Matsushita Takuya, Masaki Katsuhisa, Suzuki Satoru, Matsuoka Takeshi, Yonekawa Tomomi, Wu Xiao-Mu, Tabira Takeshi, Iwaki Toru, Kira Jun-Ichi
Department of Neurommunology, Graduate School of Medical Sciences, Kyushu University.
Rinsho Shinkeigaku. 2011 Nov;51(11):898-900. doi: 10.5692/clinicalneurol.51.898.
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) while neuromyelitis optica (NMO) is an inflammatory disease of the CNS that selectively affects the optic nerves and spinal cord. An antibody for aquaporin-4 (AQP4), which is a water channel located in astrocyte foot process, is specifically positive for NMO and antibody and complement dependent astrocytic damage is thought to be a main cause of NMO. Baló's disease is characterized by alternating rings of demyelination and preserved myelin. We pathologically compared the astrocytic changes among autopsied cases with these CNS demyelinating diseases. NMO, MS and Baló's disease shared with reduced AQP4 immunoreactivity independent of antibodies and complements. The pathological finding was accompanied with a reduced immunoreactivity of connexin 43 and perivascular lymphocytic cuffing predominantly composed by T cells. The loss of astrocytic proteins such as AQP4 and connexin 43 preceded the loss of myelin proteins in some lesions. These features suggest astrocyte damages resulting in the loss of connexin 43 cause demyelination through the impairment of interaction between astrocytes and oligodendrocytes and the pathomechanism involves a T cell reaction.
多发性硬化症（MS）是一种中枢神经系统（CNS）的脱髓鞘疾病，而视神经脊髓炎（NMO）是一种中枢神经系统的炎症性疾病，它选择性地影响视神经和脊髓。水通道蛋白4（AQP4）的抗体，AQP4是一种位于星形胶质细胞足突的水通道，NMO患者该抗体呈特异性阳性，且抗体和补体依赖性星形胶质细胞损伤被认为是NMO的主要病因。巴洛病的特征是脱髓鞘环与保留的髓鞘交替出现。我们对这些中枢神经系统脱髓鞘疾病尸检病例的星形胶质细胞变化进行了病理比较。NMO、MS和巴洛病均存在与抗体和补体无关的AQP4免疫反应性降低。这一病理发现伴随着连接蛋白43免疫反应性降低以及主要由T细胞组成的血管周围淋巴细胞套袖现象。在一些病变中，星形胶质细胞蛋白如AQP4和连接蛋白43的丢失先于髓鞘蛋白的丢失。这些特征表明，星形胶质细胞损伤导致连接蛋白43丢失，通过损害星形胶质细胞与少突胶质细胞之间的相互作用而引起脱髓鞘，并且发病机制涉及T细胞反应。
