Kusunoki S
Department of Neurology, School of Medicine, University of Tokyo.
Rinsho Shinkeigaku. 1999 Jan;39(1):93-5.
Antiganglioside antibodies are frequently detected in sera from patients with autoimmune neuropathies, such as Guillain-Barré syndrome, Miller Fisher syndrome, IgM paraproteinemic neuropathy, chronic inflammatory demyelinating polyneuropathy, and multifocal motor neuropathy. In the acute phase sera from GBS patients, antiganglioside antibodies are detected in 60-70%. Ganglioside antigens recognized by serum antibodies are varied from case to case. IgG antibody against GQ1b ganglioside is specifically raised in sera from patients with Miller Fisher syndrome and Guillain-Barré syndrome with ophthalmoplegia. That antibody may bind to the paranodal myelin of oculomotor, trochlear and abducens nerves, where GQ1b ganglioside is specifically localized, to cause ophthalmoplegia. IgM M-protein which recognizes the disialosyl residue of GD1b is specifically associated with sensory ataxic neuropathy. The IgM M-protein may bind to the primary sensory neurons, where GD1b ganglioside is localized, to cause sensory disturbance. After we confirmed the localization of GD1b in the rabbit primary sensory neurons, we sensitized rabbits with GD1b and induced sensory ataxic neuropathy in them. This is the first established animal model of autoimmune neuropathy induced by sensitization with ganglioside. Some antiganglioside antibodies may determine the clinical phenotype of neuropathy by binding specifically to the ganglioside antigens which have unique localization.
抗神经节苷脂抗体经常在自身免疫性神经病患者的血清中被检测到,如吉兰 - 巴雷综合征、米勒 - 费雪综合征、IgM副蛋白血症性神经病、慢性炎症性脱髓鞘性多发性神经病和多灶性运动神经病。在吉兰 - 巴雷综合征患者的急性期血清中,60 - 70%可检测到抗神经节苷脂抗体。血清抗体识别的神经节苷脂抗原因病例而异。抗GQ1b神经节苷脂的IgG抗体在米勒 - 费雪综合征和伴有眼肌麻痹的吉兰 - 巴雷综合征患者的血清中特异性升高。该抗体可能与动眼神经、滑车神经和展神经的结旁髓鞘结合,而GQ1b神经节苷脂特异性定位于此处,从而导致眼肌麻痹。识别GD1b双唾液酸残基的IgM M蛋白与感觉性共济失调性神经病特异性相关。IgM M蛋白可能与GD1b神经节苷脂所在的初级感觉神经元结合,导致感觉障碍。在我们确定了GD1b在兔初级感觉神经元中的定位后,我们用GD1b使兔致敏,并在它们身上诱发了感觉性共济失调性神经病。这是首个通过神经节苷脂致敏建立的自身免疫性神经病动物模型。一些抗神经节苷脂抗体可能通过特异性结合具有独特定位的神经节苷脂抗原,来决定神经病的临床表型。