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



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.


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).


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.


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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Correspondence to Benjamin Bardel.

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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).

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