Seizure semiology in leucine-rich glioma-inactivated protein 1 antibody-associated limbic encephalitis
Seizure semiology in leucine-rich glioma-inactivated protein 1 antibody-associated limbic encephalitis
复制标题
富含亮氨酸的胶质瘤灭活蛋白 1 抗体相关边缘脑炎的癫痫发作症状学
DOI：
10.1016/j.yebeh.2017.08.011
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发表时间：
2017-12-01
影响因子：
2.6
通讯作者：
Shao, Xiao-Qiu
中科院分区：
文献类型：
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作者：
Chen, Chao;Wang, Xiu;Shao, Xiao-Qiu
目的：本研究的目的是推进癫痫发作的症状学特征的富含亮氨酸的胶质瘤失活蛋白1（LGI 1）抗体相关的边缘系统脑炎（LE.Methods）：18例确诊为LGI 1 LE。癫痫发作的症状学，人口统计学特征，MRI和氟脱氧葡萄糖正电子发射断层扫描（FDG-PET），脑电图，和免疫治疗后的结果进行了evaluated.Results：患者分为以下组的基础上癫痫发作的症状学：面臂肌张力障碍性癫痫发作（仅FBDS，n = 4），癫痫发作无FBDS（非FBDS，n = 6），和FBDS+癫痫发作（FBDS+，n= 8）。在非FBDS组中，大多数患者（5/6）表现为内侧颞叶癫痫（MTLE）样症状（即，恐惧、上腹隆起、凝视和自动症），频率为每天7 +/- 5次，持续时间为15.3 +/- 14.3 s。在FBDS+组中，特征性症状是FBDS，随后是癫痫事件，特别是自动症（7/8），频率为每天16 +/- 12次，持续时间为13.0 +/- 8.0 s。在这些病例中，分别有67%和50%的患者在MRI和FDG-PET上显示异常，并且最常累及内侧颞叶结构。仅FBDS组、非FBDS组和FBDS+组中分别有0/4、6/6和8/8例患者观察到发作性放电。主要是颞叶受到影响。意义：LGI 1 LE应作为一种疾病综合征，具有一系列临床表现。识别独特的症状学特征类型将有助于早期诊断和及时启动免疫治疗。(C)2017爱思唯尔公司All rights reserved.
Objective: The objective of this study was to advance the characterization of seizure semiology in leucine-rich glioma-inactivated protein 1 (LGI1) antibody-associated limbic encephalitis (LE).Methods: Eighteen patients diagnosed with LGI1 LE were identified. Seizure semiology, demographic features, MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), electroencephalograms, and outcomes following immunotherapy were evaluated.Results: Patients were divided into the following groups based on seizure semiology: faciobrachial dystonic seizure only (FBDS-only, n = 4), epileptic seizure without FBDS (Non-FBDS, n = 6), and FBDS plus epileptic seizure (FBDS+, n= 8). In the groupwith Non-FBDS, themajority of patients (5/6) manifested mesial temporal lobe epilepsy (MTLE) like semiology (i.e., fear, epigastric rising, staring, and automatisms) with a frequency of 7 +/- 5 times per day and a duration of 15.3 +/- 14.3 s. In the group with FBDS+, the distinctive symptom was FBDS followed by epileptic events, especially automatisms (7/8), with a frequency of 16 +/- 12 times per day and a duration of 13.0 +/- 8.0 s. In these cases, 67% and 50% of the patients showed abnormalities on MRI and FDG-PET, respectively, and the mesial temporal lobe structures were most often involved. Ictal discharges were observed in 0/4, 6/6, and 8/8 of the patients in the groups with FBDS only, Non-FBDS, and FBDS+, respectively. The temporal lobe was mainly affected. Immunotherapy had favorable therapeutic effects.Significance: The LGI1 LE should be considered as one disease syndrome with a series of clinical manifestation. Identifying types of unique semiology features will facilitate the early diagnosis and the timely initiation of immunotherapy. (C) 2017 Elsevier Inc. All rights reserved.