title: Multifocal motor neuropathy: current concepts and controversies.
abstract: Multifocal motor neuropathy (MMN) is now a well-defined purely motor multineuropathy characterized by the presence of multifocal partial motor conduction blocks (CB), frequent association with anti-GM1 IgM antibodies, and usually a good response to high-dose intravenous immunoglobulin (IVIg) therapy. However, several issues remain to be clarified in the diagnosis, pathogenesis, and therapy of this condition including its nosological position and its relation to other chronic dysimmune neuropathies; the degree of CB necessary for the diagnosis of MMN; the existence of an axonal form of MMN; the pathophysiological basis of CB; the pathogenetic role of antiganglioside antibodies; the mechanism of action of IVIg treatments in MMN and the most effective regimen; and the treatment to be used in unresponsive patients. These issues are addressed in this review of the main clinical, electrophysiological, immunological, and therapeutic features of this neuropathy.
authors.affiliation: Dino Ferrari Centre and Centre of Excellence for Neurodegenerative Diseases, Department of Neurological Sciences, Milan University, IRCCS Ospedale Maggiore Policlinico, and Humanitas Clinical Institute, Milan, Italy. eduardo.nobile@unimi.it
authors.forename: Eduardo
authors.initials: E
authors.lastname: Nobile-Orazio
authors.forename: Alberto
authors.initials: A
authors.lastname: Cappellari
authors.forename: Alberto
authors.initials: A
authors.lastname: Priori
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology; D005677:G(M1) Ganglioside / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D016472:Motor Neuron Disease / Q000276:immunology / Q000503:physiopathology* / Q000628:therapy; D009046:Motor Neurons / Q000276:immunology / Q000473:pathology; D009431:Neural Conduction / Q000276:immunology; D009443:Neuritis / Q000276:immunology / Q000503:physiopathology* / Q000628:therapy; D010525:Peripheral Nerves / Q000276:immunology / Q000473:pathology / Q000503:physiopathology*
chemical_list: D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D005677:G(M1) Ganglioside