Predictive models in the diagnosis and treatment of autoimmune epilepsy
Predictive models in the diagnosis and treatment of autoimmune epilepsy
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DOI：
10.1111/epi.13797
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发表时间：
2017-07-01
期刊：
影响因子：
5.6
通讯作者：
McKeon, Andrew
中科院分区：
文献类型：
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作者：
Dubey, Divyanshu;Singh, Jaysingh;McKeon, Andrew
目的验证癫痫神经抗体阳性和免疫治疗反应的预测模型。方法我们对梅奥诊所（明尼苏达州罗彻斯特、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，其中要求进行自身免疫性脑病/癫痫/痴呆自身抗体检测（2014年6月30日-2016年6月30日）。根据临床特征，为每位患者分配癫痫抗体流行率 (APE) 评分。在接受免疫治疗的患者中，分配了癫痫免疫治疗反应（RITE）评分。良好的癫痫发作结果定义为首次随访时癫痫发作频率减少 >50%。 结果 1,736 名患者的血清和脑脊液 (CSF) 被送往 Mayo Clinic 神经免疫学实验室进行神经自身抗体评估。其中三百八十七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。某些临床特征，如新发癫痫、自主神经功能障碍、病毒前驱症状、面臂肌张力障碍性癫痫发作/口腔运动障碍、炎症性脑脊液图谱和内侧颞叶磁共振成像 (MRI) 异常与抗体结果呈显着相关。抗体阳性患者中 APE 评分为 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中 (77)，自主神经功能障碍、面臂肌张力障碍癫痫发作/口腔运动障碍、早期开始免疫治疗以及针对质膜蛋白（细胞表面抗原）的抗体的存在与良好的癫痫发作结果相关。 RITE 评分 7 预测有利癫痫发作结果的敏感性和特异性分别为 87.5% 和 83.8%。 意义 APE 和 RITE 评分可以帮助自身免疫性癫痫的诊断、治疗和预后。总结本文的 PowerPoint 幻灯片可在支持信息部分下载。
ObjectiveTo validate predictive models for neural antibody positivity and immunotherapy response in epilepsy.MethodsWe 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.ResultsSerum 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.SignificanceAPE 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 .