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Revisiting the spectrum of IgM-related neuropathies in a large cohort of IgM monoclonal gammopathy

Journal of Neurology (2022)Cite this article

Abstract

Introduction

A significant number of patients with a peripheral neuropathy have IgM monoclonal gammopathy (IgM-MG). In this work, we encompassed the spectrum and outcome of IgM-related neuropathies (IgM-NP) in a large monocentric cohort of patients with IgM-MG.

Methods

We retrospectively reviewed the neurological and hematological findings and the course of neuropathy in all patients with IgM-MG over a five-year period in our center (Henri Mondor hospital, Assistance Publique Hôpitaux de Paris (APHP), France).

Results

Among 550 patients with IgM-MG, 83 patients (15%) had IgM-NP (55 males, mean age 67 y.o.). The median serum level of IgM-MG was 3.4 g/L, mostly kappa light chain component. The hematological diagnosis was Monoclonal Gammopathy of Undetermined Significance (MGUS) in 62 patients. Anti-MAG antibodies were detected in 38 patients with heterogeneous clinical and neurophysiological features. Four patients had neurolymphomatosis presenting as a non-length dependent predominantly motor neuropathy, which occurred long after the finding of IgM-MG and was responsive to hematological treatment. Five patients had an AL amyloid neuropathy revealed by a small fiber neuropathy. Finally, 30 patients were classified as “Neuropathy of Uncertain Relationship with the IgM” (NURIM) with characteristics close to those of an anti-MAG-NP at the time of diagnosis, except for the neurophysiological features with a predominant axonal pattern.

Conclusion

This study emphasizes the wide spectrum of IgM-NP associated with a variety of hematological diagnoses. In particular, the course and prognosis vary considerably. In this setting, further studies are needed to unravel the group of patients classified as NURIM.

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Funding

This study received no funding.

Author information

Authors and Affiliations

  1. Excitabilité Nerveuse Et Thérapeutique (ENT), Univ Paris Est Creteil, EA 4391, 94010, Creteil, France

    Benjamin Bardel, Samar S. Ayache, Tarik Nordine & Jean-Pascal Lefaucheur

  2. Department of Clinical Neurophysiology, DMU FIxIT, AP-HP, Henri Mondor University Hospital, 94010, Creteil, France

    Benjamin Bardel, Samar S. Ayache, Tarik Nordine & Jean-Pascal Lefaucheur

  3. INSERM, IMRB, Univ Paris Est Creteil, 94010, Creteil, France

    Valérie Molinier-Frenkel

  4. Department of Immunology, DMU Biologie Pathologie, AP-HP, Henri Mondor University Hospital, 94010, Creteil, France

    Valérie Molinier-Frenkel

  5. Department of Lymphoid Hemopathy, DMU Cancer, AP-HP, Henri Mondor University Hospital, 94010, Creteil, France

    Fabien Le Bras

  6. Department of Neurology, DMU Médecine, AP-HP, Henri Mondor University Hospital, 94010, Creteil, France

    Violaine Planté-Bordeneuve

Authors
  1. Benjamin Bardel
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  2. Valérie Molinier-Frenkel
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  3. Fabien Le Bras
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  4. Samar S. Ayache
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  6. Jean-Pascal Lefaucheur
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  7. Violaine Planté-Bordeneuve
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Corresponding author

Correspondence to Benjamin Bardel.

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Conflicts of interest

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Ethical standard statement

The study was entirely based on register data and was approved by the French data inspection agency (Commission nationale de l’informatique et des libertés, CNIL 1685786 v 0). The participants gave their non-objection consent to the use of these data collected in the context of clinical practice, according to the French law requirement regarding ethical approval of register-based research.

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Bardel, B., Molinier-Frenkel, V., Le Bras, F. et al. Revisiting the spectrum of IgM-related neuropathies in a large cohort of IgM monoclonal gammopathy. J Neurol (2022). https://doi.org/10.1007/s00415-022-11139-2

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  • DOI: https://doi.org/10.1007/s00415-022-11139-2

Keywords

  • Peripheral neuropathy
  • IgM-related neuropathy
  • Anti-MAG neuropathy
  • IgM-monoclonal gammopathy
  • Paraproteinemic neuropathy