PT -期刊文章盟詹妮弗·威廉姆斯盟——卡伦·奥康奈尔盟——肖恩·康诺利盟——安吉拉·文森特AU -克里斯托弗McGuigan盟-迈克尔·哈钦森盟-肖恩baillie gifford TI -复发Anti-Glycine受体抗体介导的脑炎与刚度和肌阵挛(P01.235) DP - 2012年4月23日TA -神经病学PG - P01.235 P01.235 VI - 78 IP - 1补充4099 - //www.ez-admanager.com/content/78/1_Supplement/P01.235.short 4100 - //www.ez-admanager.com/content/首页78/1_Supplement/P01.235.full所以Neurology2012 4月23日;78 AB -目的:描述一个临床病例的烫(与刚度和肌阵挛进行性脑脊髓炎)造成anti-glycine受体抗体。背景进步与刚度和肌阵挛性脑脊髓炎(烫)是一种罕见的疾病呈现与刚性亚急性,肌肉痉挛,脑干和hyperekplexia迹象。Hyperekplexia结果中断正常的抑制性glycinergic途径集中在尾桥。我们报告一例复发烫次要anti-glycine受体抗体。设计/方法:一位65岁的老人在2001年提出一个两周的历史严重hyperekplexia,脊椎和下肢的刚性,双边下垂,水平凝视麻痹。调查显示脑脊液淋巴细胞脑脊液细胞增多和同时同心针肌电图与烫的诊断是相一致的。Anti-glycine受体抗体被确定病人血清检测时绑定到人类胚胎肾细胞转染EGFP-tagged甘氨酸受体α1亚基。病人的血清强烈束缚GlyR-EGFP表达细胞。诊断为anti-glycine受体介导烫。病人对最初的强烈的免疫抑制和多年保持稳定,没有进一步的免疫调节。Results: Ten years after initial presentation he presented again with a two month history of worsening ptosis, complex ophthalmoplegia, recurrent hyperekplexia, and limb rigidity. There was no evidence of intercurrent sepsis or underlying malignancy. High titres of anti-glycine receptor antibodies were again identified in serum and CSF. The recurrent symptoms improved quickly with immunomodulation.Conclusions: Relapsing PERM due to anti-glycine receptor antibodies ten years after initial presentation is a rare and interesting clinical scenario. Rather than a monophasic illness, the disease course has been relapsing and remitting requiring combined aggressive immunosuppressive treatment. This raises several interesting clinical issues including the use of serial antibody testing to predict clinical relapse and of long term, low-grade immunosuppression in relapse prevention.Disclosure: Dr. Williams has nothing to disclose. Dr. O'Connell has nothing to disclose. Dr. Connolly has nothing to disclose. Dr. Vincent has received personal compensation for activities with Athena Diagnostics as a consultant. Dr. Vincent has received royalty payments from Athena Diagnostics. Dr. McGuigan has received personal compensation for activities with Biogen Idec and Merck Serono. Dr. Hutchinson has received personal compensation for activities with Biogen Idec as a speaker. Dr. O'Riordan has nothing to disclose.Monday, April 23 2012, 14:00 pm-18:30 pm