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In controlled clinical trials, IVIg has been capable of healing the Guillian-Barre syndrome, multifocal motor neuropathy, chronic inflammatory demyelinating polyneuropathy, and dermatomyositis. In other controlled trials and case reports, IVIg produced improvement in various patients with the Lambert-Eaton myasthenic syndrome and myasthenia gravis but had a variable, mild, or unsubstantiated benefit in some patients with inclusion-body myositis, paraproteinemic IgM demyelinating polyneuropathy, certain intractable childhood epilepsies, polymyositis, multiple sclerosis, optic neuritis, and the stiff-man syndrome. The primary adverse reaction was headache, aseptic meningitis, skin reactions, thromboembolic events, and renal tubular necrosis occurred rarely. The most important immunomodulatory actions of IVIg, operating alone or in combination, are inhibition of complement deposition, neutralization of cytokines, modulation of Fc-receptor-mediated phagocytosis, and downregulation of autoantibody production. Therapy with Ivig is effective for certain autoimmune neurologic diseases, but its spectrum of efficacy has not been fully established. In 1981, it was first observed that children with idiopathic thrombocytopenic purpura respond to high-dose intravenous immune globulin therapy. This therapy has been used in a wide range of autoimmune diseases, various primary immunodeficiencies, and the Kawasaki syndrome. In neurology, the advancing use of this very costly therapy has already affected medical insurance coverage, hospital pharmacy budgets, and the ability of patients to afford care. Different understanding of clinical trial results, incomplete understanding of IVIg's mechanisms of action, and newly recognized complications of therapy are initiating confusion among practitioners. |
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