Doppler Kathrin, Appeltshauser Luise, Krämer Heidrun H, Ng Judy King Man, Meinl Edgar, Villmann Carmen, Brophy Peter, Dib-Hajj Sulayman D, Waxman Stephen G, Weishaupt Andreas, Sommer Claudia
Department of Neurology, University Hospital Würzburg, Würzburg, Germany.
Department of Neurology, University Hospital Gießen, Gießen, Germany.
PLoS One. 2015 Jul 28;10(7):e0134274. doi: 10.1371/journal.pone.0134274. eCollection 2015.
Multifocal motor neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflammatory demyelinating polyneuropathy. Recently, auto-antibodies against neurofascin-186 and gliomedin were described in more than 60% of patients with multifocal motor neuropathy. In the current study, we aimed to validate this finding, using a combination of different assays for auto-antibody detection. In addition we intended to detect further auto-antibodies against paranodal proteins, specifically contactin-1 and neurofascin-155 in multifocal motor neuropathy patients' sera. We analyzed sera of 33 patients with well-characterized multifocal motor neuropathy for IgM or IgG anti-contactin-1, anti-neurofascin-155 or -186 antibodies using enzyme-linked immunosorbent assay, binding assays with transfected human embryonic kidney 293 cells and murine teased fibers. We did not detect any IgM or IgG auto-antibodies against contactin-1, neurofascin-155 or -186 in any of our multifocal motor neuropathy patients. We conclude that auto-antibodies against contactin-1, neurofascin-155 and -186 do not play a relevant role in the pathogenesis in this cohort with multifocal motor neuropathy.
多灶性运动神经病是一种免疫介导的疾病，表现为多灶性肌无力和传导阻滞。约50%的患者可检测到抗神经节苷脂GM1的IgM自身抗体。在慢性炎症性脱髓鞘性多发性神经病患者亚组中，已检测到抗结旁蛋白contactin-1和神经束膜蛋白-155以及结蛋白神经束膜蛋白-186的自身抗体。最近，在超过60%的多灶性运动神经病患者中发现了抗神经束膜蛋白-186和神经胶质瘤素的自身抗体。在本研究中，我们旨在通过结合不同的自身抗体检测方法来验证这一发现。此外，我们还打算在多灶性运动神经病患者血清中检测更多针对结旁蛋白的自身抗体，特别是contactin-1和神经束膜蛋白-155。我们使用酶联免疫吸附测定、转染人胚肾293细胞结合测定和鼠类 teased纤维分析了33例特征明确的多灶性运动神经病患者血清中的IgM或IgG抗contactin-1、抗神经束膜蛋白-155或-186抗体。在我们所有的多灶性运动神经病患者中，均未检测到任何针对contactin-1、神经束膜蛋白-155或-186的IgM或IgG自身抗体。我们得出结论，针对contactin-1、神经束膜蛋白-155和-186的自身抗体在该多灶性运动神经病队列的发病机制中不发挥相关作用。
