McEvoy K M
Department of Neurology, Mayo Clinic and Foundation, Rochester, Minnesota.
Neurol Clin. 1994 May;12(2):387-99.
Lambert-Eaton myasthenic syndrome is a rare autoimmune neuromuscular and autonomic disease that produces fluctuating muscle weakness, hyporeflexia, and autonomic dysfunction, and often is associated with small-cell lung cancer. The pathophysiology is understood quite well; antibodies to voltage-gated calcium channels in motor and autonomic nerve terminals disrupt calcium influx and reduce acetylcholine release. The diagnosis may be suspected clinically, but must be confirmed with electrophysiologic testing. Initial and then periodic screening for malignancy is essential. Tumors other than small-cell lung cancer occasionally are found. Effective tumor treatment may induce remission. Active disease may respond to agents that enhance neuromuscular transmission or to immunosuppression. Combined therapy frequently is needed and control is often marginal despite this. It is expected that availability of 3,4-DAP will improve significantly the response to treatment in most patients.
兰伯特-伊顿肌无力综合征是一种罕见的自身免疫性神经肌肉和自主神经疾病，会导致肌肉无力波动、反射减退和自主神经功能障碍，且常与小细胞肺癌相关。其病理生理学已被充分了解；运动和自主神经末梢中电压门控钙通道的抗体破坏钙内流并减少乙酰胆碱释放。临床可能怀疑该诊断，但必须通过电生理检查来确诊。初始及随后定期筛查恶性肿瘤至关重要。偶尔会发现除小细胞肺癌以外的肿瘤。有效的肿瘤治疗可能诱导缓解。活动性疾病可能对增强神经肌肉传递的药物或免疫抑制治疗有反应。尽管如此，通常需要联合治疗且控制效果往往有限。预计3,4-二氨基吡啶的可用性将显著改善大多数患者的治疗反应。
