RT Journal Article
SR Electronic
T1 Anti-MAG antibodies in 202 patients: clinicopathological and therapeutic features
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 499
OP 505
DO 10.1136/jnnp-2017-316715
VO 89
IS 5
A1 Svahn, Juliette
A1 Petiot, Philippe
A1 Antoine, Jean-Christophe
A1 Vial, Christophe
A1 Delmont, Emilien
A1 Viala, Karine
A1 Steck, Andreas J
A1 Magot, Armelle
A1 Cauquil, Cecile
A1 Zarea, Aline
A1 Echaniz-Laguna, Andoni
A1 Iancu Ferfoglia, Ruxandra
A1 Gueguen, Antoine
A1 Magy, Laurent
A1 Léger, Jean-Marc
A1 Kuntzer, Thierry
A1 Ferraud, Karine
A1 Lacour, Arnaud
A1 Camdessanché, Jean-Philippe
A1 
YR 2018
UL http://jnnp.bmj.com/content/89/5/499.abstract
AB 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.