Treatment of immune neuropathies

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.