title: MMN: from immunological cross-talk to conduction block.
abstract: Multifocal motor neuropathy (MMN) is a rare inflammatory neuropathy characterized by progressive, asymmetric distal limb weakness and conduction block (CB). Clinically MMN is a pure motor neuropathy, which as such can mimic motor neuron disease. GM1-specific IgM antibodies are present in the serum of approximately half of all MMN patients, and are thought to play a key role in the immune pathophysiology. Intravenous immunoglobulin (IVIg) treatment has been shown to be effective in MMN in five randomized placebo-controlled trials. Despite long-term treatment with intravenous immunoglobulin (IVIg), which is efficient in the majority of patients, slowly progressive axonal degeneration and subsequent muscle weakness cannot be fully prevented. In this review, we will discuss the current understanding of the immune pathogenesis underlying MMN and how this may cause CB, available treatment strategies and future therapeutic targets.
authors.affiliation: Department of Neurology and Neurosurgery, UMC Utrecht Brain Center Rudolf Magnus, Utrecht, 3584 CG, The Netherlands.
authors.forename: Oliver
authors.initials: O
authors.lastname: Harschnitz
authors.forename: Bas A
authors.initials: BA
authors.lastname: Jongbloed
authors.forename: Hessel
authors.initials: H
authors.lastname: Franssen
authors.forename: Dirk C G
authors.initials: DC
authors.lastname: Straver
authors.forename: W Ludo
authors.initials: WL
authors.lastname: van der Pol
authors.forename: Leonard H
authors.initials: LH
authors.lastname: van den Berg
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000378:metabolism; D002450:Cell Communication; D005677:G(M1) Ganglioside / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M / Q000378:metabolism; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007167:Immunotherapy / Q000379:methods*; D009046:Motor Neurons / Q000276:immunology*; D009431:Neural Conduction / Q000187:drug effects; D011115:Polyneuropathies / Q000276:immunology / Q000503:physiopathology* / Q000628:therapy; D016032:Randomized Controlled Trials as Topic
chemical_list: D001323:Autoantibodies; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous; D005677:G(M1) Ganglioside
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