Update on paraproteinemic neuropathy
- PMID: 40549225
- DOI: 10.1007/s11910-025-01430-w
Update on paraproteinemic neuropathy
Abstract
Purpose of review: This article reviews recent clinical updates and research on the evaluation and management of patients with peripheral neuropathy in association with monoclonal gammopathies.
Recent findings: Recent studies have elucidated pathogenic mechanisms of IgM paraprotein associated neuropathies, including nodal and paranodal targets and complement mediated processes, suggesting novel therapeutic targets. New chemotherapeutic regimens have improved outcomes and neurotoxic side effect profiles in the treatment of patients with light chain (AL) amyloidosis and POEMS syndrome. Establishing when a monoclonal gammopathy is causative of a peripheral neuropathy remains a clinical challenge. New therapeutic drugs for treatment of IgM paraprotein associated neuropathies show promise. Identifying AL amyloidosis and POEMS early in patients presenting with neuropathy are important. Clinical phenotyping and antibody testing are critical to evaluating patients with paraproteins and peripheral neuropathy.
Keywords: Anti-MAG antibody neuropathy; Monoclonal gammopathy; Monoclonal gammopathy of neurological significance; Paraneoplastic neuropathy; Paraprotein.
© 2025. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
Conflict of interest statement
Declarations. Competing Interests: Rebecca Traub has received research funding and consulting fees from Argenx, research funding from Immunovant and consulting fees from Takeda. Samuel Rubinstein has received consulting fees from Johnson&Johnson, Brisol-Myers Squibb, and Sanofi. Christopher Dittus has received research funding from Segan, Genentech, and Astrazeneca and consulting fees from Beigene, Genmab, and ADC.
References
-
- Min YG, Visentin A, Briani C, Rajabally YA. Neuropathy with anti-myelin-associated glycoprotein antibodies: update on diagnosis, pathophysiology and management. J Neurol Neurosurg Psychiatry. BMJ Publishing Group; 2024.
-
- Magy L, Kaboré R, Mathis S, Lebeau P, Ghorab K, Caudie C et al. Heterogeneity of polyneuropathy associated with Anti-MAG antibodies. J Immunol Res. 2015;2015.
-
- Ferfoglia RI, Guimarães-Costa R, Viala K, Musset L, Neil J, Marin B, et al. Long-term efficacy of rituximab in IgM anti-myelin-associated glycoprotein neuropathy: RIMAG follow-up study. J Peripheral Nerv Syst. 2016;21:10–4. - DOI
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