Süße Marie, Zank Maria, von Podewils Viola, von Podewils Felix
Department of Neurology, Epilepsy Center, University Medicine Greifswald, Greifswald, Germany.
Front Neurol. 2021 Apr 7;12:633999. doi: 10.3389/fneur.2021.633999. eCollection 2021.
This study was conducted to elucidate prevalence, clinical features, outcomes, and best treatment in patients with late-onset seizures due to autoimmune encephalitis (AE). This is a single-institution prospective cohort study (2012-2019) conducted at the Epilepsy Center at the University of Greifswald, Germany. A total of 225 patients aged ≥50 years with epileptic seizures were enrolled and underwent an MRI/CT scan, profiling of neural antibodies (AB) in serum and cerebrospinal fluid (CSF), and neuropsychological testing. On the basis of their work-up, patients were categorized into the following three cohorts: definite, suspected, or no AE. Patients with definite and suspected AE were subsequently treated with immunosuppressive therapy (IT) and/or anti-seizure drug (ASD) therapy and were followed up (FU) regarding clinical and seizure outcome. Of the 225 patients, 17 (8%) fulfilled the criteria for definite or suspected AE according to their AB profile and MRI results. Compared with patients with no evidence of AE, those with AE were younger ( = 0.028), had mesial temporal neuropsychological deficits ( = 0.001), frequently had an active or known malignancy ( = 0.006) and/or a pleocytosis ( = 0.0002), and/or had oligoclonal bands in CSF ( = 0.001). All patients with follow-up became seizure-free with at least one ASD. The Modified Rankin scale (mRS) at hospital admission was low for patients with AE (71% with mRS ≤2) and further decreased to 60% with mRS ≤2 at last FU. AE is an important etiology in late-onset seizures, and seizures may be the first symptom of AE. Outcome in non-paraneoplastic AE was favorable with ASD and IT. AB testing in CSF and sera, cerebral MRI, CSF analysis, and neuropsychological testing for mesial temporal deficits should be part of the diagnostic protocol for AE following late-onset seizures.
本研究旨在阐明自身免疫性脑炎(AE)所致迟发性癫痫患者的患病率、临床特征、预后及最佳治疗方法。这是一项在德国格赖夫斯瓦尔德大学癫痫中心进行的单机构前瞻性队列研究(2012 - 2019年)。共纳入225例年龄≥50岁的癫痫发作患者,这些患者均接受了MRI/CT扫描、血清和脑脊液(CSF)神经抗体(AB)分析以及神经心理学测试。根据检查结果,患者被分为以下三组:确诊AE组、疑似AE组或无AE组。确诊和疑似AE的患者随后接受免疫抑制治疗(IT)和/或抗癫痫药物(ASD)治疗,并对其临床和癫痫发作结局进行随访(FU)。在225例患者中,17例(8%)根据其AB分析和MRI结果符合确诊或疑似AE的标准。与无AE证据的患者相比,有AE的患者更年轻(P = 0.028),有内侧颞叶神经心理学缺陷(P = 0.001),经常有活跃或已知的恶性肿瘤(P = 0.006)和/或脑脊液细胞增多(P = 0.0002),和/或脑脊液中有寡克隆带(P = 0.001)。所有接受随访的患者使用至少一种ASD后均无癫痫发作。AE患者入院时改良Rankin量表(mRS)评分较低(71%的患者mRS≤2),最后一次随访时mRS≤2的患者比例进一步降至60%。AE是迟发性癫痫的一个重要病因,癫痫发作可能是AE的首发症状。非副肿瘤性AE患者使用ASD和IT治疗的预后良好。脑脊液和血清中的AB检测、脑部MRI、脑脊液分析以及内侧颞叶缺陷的神经心理学测试应成为迟发性癫痫后AE诊断方案的一部分。