Bridel Claire, Horvath Judit, Kurian Mary, Truffert André, Steck Andreas, Lalive Patrice H
Department of Clinical Neurosciences, Division of Neurology, Geneva University Hospital, Geneva, Switzerland.
J Peripher Nerv Syst. 2014 Jun;19(2):180-2. doi: 10.1111/jns5.12069.
Multifocal motor neuropathy (MMN) and anti-myelin-associated glycoprotein (anti-MAG)-associated neuropathy are clinically and electrophysiologically distinct entities. We describe a patient with characteristic features of both neuropathies, raising the possibility of an overlap syndrome. A 49-year-old patient reported a history of slowly progressive predominantly distal tetraparesis, with mild sensory deficits. Nerve conduction studies demonstrated persistent motor conduction blocks outside compression sites, typical of MMN. Laboratory findings revealed persistently high titers of anti-MAG immunoglobulin Mλ (IgMλ) paraprotein in the context of a monoclonal gammapathy of unknown significance. Skin biopsy of distal lower limb revealed IgM positive terminal nerve perineurium deposits. This case suggests that the distinction between subtypes of chronic inflammatory neuropathies may not be as clear as initially thought, and that the pattern of pathogenicity of anti-MAG antibodies may vary.
多灶性运动神经病（MMN）和抗髓鞘相关糖蛋白（抗-MAG）相关神经病在临床和电生理方面是不同的实体。我们描述了一名具有这两种神经病特征的患者，提示存在重叠综合征的可能性。一名49岁患者报告有缓慢进展的以远端为主的四肢轻瘫病史，伴有轻度感觉障碍。神经传导研究显示在非压迫部位存在持续性运动传导阻滞，这是MMN的典型表现。实验室检查结果显示，在意义未明的单克隆丙种球蛋白病背景下，抗-MAG免疫球蛋白Mλ（IgMλ）副蛋白水平持续升高。下肢远端皮肤活检显示IgM阳性的终末神经束膜沉积。该病例表明，慢性炎症性神经病亚型之间的区别可能不像最初认为的那么清晰，并且抗-MAG抗体的致病模式可能有所不同。
