Stanford Comprehensive Epilepsy Center, Stanford University, Palo Alto, CA, USA.
Department of Psychiatry, University of Alberta Hospital, Alberta, Canada.
Epilepsia. 2021 Mar;62(3):720-728. doi: 10.1111/epi.16820. Epub 2021 Jan 19.
There is a growing recognition of immune-mediated causes in patients with focal drug-resistant epilepsy (DRE); however, they are not systematically assessed in the pre-surgical diagnostic workup. Early diagnosis and initiation of immunotherapy is associated with a favorable outcome in immune-mediated seizures. Patients with refractory focal epilepsy with neuronal antibodies (Abs) tend to have a worse surgical prognosis when compared to other etiologies.
We studied the prevalence of serum Abs in patients ≥18 years of age with DRE of unknown cause before surgery. We proposed and calculated a clinical APES (Antibody Prevalence in Epilepsy before Surgery) score for each subject, which was modified based on Dubey's previously published APE score. RESULTS`: A total of 335 patients were screened and 86 subjects were included in final analysis. The mean age at the time of recruitment was 44.84 ± 14.86 years, with age at seizure onset 30.89 ± 19.88 years. There were no significant differences among baseline clinical features between retrospective and prospective sub-cohorts. The prevalence of at least one positive Ab was 33.72%, and central nervous system (CNS)-specific Abs was 8.14%. APES score ≥4 showed slightly better overall prediction (area under the curve [AUC]: 0.84 vs 0.74) and higher sensitivity (100% vs 71.4%), with slightly lower but similar specificity (44.3% vs 49.4%), when compared to APE score ≥4. For subjects who had available positron emission tomography (PET) results and all components of APES score (n = 60), the sensitivity of APES score ≥4 yielded a similar prediction potential with an AUC of 0.80.
Our findings provide persuasive evidence that a subset of patients with focal DRE have potentially immune-mediated causes. We propose an APES score to help identify patients who may benefit from a workup for immune etiologies during the pre-surgical evaluation for focal refractory epilepsy with unknown cause.
越来越多的人认识到,在局灶性耐药性癫痫(DRE)患者中存在免疫介导的病因;然而,在术前诊断中并未对其进行系统评估。免疫介导性癫痫发作早期诊断和免疫治疗的启动与良好的预后相关。与其他病因相比,伴有神经元抗体(Abs)的难治性局灶性癫痫患者的手术预后往往较差。
我们研究了术前病因不明的 DRE 患者中血清 Abs 的患病率。我们为每位患者提出并计算了一个临床 APES(手术前癫痫中的抗体患病率)评分,该评分基于 Dubey 先前发表的 APE 评分进行了修改。
共筛选了 335 名患者,最终有 86 名患者纳入最终分析。招募时的平均年龄为 44.84±14.86 岁,发病年龄为 30.89±19.88 岁。回顾性和前瞻性亚组之间的基线临床特征无显著差异。至少有一种阳性 Ab 的患病率为 33.72%,中枢神经系统(CNS)特异性 Abs 的患病率为 8.14%。APES 评分≥4 时,总体预测效果略好(曲线下面积[AUC]:0.84 对 0.74),敏感性更高(100%对 71.4%),特异性略低但相似(44.3%对 49.4%),与 APE 评分≥4 相比。对于有可用正电子发射断层扫描(PET)结果和 APES 评分所有成分的患者(n=60),APES 评分≥4 的敏感性具有相似的预测潜力,AUC 为 0.80。
我们的研究结果提供了有说服力的证据,表明一部分局灶性 DRE 患者可能存在免疫介导的病因。我们提出了一个 APES 评分,以帮助识别那些在术前评估病因不明的局灶性难治性癫痫时可能受益于免疫病因检查的患者。