National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière, University Pierre et Marie Curie, Paris VI, Paris, France.
Curr Opin Neurol. 2013 Oct;26(5):503-9. doi: 10.1097/WCO.0b013e328364caca.
Multifocal motor neuropathy (MMN) remains a difficult issue for neurologists, as its clinical and electrophysiological presentation may be atypical, and because no alternative treatment to periodic immunoglobulin infusions has been assessed in its long-term management. This review intends to summarize the most recent advances in the diagnosis and treatment of MMN.
Recent reports have focused on atypical onset and unusual clinical presentation. Several sophisticated electrophysiological techniques, as triple stimulation, may help establish the presence of conduction blocks, as well as MRI findings. A recent immunological study focused on the detection of serum IgM binding to NS6S heparin disaccharide. In another research article, it was proposed that the use of combinatorial glycoarray or ELISA may increase the diagnostic sensitivity of antiglycolipid antibody testing. Subcutaneous immunoglobulin may represent an interesting alternative option to intravenous immunoglobulin. Lastly, recently reported open-label clinical trials with complement inhibitors and anti-CD20 monoclonal antibody may constitute a first step for further developments.
Diagnostic criteria for MMN are well established, but challenging situations still occur. Progresses in neurophysiologic and other laboratory tests may help in clarifying doubtful diagnoses. Current research into the pathophysiology of MMN is required to determine the future treatment targets.
多灶性运动神经病(MMN)仍然是神经科医生面临的难题,因为其临床表现和电生理表现可能不典型,并且在其长期管理中尚未评估过除周期性免疫球蛋白输注以外的替代治疗方法。本篇综述旨在总结 MMN 的最新诊断和治疗进展。
最近的报告重点关注不典型的发病和不常见的临床表现。一些复杂的电生理技术,如三重刺激,可能有助于确定存在传导阻滞以及 MRI 发现。最近的一项免疫学研究集中在检测血清 IgM 与 NS6S 肝素二糖的结合。在另一项研究文章中,有人提出使用组合糖基阵列或 ELISA 可能会增加抗神经节苷脂抗体检测的诊断灵敏度。皮下免疫球蛋白可能是静脉免疫球蛋白的一种有趣替代选择。最后,最近报道的补体抑制剂和抗 CD20 单克隆抗体的开放性临床试验可能是进一步发展的第一步。
MMN 的诊断标准已经确立,但仍存在具有挑战性的情况。神经生理和其他实验室检测的进展可能有助于澄清可疑的诊断。需要对 MMN 的病理生理学进行研究,以确定未来的治疗靶点。