Departments of Neurosciences, University Hospital Modena, Italy.
Int J Neurosci. 2012 Jul;122(7):395-400. doi: 10.3109/00207454.2012.660587. Epub 2012 Feb 27.
Multifocal motor neuropathy (MMN) shows stepwise progression over decades. The multifocal weakness usually remains asymmetric, confined to distal limb muscles, while sparing cranial, phrenic, and sensory nerves. One electrophysiological hallmark is partial motor conduction block (CB) at sites not exposed to compression; whether CB is an essential feature remains debatable. High titer of anti-GM1 antibodies is found with figures usually between 40% and 50% of patients. Intravenous immuneglobulin (IVIg) is effective in almost 80%, but plasmapheresis and steroids are not. The condition is reported as lethal exceptionally, mimicking motor neuron diseases (MND). We have studied two patients who failed to respond to treatment and who died with respiratory failure; one of the two had high titer of IgM antibody to the ganglioside GM1. Our cases confirm that great attention should be paid in order to define the borderland between MMN and MND and the entity of their clinical and electrophysiological overlaps.
多灶性运动神经病 (MMN) 在几十年中呈阶梯式进展。多灶性肌无力通常仍然不对称，局限于远端肢体肌肉，而颅神经、膈神经和感觉神经不受累。一个电生理学标志是在未受压部位出现部分运动传导阻滞 (CB)；CB 是否是一个基本特征仍存在争议。高滴度的抗 GM1 抗体通常在 40%至 50%的患者中发现。静脉注射免疫球蛋白 (IVIg) 在近 80%的患者中有效，但血浆置换和类固醇无效。这种情况极少见致命，类似于运动神经元病 (MND)。我们研究了两名对治疗无反应并死于呼吸衰竭的患者；其中一名患者的 GM1 神经节苷脂 IgM 抗体滴度较高。我们的病例证实，为了明确 MMN 和 MND 之间的界限以及它们在临床和电生理上的重叠实体，应给予高度重视。
