Ito H, Saito T
Department of Neurology, Kitasato University East Hospital.
Rinsho Shinkeigaku. 1997 Dec;37(12):1111-2.
Most of acquired demyelinating neuropathies are caused by immune-mediated processes. Therefore, we can treat these neuropathies to control underlying immune abnormality. We review the recent therapies and show possible strategies in the future. (1) Recent therapies Guillain-Barré syndrome should be treated quickly and intensively in the first week of the disease with plasmapheresis (PP). Intravenous immunoglobulin (IVIG) is as effective as PP. IVIG is under phase 3 clinical trial in Japan. Chronic inflammatory demyelinating polyneuropathy (CIDP) is treated with corticosteroids, PP or IVIG. Corticosteroids or PP is the first choice. For severe or resistant cases, combined therapy or immunosuppressants, such as cyclophosphamide pulse therapy, should be considered. Polyneuropathy with IgM-MGUS (monoclonal gammopathies of undetermined significance) is treated with PP, immunosuppressants or these combinations to decrease serum IgM, especially for cases with anti-MAG antibody, because the autoantibody induces demyelination by itself. (2) Future strategies Immunopathogenesis may differ among cases with Guillain-Barré syndrome or CIDP. The best therapy will be chosen individually, according to appropriate immunologic tests. In CIDP, immunomodulation therapies, that are similar ones in multiple sclerosis, will be considered to maintain improvement.
大多数获得性脱髓鞘性神经病由免疫介导过程引起。因此,我们可以通过治疗这些神经病来控制潜在的免疫异常。我们回顾了近期的治疗方法并展示了未来可能的策略。(1)近期治疗方法 吉兰 - 巴雷综合征在疾病的第一周应迅速且强化地进行血浆置换(PP)治疗。静脉注射免疫球蛋白(IVIG)与PP效果相同。IVIG在日本正处于3期临床试验阶段。慢性炎症性脱髓鞘性多发性神经病(CIDP)采用皮质类固醇、PP或IVIG治疗。皮质类固醇或PP是首选。对于严重或难治性病例,应考虑联合治疗或使用免疫抑制剂,如环磷酰胺脉冲疗法。伴有IgM - MGUS(意义未明的单克隆丙种球蛋白病)的多发性神经病采用PP、免疫抑制剂或这些方法的联合治疗以降低血清IgM,特别是对于抗MAG抗体阳性的病例,因为自身抗体可自行诱导脱髓鞘。(2)未来策略 吉兰 - 巴雷综合征或CIDP病例的免疫发病机制可能不同。根据适当的免疫检测结果,将为个体选择最佳治疗方法。在CIDP中,将考虑采用与多发性硬化症中类似的免疫调节疗法来维持病情改善。