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

J Neurol. 2020 Feb;267(2):561-571. doi: 10.1007/s00415-019-09618-0. Epub 2019 Nov 8.


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.

Keywords: MAG; Neuropathy; Prognosis; Rituximab; Sural nerve.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Disease Progression*
  • Electrophysiological Phenomena*
  • Female
  • Follow-Up Studies
  • France
  • Humans
  • Immunologic Factors / administration & dosage*
  • Male
  • Middle Aged
  • Myelin-Associated Glycoprotein / immunology*
  • Outcome Assessment, Health Care
  • Polyradiculoneuropathy / classification
  • Polyradiculoneuropathy / drug therapy*
  • Polyradiculoneuropathy / pathology*
  • Polyradiculoneuropathy / physiopathology*
  • Prognosis
  • Retrospective Studies


  • Immunologic Factors
  • MAG protein, human
  • Myelin-Associated Glycoprotein