TY - JOUR T1 - Anti-MAG antibodies in 202 patients: clinicopathological and therapeutic features JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry DO - 10.1136/jnnp-2017-316715 SP - jnnp-2017-316715 AU - Juliette Svahn AU - Philippe Petiot AU - Jean-Christophe Antoine AU - Christophe Vial AU - Emilien Delmont AU - Karine Viala AU - Andreas J. Steck AU - Armelle Magot AU - Cecile Cauquil AU - Aline Zarea AU - Andoni Echaniz-Laguna AU - Ruxandra Iancu Ferfoglia AU - Antoine Gueguen AU - Laurent Magy AU - Jean-Marc Léger AU - Thierry Kuntzer AU - Karine Ferraud AU - Arnaud Lacour AU - Jean-Philippe Camdessanché A2 - , Y1 - 2017/10/25 UR - http://jnnp.bmj.com/content/early/2017/10/25/jnnp-2017-316715.abstract N2 - Objective To 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.Methods We retrospectively and prospectively analysed standardised report forms and medical records of 202 patients from 14 neuromuscular centres.Results Mean 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.Conclusion Our 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. ER -