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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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 269, 4955–4960 (2022). https://doi.org/10.1007/s00415-022-11139-2
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DOI: https://doi.org/10.1007/s00415-022-11139-2