Multifocal motor neuropathy.
Multifocal motor neuropathy (MMN) is a recently identified peripheral nerve disorder characterized by progressive, predominantly distal, asymmetric limb weakness mostly affecting upper limbs, minimal or no sensory impairment, and by the presence on nerve conduction studies of multifocal persistent partial conduction blocks on motor but not sensory nerves. The etiopathogenesis of MMN is not known, but there is some evidence, based mostly on the clinical improvement after immunological therapies, that the disease has an immunological basis. Antibodies, mostly IgM, to the gangliosides GM1, and though less frequently, GM2 and GD1a, are frequently detected in patients' sera, helping in the diagnosis of this disease. Even if there is some experimental evidence that these antibodies may be pathogenic in vitro, their role in the neuropathy remains to be established. Patients with MMN do not usually respond to steroids or plasma exchange, which may occasionally worsen the symptoms, while the efficacy of cyclophosphamide is limited by its relevant side effects. More than 80% of MMN patients rapidly improve with high dose intravenous immunoglobulin therapy (IVIg). The effect of this therapy is, however, transient and improvement has to be maintained with periodic infusions. A positive response to interferon-beta has been recently reported in a minority of patients, some of whom were resistant to IVIg. Even if many progresses have been made on the diagnosis and therapy of MMN, there are still several issues on the nosological position, etiopathogenesis and long-term treatment of this neuropathy that need to be clarified.
Nobile-Orazio
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"Giorgio Spagnol" Service of Clinical Neuroimmunology, Dino Ferrari Centre, Department of Neurological Sciences, University of Milan, IRCCS Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, Italy. eduardo.nobile@unimi.it
D001323:Autoantibodies / Q000097:blood; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004568:Electrodiagnosis; D005260:Female; D005677:G(M1) Ganglioside / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D016899:Interferon-beta / Q000627:therapeutic use; D008297:Male; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D009431:Neural Conduction; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy*; D011129:Polyradiculoneuropathy / Q000175:diagnosis
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D005677:G(M1) Ganglioside; D016899:Interferon-beta; D003520:Cyclophosphamide