Intravenous Immunoglobulin Treatment in Multifocal Motor Neuropathy.
J Clin Immunol. 2010 Apr 20. [Epub ahead of print]
van der Pol WL, Cats EA, van den Berg LH.
Rudolf Magnus Institute of Neuroscience, Department of Neurology, University Medical Center Utrecht, Heidelberglaan 100, 3584, CX, Utrecht, the Netherlands.
INTRODUCTION: Multifocal motor neuropathy (MMN) is characterized by asymmetric weakness of limbs and the electrophysiological finding of conduction block in motor nerves. Conduction block is the inability of nerves to propagate action potentials and is probably caused by immune-mediated dysfunction of the axon at the nodes of Ranvier or the myelin sheath. MMN immune pathogenesis has not been elucidated. RESULTS: In approximately 50% of all patients, IgM antibodies that bind to the glycolipid GM1, which is abundantly expressed in peripheral motor nerves, can be detected. A recent study showed an association with HLA-DRB1*15, and virtually all patients respond to treatment with intravenous immunoglobulin (IVIG) in at least the early stages of the disease. CONCLUSION: This review aims at providing a concise overview of what is known about MMN pathogenesis, and how the beneficial effect of IVIG might be explained.