RT期刊文章SR电子T1分析Antineuronal疑似患者的抗体和病理证实克雅二氏症(P04.218)摩根富林明神经病学神经学乔FD Lippincott Williams &威尔金斯SP P04.218 OP P04.218 VO 80是7补充A1 Oriol格劳A1拉奎尔桑切斯Del Valle Diaz A1艾伯特Sa首页iz A1何塞Molinuevo Guix A1约瑟Dalmau A1弗朗西斯克格劳年2013 UL //www.ez-admanager.com/content/80/7_Supplement/P04.218.abstract AB目的:报告的频率CSF Antineuronal抗体(ANA-ab)疑似或病理证实患者克雅氏病(CJD)。背景:先前的研究描述了临床重叠库贾氏症和免疫介导性脑炎、和NMDA-R VGKC-complex定库贾氏症患者血清中抗体最近描述。设计/方法:通过免疫组织化学方法分析ANA-ab在冰冻的老鼠大脑与多聚甲醛固定的两组脑脊液样本:243在2012年发送到我们实验室14-3-3测试和48例病理证实库贾氏症。在第一组阳性病例进一步研究抗原特异性和所有48个库贾氏症患者的脑脊液样本还测试了NMDAR-ab使用一个非常具体的存在NR1/2细胞分析。结果:ANA-ab被发现在6(2.4%)的样品。迦得,目标抗原与CASPR2 NMDA-R,动,Tr (dn)和一个non-identified抗原。五个病人出现快速进行性认知能力下降,病人与Tr-ab亚急性小脑综合症。只有CSF Tr-ab也是14-3-3积极。MRI特征表明边缘脑炎被观察到在两个病人和CSF脑脊液细胞增多。小细胞肺癌诊断不明的患者抗体。所有患者改善(三个接受免疫疗法,两个症状治疗,和一个肿瘤治疗)。3/48库贾氏症患者的CSF与老鼠大脑显示典型的反应性; none of the 48 cases had NMDAR or VGKC-complex antibodies.CONCLUSIONS: A low but clinically relevant number of patients with suspected CJD have other potentially treatable disorders in association with ANA-ab. This finding reinforces the importance of including ANA-ab analysis in the diagnostic work-up of patients with suspected CJD. Patients with definite CJD do not have VGKC-complex or NMDAR-ab; our findings stress the need to examine CSF to avoid false positive antibody testing.Supported by: Fondo de Investigaciones Sanitarias. Madrid. Spain (FIS PS09/0193 and 11/01780).Disclosure: Dr. Grau has nothing to disclose. Dr. Sanchez Del Valle Diaz has nothing to disclose. Dr. Saiz has received personal compensation for activities with Bayer-Schering, Merck-Serono, Biogen Idec, Sanofi-Aventis, Teva Pharmaceutical Industries Ltd and Novartis as consultant and speaker. Dr. Molinuevo Guix has nothing to disclose. Dr. Dalmau has received personal compensation in an editorial capacity for UpToDate. Dr. Dalmau has received royalty payments from Athena Diagnostics. Dr. Dalmau has received research support from Euroimmun. Dr. Graus has nothing to disclose.Wednesday, March 20 2013, 7:30 am-12:00 pm