Baron M, Lozeron P, Harel S, Bengoufa D, Vignon M, Asli B, Malphettes M, Parquet N, Brignier A, Fermand J P, Kubis N, Arnulf Bertrand
Département d'Immuno-Hématologie, APHP, Hôpital Saint Louis, Paris, France.
Service de Physiologie Clinique-Explorations Fonctionnelles, AP-HP, Hôpital Lariboisière, INSERM UMR965, Université Paris Diderot, Sorbonne Paris Cité, Paris, France.
J Neurol. 2017 Jun;264(6):1132-1135. doi: 10.1007/s00415-017-8502-3. Epub 2017 May 8.
Monoclonal IgM anti-myelin-associated glycoprotein (MAG) antibody-related peripheral neuropathy (anti-MAG neuropathy) is predominantly a demyelinating sensory neuropathy with ataxia and distal paresthesia. The clinical course of anti-MAG neuropathy is usually slowly progressive making difficult the identification of clear criteria to start a specific treatment. Although no consensus treatment is yet available, a rituximab-based regimen targeting the B-cell clone producing the monoclonal IgM may be proposed, alone or in combination with alkylating agents or purine analogs. However, in some rare cases, an acute and severe neurological deterioration can occur in few days leading to a rapid loss of autonomy. In these cases, a treatment rapidly removing the monoclonal IgM from the circulation might be useful before initiating a specific therapy. We report successful treatment with plasma exchanges (PE) in four patients presenting with acute neurological deterioration. PE allowed a dramatic and rapid neurological improvement in all patients. PE are safe and may be useful at the initial management of these cases of anti-MAG neuropathy.
单克隆IgM抗髓鞘相关糖蛋白（MAG）抗体相关的周围神经病（抗MAG神经病）主要是一种伴有共济失调和远端感觉异常的脱髓鞘性感觉神经病。抗MAG神经病的临床病程通常进展缓慢，难以确定启动特异性治疗的明确标准。尽管尚无共识性治疗方案，但可提出以利妥昔单抗为基础的方案，靶向产生单克隆IgM的B细胞克隆，单独使用或与烷化剂或嘌呤类似物联合使用。然而，在一些罕见情况下，可能在数天内发生急性严重神经功能恶化，导致自主能力迅速丧失。在这些情况下，在开始特异性治疗前，迅速从循环中清除单克隆IgM的治疗可能有用。我们报告了4例出现急性神经功能恶化的患者接受血浆置换（PE）治疗成功的病例。PE使所有患者的神经功能得到显著且快速的改善。血浆置换安全，在这些抗MAG神经病病例的初始治疗中可能有用。
