文摘
目的:确定抗体的曲目群体的患者GAD65 antibody-associated综合症。背景:高滴度抗体GAD65一直在描述各种各样的障碍,包括小脑性共济失调(CA), stiff-person综合症(SMS)和边缘脑炎/癫痫(LE)。的各种表型表明差异GAD65-antibody浓度和/或共存自身免疫可能出现在这些病人。方法:109例GAD65-antibody-associated综合症(40 CA, 32 SMS和37例)被纳入研究。GAD65-antibodies的存在被证实与鼠小脑,免疫组织化学和ELISA或细胞试验(CBA)。GAD67-antibodies测定CBA,神经细胞表面抗原的抗体与免疫荧光检查培养海马神经元和抗原CBA (NMDAR、GABAaR GABAbR, GlyR)。结果:GAD67-antibodies被确定在82/109(75%)的患者anti-GAD65综合症有关。有相关性的存在更高浓度的GAD65-antibodies和GAD67-antibodies的存在(p = 0.0054)。所有临床表型与类似的一系列GAD65-antibody滴度。在所有患者GAD65-antibodies识别线性抗原表位免疫印迹(可见的)。 Thirteen patients had co-existing cell surface antibodies: 5 GlyR, 5 GABAaR, 1 GABAb receptor, and 1 NMDA receptor. The presence of cell surface antibodies was more frequent in patients with LE (7/37, 19%) compared with patients with CA (4/40, 10%) or SMS (2/32, 6%). Conclusions: 75% of patients with GAD65-antibody-associated syndromes have GAD67-antibodies. The association of GAD67-antibodies with LE has not been previously reported. The variety of phenotypes occurring in association with GAD65-antibodies cannot be explained by differences in GAD65-antibody titers (similar range in all phenotypes) or the presence of GAD67-antibodies. In a subset of patients with LE the clinical picture is likely related to coexisting and more relevant antibodies to cell-surface antigens. Funded by FIS PI11/01780 and PI12/00611, the NIH RO1NS077851 and Fundació la Marató de TV3 (101530)
披露:Gresa-Arribas博士没有披露。Arino博士没有披露。萨瓦特博士没有披露。卡巴雷若没有披露博士。阿尔巴博士没有披露。马丁斯博士没有披露。小博士没有披露。Saiz博士已经收到个人赔偿的活动与拜耳先灵葆雅,默克公司Serono, Idec,赛诺菲-安万特制药公司,Teva神经科学和诺华。Dalmau博士已经收到个人薪酬在一篇社论中更新的能力。从雅典娜诊断Dalmau博士获得了特许使用金。 Dr. Dalmau has received research support from Euroimmun. Dr. Graus has nothing to disclose.
周三,2014年4月30日,下午三点pm-6:30
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