Prognostic factor of poor outcome in anti-MAG neuropathy: clinical and electrophysiological analysis of a French Cohort

  • Marie-Ange Hoang Tang
  • Stéphane Mathis
  • Pierre Duffau
  • Philippe Cazenave
  • Guilhem Solé
  • Fanny Duval
  • Antoine Soulages
  • Gwendal Le MassonEmail author
Original Communication



Anti-MAG polyneuropathy (anti-MAG PN) is an immune-mediated peripheral sensorimotor neuropathy characterized by distal demyelination and ataxia. However, this disorder, unlike other immune-mediated neuropathies, is difficult to treat in most cases.


We retrospectively collected all anti-MAG PN patients followed in two hospitals for a period of 12 years to determine prognostic factors, especially those that indicated a good response to the various therapeutic strategies used.


Forty-seven patients were included in the study; of these, 61% had a classical ‘distal demyelinating pattern’, 34.2% had a ‘CIDP-like pattern’, and the others had an ‘axonal pattern’. The most commonly used treatments were intravenous immunoglobulin (IVIg) as the first-line treatment and rituximab as the second- or third-line treatment. No prognostic factor was identified for IVIg, but electrophysiological parameters at onset were better in patients with a good response to rituximab than in non-responder patients, even though mild or high disability was observed in nearly half the patients at last examination.


Even though disability seems to progress in most cases despite the treatments used, our results suggest that an early electrophysiological reduction in sensory nerves could be considered a ‘red flag’ for the prompt initiation of rituximab to try to delay long-term disability.


MAG Neuropathy Prognosis Sural nerve Rituximab 



This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Compliance with ethical standards

Conflicts of interest

The authors certify that they have no affiliations with or involvement in any organization or entity with any financial interest or non-financial interest in the subject matter or materials discussed in this manuscript.

Ethical standards

This study was in compliance with the ethical principles of the conference of Helsinki.

Supplementary material

415_2019_9618_MOESM1_ESM.docx (13 kb)
Supplementary material 1 (DOCX 13 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Neurology, Nerve-Muscle Unit, CHU Bordeaux (Groupe Hospitalier Pellegrin)University of BordeauxBordeauxFrance
  2. 2.Centre de Référence des Pathologies Neuromusculaires, CHU Bordeaux (Groupe Hospitalier Pellegrin)University of BordeauxBordeauxFrance
  3. 3.Department of Internal Medicine and Clinical Immunology, CHU Bordeaux (Groupe Hospitalier Saint-André)University of BordeauxBordeauxFrance
  4. 4.Department of NeurologyRobert Boulin HospitalLibourneFrance

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