自己抗体による末梢神経疾患の発症機序

鞘糖脂是神经细胞的组成成分，可作为重要抗原参与体液和细胞免疫反应。糖脂抗体已被证明存在于各种免疫介导的神经系统疾病患者中。在这项研究中，我们旨在阐明这些自身抗体的意义和独特的糖脂家族的作用，以及可能在这些疾病的免疫发病机制下。在患有这种疾病的患者中检测到高抗神经节苷脂抗体滴度。我们已经证明神经节苷类与先前感染的细菌具有共同的碳水化合物表位，是格林-巴利综合征和费舍尔病的潜在靶抗原。在许多其他周围神经病变中，各种次要神经节苷也可以成为抗体的靶标。在慢性炎症性脱髓鞘性多神经病变患者中，经常检测到高滴度的抗硫酸葡萄糖醛基糖脂。该抗体的高滴度与m蛋白的存在和血清IgM值无关。我们认为IgM抗MAG/SGPG抗体的多发性神经病形成了CIDP的一个亚群。梅尼埃病患者血清抗糖脂抗体活性显示抗唾液酰副叶皂苷抗体滴度阳性。通过对听神经瘤样品的化学分析，检测了新乳酸系列糖脂。这些结果表明抗糖脂抗体在这些疾病的免疫发病机制中起着重要的作用。

Glycosphingolipids are constituents of nerve cells that may serve as important antigens prticipating in humoral and cellular immune responses. Antibodies to glycolipids have been shown to be present in patients with variety of immune-mediated neurological disorders. In this research, we aimed to elucidate the significance of these autoantibodies and the roles of unique family of glycolipids as well as mechanisms that may under lie the immunopathogenesis of these disorders.High anti-ganglioside antibodies titers are detected in patients suffering this disorders. We have demonstrated that gangliosides, which possess a common carbohydrate epitope with bacteria of the preceding infection are potential target antigens for the Guillain-Barre syndrome and Fisher disease. Various minor gangliosides can also be the target of antibodies in many other peripheral neuropathies. In patients with chronic inflammatory demyelinating polyneuropathy, high titer anti-sulfated glucuronosyl glycolipids were frequently detected. This high titer of antibody was not related with the presence of M-protein nor serum IgM value. We propose that polyneuropathy with IgM anti MAG/SGPG antibody form a subgroup of CIDP.Serum anti-glycolipids antibody activities in patients with Meniere's disease showed positive anti-sialylparagloboside antibody titers. by the chemical analysis of acoustic neurinoma samples, neolacto-series glycolipids were detected. These results indicate the significant roles of the anti-glycolipid antibody for the immunopathogenesis of these disorders.