Intravenous immunoglobulin (IVIg) has made a major impact in the treatment of certain autoimmune neurological disorders by providing therapy for previously untreatable, or poorly managed, conditions. Based on controlled trials, IVIg is approved as first-line therapy for patients with GBS, CIDP, and multifocal motor neuropathy. In CIDP, where the largest controlled study was performed, IVIg is also effective in the chronic management of the disease by preventing relapses and axonal loss and maintaining remissions over a 48-week period. Controlled studies have also shown that IVIg is effective as second-line therapy in dermatomyositis, myasthenia gravis, and stiff-person syndrome; it is however ineffective in paraproteinemic IgM-anti-MAG demyelinating polyneuropathies, inclusion body myositis, and Alzheimer disease. IVIg exerts multiple actions on the immunoregulatory network by variably affecting complement activation, autoantibodies, and inflammatory mediators relevant for each neurological disorder. Biomarkers are needed to identify patients who require continuous IVIg immunotherapy for chronic management and for patients more likely to respond to IVIg from the outset.
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