• Juliette Svahn, Philippe Petiot, Jean-Christophe Antoine, Christophe Vial, Emilien Delmont, Karine Viala, Andreas J Steck, Armelle Magot, Cecile Cauquil, Aline Zarea, Andoni Echaniz-Laguna, Iancu Ferfoglia Ruxandra R Electroneuromyography and Neuromuscular Disorders Unit, University Hospital Geneva, Geneva, Switzerland., Antoine Gueguen, Laurent Magy, Jean-Marc Léger, Thierry Kuntzer, Karine Ferraud, Arnaud Lacour, Jean-Philippe Camdessanché, and Francophone anti-MAG cohort Group.
• Electroneuromyography and Neuromuscular Department, University Hospital Lyon, Lyon, France.
• J. Neurol. Neurosurg. Psychiatr. 2018 May 1; 89 (5): 499-505.

ObjectiveTo assess the clinicopathological and therapeutic features of patients with low (≥1000 to <10 000 Bühlmann Titre Units) (BTU), medium (10 000-70 000) or high (≥70 000) anti-myelin-associated glycoprotein (anti-MAG) antibody titres.MethodsWe retrospectively and prospectively analysed standardised report forms and medical records of 202 patients from 14 neuromuscular centres.ResultsMean age at onset and mean time between symptom onset to last follow-up were respectively 62.6 years (25-91.4) and 8.4 years (0.3-33.3). Anti-MAG antibody titres at diagnosis were low, medium or high in 11%, 51% and 38% of patients. Patients presented with monoclonal gammopathy of undetermined significance in 68% of cases. About 17% of patients presented with 'atypical' clinical phenotype independently of anti-MAG titres, including acute or chronic sensorimotor polyradiculoneuropathies (12.4%), and asymmetric or multifocal neuropathy (3%). At the most severe disease stage, 22.4% of patients were significantly disabled. Seventy-eight per cent of patients received immunotherapies. Transient clinical worsening was observed in 12% of patients treated with rituximab (11/92). Stabilisation after rituximab treatment during the 7-12-month follow-up period was observed in 29% of patients. Clinical response to rituximab during the 6-month and/or 7-12-month follow-up period was observed in 31.5% of patients and correlated with anti-MAG titre ≥10 000 BTU.ConclusionOur study highlights the extended clinical spectrum of patients with anti-MAG neuropathy, which appears unrelated to antibody titre. Besides, it may also suggest beneficial use of rituximab in the early phase of anti-MAG neuropathy.© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

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