Motomura M, Iwanaga H
First Department of Internal Medicine, Nagasaki University School of Medicine.
Clin Calcium. 2001 Nov;11(11):1468-74.
Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of neuromuscular and autonomic transmission in which IgG autoantibodies lead to presynaptic voltage-gated calcium channel (VGCC) loss, or as a paraneoplastic disorder in association with small cell lung carcinoma (SCLC). Recent results strongly suggest that the antibodies to P/Q-type VGCC are the principal pathogenic factors in LEMS. Here, we present diagnosis and treatment of LEMS patients. Proximal weakness, depressed tendon reflexes, autonomic symptoms, and electrical posttetanic potentiation together are essential to accurately diagnose LEMS. The diagnosis is established immunologically by the presence of anti-P/Q-type VGCC antibodies, detected using the (125)I-omega-conotoxin MVIIC radioimmunoassay, which will be present in 85% of LEMS patients. The drug 3,4-diaminopyridine with anti-cholinesterase inhibitor is most effective in LEMS patients with or without SCLC. In LEMS with SCLC, specific tumor therapy will often improve the neurological disorder. In some cases plasmapheresis or intravenous immunoglobulin may be indicated.
兰伯特-伊顿肌无力综合征（LEMS）是一种神经肌肉和自主神经传递的自身免疫性疾病，其中IgG自身抗体导致突触前电压门控钙通道（VGCC）丧失，或作为与小细胞肺癌（SCLC）相关的副肿瘤性疾病。最近的结果强烈表明，抗P/Q型VGCC抗体是LEMS的主要致病因素。在此，我们介绍LEMS患者的诊断和治疗。近端肌无力、腱反射减弱、自主神经症状以及强直后电位增强共同对准确诊断LEMS至关重要。通过使用（125）I-ω-芋螺毒素MVIIC放射免疫测定法检测到抗P/Q型VGCC抗体，从免疫学角度确立诊断，85%的LEMS患者会出现该抗体。对于伴有或不伴有SCLC的LEMS患者，药物3,4-二氨基吡啶与抗胆碱酯酶抑制剂最为有效。对于伴有SCLC的LEMS患者，特异性肿瘤治疗通常会改善神经功能障碍。在某些情况下，可能需要进行血浆置换或静脉注射免疫球蛋白。
