% 0期刊文章%一个Avi Gadoth Anastasia Zekeridou % %一个科尔顿Thoreson克里斯托弗·克莱因% %一个Masoud马吉德% Eoin P·弗拉纳根%莎拉·詹金斯安德鲁·麦肯% %万带兰A·列侬%肖恩·J·Pittock % T升高LGI1-IgG CSF指数预测较差的神经学结果(P1.397) % D J神经病学2018% % P P1.397 % V 90% N 15补充% X目的:确定CSF leucine-rich-glioma-inactivated-1 (LGI1)免疫球蛋白效价、指数或IgG-subclass LGI1患者自首页身免疫有预后意义。背景:LGI1-IgG有可能导致神经自身免疫,影响中枢和周围神经系统。大多数患者是immunotherapy-responsive;启动较晚,治疗,缺乏应对初始免疫疗法和已报告发生复发与糟糕的结果。数据是缺乏关于鞘内LGI1-IgG合成及其关系的结果。设计/方法:临床资料的回顾性研究(病人的图表或医生提供信息)39伴随患者血清和脑脊液标本梅奥诊所的神经免疫学实验室。结果计算使用夫人得分(有利≤2)。LGI1-IgG检测、滴定和子类被确定通过临床验证使用HEK293细胞间接免疫荧光法测定人类LGI1 overexpressing重组。LGI1-IgG特定指数计算如下:[LGI1-IgG-titer CSF /血清]/[血清白蛋白CSF /]结果:患者有利(77%)和不利结果年龄之间没有显著性差异,性别,夫人在最低点和所用时间治疗。所有可用的病人的血清特异性LGI1-IgG4子类。只有26%的人血清LGI1-IgG1共存。 There was no difference in demographics, relapse rate and MRI findings in patients with co-existing LGI1-IgG1.LGI1-IgG index was elevated (>1) in 21 patients (54%), suggesting intrathecal synthesis. Patients with worse outcome at last follow-up had significantly higher LGI1-IgG-CSF index (median 6.57 vs 0.5, p<0.05) compared to those with better outcome. Higher CSF LGI1-IgG4-subclass-specific titer and index also correlated with worse outcome (p<0.005 for both).Conclusions: We suggest that evidence of intrathecal LGI1-IgG synthesis is associated with worse clinical outcome and may correlate with neuronal injury. A higher index in initial non-responders to immunotherapy may indicate the need for more aggressive immunotherapyDisclosure: Dr. Zekeridou has nothing to disclose. Dr. Gadoth has nothing to disclose. Dr. Klein has nothing to disclose. Dr. Thoreson has nothing to disclose. Dr. Majed has nothing to disclose. Dr Flanagan has nothing to disclose. Dr. McKeon has received research support from Medimmune, Euroimmun, Grifols and Alexion. Dr. Jenkins has nothing to disclose. Dr. Lennon has received royalty, license fees, or contractual rights payments from RSR, royalties from sale kits for AQP4 IgG detetction and from clinical service assays performed outside Mayo clinic. Dr. Pittock has nothing to disclose. %U