% 0期刊文章%伊利亚Sechi % P·皮尔斯莫里斯%安德鲁·麦肯%一个香农Hinson肖恩Pittock % %阿伦·Aksamit布莱恩Weinshenker % %阿纳斯塔西亚Zekeridou埃文·A . Jolliffe % %院长Wingerchuk % Eoin P·弗拉纳根% T胶质原纤维酸性蛋白免疫球蛋白G (GFAP-IgG)相关脊髓炎:描述和比较Aquaporin-4-IgG脊髓炎(S13.006) % D J神经病学2018% % P S13.006 % V 90% N 15补充% X目的:比较脊髓炎陪同GFAP-IgG astrocytopathy脊髓炎的Aquaporin-4-IgG血清反应阳性的视neuromyelitis谱系障碍(NMOSD)。首页背景:我们最近报道GFAP-IgG meningo-encephalo-myelitis或有限形式的新颖的生物标志物。这已经由其他独立复制的调查人员在世界的其他地区。两个aquaporin-4-IgG血清反应阳性的NMOSD和GFAP-IgG可以与longitudinally-extensive(≥3椎段)T2-hyperintense病变(LETM)脊柱MRI。设计/方法:我们回顾性14 GFAP-IgG脊髓炎患者血清反应阳性的在梅奥诊所(2000 - 2017)。GFAP-IgG中检测出血清、脑脊液或通过组织和细胞免疫荧光检测;14都是对AQP4-IgG不利。我们比较这些病人41 aquaporin-4-IgG血清反应阳性的NMOSD脊髓炎(通过组织的免疫荧光或细胞测定[固定或生活]);所有41 GFAP-IgG血清反应阴性的。结果:GFAP-IgG脊髓炎通常呈现在不知不觉中(> 6周)比NMOSD(9/14(64%)和1/38 (3%);p < 0.01),总是(14/14[100%])同时或之前meningo-encephalitis症状/体征,包括头痛(79%)、地震(71%)、脑病(71%)和视神经盘水肿(57%)。最常见的骨髓炎表现肠/膀胱功能障碍(11/14[79%]),痉挛状态/反射亢进(7/14[50%])和步态失调(6/14 [43%])。 Unlike NMOSD, weakness (5/14[36%] vs 36/41[88%]) and numbness (4/14[29%] vs 41/41[100%]) were less common (p<0.01).On MRI, LETM predominated in both GFAP-IgG (8/11[73%]) and AQP4-IgG (29/33[86%]). T2-signal was often fainter and less well-demarcated in GFAP-IgG myelitis with more frequent conus involvement (7/11[64%] vs 1/24[4%]; p<0.01). Cord swelling was less frequent (4/13[31%] vs 27/32[84%], p<0.01). Additional distinguishing features (p<0.05) included central canal enhancement (3/12[25%] vs 0/33[0%]), diffuse punctate cord parenchymal enhancement (5/12[42%] vs 0/33[0%]), cord leptomeningeal enhancement (9/12[75%] vs 1/33[3%]) and radial periventricular enhancement on brain MRI (10/13[77%] vs 1/21[5%]). Brisk improvement following corticosteroids occurred in 93% with GFAP-IgG myelitis; 80% relapsed with taper.Conclusions: In contrast to myelitis with aquaporin-4-IgG seropositive NMOSD, myelitis accompanying GFAP-IgG presents more insidiously and is typically accompanied by meningo-encephalitis manifestations.Disclosure: Dr. Sechi has nothing to disclose. Dr. Morris has nothing to disclose. Dr. McKeon has received research support from Medimmune, Euroimmun, Grifols and Alexion. Dr. Pittock has nothing to disclose. Dr. Hinson has nothing to disclose. Dr. Weinshenker has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Novartis, Alexion, MedImmune, Caladrius Biosciences, Brainstorm Therapeutics. Dr Aksamit has nothing to disclose. Dr. Jolliffe has nothing to disclose. Dr. Zekeridou has nothing to disclose. Dr. Wingerchuk has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Caladrius and MedImmune. Dr Flanagan has nothing to disclose. %U

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