晚发型NMO和NMOSD患者的特征分析(P02.152)

摘要

摘要目的:分析LONMO/NMOSD患者的特征。

背景:视神经脊髓炎(NMO)是一种罕见的炎性疾病。关于50岁以后发病的迟发性NMO (LONMO)或NMO谱系疾病(NMOSD，即视神经炎/长广泛性横脊炎伴AQP4-Ab)患者的资料较少。

设计/方法:对成人医疗中心的LONMO/NMOSD患者进行回顾性研究，包括NOMADMUS(法国)、德国NMO中心、土耳其LONMO组(土耳其)和英国队列的四个国家队列。这些队列参加了EDEN(欧洲设备视神经脊髓炎)项目。

结果:67例LONMO患者和45例LONMOSD患者平均随访5.2年。LONMO/NMOSD队列主要为白种人(93.2%)，女性(81.2%)，血清抗aqp4抗体阳性(90%)。NMO发病时，中位年龄为58.5岁，年龄最大的患者为82.5岁。在80%的病例中观察到复发形式，无进展形式。平均年复发率为1.6，首次发作间隔15.6个月。初次脑MRI显示T2病变的平均数量为1.5个，8%的患者符合Barkhof标准。23%的患者观察到脑脊液寡克隆条带。LONMO/NMOSD患者到EDSS 4的中位时间为1.5年，到免疫抑制的中位时间为1.4年。在最后一次随访中，16例患者死亡(2例在第一次发作后死亡)，LONMO组和LONMOSD组的中位EDSS为5.5，锥体和肠/膀胱功能严重残疾(中位EDSS=3)。根据LONMO或LONMOSD病理的生存曲线与AQP4血清学状态之间无差异。

结论:与文献中描述的早发性NMO相比，LONMO/NMOSD特别严重，死亡率高。我们也证实了运动障碍在这些患者中的主导地位。

得到:法国国家研究机构在投资未来方案范围内的公共赠款，编号ANR-10-COHO-002。

披露:他在Biogen Idec, Bayer Schering, Teva Neuroscience, Merck Serono, Sanofi-Aventis Pharmaceuticals, Inc.和Novartis的活动中获得个人报酬。马里尼耶医生没有什么要透露的。雅各布医生没什么好说的。Siva博士在去年获得拜耳-先ering AG，诺华，Biogen Idec和Allergan的科学顾问委员会的咨询和服务的个人报酬，以及差旅和注册保险。Siva博士获得了伊斯坦布尔大学Cerrahpasa医学院神经内科临床神经免疫学部门多发性硬化症中心的研究支持。首页该中心获得了拜耳先灵公司的无限制研究资助。Paul博士曾因与梯瓦、赛诺菲安万特、默克雪兰诺、百健爱德、拜耳、诺华和Guthy Jackson慈善基金会的合作而获得个人补偿。Paul博士曾获得拜耳先灵、默克雪兰诺、梯瓦制药和诺华制药的研究支持。Zephir博士在Biogen Idec, Bayer Pharmaceuticals Corporation, Merck Serono, Teva Neuroscience, Sanofi-Aventis Pharmaceuticals, Inc.和Novartis担任顾问的活动中获得个人报酬。Leite博士因在Biogen Idec担任演讲嘉宾而获得个人报酬。 Dr Akman-Demir has received personal compensation for activities with Merck Serono, Bayer, Novartis, Teva, for consulting or giving lectures. Dr. Elsone has nothing to disclose. Dr. Jarius has received research support from Bayer Healthcare and Merck Serono. Dr. Papeix has received personal compensation for activities with Bayer Schering, Biogen Idec, Merck Serono, Novartis, Sanofi and Teva Pharma, Almirall, Roche, Genzyme as consultant. Dr. Mutch has nothing to disclose. Dr. Saipoglu received personal compensation for activities with Merck Serono and Biogen Idec. Dr. Wildemann has received personal compensation for activities with Merck-Serono and Teva/Sanofi Aventis. Dr. Wildemann has received research support from Novartis Pharma GmbH and the German Federal Ministry of Education and Research. Dr. Kitley has received personal compensation for activities with Novartis, Biogen Idec, and Teva. Dr. Karabudak has received honorarium from Bayer-Schering. Dr. Aktas has received personal compensation for activities with the German Research Council (DFG SFB-TRR43, DFG GK-1033) and honoraria & travel grants from Bayer HealthCare, BiogenIdec, Merck-Sorono, Novartis, and Teva/Sanofi-Aventis. Dr. Aktas has received research support from Bayer Schering Pharma and Novartis and consulting/speaker fees from Bayer Schering Pharma, Biogen Idec, Merck Serono, Novartis, and Sanofi-Aventis. Dr. Kuscu has nothing to disclose. Dr. Altintas has nothing to disclose. Dr. Palace has received personal compensation from Teva, Biogen, Merck Serono, GSK, and Bayer Schering Pharma for clinical trials, running a database, attending advisory committees, giving lectures, and attending international meetings. Dr. Confavreux has received personal compensation for activities with Biogen Dompe, Biogen Idec, Gemacbio, Genzyme Corporation, Hertie Foundation, Novartis, Sanofi-Aventis Pharmaceuticals, Inc., Teva Neuroscience, UCB Pharma, Bayer Schering, and Merck Serono. Dr. Confavreux has received research support from Bayer Schering, Biogen Idec, Merck Serono, Novartis, Sanofi-Aventis Pharmaceuticals, Inc., and Teva Neuroscience. Dr. De Seze has received personal compensation for activities with Allergan, Inc., Almirall, Bayer Schering, Biogen Idec, Genzyme Corporation, LFB, Merck Serono, Sanofi-Aventis Pharmaceuticals, Inc., and Teva Neuroscience.