% 0期刊文章%玛丽安娜Spatola %一Eugenia马丁斯·莉迪亚•萨瓦特% %一个默娜·罗森菲尔德%弗朗西斯克格劳%约瑟Dalmau % T小说发现自身免疫性脑炎抗体metabotropic谷氨酸受体5(受体)(P1.333) % D J神经病学2017% % P P1.333 X % V % 88% N 16补充目的:报告10例的临床特征和结果anti-mGluR5脑炎和抗体对培养神经细胞的影响。首页背景:受体抗体在自身免疫性脑炎患者和已报告霍奇金淋巴瘤(欧菲莉亚综合症)。设计/方法:临床治疗的医生提供的信息。抗体测定大脑使用免疫组织化学和细胞化验;抗体效果测定海马神经元的文化。结果:我们发现10例(2006年1月- 2016年10月):平均年龄29岁(范围6 - 75),4/10的女性。主要临床表现是精神(9/10),认知(8/10),运动障碍(7/10),癫痫发作(6/10),改变意识(4/10),和睡眠障碍(5/8)。夫人在疾病高峰中值为4.5;5/10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;后者有进步眼肌麻痹,认知变化,步态共济失调和SOX1抗体。6的肿瘤患者,3接受免疫治疗和癌症治疗,3只癌症治疗。 The 4 patients without tumor received immunotherapy. At last follow-up (median 30 months) 9/10 patients had complete (6) or partial (3) recovery; 1 patient died. One patient with Hodgkin’s lymphoma had a neurological relapse 2.5 years after the first episode of encephalitis. Ancillary tests showed: CSF pleocytosis in 10/10 (median WBC 22) patients; oligoclonal bands in 4/7; MRI abnormalities in 5/10 involving temporal (2/5) or extra-temporal regions (3/5), and EEG abnormalities in 5/7. Antibodies were identified in serum and CSF of all paired samples (5/10). The effects of the antibodies in neuronal cultures will be presented.Conclusions: Anti-mGluR5 encephalitis is characterized by limbic and extra-limbic manifestations and can occur in the absence of a tumor. Patients with tumors other than Hodgkin’s lymphoma may develop an atypical clinical syndrome. Patients respond to immunotherapy (and cancer treatment), but relapses can occur.Disclosure: Dr. Spatolas has received research support from Pierre Mercier Foundation for Science and the Societe Acadmique Vaudoise. Dr. Sabater has nothing to disclose. Dr. Martinez-Hernandez has nothing to disclose. Dr. Rosenfeld has received personal compensation from Athena Diagnostics and Euroimmun. Dr. Graus has nothing to disclose. Dr. Dalmau receives royalties from the editorial board of Up-To-Date. Dr. Dalmau has received royalties from a patent from Memorial Sloan-Kettering Cancer Center and royalty payments and/or for technology and invention on NMDAR, GABA(B), GABAaR, DPPX, and IgLON5 antibody test. Dr. Dalmau has received research support from Euroimmun. %U
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