临床特征、实验室调查和应对自身免疫性脑病患者的免疫治疗:一项观察性研究(P6.128)

文摘

目的:本研究的目的是报告的临床和免疫学特征和应对可能的自身免疫性脑病患者的免疫治疗。背景:自身免疫性脑病代表一个复杂综合征与不同的临床表现和治疗结果。设计/方法:所有脑病患者包括下列一个或多个:癫痫、精神障碍、运动障碍、认知功能障碍,承认我们的机构从2012年1月至2015年9月,被录取。患者血清和脑脊液样本检测neural-specific自身抗体的免疫组织化学在老鼠大脑的衬底,肾和胃和免疫细胞化学在活体大鼠海马和皮层神经元。样品与胞内抗体的免疫球蛋白g绑定onconeural抗原特异性分析的屁股。所有样品都测试通过为NMDAR-IgG细胞化验,LGI1-IgG, CASPR2-IgG, AMPAR-IgG, GABA (B) R-IgG, GABA (A) R-IgG, mGluR5-IgG, mGluR1-IgG, DNER-IgG, GlycineR-IgG, IGLON5-IgG GAD65-IgG未及。结果:九十六例患者(女性,47)都包括在内。的平均年龄为44.3岁。Neural-specific免疫球蛋白检测35/96例(36岁,5 [percnt])。自身抗体绑定到NMDAR 4 (11 [percnt]); GABAaR, 2 (6[percnt]); LGI1, 2 (6[percnt]); LGI1 and CASPR2, 1 (3[percnt]); Hu, in 2 (6[percnt]); GAD65, in 3 (8,5[percnt]); SOX1,1 (3[percnt]), and to unclassified antigens (UNCA), in 19 (54 [percnt]). Associated tumors were found in 6/35 patients (17[percnt]) (thymoma, 3; non-Hodgkin lymphoma, 1; breast carcinoma, 1; lung adenocarcinoma, 1). In thirty-six patients who tested negative for neural autoantibodies an autoimmune etiology was excluded because other causes of the encephalopathy were identified. Thirty-three patients with and 21 patients without neural antibodies received immunotherapy. After immunotherapy, 30/33 patients with neural autoantibodies showed clinical improvement compared to 8/21 patients without autoimmune encephalopathy (p=<0.0001). CONCLUSIONS: the majority of neural autoantibodies detected in our cohort bound to unclassified antigens. The detection of neural autoantibodies specific for known or unclassified antigens predicts a response to immunotherapy.

披露:Damato博士没有披露。Evoli博士已经收到个人补偿活动与Biomarin科学顾问委员会成员。人工博士没有披露。