PT -期刊文章盟Yujuan娇AU -詹姆斯·弗莱尔AU -万带兰列侬AU -艾米Quek Chiara Costanzi AU -安德鲁·麦肯盟盟莎拉·詹金斯-拉斐尔人工盟盟-史密斯Carin盟-布莱恩Weinshenker AU -迪恩Wingerchuk AU -伊丽莎白•舒斯特盟克劳迪娅Lucchinetti AU -肖恩Pittock TI -什么是“真正”的频率AQP4-IgG-Negative动和表型上不同于血清反应阳性的疾病吗?(P02.136) DP - 2013年2月12日TA -神经病首页学PG - P02.136 P02.136 VI - 80 IP - 7补充4099 - //www.ez-admanager.com/content/80/7_Supplement/P02.136.short 4100 - //www.ez-admanager.com/content/80/7_Supplement/P02.136.full所以Neurology2013 2月12;80 AB -目的:调查1999年病人履行Wingerchuk动校正的诊断标准:1)AQP4-IgG检出率在重组antigen-based化验血清得分为负1代组织的间接免疫荧光试验(IIF),和2)持续血清反应阴性的患者的临床特点。背景:AQP4-IgG临床验证生物标志物统一动谱系障碍。试验敏感性/特异性改变。假消极无效表型的比较。设计/方法:从2005年到2011年,5349年梅奥诊所的病人样本被国际分析测试。临床记录了159例动。其中,109例(69%)阳性、50(31%)负面;47个50的血清得分负被ELISA用于测试(RSR / Kronus)和AQP4-transfected细胞绑定化验(CBA幻灯片(Euroimmun)和内部fluorescence-activated细胞排序(流式细胞仪))。临床和人口特征基于最终serostatus比较。结果:30的47个IIF-negatives(64%)评审鉴定血清反应阳性的。 Overall, AQP4-IgG detection rate was improved to 88% by FACS assay, 85% by CBA and 80% by ELISA. For 24 of these 30 patients, serial specimens were available. Subsequent sera yielded a positive result in only 2 patients whose initial serum was negative by FACS assay: the 4th of 6 specimens in one, and the subsequent 3 in the other. Of the 17 cases finally scored seronegative, only one specimen was available for 7, and 14 were receiving immunotherapy when blood was drawn. By comparison with seropositive cases, sexes were equally represented in seronegatives (female:male = 8:1 in seropositives; p<0.0001); simultaneous optic neuritis and transverse myelitis at disease onset was 10-fold more frequent in seronegatives (29% versus 3%, p=0.0001).CONCLUSIONS: Serological tests employing recombinant AQP4 antigen are significantly more sensitive than tissue-based IIF for NMO-IgG detection. Initial testing should be done before commencing immunotherapy and, if negative, a subsequent specimen tested. Patients seronegative by most sensitive (FACS) assay differ phenotypically from AQP4-IgG-seropositive patients.Supported by: Mayo Clinic Foundation, Guthy-Jackson Charitable Foundation (VL, CL, SP, BW, DW) and NIH (R01-NS65829, SP).Disclosure: Dr. Jiao has nothing to disclose. Dr. Fryer has nothing to disclose. Dr. Lennon stands to receive royalties for commercial assays to detect of aquaporin 4-specific autoan. Dr. Quek has nothing to disclose. Dr. McKeon has nothing to disclose. Dr. Costanzi has nothing to disclose. Dr. Iorio has nothing to disclose. Dr. Jenkins has nothing to disclose. Ms. Smith has received research support from Abbott Laboratories. Dr. Weinshenker has received personal compensation for activities with Novartis, Biogen Idec, Elan Corporation, GlaxoSmithKline Inc., Asahi Kasei Kuraray Medical Company as a consultant and/or participant on a data safety monitoring board. Dr. Weinshenker has received (royalty or license fee or contractual rights) payments from Mayo Medical Ventures. Dr. Wingerchuk has received research support from Genentech, Genzyme, Alexion, TerumoBCT and the Guthy-Jackson Charitable Foundation. Dr. Shuster has nothing to disclose. Dr. Lucchinetti stands to receive royalties for commercial assays to detect aquaporin 4-specific autoantibody. Dr. Pittock and Mayo Clinic have a financial interest in the technology entitled "Neuromyelitis Optica Autoantibodies as a Marker for Neoplasia." This technology has been licensed to a commercial entity but no royalties have been received. Dr Sean Pittock has received research support from Alexion Pharmaceuticals, Inc.Tuesday, March 19 2013, 7:30 am-12:00 pm