RT期刊文章SR电子T1神经节苷脂复合测试确定免疫响应motor-onset MADSAM和MMN乔(P5.111)摩根富林明神经病学神经病学FD Lippincott Williams &威尔金斯SP P5.111 VO 88是16补充A1詹妮弗Ma188金宝慱官网下载rtinez-T首页hompson A1梅丽莎·斯奈德A1迈克尔埃托雷A1 Jayawant Mandrekar A1安德鲁·麦肯A1肖恩Pittock A1安东尼Windebank A1克里斯托弗·克莱因年2017 UL //www.ez-admanager.com/content/88/16_Supplement/P5.111.abstract AB目的:评估如果复合神经节苷脂自身抗体检测可以帮助预测免疫疗法反应多病灶的运动神经病变(MMN)和识别motor-onset多病灶的病例获得脱髓鞘感觉和运动神经病变(MADSAM)。背景:MMN马达接通状态,传统上难以和有时很难区分motor-predominant MADSAM病例。这对预后和治疗反应的两种疾病的临床过程不同。设计/方法:我们回顾了1112年梅奥诊所的病人接受复合神经节苷脂GM1自身抗体检测(monosialo-asialo [IgM,免疫球蛋白g])和GD1b (disialo [IgG、IgM])从2007 - 2015年的自身抗体。结果:至少一个神经节苷脂自身抗体检测在66年(6%)的人在这个群。其中,19(29%)最初诊断为MMN, 10 (15%) CIDP与MADSAM(6), 7(11%)和肌萎缩性侧索硬化症。平均3自身抗体在MMN情况下被发现。发现IgM-monosialo GM1在MMN病例(79%)最常见的中位数1:16,000的效价。这个自身抗体存在于13%的MADSAM病例1:2,500平均效价。百分之七十九的血清反应阳性的MMN患者免疫治疗响应(通常丙种球蛋白)相比,血清反应阴性的病例的47% (p = 0.045),平均反应治疗60岁和12个月,分别。八个最初的19例(42%)诊断为MMN最终被诊断为MADSAM开发感觉症状或异常后感觉神经传导研究。百分之七十五的病例免疫治疗反应。Conclusions: The presence of multiple ganglioside autoantibodies including IgM-monosialo GM1 lend support to a diagnosis of MMN, but the presence of IgM-monosialo GM1 does not exclude the possibility of motorpredominant MADSAM. A sustained immunotherapy response should raise suspicion for MADSAM even if the patient initially manifests with motor-only or motor-predominant symptoms.Disclosure: Dr. Martinez-Thompson has nothing to disclose. Dr. Snyder has nothing to disclose. Dr. Ettore has nothing to disclose. Dr. Mandrekar has nothing to disclose. Dr. McKeon has received research support from MedImmune and Euroimmun. Dr. Pittock's institution has received compensation for Dr. Pittock's activities with Alexion Pharmaceuticals, Medimmune and Chugai Pharma USA. Dr. Pittock and Dr. Pittock's institution stand to receive patent payments that relate to patents for functional AQP4/NMO-IgG assays and NMO-IgG as a cancer marker. Dr. Pittock has received research support from NIH, Alexion Pharmaceuticals and Medimmune related to autoimmune NMO/AQP4 channelopathy. Dr. Windebank has nothing to disclose. Dr. Klein has received personal compensation for activities with Genzyme, Inc. as a speaker.