J Neurol (2005) 252 : 385–395 DOI 10.1007/s00415-005-0805-0 ENS TEACHING REVIEW Eduardo Nobile-Orazio Treatment of dysimmune neuropathies Introduction Immune-mediated neuropathies, including Guillain- Barré syndrome, chronic inflammatory demyelinating neuropathy,multifocal motor neuropathy,multifocal de- myelinating neuropathy (Lewis-Sumner syndrome), neuropathies associated with monoclonal gammopathy, and paraneoplastic neuropathies, are a group of disor- ders deemed to be caused by an immune response di- rected against peripheral nerve antigens. The role of the immune system in the pathogenesis of these neu- ropathies is supported not only by their frequent associ- ation with a number of anti-neural antibodies (whose pathogenetic role remains, however, to be established) [77],but also by their frequent response to immune ther- apies. Several immune therapies have been reported to be effective in these neuropathies including steroids, plasma exchange (PE), high-dose intravenous im- munoglobulins (IVIg), as well as a number of immuno- JON 1805 ■ Abstract Several therapies are currently used in dysimmune neuropathies including steroids, plasma exchange (PE), high-dose intravenous immunoglobulins (IVIg), and immunosuppressive agents (IS). Even if there is sub- stantial evidence that these treat- ments may improve the course of the neuropathy, their effectiveness is far from being complete and is sometime hampered by the occur- rence of associated side effects. In Guillain-Barré syndrome (GBS), IVIg and PE are similarly effective in accelerating the recovery but there is still little evidence that they can reduce mortality or long-term disability. Recent reports on the association of intravenous methyl- prednisolone or interferon-β (IFN- β) to IVIg did not result in signifi- cant further improvement. In chronic inflammatory demyelinat- ing polyradiculoneuropathy (CIDP) steroids, PE, and IVIG are initially similarly effective. The short-term effect of PE and IVIg and the side effects associated with the long-term use of steroids have prompted the use of several IS, in- terferon and, more recently, the anti-CD20 monoclonal-antibody Rituximab, but their efficacy has still to be proved in controlled studies. The recent identification of multifocal motor neuropathy (MMN) was shortly followed by the finding of an effective therapy.Al- most 80 % of patients respond to IVIg whose effect needs to be maintained with periodic infusions for long periods of time, and tends to decrease after several years. Also in this condition a number of im- mune modulating agents have been used to reduce the frequency or improve the effectiveness of IVIg, but their efficacy has not been so far confirmed in randomized trials. Similar conclusions can be drawn for neuropathies associated with monoclonal gammopathies where only PE and IVIg have proved to be effective in controlled studies, while the promising initial results obtained with Rituximab in neu- ropathy associated IgM mono- clonal gammopathy awaits confir- mation from controlled trials. ■ Key words chronic inflammatory demyelinating polyradiculoneuropathy · Guillain- Barré syndrome · monoclonal gammopathy · multifocal motor neuropathy · therapy Received: 31 December 2004 Accepted: 5 January 2005 E. Nobile-Orazio, M.D., Ph.D. () Department of Neurological Sciences Dino Ferrari Center, University of Milan IRCCS Humanitas Clinical Institute Via Manzoni 56 20089, Rozzano, Milan, Italy Tel.: +39-02/8224-2209 Fax: +39-02/8224-2298 E-Mail: eduardo.nobile@unimi.it