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Research paper
Anti-MAG antibodies in 202 patients: clinicopathological and therapeutic features
  1. Juliette Svahn1,
  2. Philippe Petiot1,
  3. Jean-Christophe Antoine2,
  4. Christophe Vial1,
  5. Emilien Delmont3,
  6. Karine Viala4,
  7. Andreas J. Steck5,
  8. Armelle Magot6,
  9. Cecile Cauquil7,
  10. Aline Zarea8,
  11. Andoni Echaniz-Laguna9,
  12. Ruxandra Iancu Ferfoglia10,
  13. Antoine Gueguen11,
  14. Laurent Magy12,
  15. Jean-Marc Léger13,
  16. Thierry Kuntzer14,
  17. Karine Ferraud2,
  18. Arnaud Lacour2,
  19. Jean-Philippe Camdessanché2
  20. The Francophone anti-MAG cohort Group
  1. 1Electroneuromyography and Neuromuscular Department, University Hospital Lyon, Lyon, France
  2. 2Department of Neurology, University Hospital Saint-Etienne, Saint-Etienne, France
  3. 3Department of Neurology, University Hospital Timone, Marseille, France
  4. 4Department of Neurophysiology and Neuropathology, University Hospital Pitié-Salpêtrière, Paris, France
  5. 5Department of Neurology, University Hospital Basel, Basel, Switzerland
  6. 6Neuromuscular Reference Center, University Hospital Nantes, Nantes, France
  7. 7Department of Neurology, University Hospital Bicêtre, Le Kremlin Bicêtre, France
  8. 8Neuromuscular Competence Center, University Hospital Rouen, Rouen, France
  9. 9Department of Neurology, University Hospital Strasbourg, Strasbourg, France
  10. 10Electroneuromyography and Neuromuscular Disorders Unit, University Hospital Geneva, Geneva, Switzerland
  11. 11Department of Neurology, Fondation Ophtalmologique A. de Rothschild, Paris, France
  12. 12Department of Neurology, University Hospital Limoges, Limoges, France
  13. 13Department of Neurology, University Hospital Pitié-Salpêtrière, Paris, France
  14. 14Department of Clinical Neurosciences, Nerve-Muscle Unit, Neurology Service, Lausanne University Hospital (CHUV), Lausanne, Switzerland
  1. Correspondence to Dr Juliette Svahn, Electroneuromyography and Neuromuscular Department, University Hospital Lyon, 69677 Lyon, Bron Cedex – France; juliette.svahn{at}chu-lyon.fr

Abstract

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.

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Footnotes

  • Contributors JS, PP, JCA and JPC contributed to the study concept, analysis or interpretation of data. JS and KF contributed to the acquisition of data. JS, JPC, PP, JCA, AS, KV, AEL and TK contributed to drafting/revising of the manuscript. All other authors provide clinical care to patients and detailed clinical information for the study.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Ethics approval The project was approved by the Advisory Committee for Data Processing in Health Research (CCTIRS).

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement All relevant data are presented in the article. Further information is available from the corresponding author.

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