Seizure semiology: an important clinical clue to the diagnosis of autoimmune epilepsy.
Seizure semiology: an important clinical clue to the diagnosis of autoimmune epilepsy.
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癫痫症状学：诊断自身免疫性癫痫的重要临床线索
DOI：
10.1002/acn3.520
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发表时间：
2018-03
影响因子：
5.3
通讯作者：
Shao XQ
中科院分区：
文献类型：
--
作者：
Lv RJ;Ren HT;Guan HZ;Cui T;Shao XQ
本研究的目的是分析自身免疫性癫痫（AE）患者的癫痫发作症状学特征，并在更大的样本量下描述AE的调查特征。这项观察性回顾性病例系列研究于2014年5月至2017年3月在三级癫痫中心进行。根据自身免疫的实验室证据选择新发癫痫发作病例。同时从同期接受术前评估的受试者中招募典型内侧颞叶癫痫（MTLE）伴海马硬化（HS）患者作为对照组。共确定了61例发生AE的患者。39例患者（63.93%）检测到特异性自身免疫抗体，其中抗VGKC 23例（37.70%），抗NMDA-R 9例（14.75%），抗GABAB-R 6例（9.84%），抗Amphiphysin 1例（1.64%）。关于癫痫发作症状学，在有自身抗体的AE患者和无抗体的疑似AE患者之间未观察到显著差异。与典型的伴有HS的MTLE患者相比，有自身抗体的AE患者和无抗体的疑似AE患者具有相同的癫痫发作症状学特征，包括更频繁的SPS或CPS、更短的癫痫发作持续时间、罕见的发作后意识模糊和常见的睡眠SGTC癫痫发作。本研究强调了AE的重要癫痫发作症状学特征。自身免疫性癫痫患者具有特殊的发作症状学特征。对于单独出现新发癫痫发作或癫痫发作为主的神经系统疾病的自身免疫性癫痫患者，特殊的癫痫发作症状学特征可能会提醒我们早期检测神经元核/胞质抗体，并尽快开始免疫调节治疗。此外，不存在神经特异性自身抗体并不能排除AE。
The purpose of this study is to analyze the seizure semiologic characteristics of patients with autoimmune epilepsy (AE) and describe the investigation characteristics of AE using a larger sample size. This observational retrospective case series study was conducted from a tertiary epilepsy center between May 2014 and March 2017. Cases of new‐onset seizures were selected based on laboratory evidence of autoimmunity. At the same time, typical mesial temporal lobe epilepsy (MTLE) patients with hippocampal sclerosis (HS) were recruited as the control group from the subjects who underwent presurgical evaluation during the same period. A total of 61 patients with AE were identified. Specific autoimmune antibodies were detected in 39 patients (63.93%), including anti‐VGKC in 23 patients (37.70%), anti‐NMDA‐R in 9 patients (14.75%), anti‐GABAB‐R in 6 patients (9.84%), and anti‐amphiphysin in 1 patient (1.64%). Regarding the seizure semiology, no significant differences were noted between AE patients with autoantibody and patients with suspected AE without antibody. Compared to typical MTLE patients with HS, both AE patients with autoantibody and patients with suspected AE without antibody had the same seizure semiologic characteristics, including more frequent SPS or CPS, shorter seizure duration, rare postictal confusion, and common sleeping SGTC seizures. This study highlights important seizure semiologic characteristics of AE. Patients with autoimmune epilepsy had special seizure semiologic characteristics. For patients with autoimmune epilepsy presenting with new‐onset seizures in isolation or with a seizure‐predominant neurological disorder, the special seizure semiologic characteristics may remind us to test neuronal nuclear/cytoplasmic antibodies early and initiate immunomodulatory therapies as soon as possible. Furthermore, the absence of neural‐specific autoantibodies does not rule out AE.
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