Statland Jeffrey M, Ciafaloni Emma
Department of Neurology, University of Rochester Medical Center, Rochester, NY.
Neurol Clin Pract. 2013 Apr;3(2):126-133. doi: 10.1212/CPJ.0b013e31828d9fec.
Myasthenia gravis (MG) is the most common autoimmune disease affecting neuromuscular junction transmission. MG is characterized by muscle weakness that worsens with activity and fluctuates over the course of the day. Involvement of respiratory musculature can lead to life-threatening crisis requiring intensive care unit care. Antibody testing is positive in most patients with MG. Treatment of MG includes short-term symptomatic treatment, chronic immunosuppression, surgical intervention, and immunomodulatory therapies for severe disease or crisis. We review advances in 5 areas relevant to diagnosis and management of MG: the role of IV immunoglobulin vs plasmapharesis in myasthenic crisis and severe disease; the clinical characterization of patients with antibodies to muscle-specific tyrosine kinase receptors; old and new investigational treatments; management of MG in pregnancy; and new confirmatory diagnostic tests.
重症肌无力（MG）是影响神经肌肉接头传递的最常见自身免疫性疾病。重症肌无力的特征是肌无力，活动时加重且在一天中波动。呼吸肌受累可导致危及生命的危象，需要重症监护病房护理。大多数重症肌无力患者的抗体检测呈阳性。重症肌无力的治疗包括短期对症治疗、慢性免疫抑制、手术干预以及针对严重疾病或危象的免疫调节疗法。我们综述了与重症肌无力诊断和管理相关的5个领域的进展：静脉注射免疫球蛋白与血浆置换在重症肌无力危象和严重疾病中的作用；抗肌肉特异性酪氨酸激酶受体抗体患者的临床特征；新旧研究性治疗方法；妊娠重症肌无力的管理；以及新的确诊诊断试验。
