RT期刊文章SR电子T1临床发现,免疫球蛋白子类,在脑炎和抗体效果与metabotropic谷氨酸受体5(受体)抗体(P5.390)摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP P5.390 VO 90 15补充A1玛丽安娜Spatola A1莉迪亚萨瓦特A1耶稣Planaguma A1孝宏Iizuka A1哈拉尔德Pruss A1首页 Eugenia马丁斯A1泰国Armangue A1默娜·罗森菲尔德A1弗朗西斯克格劳A1约瑟Dalmau年2018 UL //www.ez-admanager.com/content/90/15_Supplement/P5.390.abstract AB目的:报告的临床特征和结果11 anti-mGluR5脑炎患者,免疫球蛋白g antibody-subclass,抗体对培养的神经元的影响。背景:受体抗体已报告在一些自身免疫性脑炎和霍奇金淋巴瘤患者;潜在的致病性抗体是未知的。设计/方法:临床信息是由作者或提供治疗的医生。抗体和免疫球蛋白子类使用大脑免疫组织化学和细胞化验测定;抗体致病效果测定海马神经元的文化。结果:从2006年1月到2017年5月,11个患者,平均年龄29岁(范围6 - 75),5/11的女性,被确定。的主要临床特征是精神(10/11),认知(10/11),运动障碍(7/11),睡眠障碍(7/11),癫痫发作(6/11)。在疾病中改良Rankin规模峰值为4.0;6/11的病人需要重症监护。六个患者肿瘤:5霍奇金淋巴瘤,1小细胞肺癌; the latter had progressive ophthalmoplegia, executive dysfunction, and ataxia. Of the 6 patients with tumor, 3 received immunotherapy and cancer treatment, and 3 cancer treatment only. At the last follow-up (median, 48 months) 6 patients had complete and 5 partial recovery. Neurologic relapse occurred in 2 patients. CSF showed pleocytosis (median WBC 22) in 11/11 patients; brain MRI was abnormal in 5/11 patients, involving limbic (1) or extra-limbic regions (4), and the EEG was abnormal in 5/11. Patients’ mGluR5 antibodies were IgG1, alone (2) or in combination with IgG2 (2) and IgG3 (3). Patients’ IgG caused a significant and specific decrease of total cell surface and synaptic mGluR5 clusters without altering PSD95 clusters.Conclusions: Anti-mGluR5 encephalitis is characterized by a complex neuropsychiatric syndrome, not restricted to limbic encephalitis, and can occur without tumor. Patients respond to immunotherapy and cancer treatment, but relapses can occur. The antibodies have pathogenic effects altering the levels of synaptic and extrasynaptic mGluR5.Study Supported by: MS received research support from the University of Lausanne and CHUV Hospital of Lausanne joint Foundation, Lausanne, SwitzerlandDisclosure: Dr. Spatola has nothing to disclose. Dr. Sabater has nothing to disclose. Dr. Planagumà has nothing to disclose. Dr. Iizuka has nothing to disclose. Dr. Pruss has nothing to disclose. Dr. Martinez-Hernandez has nothing to disclose. Dr Armangue has nothing to disclose. Dr. Rosenfeld has nothing to disclose. Dr. Graus has nothing to disclose. Dr. Dalmau has received personal compensation in an editorial capacity for Editor: Neurology, Neuroimmunology, Neuroinflammation; and UpToDate. Dr. Dalmau has received royalty, license fees, or contractual rights payments from Euroimmune. Dr. Dalmau has received research support from Euroimmun.