Pihan M, Decaux O, Marcorelles P, Bahon-Riedinger I, Lemercier S, Gainche-Salmon A, Doncker A-V
Service des explorations fonctionnelles neurologiques, hôpital Pontchaillou, CHU de Rennes, rue Henri-Le-Guilloux, 35033 Rennes cedex 09, France.
Rev Med Interne. 2012 Dec;33(12):686-92. doi: 10.1016/j.revmed.2012.05.009. Epub 2012 Jun 20.
Monoclonal IgM anti-MAG associated peripheral neuropathies are part of demyelinating dysimmune peripheral neuropathies. The hematological disease probably does not influence the outcome of the neuropathy. Neuropathies associated with IgM anti-MAG antibodies are predominantly sensory and distal polyneuropathies associated with ataxia, unsteadiness and tremor. The neurophysiological features include a symmetric sensorimotor demyelinating neuropathy with more slowing of conduction in the distal than in the proximal nerve segments, a length-dependence, and a variable degree of denervation. High titers of IgM anti-MAG antibodies confirm the diagnosis. The natural history is mostly slow with mild to moderate functional impairment. However, some patients have a faster evolution associated with a more severe handicap. Immunotherapies studies have failed to demonstrate significant efficacy of these treatments. Furthermore, severe adverse effects are not uncommon with any of these therapies. Thus, the risk of possible severe adverse effects must be balanced against any possible benefits. More research is needed to improve the management of anti-MAG neuropathies: research on treatment, on prognostic factors, and development of specific assessment scales adapted to the particularities of anti-MAG neuropathies.
单克隆IgM抗MAG相关周围神经病是脱髓鞘性免疫性周围神经病的一部分。血液系统疾病可能不影响周围神经病的预后。与IgM抗MAG抗体相关的周围神经病主要是感觉性和远端多发性神经病，伴有共济失调、步态不稳和震颤。神经生理学特征包括对称性感觉运动性脱髓鞘性周围神经病，远端神经节段的传导速度比近端神经节段更慢，具有长度依赖性，以及不同程度的失神经。高滴度的IgM抗MAG抗体可确诊。其自然病程大多缓慢，伴有轻至中度功能障碍。然而，一些患者病情进展较快，伴有更严重的残疾。免疫治疗研究未能证明这些治疗有显著疗效。此外，这些治疗中任何一种出现严重不良反应都并不罕见。因此，必须权衡可能的严重不良反应风险与任何可能的益处。需要更多研究来改善抗MAG周围神经病的管理：关于治疗、预后因素的研究，以及开发适合抗MAG周围神经病特点的特定评估量表。
