High-dose intravenous immunoglobulin therapy in juvenile myasthenia gravis

Autoimmune neurologic disease management has been significantly modified by the use of high-dose intravenous immunoglobulin (HDIVIG) during the past 1 5 years. Venous access, readily available IgG (until recently), and the rel ative lack of serious identifiable complications have prompted its use in m yasthenia gravis. In adults, its effectiveness has been inconsistent, with variable acetylcholine receptor (AChR) antibody responses. Ten children wer e evaluated for clinical responses to, and complications of, HDIVIG. Weekly anti-AChR antibody Liters in three patients were obtained. The HDIVIG dosa ge was 2 gm/kg body weight, infused at variable rates of 2 gm/kg for 1 day, 0.66 gm/kg daily for 3 days, and 0.5 g/kg daily for 4 days; in one patient the total dose was 0.8 gm/kg to correct to the ideal body weight. All chil dren but one tolerated HDIVIG without complications. Eight patients exhibit ed definite improvement in functional strength after HDIVIG, but a decreasi ng response to HDIVIG was evident after multiple monthly treatments, warran ting the additional use of corticosteroids in two patients. A decrease in a nti-AChR antibody levels was observed in the three patients tested, but thi s decrease was constant in one patient. No correlation was observed between clinical response and antibody Liters. HDIVIG is safe and effective in mos t patients for shortterm management of juvenile myasthenia gravis, in myast henic crises, and in preparing patients for surgery but appears to be of li mited long-term benefit. (C) 2000 by Elsevier Science Inc. AII rights reser ved.