Treatment of Guillain-Barre syndrome and CIDP
ABSTRACT Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating poly-(radiculo)neuropathy (CIDP) are immune-mediated disorders with a variable duration of progression and a range in severity of weakness. Infections can trigger GBS and exacerbate CIDP. Anti-ganglioside antibodies are important, but there is debate on the role of genetic factors in the pathogenesis of these disorders. Randomized controlled trials (RCT) have shown that intravenous immunoglobulin (IVIg) and plasma exchange (PE) are effective in both GBS and CIDP. Most CIDP patients also improve after steroid therapy. Despite current treatment options, many patients have residual deficits or need to be treated for a long period of time. Therefore, new treatment trials are highly indicated. This review focuses on the current and possible new treatment options that could be guided by recent results from laboratory experiments.
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ABSTRACT: Conventional treatment options, including corticosteroids, intravenous immunoglobulin, or plasma exchange, often fail to treat dysimmune neuropathies, such as chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and monoclonal gammopathy with its subtypes. Therefore, a significant percentage of patients require adjunctive immunosuppressive therapies. Considering that even immunosuppressive agents often are ineffective and/or associated with significant toxicities, the need for the development of safe and effective new treatment options is rising. Currently, several monoclonal antibodies (MAbs) have been tested in open-label small-sized studies or even in single cases so as to establish future directions in the therapy of diseases of the peripheral nervous system (PNS). Rituximab, an MAb targeting against the B cell surface membrane protein CD20, is the most widely used and promising MAb for the treatment of dysimmune neuropathies, especially for those in which immunoglobulin M (IgM) autoantibodies are pathogenetically involved. The efficacy of alemtuzumab, bevacizumab, and etanercept to treat various forms of dysimmune neuropathies is currently under investigation. This review looks critically at recent developments in molecularly targeted therapies for dysimmune neuropathies and also highlights areas of future research to pursue.Molecular Medicine 15(7-8):283-7. DOI:10.2119/molmed.2009.00041 · 4.82 Impact Factor
Article: Treatment of immune neuropathies.[Show abstract] [Hide abstract]
ABSTRACT: Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating poly(radiculo)neuropathy (CIDP) and multifocal motor neuropathy (MMN) are potentially treatable disorders. The use of appropriate assessment scales to evaluate the effects of treatment is essential. Recent therapeutic trials and the question of whether patients with mild disease or other variants of these disorders need to be treated are discussed. Recent clinical trials and Cochrane reviews give new information on the effect of various treatments in patients with GBS, CIDP and MMN. Intravenous immunoglobulin remains the only treatment proven to be effective in MMN. Combinations of treatment may be even more effective in GBS. Studies on prognostic factors related to improvement have been reported. Whether patients with Miller-Fisher syndrome or those with mild GBS should also be treated is still debated. New assessment scales at the disability and handicap level have now been evaluated for GBS and CIDP, and are ready for use. Results of studies in experimental models contribute to our understanding of the mechanism of action of intravenous immunoglobulin. Recent new information on the use of intravenous immunoglobulin and steroids indicates that the former should remain the cornerstone of treatment for GBS and MMN, and probably also for CIDP. Whether steroids not only suppress disease activity in CIDP but also eradicate the disease remains to be established. Some GBS patients have secondary deterioration or finally turn out to have CIDP; additional information in this group of patients may lead to more appropriate disease management. Most patients with CIDP and those with MMN need long-term treatment. New treatment strategies should now focus also on the effect and the costs of treatment over long-term follow up.Current Opinion in Neurology 11/2002; 15(5):623-31. DOI:10.1097/00019052-200210000-00014 · 5.73 Impact Factor