Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy
Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy
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DOI：
10.1212/wnl.0000000000000383
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发表时间：
2014-05-06
期刊：
影响因子：
9.9
通讯作者：
Pittock, S. J.
中科院分区：
文献类型：
--
作者：
Toledano, M.;Britton, J. W.;Pittock, S. J.
目的：评价免疫治疗对自身免疫性癫痫的诊断价值。方法：回顾性分析110例以癫痫发作为主诉的自身免疫性神经病患者的临床资料。29例患者符合以下入选标准：（1）基于存在1种神经自身抗体（n = 23）、自身免疫的个人或家族史或身体特征以及频繁或药物难治性癫痫发作而怀疑自身免疫性癫痫;（2）开始IV甲泼尼龙（IVMP）、IV免疫球蛋白（IVIg）或两者的6- 12周试验。患者被定义为响应者，如果有一个50%或更大的减少癫痫frequency.Results：18例（62%）响应，其中10例（34%）成为无癫痫; 52%改善与第一代理。在对第一种药物没有反应后接受第二种药物的人中，43%的人有所改善。良好的反应与症状发作和治疗开始之间的较短间隔相关（中位数9.5 vs 22个月; p = 0.048）。应答者包括14/16例（87.5%）质膜抗原抗体阳性患者，2/6例（33%）谷氨酸脱羧酶65抗体血清阳性患者，2/6例（33%）未检测到抗体的患者。13个应答者随后超过6个月后，开始长期口服免疫抑制剂，反应持续在11（85%）conclusions：这些回顾性研究结果证明考虑一项试验的免疫治疗在疑似自身免疫性epilepsy.Classification的证据：这项研究提供了IV类证据，在疑似自身免疫性epilepsy，IVMP，IVIg，或两者都提高癫痫发作控制。
Objective:To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy.Method:We reviewed the charts of 110 patients seen at our autoimmune neurology clinic with seizures as a chief complaint. Twenty-nine patients met the following inclusion criteria: (1) autoimmune epilepsy suspected based on the presence of 1 neural autoantibody (n = 23), personal or family history or physical stigmata of autoimmunity, and frequent or medically intractable seizures; and (2) initiated a 6- to 12-week trial of IV methylprednisolone (IVMP), IV immune globulin (IVIg), or both. Patients were defined as responders if there was a 50% or greater reduction in seizure frequency.Results:Eighteen patients (62%) responded, of whom 10 (34%) became seizure-free; 52% improved with the first agent. Of those receiving a second agent after not responding to the first, 43% improved. A favorable response correlated with shorter interval between symptom onset and treatment initiation (median 9.5 vs 22 months; p = 0.048). Responders included 14/16 (87.5%) patients with antibodies to plasma membrane antigens, 2/6 (33%) patients seropositive for glutamic acid decarboxylase 65 antibodies, and 2/6 (33%) patients without detectable antibodies. Of 13 responders followed for more than 6 months after initiating long-term oral immunosuppression, response was sustained in 11 (85%).Conclusions:These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy.Classification of evidence:This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control.