PT -期刊文章盟Marienke de Bruijn AU -罗兰Thijs AU -玛丽安Majoie盟-罗伯Rouhl盟-蒂斯van Asseldonk AU -齐斯·范Donselaar盟Frans Leijten AU -保罗Wirtz AU -安娜Bastiaansen AU -艾格尼丝·Sonderen AU -马可Schreurs盟彼得Sillevis Smitt AU - Maarten Titulaer TI -神经元抗体。她们在一次前瞻性多中心群来历不明的局灶性癫痫患者(S11.001) DP - 2019年4月09年TA -神经病学PG - S11.001 VI - 92 IP - 15补充4099 - //www.ez-admanager.com/content/92/15_Supplement/S11.001.short 4100 - //www.ez-admanager.com/content/92/15_Supplement/S11.首页001.full所以Neurology2019 4月09年;92 AB -目的:确定频率的抗体介入脑病来历不明的局灶性癫痫患者,并评估结果。背景:自身免疫性脑炎(AIE)出现了过去十年作为一个严重但可治疗神经系统疾病。大多数患者有明确的脑炎的迹象,包括癫痫发作。然而,还可以减少暴发性症状的患者,一些甚至可能有慢性癫痫没有其它脑炎的迹象。设计/方法:在这个荷兰多中心,前瞻性群组研究中,执行从2014 - 2017年,成年人与来历不明的局灶性癫痫,也没有怀疑AIE,被epileptologists包括。得到了病人和癫痫特征数据登记。数据对癫痫发作频率、治疗和夫人得到预期,4、8和12个月后包容。血清学分析完成,如果可用,CSF分析。使用免疫组织化学检测神经元抗体样本测试(包含IHC),细胞分析(CBA),海马神经元(LN)和ELISA生活。结果:患者知情同意在634年获得,包括590名患者。抗体检测19例(2%):14个高效价anti-GAD65(10000国际单位/毫升,证实CSF),一个anti-LGI1,两个anti-Caspr2,两个anti-GlycineR。 In 16 patients IHC was positive, and two also had positive LN, without known antibodies. These samples are currently being analyzed. Focusing on the patients with known antibodies, all 19 patients had subtle encephalitis signs (i.e. cognitive dysfunction, stiff person syndrome, autonomic disturbances). Drug-resistant seizures occurred in 17/19 patients (89%), 13 of them were treated with immunotherapy. All three patients with anti-LGI1 or anti-Caspr2 became seizure-free, while 6/14 GAD65 patients had >50% seizure reduction without becoming seizure-free. Other follow-up data are currently being collected.Conclusions: In this large patient cohort we found a low prevalence of antibody-mediated encephalopathies, including anti-LGI1, anti-Caspr2, anti-GAD65 and anti-GlycineR. All patients with neuronal antibodies had subtle other encephalitis signs, and most patients had drug-resistant seizures.Disclosure: Dr. De Bruijn has nothing to disclose. Dr. Thijs has nothing to disclose. Dr. Majoie has nothing to disclose. Dr. Rouhl has nothing to disclose. Dr. van Asseldonk has nothing to disclose. Dr. van Donselaar has nothing to disclose. Dr. Leijten has nothing to disclose. Dr. Wirtz has nothing to disclose. Dr. Bastiaansen has nothing to disclose. Dr. Van Sonderen has nothing to disclose. Dr. Schreurs has nothing to disclose. Dr. Sillevis Smitt has nothing to disclose. Dr. Titulaer has received research support from MedImmune LLC., Guidepoint LLC.