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慢性炎症性脱髓鞘性多发性神经病和多灶性运动神经病的门诊免疫治疗

Office immunotherapy in chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy.

作者信息

Dyck Peter J, Taylor Bruce V, Davies Jenny L, Mauermann Michelle L, Litchy William J, Klein Christopher J, Dyck P James B

机构信息

Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, Minnesota, 55905, USA.

Menzies Research Institute, Tasmania, Australia.

出版信息

Muscle Nerve. 2015 Oct;52(4):488-97. doi: 10.1002/mus.24707. Epub 2015 Aug 13.

Abstract

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).

摘要

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。

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