Dyck Peter J, Taylor Bruce V, Davies Jenny L, Mauermann Michelle L, Litchy William J, Klein Christopher J, Dyck P James B
Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.
Menzies Research Institute, Tasmania, Australia.
Muscle Nerve. 2015 Oct;52(4):488-97. doi: 10.1002/mus.24707. Epub 2015 Aug 13.
Intravenous immunoglobulin [IVIg], plasma exchange [PE], and corticosteroids are efficacious treatment in chronic inflammatory demyelinating polyneuropathy [CIDP]. IVIg is effective in multifocal motor neuropathy [MMN]. NIS, NIS-weakness, sum scores of raw amplitudes of motor fiber (CMAPs) amplitudes, and Dyck/Rankin score provided reliable measures to detect and scale abnormality and reflect change; they are therefore ideal for office management of response-based immunotherapy (R-IRx) of CIDP. Using efficacious R-IRx, a large early and late therapeutic response (≥ one-fourth were in remission or had recovered) was demonstrated in CIDP. In MMN only an early improvement with late non-significant worsening was observed. The difference in immunotherapy response supports a fundamental difference between CIDP (immune attack on Schwann cells and myelin) and MMN (attack on nodes of Ranvier and axons).
静脉注射免疫球蛋白（IVIg）、血浆置换（PE）和皮质类固醇是慢性炎症性脱髓鞘性多发性神经病（CIDP）的有效治疗方法。IVIg对多灶性运动神经病（MMN）有效。神经指数（NIS）、NIS-虚弱评分、运动纤维复合肌肉动作电位（CMAPs）波幅原始波幅总和评分以及Dyck/Rankin评分提供了检测和量化异常以及反映变化的可靠指标；因此，它们是CIDP基于反应的免疫治疗（R-IRx）门诊管理的理想选择。使用有效的R-IRx，在CIDP中显示出较大的早期和晚期治疗反应（≥四分之一达到缓解或恢复）。在MMN中，仅观察到早期改善，晚期无明显恶化。免疫治疗反应的差异支持了CIDP（对施万细胞和髓鞘的免疫攻击）和MMN（对郎飞结和轴突的攻击）之间的根本差异。
