RT期刊文章SR电子T1 Thymoma-associated神经元表面抗体和临床表现(4620)摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP 4620签证官96年3月15 A1补充Guasp A1 首页Eugenia马丁斯A1乔恩·兰达A1莉迪亚萨瓦特A1艾伯特Saiz A1弗朗西斯克格劳A1约瑟Dalmau年2021 UL //www.ez-admanager.com/content/96/15_Supplement/4620.abstract AB目的:描述的临床特征和抗体曲目Thymoma-associated自身免疫性脑炎。背景:胸腺瘤是最常见的肿瘤之一,与自身免疫性脑炎;的频率神经表面抗体(NS-ab)是未知的。设计/方法:回顾性评估50胸腺瘤患者和疑似自身免疫性脑炎研究在医院诊所,大学的巴塞罗那,2011年1月至2020年9月。NS-ab和onconeuronal抗体测定大鼠大脑免疫组织化学,内部细胞化验,和商业免疫印迹分析。结果:发现了NS-ab在31/50(62%)的病人。与19 NS-ab消极的病人相比,那些NS-ab更有可能有边缘脑炎(20% vs 5%), Morvan综合症(14% vs 5%),癫痫发作(55% vs 25%),多病灶的cortico-subcortical天赋MRI异常(43% vs 25%)和临床复发(27% vs 5%) (p < 0 05)。其他临床特征没有显著不同:neuromyotonia (7% vs 10%), stiff-person综合征/烫发(9% vs 5%),或肌无力(36% vs 42%)。GABAaR抗体是最普遍的(12例)。其他NS-ab包括:4 LGI-1;2 AMPAR;1 CASPR2; 1 NMDAR; 2 GlyR (1 with concurrent GAD); 1 AQP4; 2 LGI1 + CASPR2; 1 GABAaR + LGI1; 1 GABAaR + AMPAR; and 1 CASPR2 + AMPAR + GABAbR; in the remaining 3 patients the identity of the antigens was unknown. Antibodies against onconeuronal antigens (2 CRMP5, 1 Hu, 1 Ma2) were only identified in the group of patients with NS-ab. Age, sex, tumor characteristics, inflammatory CSF, EEG findings, treatments, and outcome were similar in NS-ab positive and negative patients (not shown).Conclusions: Among patients with thymoma and autoimmune encephalitis, 62% had NS-ab. The most prevalent antibody was GABAaR, usually associated with seizures and multifocal FLAIR MRI abnormalities. Several concurrent NS-Ab or onconeuronal antibodies occurred in 26% of antibody positive cases.Disclosure: Mar Guasp has nothing to disclose. Dr. Martinez-Hernandez has nothing to disclose. Jon Landa has nothing to disclose. Lidia Sabater has nothing to disclose. Dr. Graus has nothing to disclose. Dr. Dalmau has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for American Academy of Neurology. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer Nature. An immediate family member of Dr. Dalmau has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Lamson Dugan & Murray LLP. The institution of Dr. Dalmau has received research support from Sage Therapeutics. The institution of Dr. Dalmau has received research support from Edmond J. Safra Foundation . The institution of Dr. Dalmau has received research support from La Caixa Foundation. The institution of Dr. Dalmau has received research support from Spanish Ministry of Health (ISCIII). The institution of Dr. Dalmau has received research support from Department of Health, Government of Catalunya. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. An immediate family member of Dr. Dalmau has received intellectual property interests from a discovery or technology relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. Dr. Dalmau has received publishing royalties from a publication relating to health care. An immediate family member of Dr. Dalmau has received publishing royalties from a publication relating to health care.