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Treatment of Multifocal Motor Neuropathy with Intravenous Immunoglobulin

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Abstract

Multifocal motor neuropathy (MMN) is a rare inflammatory, chronically progressive, unremitting disorder affecting the peripheral nervous system. Although the etiology of this condition is not known, high titers of IgM Ab to GM1 may serve as a biomarker for this disease. Clinical findings of motor weakness are associated with focal conduction blocks and with time, axonal destruction. Evidence supporting an immune etiology as well as the use of intravenous immunoglobulin to limit the disease progression is reviewed.

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References

  1. Dimachkie MM, Barohn RJ, Katz J. Multifocal motor neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, and other chronic acquired demyelinating polyneuropathy variants. Neurol Clin. 2013;31(2):533–55.

    Article  PubMed  Google Scholar 

  2. Van den Berg-Vos RM, Franssen H, Wokke JH, Van Es HW, Van den Berg LH. Multifocal motor neuropathy: diagnostic criteria that predict the response to immunoglobulin treatment. Ann Neurol. 2000;48(6):919–26.

    Article  PubMed  Google Scholar 

  3. Nobile-Orazio E, Cappellari A, Meucci N, Carpo M, Terenghi F, Bersano A, et al. Multifocal motor neuropathy: clinical and immunological features and response to IVIg in relation to the presence and degree of motor conduction block. J Neurol Neurosurg Psychiatry. 2002;72(6):761–6.

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  4. Vlam L, van der Pol WL, Cats EA, Straver DC, Piepers S, Franssen H, et al. Multifocal motor neuropathy: diagnosis, pathogenesis and treatment strategies. Nat Rev Neurol. 2012;8(1):48–58.

    CAS  Google Scholar 

  5. Eftimov F, Van Schaik IN. Immunotherapy of multifocal motor neuropathy. Expert Opin Biol Ther. 2011;11(3):329–42.

    Article  CAS  PubMed  Google Scholar 

  6. Meuth SG, Kleinschnitz C. Multifocal motor neuropathy: update on clinical characteristics, pathophysiological concepts and therapeutic options. Eur Neurol. 2010;63(4):193–204.

    Article  PubMed  Google Scholar 

  7. Taylor BV, Dyck PJ, Engelstad J, Gruener G, Grant I, Dyck PJ. Multifocal motor neuropathy: pathologic alterations at the site of conduction block. J Neuropathol Exp Neurol. 2004;63(2):129–37.

    PubMed  Google Scholar 

  8. Corse AM, Chaudhry V, Crawford TO, Cornblath DR, Kuncl RW, Griffin JW. Sensory nerve pathology in multifocal motor neuropathy. Ann Neurol. 1996;39(3):319–25.

    Article  CAS  PubMed  Google Scholar 

  9. Cats EA, van der Pol WL, Piepers S, Franssen H, Jacobs BC, van den Berg-Vos RM, et al. Correlates of outcome and response to IVIg in 88 patients with multifocal motor neuropathy. Neurology. 2010;75(9):818–25.

    Article  CAS  PubMed  Google Scholar 

  10. Hahn AF, Beydoun SR, Lawson V, Oh MS, Empson VG, Leibl H, et al. A controlled trial of intravenous immunoglobulin in multifocal motor neuropathy. J Peripher Nerv Syst. 2013;18(4):321–30.

    Article  CAS  PubMed  Google Scholar 

  11. Cats EA, Jacobs BC, Yuki N, Tio-Gillen AP, Piepers S, Franssen H, et al. Multifocal motor neuropathy: association of anti-GM1 IgM antibodies with clinical features. Neurology. 2010;75(22):1961–7.

    Article  CAS  PubMed  Google Scholar 

  12. Pestronk A, Cornblath DR, Ilyas AA, Baba H, Quarles RH, Griffin JW, et al. A treatable multifocal motor neuropathy with antibodies to GM1 ganglioside. Ann Neurol. 1988;24(1):73–8.

    Article  CAS  PubMed  Google Scholar 

  13. Galban-Horcajo F, Fitzpatrick AM, Hutton AJ, Dunn SM, Kalna G, Brennan KM, et al. Antibodies to heteromeric glycolipid complexes in multifocal motor neuropathy. Eur J Neurol Off J Eur Fed Neurol Soc. 2013;20(1):62–70.

    CAS  Google Scholar 

  14. Willison HJ, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies. Brain. 2002;125(Pt 12):2591–625.

    Article  PubMed  Google Scholar 

  15. Yuki N, Kuwabara S. Axonal Guillain-Barre syndrome: carbohydrate mimicry and pathophysiology. J Peripher Nerv Syst. 2007;12(4):238–49.

    Article  CAS  PubMed  Google Scholar 

  16. Yuki N, Watanabe H, Nakajima T, Spath PJ. IVIG blocks complement deposition mediated by anti-GM1 antibodies in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry. 2011;82(1):87–91.

    Article  CAS  PubMed  Google Scholar 

  17. Takigawa T, Yasuda H, Kikkawa R, Shigeta Y, Saida T, Kitasato H. Antibodies against GM1 ganglioside affect K+ and Na+ currents in isolated rat myelinated nerve fibers. Ann Neurol. 1995;37(4):436–42.

    Article  CAS  PubMed  Google Scholar 

  18. Duarte-Rey C, Bogdanos DP, Leung PS, Anaya JM, Gershwin ME. IgM predominance in autoimmune disease: genetics and gender. Autoimmun Rev. 2012;11(6–7):A404–12.

    Article  CAS  PubMed  Google Scholar 

  19. Chaudhry V, Cornblath DR. An open-label trial of rituximab (Rituxan(R)) in multifocal motor neuropathy. J Peripher Nerv Syst. 2010;15(3):196–201.

    Article  CAS  PubMed  Google Scholar 

  20. Carpo M, Cappellari A, Mora G, Pedotti R, Barbieri S, Scarlato G, et al. Deterioration of multifocal motor neuropathy after plasma exchange. Neurology. 1998;50(5):1480–2.

    Article  CAS  PubMed  Google Scholar 

  21. Umapathi T, Hughes RA, Nobile-Orazio E, Leger JM. Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy. Cochrane Database Syst Rev. 2009;1, CD003217.

    PubMed  Google Scholar 

  22. Van Asseldonk JT, Van den Berg LH, Kalmijn S, Van den Berg-Vos RM, Polman CH, Wokke JH, et al. Axon loss is an important determinant of weakness in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry. 2006;77(6):743–7.

    Article  PubMed Central  PubMed  Google Scholar 

  23. Vucic S, Black KR, Chong PS, Cros D. Multifocal motor neuropathy: decrease in conduction blocks and reinnervation with long-term IVIg. Neurology. 2004;63(7):1264–9.

    Article  PubMed  Google Scholar 

  24. Terenghi F, Cappellari A, Bersano A, Carpo M, Barbieri S, Nobile-Orazio E. How long is IVIg effective in multifocal motor neuropathy? Neurology. 2004;62(4):666–8.

    Article  CAS  PubMed  Google Scholar 

  25. Joint Task Force of the E, the PNS. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of multifocal motor neuropathy. Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society--first revision. J Peripher Nerv Syst. 2010;15(4):295–301.

    Article  Google Scholar 

  26. van Schaik IN, van den Berg LH, de Haan R, Vermeulen M. Intravenous immunoglobulin for multifocal motor neuropathy. Cochrane Database Syst Rev. 2005;2, CD004429.

    PubMed  Google Scholar 

  27. American Association of Electrodiagnostic M, Olney RK. Guidelines in electrodiagnostic medicine. Consensus criteria for the diagnosis of partial conduction block. Muscle Nerve Suppl. 1999;8:S225–9.

    CAS  PubMed  Google Scholar 

  28. Sharrack B, Hughes RA. The Guy’s Neurological Disability Scale (GNDS): a new disability measure for multiple sclerosis. Mult Scler. 1999;5(4):223–33.

    Article  CAS  PubMed  Google Scholar 

  29. Stangel M, Kiefer R, Pette M, Smolka MN, Marx P, Gold R. Side effects of intravenous immunoglobulins in neurological autoimmune disorders—a prospective study. J Neurol. 2003;250(7):818–21.

    Article  PubMed  Google Scholar 

  30. Wittstock M, Benecke R, Zettl UK. Therapy with intravenous immunoglobulins: complications and side-effects. Eur Neurol. 2003;50(3):172–5.

    Article  CAS  PubMed  Google Scholar 

  31. Caress JB, Hobson-Webb L, Passmore LV, Finkbiner AP, Cartwright MS. Case–control study of thromboembolic events associated with IV immunoglobulin. J Neurol. 2009;256(3):339–42.

    Article  CAS  PubMed  Google Scholar 

  32. Rajabally YA, Kearney DA. Thromboembolic complications of intravenous immunoglobulin therapy in patients with neuropathy: a two-year study. J Neurol Sci. 2011;308(1–2):124–7.

    Article  CAS  PubMed  Google Scholar 

  33. Gelfand EW. Differences between IGIV products: impact on clinical outcome. Int Immunopharmacol. 2006;6(4):592–9.

    Article  CAS  PubMed  Google Scholar 

  34. Ballow M. Safety of IGIV therapy and infusion-related adverse events. Immunol Res. 2007;38(1–3):122–32.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

The Randomized controlled study of IGIV in MMN (Hahn A et al., Journal of the Peripheral Nervous System. 2013;18(4):321–30.) was funded in full by Baxter Healthcare Corporation.

Conflict of Interest

Carol Lee Koski MD served as a consultant to Baxter Healthcare for the planning and monitoring of the above MMN IGIV trial.

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Correspondence to Carol Lee Koski.

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Koski, C.L. Treatment of Multifocal Motor Neuropathy with Intravenous Immunoglobulin. J Clin Immunol 34 (Suppl 1), 127–131 (2014). https://doi.org/10.1007/s10875-014-0016-5

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  • DOI: https://doi.org/10.1007/s10875-014-0016-5

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