抗肌肉特异性激酶抗体阳性的女性广泛性重症肌无力患者对沙丁胺醇有反应而对吡哆斯的明无反应:1例报告

Konishi Tetsuro
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摘要

抗肌肉特异性激酶(MuSK)抗体阳性的全身性重症肌无力(MuSK- mg)是一种自身免疫性疾病,胆碱酯酶抑制剂(ChE-I)治疗可使重症肌无力症状恶化。沙丁胺醇是一种交感β2受体激动剂,在麝香基因异常的先天性肌无力综合征患者和麝香- mg动物模型中是有效的治疗药物。沙丁胺醇曾试图治疗一名对ChE-I无反应的麝香mg女性患者。沙丁胺醇没有任何急性疗效,但她的肌无力症状在一年内逐渐改善,没有不良反应。沙丁胺醇可能是缓解麝香- mg患者肌无力症状的治疗药物。
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A Female Patient with Anti-Muscle-Specific Kinase Antibody Positive Generalized Myasthenia Gravis Responded to Salbutamol but not Pyridostigmine: A Case Report
Anti-muscle-specific kinase (MuSK) antibody positive generalized myasthenia gravis (MuSK-MG) is an autoimmune disease, in which deterioration of myasthenic symptoms may occur by cholinesterase inhibitor (ChE-I) treatment. Salbutamol, a sympathetic β2 receptor agonist, was an effective therapeutic drug in congenital myasthenic syndrome patients with MuSK gene abnormalities and in MuSK-MG animal model. Salbutamol was attempted to treat a female MuSK-MG patient who did not respond to ChE-I. Any acute benefit of salbutamol was not observed but her myasthenic symptoms gradually improved over a year without adversive effects. Salbutamol may be a therapeutic drug to alleviate myasthenic symptoms in MuSK-MG patients.
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