Multifocal motor neuropathy (MMN) is a debilitating and rare disease causing profound weakness with minimal to no sensory symptoms. Conduction block is frequently seen on electrodiagnostic testing. An immune-mediated pathology is suspected though the exact underlying pathophysiology has yet to be elucidated. The presence of anti-GM1 ganglioside IgM antibodies coupled with favorable response to intravenous and subcutaneous immunoglobulins supports a complement-mediated mechanism which leads to destruction of nerve tissue with probable predilection to the nodes of Ranvier. High-dose immunoglobulin currently is the only treatment with proven efficacy for MMN patients. Unfortunately, many patients experience decreased responsiveness to immunoglobulins over time, requiring higher and more frequent dosing. In this review, we will focus on the pharmacology, efficacy, safety, and tolerability of intravenous and subcutaneous immune globulin infusion for treatment of MMN.
Keywords: anti-GM1 ganglioside IgM antibodies; conduction block; immunosuppressive therapies; intravenous immunoglobulin; multifocal motor neuropathy; subcutaneous immunoglobulin.
Conflict of interest statement
Disclosure The authors report no conflicts of interest in this work.
The pathogenesis of multifocal motor neuropathy and an update on current management options.Ther Adv Neurol Disord. 2015 May;8(3):109-22. doi: 10.1177/1756285615575269. Ther Adv Neurol Disord. 2015. PMID: 25941538 Free PMC article. Review.
IVIG blocks complement deposition mediated by anti-GM1 antibodies in multifocal motor neuropathy.J Neurol Neurosurg Psychiatry. 2011 Jan;82(1):87-91. doi: 10.1136/jnnp.2010.205856. Epub 2010 Jul 28. J Neurol Neurosurg Psychiatry. 2011. PMID: 20667861
Treatment of multifocal motor neuropathy.Curr Treat Options Neurol. 2014 Feb;16(2):269. doi: 10.1007/s11940-013-0269-y. Curr Treat Options Neurol. 2014. PMID: 24395647
Multifocal motor neuropathy.Curr Opin Neurol. 2018 Oct;31(5):559-564. doi: 10.1097/WCO.0000000000000605. Curr Opin Neurol. 2018. PMID: 30102608 Review.
Multifocal Motor Neuropathy (MMN).2020 Feb 7. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan–. StatPearls. 2020 Jan–. PMID: 32119411 Free Books & Documents. Review.
- Cats EA, van der Pol WL, Piepers S, et al. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. Neurology. 2010;75(9):818–825. - PubMed
- Moroni I, Bugiani M, Ciano C, Bono R, Pareyson D. Childhood-onset multifocal motor neuropathy with conduction blocks. Neurology. 2006;66(6):922–924. - PubMed
- Nguyen TP, Chaudhry V. Multifocal motor neuropathy. Neurol India. 2011;59(5):700–706. - PubMed
- Vlam L, van der Pol WL, Cats EA, et al. Multifocal motor neuropathy: diagnosis, pathogenesis and treatment strategies. Nat Rev Neurol. November 22. 2011;8(1):48–58. - PubMed
- Taylor BV, Wright RA, Harper CM, Dyck PJ. Natural history of 46 patients with multifocal motor neuropathy with conduction block. Muscle Nerve. 2000;23(6):900–908. - PubMed