Department of Neurology, Mayo Clinic, Rochester, Minnesota, U.S.A.
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, U.S.A.
Epilepsia. 2017 Jul;58(7):1181-1189. doi: 10.1111/epi.13797. Epub 2017 May 26.
To validate predictive models for neural antibody positivity and immunotherapy response in epilepsy.
We conducted a retrospective study of epilepsy cases at Mayo Clinic (Rochester-MN; Scottsdale-AZ, and Jacksonville-FL) in whom autoimmune encephalopathy/epilepsy/dementia autoantibody testing profiles were requested (06/30/2014-06/30/2016). An Antibody Prevalence in Epilepsy (APE) score, based on clinical characteristics, was assigned to each patient. Among patients who received immunotherapy, a Response to Immunotherapy in Epilepsy (RITE) score was assigned. Favorable seizure outcome was defined as >50% reduction of seizure frequency at the first follow-up.
Serum and cerebrospinal fluid (CSF) from 1,736 patients were sent to the Mayo Clinic Neuroimmunology Laboratory for neural autoantibody evaluation. Three hundred eighty-seven of these patients met the diagnostic criteria for epilepsy. Central nervous system (CNS)-specific antibodies were detected in 44 patients. Certain clinical features such as new-onset epilepsy, autonomic dysfunction, viral prodrome, faciobrachial dystonic seizures/oral dyskinesia, inflammatory CSF profile, and mesial temporal magnetic resonance imaging (MRI) abnormalities had a significant association with positive antibody results. A significantly higher proportion of antibody-positive patients had an APE score ≥4 (97.7% vs. 21.6%, p < 0.01). Sensitivity and specificity of an APE score ≥4 to predict presence of specific neural auto-antibody were 97.7% and 77.9%, respectively. In the subset of patients who received immunotherapy (77), autonomic dysfunction, faciobrachial dystonic seizures/oral dyskinesia, early initiation of immunotherapy, and presence of antibodies targeting plasma membrane proteins (cell-surface antigens) were associated with favorable seizure outcome. Sensitivity and specificity of a RITE score ≥7 to predict favorable seizure outcome were 87.5% and 83.8%, respectively.
APE and RITE scores can aid diagnosis, treatment, and prognostication of autoimmune epilepsy. A PowerPoint slide summarizing this article is available for download in the Supporting Information section here.
验证预测神经抗体阳性和免疫治疗反应的模型在癫痫中的作用。
我们对梅奥诊所（明尼苏达州罗切斯特；亚利桑那州斯科茨代尔和佛罗里达州杰克逊维尔）的癫痫病例进行了回顾性研究，这些病例请求进行自身免疫性脑炎/癫痫/痴呆自身抗体检测分析（2014 年 6 月 30 日至 2016 年 6 月 30 日）。为每位患者分配了基于临床特征的抗体阳性在癫痫中的评分（APE）。在接受免疫治疗的患者中，分配了癫痫免疫治疗反应评分（RITE）。良好的癫痫发作结局定义为首次随访时癫痫发作频率减少≥50%。
1736 名患者的血清和脑脊液（CSF）被送到梅奥诊所神经免疫实验室进行神经自身抗体评估。其中 387 名患者符合癫痫的诊断标准。在 44 名患者中检测到中枢神经系统（CNS）特异性抗体。新发病的癫痫、自主神经功能障碍、病毒前驱期、面肩肱型肌张力障碍性发作/口腔运动障碍、炎症性 CSF 特征和内侧颞叶磁共振成像（MRI）异常等某些临床特征与抗体阳性结果有显著相关性。抗体阳性患者的 APE 评分≥4 的比例明显更高（97.7% vs. 21.6%，p<0.01）。APE 评分≥4 预测特定神经自身抗体存在的敏感性和特异性分别为 97.7%和 77.9%。在接受免疫治疗的患者亚组（77 名）中，自主神经功能障碍、面肩肱型肌张力障碍性发作/口腔运动障碍、早期开始免疫治疗和针对血浆膜蛋白（细胞表面抗原）的抗体与良好的癫痫发作结局相关。RITE 评分≥7 预测良好的癫痫发作结局的敏感性和特异性分别为 87.5%和 83.8%。
APE 和 RITE 评分可辅助自身免疫性癫痫的诊断、治疗和预后判断。本文的幻灯片摘要可在支持信息部分下载。
