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

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Abstract

Background

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.

Method

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.

Results

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.

Conclusion

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.

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Funding

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

Author information

Authors and Affiliations

  1. Department of Neurology, Nerve-Muscle Unit, CHU Bordeaux (Groupe Hospitalier Pellegrin), University of Bordeaux, Place Amélie Raba-Léon, 33000, Bordeaux, France

    Marie-Ange Hoang Tang, Stéphane Mathis, Guilhem Solé, Fanny Duval, Antoine Soulages & Gwendal Le Masson

  2. Centre de Référence des Pathologies Neuromusculaires, CHU Bordeaux (Groupe Hospitalier Pellegrin), University of Bordeaux, Place Amélie Raba-Léon, 33000, Bordeaux, France

    Stéphane Mathis, Guilhem Solé, Fanny Duval & Gwendal Le Masson

  3. Department of Internal Medicine and Clinical Immunology, CHU Bordeaux (Groupe Hospitalier Saint-André), University of Bordeaux, Place Amélie Raba-Léon, 33000, Bordeaux, France

    Pierre Duffau

  4. Department of Neurology, Robert Boulin Hospital, 112 rue de la Marne, 33505, Libourne, France

    Philippe Cazenave

Authors
  1. Marie-Ange Hoang Tang
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  2. Stéphane Mathis
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  3. Pierre Duffau
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  4. Philippe Cazenave
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  5. Guilhem Solé
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  6. Fanny Duval
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  7. Antoine Soulages
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  8. Gwendal Le Masson
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Correspondence to Gwendal Le Masson.

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

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Tang, MA.H., Mathis, S., Duffau, P. et al. Prognostic factor of poor outcome in anti-MAG neuropathy: clinical and electrophysiological analysis of a French Cohort. J Neurol 267, 561–571 (2020). https://doi.org/10.1007/s00415-019-09618-0

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  • DOI: https://doi.org/10.1007/s00415-019-09618-0

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