文摘
摘要目的:评估临床、放射学和预后特点MOG-IgG脊髓炎,比较水通道蛋白4 (AQP4)免疫球蛋白和多发性硬化症(MS)脊髓炎。
背景:越来越多的证据表明,检测MOG-IgG定义了一个临床综合征不同于女士和AQP4-IgG血清反应阳性的NMOSD。的临床和影像学特征识别MOG-IgG骨髓炎可以早期诊断,从而提高预测和治疗决策。
设计/方法:梅奥诊所的病人(2000 - 2017)与骨髓炎和血清阳性MOG-IgG被包括在内。AQP4-IgG脊髓炎(n = 46)和多发性硬化症(MS)脊髓炎(n = 26)患者用作比较。结果变量包括改良Rankin得分(夫人)和步态援助的必要性。MRI特征比较neuro-radiologist蒙蔽的方式。
结果:54个MOG-IgG脊髓炎患者包括在内。孤立的横向脊髓炎是29的初始临床表现(53.5%)和10(19%)最初诊断为病毒性/ post-viral急性弛缓性脊髓炎。某些特性有利于MOG-IgG AQP4-IgG或骨髓炎是女士T2-signal异常局限于灰质(矢状线和轴向H-sign)和缺乏增强(p < 0.05)。Longitudinally-extensive t2损伤在MOG-IgG相似的频率和AQP4-IgG脊髓炎(80% vs 79%;p = 0.52),但没有找到女士在一个以上的脊髓损伤和圆锥与MOG-IgG更频繁的参与比AQP4-IgG但没有不同于依赖轮椅女士脊髓炎最低点发生在三分之一的MOG-IgG和AQP4-IgG患者但从未女士然而,复苏与MOG-IgG比AQP4-IgG病人。
结论:骨髓炎是一种早期的表现MOG-IgG相关疾病和急性弛缓性脊髓炎临床表型。我们确定了临床和MRI属性给出线索MOG-IgG脊髓炎和帮助区别于AQP4-IgG和脊髓炎女士。
披露:Dubey博士没有披露。Pittock接收博士研究支持Alexion制药、落实和Grifols。Krecke博士没有披露。莫里斯博士没有披露。Sechi博士没有披露。Zalewski博士没有披露。Weinshenker博士已经收到个人赔偿咨询、担任科学顾问委员会说,或其他活动与诺华,Alexion,落实Caladrius生物科学,头脑风暴疗法,Chugai, Roivant科学。Weinshenker博士已经收到版税、许可费或合同权利从RSR有限公司支付,牛津大学,济贫院民用里昂,MVZ劳动PD福博士和同事GbR。综合商社没有披露博士。Lucchinetti博士没有披露。 Dr. Fryer has nothing to disclose. Dr. Lopez has nothing to disclose. Dr. Chen has nothing to disclose. Dr. Jitprapaikulsan has nothing to disclose. Dr. McKeon has received research support from Medimmune, Euroimmun, Grifols and Alexion. Dr. Gadoth has nothing to disclose. Dr. Keegan has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Cambridge University Press. Dr. Keegan has received research support from Biogen. Dr. Tillema has nothing to disclose. Dr. Naddaf has nothing to disclose. Dr. Patterson has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Actelion, Amicus, Intrabio, Orphazyme, and Vtesse. Dr. Patterson has received personal compensation in an editorial capacity for Sage, the Society for the Study of Inborn Errors of Metabolism, and Up-To-Date. Dr. Patterson has received research support from Actelion and Orphazyme. Dr. Messacar has received research support from Dr. Messacar receives grant support from the NIH. Dr. Tyler has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with DNAtrix Therapeutics. Dr. Tyler has received research support from Taiga Biotechnologies Inc. Dr. Flanagan has received research support from Medimmune.
