Etemadifar Masoud, Abtahi Seyed-Hossein, Dehghani Alireza, Abtahi Mohammad-Ali, Akbari Mojtaba, Tabrizi Nasim, Goodarzi Tannaz
Medical School, Isfahan University of Medical Sciences, Isfahan.
Case Rep Neurol. 2011 Sep;3(3):268-73. doi: 10.1159/000334128. Epub 2011 Oct 21.
Neuromyelitis optica (NMO) is an inflammatory demyelinating disorder of the central nervous system that has been thought to be a severe subtype of multiple sclerosis for a long time. The discovery of aquaporin-4 (AQP4) antibody as a highly specific marker responsible for the pathogenesis of NMO, not only has made a revolutionary pace in establishing a serologic distinction between the two diseases, but it has also classified NMO as an antibody-mediated disorder. Similarly, myasthenia gravis (MG) is a well-known antibody-mediated disorder. In this report, we describe the case of a middle-aged female patient who experienced definite MG with an unclear clinical picture of chronic demyelinating disease that initially reflected the diagnosis of MS, but further imaging and paraclinical workup (e.g. positive AQP4 antibody test) revealed NMO. The coexistence of NMO and MG is previously described. However, this is the first case with NMO symptoms preceding the onset of MG. Of note, the development of MG occurred after a 2-year period of interferon β-1b (IFN β-1b) administration. This calls the question to mind of whether in our case MG is induced by the administration of interferon, instead of an original pathogenic link between MG and NMO. In other words, immunomodulatory treatments can slip the immunity towards T-helper II predominant pathways that can trigger MG. However, if we assume that such an explanation (i.e. increased susceptibility to autoantibody-mediated disorders) is true, our case can be considered the first case of NMO who developed MG following IFN β-1b treatment.
视神经脊髓炎(NMO)是一种中枢神经系统炎性脱髓鞘疾病,长期以来一直被认为是多发性硬化症的一种严重亚型。水通道蛋白4(AQP4)抗体作为NMO发病机制的高度特异性标志物被发现,这不仅在区分这两种疾病的血清学方面取得了革命性进展,还将NMO归类为抗体介导的疾病。同样,重症肌无力(MG)是一种众所周知的抗体介导的疾病。在本报告中,我们描述了一名中年女性患者的病例,该患者患有明确的MG,伴有慢性脱髓鞘疾病的不明确临床表现,最初提示为MS诊断,但进一步的影像学和辅助检查(如AQP4抗体检测阳性)显示为NMO。NMO和MG共存的情况此前已有描述。然而,这是首例NMO症状先于MG发作的病例。值得注意的是,MG的发生是在使用干扰素β-1b(IFN β-1b)两年后。这让人不禁思考,在我们的病例中,MG是否是由干扰素的使用诱发的,而非MG与NMO之间原本就存在致病联系。换句话说,免疫调节治疗可能会使免疫反应偏向以辅助性T细胞II为主导的途径,从而引发MG。然而,如果我们假设这种解释(即对自身抗体介导疾病的易感性增加)是正确的,那么我们的病例可被视为首例在IFN β-1b治疗后发生MG的NMO病例。