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Approximately 5–8 % of myasthenia gravis (MG) patients test positive for antibodies against muscle- specific tyrosine kinase (MuSK) receptors. Except in extremely rare reports, all are acetylcholine receptor (AChR) antibody-negative. While MuSK myasthenia gravis (MMG) patients have distinct clinical phenotypes and may differ from AChR-positive patients in diagnostic testing and response to treatment, goals for the treatment of MMG are similar to those in non-MMG. Priority of treatment should be directed toward reducing weakness as much and as quickly as possible. This is particularly true in patients with bulbar or respiratory weakness in order to avoid progression to respiratory failure. After this initial phase, medications should be slowly tapered to the minimum effective dose. Considering the natural history of MMG, a small proportion of patients can be completely taken off treatment …