Keys P A, Blume R P
Department of Clinical Pharmacy, School of Pharmacy, Duquesne University, Pittsburgh, PA 15219.
DICP. 1991 Oct;25(10):1101-8. doi: 10.1177/106002809102501016.
Myasthenia gravis (MG) is a complex autoimmune neurologic disorder of unknown etiology, characterized by fluctuating skeletal muscle weakness most commonly involving the muscles of the head, neck, and upper extremities. Autoantibodies directed against acetylcholine receptors on the postjunctional membrane decrease the numbers of functional acetylcholine receptors and cause membrane alterations, resulting in neuromuscular transmission failure. Diagnosis is established by history and physical examination, the "Tensilon Test," and acetylcholine receptor antibody titers. Treatment modalities include drug therapy, thymectomy, and plasmapheresis. The drugs most commonly employed are anticholinesterases, corticosteroids, and immuno-suppressive agents. Cyclosporine and intravenous immunoglobulin are promising investigational treatments. The purpose of the article is to review current concepts in the pathophysiology, immunopathology, diagnosis, and treatment of MG. Special emphasis is placed on the autoimmune form of the disease and the drugs employed in its management. Standard regimens as well as some experimental treatment modalities are reviewed.
重症肌无力(MG)是一种病因不明的复杂自身免疫性神经疾病,其特征为骨骼肌无力波动,最常累及头部、颈部和上肢的肌肉。针对突触后膜上乙酰胆碱受体的自身抗体减少了功能性乙酰胆碱受体的数量,并导致膜改变,从而导致神经肌肉传递失败。通过病史、体格检查、“腾喜龙试验”和乙酰胆碱受体抗体滴度来确诊。治疗方式包括药物治疗、胸腺切除术和血浆置换。最常用的药物是抗胆碱酯酶药、皮质类固醇和免疫抑制剂。环孢素和静脉注射免疫球蛋白是有前景的研究性治疗方法。本文的目的是综述重症肌无力病理生理学、免疫病理学、诊断和治疗的当前概念。特别强调了该疾病的自身免疫形式及其治疗中使用的药物。对标准治疗方案以及一些实验性治疗方式进行了综述。