Department of Neurology, Haukeland University Hospital, 5021, Bergen, Norway.
Department of Clinical Medicine, University of Bergen, Bergen, Norway.
J Neurol. 2023 Jul;270(7):3329-3340. doi: 10.1007/s00415-023-11733-y. Epub 2023 Apr 26.
Myasthenia gravis (MG) is characterized by muscle weakness caused by autoantibodies that bind to the postsynaptic membrane at the neuromuscular junction and impair acetylcholine receptor function. Weakness of respiratory muscles represents the most severe MG manifestation, and 10-15% of all patients experience an MG crisis with the need of mechanical ventilatory support at least once in their life. MG patients with respiratory muscle weakness need active immunosuppressive drug treatment long term, and they need regular specialist follow-up. Comorbidities affecting respiratory function need attention and optimal treatment. Respiratory tract infections can lead to MG exacerbations and precipitate an MG crisis. Intravenous immunoglobulin and plasma exchange are the core treatments for severe MG exacerbations. High-dose corticosteroids, complement inhibitors, and FcRn blockers represent fast-acting treatments that are effective in most MG patients. Neonatal myasthenia is a transient condition with muscle weakness in the newborn caused by mother's muscle antibodies. In rare cases, treatment of respiratory muscle weakness in the baby is required.
重症肌无力（MG）的特征是肌肉无力，由自身抗体引起，这些自身抗体与神经肌肉接头的突触后膜结合，损害乙酰胆碱受体的功能。呼吸肌无力是 MG 最严重的表现，10-15%的所有患者一生中至少经历一次 MG 危象，需要机械通气支持。有呼吸肌无力的 MG 患者需要长期积极的免疫抑制药物治疗，需要定期专科随访。影响呼吸功能的合并症需要注意并进行最佳治疗。呼吸道感染可导致 MG 恶化并引发 MG 危象。静脉注射免疫球蛋白和血浆置换是严重 MG 恶化的核心治疗方法。大剂量皮质类固醇、补体抑制剂和 FcRn 阻断剂是大多数 MG 患者有效的快速作用治疗方法。新生儿肌无力是一种短暂的疾病，新生儿出现肌肉无力，是由母亲的肌肉抗体引起的。在极少数情况下，婴儿的呼吸肌无力需要治疗。
