Verschuuren Jan J G M, Wirtz Paul W, Titulaer Maarten J, Willems Luuk N A, van Gerven Joop
Leiden University Medical Centre, Department of Neurology, PO Box 9600, 2300 RC Leiden, Leiden, The Netherlands.
Expert Opin Pharmacother. 2006 Jul;7(10):1323-36. doi: 10.1517/14656566.7.10.1323.
Lambert-Eaton myasthenic syndrome is a rare, but reasonably well-understood, antibody-mediated autoimmune disease that is caused by serum auto-antibodies and results in muscle weakness and autonomic dysfunction. One half of the patients have an idiopathic form, the other half a tumour-associated form of the disease. Three randomised trials and a large number of smaller clinical studies have resulted in a number of drugs becoming available for the treatment of Lambert-Eaton myasthenic syndrome. Several drugs are available for the symptomatic treatment of the disease, including guanidine, aminopyridines or acetylcholinesterase inhibitors. Other therapies aim to deplete the serum autoantibodies or to suppress the immune system. For this purpose, immunomodulating strategies, such as intravenous immunoglobulins or plasmapheresis, or several immunosuppressive agents are available. Chemotherapy has successfully ameliorated the course of disease in Lambert-Eaton myasthenic syndrome patients with an underlying tumour.
兰伯特-伊顿肌无力综合征是一种罕见但理解较为充分的抗体介导的自身免疫性疾病,由血清自身抗体引起,导致肌肉无力和自主神经功能障碍。一半患者为特发性形式,另一半为肿瘤相关性形式。三项随机试验和大量较小规模的临床研究已使多种药物可用于治疗兰伯特-伊顿肌无力综合征。有几种药物可用于该疾病的对症治疗,包括胍、氨基吡啶或乙酰胆碱酯酶抑制剂。其他疗法旨在清除血清自身抗体或抑制免疫系统。为此,有免疫调节策略,如静脉注射免疫球蛋白或血浆置换,或几种免疫抑制剂可供使用。化疗已成功改善了患有潜在肿瘤的兰伯特-伊顿肌无力综合征患者的病程。