Lv Rui-Juan, Ren Hai-Tao, Guan Hong-Zhi, Cui Tao, Shao Xiao-Qiu
Department of Neurology Beijing Tiantan Hospital Capital Medical University 6 Tiantanxili, Dongcheng District Beijing 100050 China.
China National Clinical Research Center for Neurological Diseases 6 Tiantanxili, Dongcheng District Beijing 100050 China.
Ann Clin Transl Neurol. 2018 Jan 22;5(2):208-215. doi: 10.1002/acn3.520. eCollection 2018 Feb.
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-GABA-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.
本研究旨在分析自身免疫性癫痫(AE)患者的发作症状学特征,并采用更大样本量描述AE的检查特征。
本观察性回顾性病例系列研究于2014年5月至2017年3月在一家三级癫痫中心进行。根据自身免疫的实验室证据选择新发癫痫病例。同时,从同期接受术前评估的受试者中招募典型的伴有海马硬化(HS)的内侧颞叶癫痫(MTLE)患者作为对照组。
共识别出61例AE患者。39例患者(63.93%)检测到特异性自身抗体,其中23例患者(37.70%)检测到抗电压门控钾通道(anti-VGKC)抗体,9例患者(14.75%)检测到抗N-甲基-D-天冬氨酸受体(anti-NMDA-R)抗体,6例患者(9.84%)检测到抗γ-氨基丁酸受体(anti-GABA-R)抗体,1例患者(1.64%)检测到抗 amphiphysin 抗体。关于发作症状学,有自身抗体的AE患者与疑似无抗体的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患者和疑似无抗体的AE患者具有相同的发作症状学特征,包括更频繁的简单部分性发作(SPS)或复杂部分性发作(CPS)、发作持续时间较短、发作后罕见意识模糊以及常见睡眠期全面性强直阵挛发作(SGTC)。
本研究突出了AE重要的发作症状学特征。自身免疫性癫痫患者具有特殊的发作症状学特征。对于以新发孤立性发作或发作性为主的神经系统疾病就诊的自身免疫性癫痫患者,这些特殊的发作症状学特征可能提醒我们尽早检测神经元核/胞浆抗体并尽快启动免疫调节治疗。此外,未检测到神经特异性自身抗体并不排除AE。