Kawachi Izumi, Nishizawa Masatoyo
Department of Neurology, Clinical Neuroscience Branch, Brain Research Institute, Niigata University.
Brain Nerve. 2015 Apr;67(4):497-504. doi: 10.11477/mf.1416200166.
Multiple sclerosis (MS) and neuromyelitis optica (NMO) are the two main autoimmune and inflammatory diseases of the central nervous system. MS is considered an 'oligodendrocytopathy' with undefined autoantibodies, whereas NMO is considered an 'astrocytopathy' with aquaporin-4 antibodies. Both have long been considered autoimmune diseases that primarily affect the white matter (WM). However, recent interest in gray matter (GM) pathology is emerging. Pathological evaluations reveal that substantial cortical demyelination is prominent at all stages or courses of MS. In addition, the neurodegeneration in MS is present not only in GM demyelination but also in normal-appearing GM. NMO involves cortical neurodegeneration and unique dynamics of astrocytes; however, no cortical demyelination is seen. Thus, MS and NMO are distinct diseases with unique pathological features in the brain WM and GM. Although the cause of GM and WM lesions in MS and NMO has not been fully determined, improved knowledge about the pathomechanisms in affected brains is desired. This would result in a more targeted pharmacotherapeutic approach to these diseases.
多发性硬化症(MS)和视神经脊髓炎(NMO)是中枢神经系统的两种主要自身免疫性和炎性疾病。MS被认为是一种伴有未明确自身抗体的“少突胶质细胞病”,而NMO被认为是一种伴有水通道蛋白4抗体的“星形胶质细胞病”。长期以来,两者都被视为主要影响白质(WM)的自身免疫性疾病。然而,最近对灰质(GM)病理学的关注正在兴起。病理评估显示,在MS的所有阶段或病程中,大量皮质脱髓鞘都很突出。此外,MS中的神经退行性变不仅存在于GM脱髓鞘中,也存在于外观正常的GM中。NMO涉及皮质神经退行性变和星形胶质细胞的独特动态变化;然而,未见皮质脱髓鞘。因此,MS和NMO是在脑WM和GM中具有独特病理特征的不同疾病。尽管MS和NMO中GM和WM病变的原因尚未完全确定,但仍需要更好地了解受影响大脑中的发病机制。这将导致对这些疾病采取更有针对性的药物治疗方法。