Neuromyelitis Optica and Asian Phenotype of Multiple Sclerosis
Neuromyelitis Optica and Asian Phenotype of Multiple Sclerosis
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DOI：
10.1196/annals.1444.002
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发表时间：
2008-01-01
期刊：
影响因子：
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通讯作者：
Kira, Jun-ichi
中科院分区：
文献类型：
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作者：
Kira, Jun-ichi
多发性硬化（MS）是中枢神经系统（CNS）的脱髓鞘疾病，而视神经肌炎（NMO）是选择性影响视神经和脊髓的CNS炎性疾病。MS的病理标志是明显分界的脱髓鞘斑块，轴突相对保存，而在NMO中轴突和髓鞘都参与，导致坏死性空洞。NMO的疾病分类学地位长期以来一直是一个有争议的问题。在亚洲人中，MS是罕见的;然而，当它出现时，选择性但严重的视神经和脊髓受累是特征。这种形式，称为opticosporinal MS（OSMS），具有类似的功能，复发形式的NMO在西方人群。最近发现的针对NMO的特异性免疫球蛋白G（IgG），命名为NMO-IgG，表明NMO是一种独特的疾病实体，其病因与MS的病因根本不同。因为NMO-IgG已被报道存在于约50%-60%的具有纵向广泛脊髓病变（LESCL）的OSMS患者中，所以亚洲人的OSMS已被认为与NMO是相同的实体。大约一半的抗水通道蛋白4（AQP 4）抗体的患者表现出符合Barkhof标准的脑病变，而没有抗AQP 4抗体的OSMS患者表现出显着较少的脑病变。这些发现表明，亚洲人LESCL的机制是异质性的，既与抗AQP 4抗体相关，也与抗AQP 4抗体无关，并且抗AQP 4抗体的疾病状况与亚洲人的OSMS不完全重叠。本文就OSMS和抗AQP 4自身免疫综合征的可能机制进行综述。
Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS), whereas neuromyclitis optica (NMO) is an inflammatory disease of the CNS selectively affecting the optic nerves and spinal cord. The pathological hallmark in MS is sharply demarcated demyelinating plaque with axons relatively preserved, whereas in NMO both axons and myelin are involved, resulting in necrotic cavitation. The nosological position of NMO has long been a matter of debate. In Asians, MS is rare; however, when it appears, the selective but severe involvement of the optic nerves and spinal cord is characteristic. This form, termed opticospinal MS (OSMS), has similar features to those of the relapsing form of NMO in Western populations. Recent discovery of a specific immunoglobulin G (IgG) against NMO, designated NMO-IgG, suggests that NMO is a distinct disease entity with a fundamentally different etiology from that of MS. Because NMO-IgG has been reported to be present in about 50%-60% of OSMS patients with longitudinally extensive spinal cord lesions (LESCLs), OSMS in Asians has been suggested to be the same entity as NMO. About half of the patients with the anti-aquaporin 4 (AQP4) antibody demonstrate brain lesions fulfilling the Barkhof criteria, whereas OSMS patients without the anti-AQP4 antibody show significantly fewer brain lesions. These findings indicate that the mechanism of LESCLs in Asians is heterogeneous, both related and unrelated to anti-AQP4 antibody, and that the disease condition with anti-AQP4 antibody does not completely overlap OSMS in Asians. This review discusses possible mechanisms for OSMS and anti-AQP4 autoimmune syndrome of the CNS.