人免疫球蛋白输注治疗多灶性运动神经病变。

Degenerative Neurological and Neuromuscular Disease Pub Date : 2015-12-22 eCollection Date: 2016-01-01 DOI:10.2147/DNND.S96258
Elizabeth Jovanovich, Chafic Karam
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引用次数: 4

摘要

多灶性运动神经病(MMN)是一种使人衰弱和罕见的疾病,引起严重的虚弱,很少或没有感觉症状。传导阻滞常见于电诊断试验。一种免疫介导的病理被怀疑,虽然确切的潜在病理生理尚未阐明。抗gm1神经节苷脂IgM抗体的存在,加上对静脉注射和皮下免疫球蛋白的良好反应,支持补体介导的机制,导致神经组织的破坏,可能倾向于Ranvier淋巴结。目前,高剂量免疫球蛋白是MMN患者唯一被证实有效的治疗方法。不幸的是,随着时间的推移,许多患者对免疫球蛋白的反应性降低,需要更高和更频繁的剂量。在这篇综述中,我们将重点讨论静脉注射和皮下注射免疫球蛋白治疗MMN的药理学、疗效、安全性和耐受性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Human immune globulin infusion in the management of multifocal motor neuropathy.

Multifocal motor neuropathy (MMN) is a debilitating and rare disease causing profound weakness with minimal to no sensory symptoms. Conduction block is frequently seen on electrodiagnostic testing. An immune-mediated pathology is suspected though the exact underlying pathophysiology has yet to be elucidated. The presence of anti-GM1 ganglioside IgM antibodies coupled with favorable response to intravenous and subcutaneous immunoglobulins supports a complement-mediated mechanism which leads to destruction of nerve tissue with probable predilection to the nodes of Ranvier. High-dose immunoglobulin currently is the only treatment with proven efficacy for MMN patients. Unfortunately, many patients experience decreased responsiveness to immunoglobulins over time, requiring higher and more frequent dosing. In this review, we will focus on the pharmacology, efficacy, safety, and tolerability of intravenous and subcutaneous immune globulin infusion for treatment of MMN.

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