Jiang Jung-Rern, Shih Jin-Yuan, Wang Hao-Chien, Wu Ruey-Meei, Yu Chong-Jen, Yang Pan-Chyr
Department of Internal Medicine, National Taiwan University Hospital, 7 Chung-Shan South Road, Taipei, Taiwan.
J Formos Med Assoc. 2002 Dec;101(12):871-4.
Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disorder characterized by defective neurotransmitter release at presynaptic terminals. It is caused by an IgG autoantibody reacting against voltage-gated calcium channels. Severe LEMS complicated by ventilatory failure is rare. We report a case of small-cell lung cancer (SCLC) presenting with LEMS and ventilatory failure in a 67-year-old man who initially presented with progressive limb weakness for 6 months and tachypnea with shallow breathing for 1 week. LEMS was diagnosed through electrophysiologic studies. Chest radiography and computerized tomography showed a huge mass lesion over the left anterior and middle mediastinum with an encasement of the left pulmonary artery. Cytologic examination of ultrasound-guided fine needle aspiration disclosed SCLC. Successful treatment in combination with plasma exchange and chemotherapy resulted in dramatic tumor regression and LEMS remission, which were confirmed by chest radiography and electrophysiologic studies. This case suggests that plasma exchange and chemotherapy can be effective in treating SCLC with severe LEMS that produces ventilatory failure.
兰伯特-伊顿肌无力综合征(LEMS)是一种神经肌肉疾病,其特征是突触前终末神经递质释放缺陷。它由一种针对电压门控钙通道的IgG自身抗体引起。并发呼吸衰竭的严重LEMS较为罕见。我们报告一例67岁男性小细胞肺癌(SCLC)合并LEMS及呼吸衰竭的病例,该患者最初表现为进行性肢体无力6个月,呼吸急促伴浅呼吸1周。通过电生理研究诊断为LEMS。胸部X线和计算机断层扫描显示左前纵隔和中纵隔有巨大肿块病变,左肺动脉被包绕。超声引导下细针穿刺的细胞学检查发现为SCLC。血浆置换和化疗联合成功治疗导致肿瘤显著消退和LEMS缓解,胸部X线和电生理研究证实了这一点。该病例表明,血浆置换和化疗可有效治疗伴有严重LEMS并导致呼吸衰竭的SCLC。