Rivner Michael H, Pasnoor Mamatha, Dimachkie Mazen M, Barohn Richard J, Mei Lin
EMG Lab, Augusta University, 1120 15th Street, BP-4390, Augusta, GA 30912, USA.
Department of Neurology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
Neurol Clin. 2018 May;36(2):293-310. doi: 10.1016/j.ncl.2018.01.004.
Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors. Other antibodies associated with myasthenia gravis, including low-density lipoprotein receptor-related protein 4, are discussed.
约20%的重症肌无力患者乙酰胆碱受体抗体呈阴性;在这些病例中,30%至40%的患者被确定重症肌无力的病因是肌肉特异性酪氨酸激酶抗体(MuSK)。抗MuSK重症肌无力与特定的临床表型相关。一种是球部型,眼部症状较少。其他表现为孤立性头部下垂或症状与乙酰胆碱受体阳性重症肌无力难以区分。这些患者通常对免疫抑制治疗反应良好,但对胆碱酯酶抑制剂反应欠佳。还讨论了与重症肌无力相关的其他抗体,包括低密度脂蛋白受体相关蛋白4。