title: Multifocal motor neuropathy.
abstract: OBJECTIVE
  Multifocal motor neuropathy (MMN) remains a difficult issue for neurologists, as its clinical and electrophysiological presentation may be atypical, and because no alternative treatment to periodic immunoglobulin infusions has been assessed in its long-term management. This review intends to summarize the most recent advances in the diagnosis and treatment of MMN.
  
  
  RESULTS
  Recent reports have focused on atypical onset and unusual clinical presentation. Several sophisticated electrophysiological techniques, as triple stimulation, may help establish the presence of conduction blocks, as well as MRI findings. A recent immunological study focused on the detection of serum IgM binding to NS6S heparin disaccharide. In another research article, it was proposed that the use of combinatorial glycoarray or ELISA may increase the diagnostic sensitivity of antiglycolipid antibody testing. Subcutaneous immunoglobulin may represent an interesting alternative option to intravenous immunoglobulin. Lastly, recently reported open-label clinical trials with complement inhibitors and anti-CD20 monoclonal antibody may constitute a first step for further developments.
  
  
  CONCLUSIONS
  Diagnostic criteria for MMN are well established, but challenging situations still occur. Progresses in neurophysiologic and other laboratory tests may help in clarifying doubtful diagnoses. Current research into the pathophysiology of MMN is required to determine the future treatment targets.
authors.affiliation: National Referral Center for Rare Neuromuscular Diseases, Institut Hospitalo-Universitaire de Neurosciences, University Hospital Pitié-Salpêtrière, University Pierre et Marie Curie, Paris VI, Paris, France.
authors.forename: Raquel
authors.initials: R
authors.lastname: Guimarães-Costa
authors.forename: Francesco
authors.initials: F
authors.lastname: Bombelli
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Léger
mesh_terms: D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D004187:Disaccharides / Q000627:therapeutic use; D006493:Heparin / Q000031:analogs & derivatives / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D009431:Neural Conduction / Q000187:drug effects / Q000502:physiology; D011115:Polyneuropathies / Q000175:diagnosis* / Q000188:drug therapy* / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D004187:Disaccharides; D016756:Immunoglobulins, Intravenous; C464536:heparin disaccharide; D006493:Heparin