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Multifocal motor neuropathy: controversies and priorities.
Journal of Neurology, Neurosurgery, and Psychiatry ( IF 8.7 ) Pub Date : 2019-09-11 , DOI: 10.1136/jnnp-2019-321532
Wei Zhen Yeh 1, 2 , P James Dyck 3 , Leonard H van den Berg 4 , Matthew C Kiernan 5, 6 , Bruce V Taylor 7, 8
Affiliation  

Despite 30 years of research there are still significant unknowns and controversies associated with multifocal motor neuropathy (MMN) including disease pathophysiology, diagnostic criteria and treatment. Foremost relates to the underlying pathophysiology, specifically whether MMN represents an axonal or demyelinating neuropathy and whether the underlying pathophysiology is focused at the node of Ranvier. In turn, this discussion promotes consideration of therapeutic approaches, an issue that becomes more directed in this evolving era of precision medicine. It is generally accepted that MMN represents a chronic progressive immune-mediated motor neuropathy clinically characterised by progressive asymmetric weakness and electrophysiologically by partial motor conduction block. Anti-GM1 IgM antibodies are identified in at least 40% of patients. There have been recent developments in the use of neuromuscular ultrasound and MRI to aid in diagnosing MMN and in further elucidation of its pathophysiological mechanisms. The present Review will critically analyse the knowledge accumulated about MMN over the past 30 years, culminating in a state-of-the-art approach to therapy.

中文翻译:

多灶性运动神经病:争议和优先事项。

尽管进行了30年的研究,但仍存在与多灶性运动神经病(MMN)相关的重大未知和争议,包括疾病的病理生理学,诊断标准和治疗方法。首先涉及基础病理生理,特别是MMN是代表轴突还是脱髓鞘性神经病,以及基础病理生理是否集中在Ranvier的结节上。反过来,这种讨论促进了对治疗方法的考虑,在这个不断发展的精密医学时代,这个问题变得更加直接。人们普遍认为,MMN代表一种临床上以进行性不对称无力为特征,在电生理上以部分运动传导阻滞为特征的慢性进行性免疫介导的运动神经病。在至少40%的患者中鉴定出了抗GM1 IgM抗体。在使用神经肌肉超声和MRI来协助诊断MMN以及进一步阐明其病理生理机制方面已有新的发展。本综述将对过去30年中积累的有关MMN的知识进行批判性分析,最终形成最先进的治疗方法。
更新日期:2020-01-10
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