山东大学齐鲁医院(青岛)确诊的LGI1抗体相关边缘叶脑炎5例,分析其临床表现、实验室检查结果、影像学、长时程视频脑电图表现及治疗随访情况。结果男性3例,女性2例,其中4例短期内出现记忆力减退,3例有癫痫发作,5例有面臂肌张力障碍发作(FBDS);3例出现低钠血症,5例血清及脑脊液LGI1抗体均阳性, 5例磁共振检查Flair及T₂序列均未见颞叶内侧高信号,1例左侧基底节出现T₁序列高信号。4例完善了长时程视频脑电图,其中3例监测过程中有FBDS多次发作。5例患者均接受了免疫治疗;随访至目前,2例有癫痫的再发作、1例再次出现精神症状,调整药物后好转。结论 LGI1抗体相关边缘叶脑炎是一种早期以记忆力减退、FBDS及癫痫发作为特征的自身免疫性脑炎,常伴有低钠血症,影像学以颞叶、海马受累为主,免疫治疗效果好,临床可复发。

关键词: 富亮氨酸胶质瘤失活1蛋白, 边缘叶脑炎, 记忆力下降, 面臂肌张力障碍发作

Abstract: Objective To explore the clinical features, auxiliary examination results and therapeutic outcomes of leucine-rich glioma inactived-1 antibody(anti-LGI1)associated limbic encephalitis. Methods The clinical data of 5 cases of anti-LGI1 associated limbic encephalitis treated during July 2017 and May 2018 were retrospectively analyzed, including the clinical manifestations, laboratory examinations, radiological features, long-term video-electroencephalogram, treatments and prognosis. Results The patients were three male and two female. Memory deficit was observed in 4 cases, seizure in 3 cases, faciobrachial dystonic seizures(FBDS)in all cases, hyponatremia in 3 cases, and positive anti-LGI1 antibodies both in the cerebrospinal fluid and blood serum in all cases. Magnetic resonance imaging(MRI)showed no T₂WI or flair-phase high signal in the medial temporal lobes in 5 cases, but high signal in basal ganglia in T₁WI in 1 case. Long-term video-electroencephalographs of 4 cases showed frequent FBDS. All patients received immunotherapy. During the follow-up, seizure recurred in 2 cases, and mental disturbance recurred in 1 case. Conclusion Anti-LGI1 associated limbic encephalitis is an autoimmune encephalitis characterized by early memory loss, FBDS and seizure. It is often complicated with hyponatremia. Temporallobe and hippocampus are often involved. Immunotherapy is effective but relapses are common.

Key words: Leucine-rich glioma inactivated-1 protein, Limbic encephalitis, Memory loss, Faciobrachial dystonic seizures

中图分类号:

R742

李洪燕,房雷,张红英,苏源,李玲. 富亮氨酸胶质瘤失活1蛋白抗体相关边缘叶脑炎5例临床分析[J]. 山东大学学报 (医学版), 2019, 57(5): 105-109.

LI Hongyan, FANG Lei, ZHANG Hongying, SU Yuan, LI Ling. A clinical study of 5 cases of leucine-rich glioma inactivated 1 protein antibody associated limbic encephalitis[J]. Journal of Shandong University (Health Sciences), 2019, 57(5): 105-109.

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