Yamada A, Takeuchi H, Touge T, Miki H, Kuwabara H
Third Department of Internal Medicine, Kagawa Medical School.
Rinsho Shinkeigaku. 1990 Jul;30(7):780-3.
A 63-year-old man with Eaton-Lambert syndrome manifested by marked respiratory failure was reported. He began to notice blepharoptosis and diplopia in September, 1987, followed by weakness and easy fatigability in bulbar, neck and limb muscles in association with impotence by February, 1988. On admission in August, 1988, Gowers' sign, decreased tendon reflexes and muscle weakness improved by the injection of edrophonium were found. Anti-acetylcholine receptor antibody was negative. Single muscle action potential evoked in the thenar muscle was abnormally low in amplitude with the stimulation of the median nerve: repetitive nerve stimulation study revealed the waning at the low rates, but the waxing at the high rate (30 Hz), suggesting the diagnosis of Eaton-Lambert syndrome. Early gastric cancer (adenocarcinoma) was diagnosed from needle biopsy specimens in August, 1988, but no other neoplasm including thymoma or lung cancer was found. After subtotal gastric resection in September, 1988, he failed into respiratory failure, requiring artificial ventilation for seven months. Plasmapheresis and drugs such as anticholinesterase, guanidine hydrochloride, and corticosteroid were ineffective for the recovery from weakness in respiratory muscle. Lung cancer was suspected, based on a chest X-ray in March, 1989, and one month later he died of pneumonia. At autopsy, small cell carcinoma of the lung was observed, but there was neither recurrence nor metastasis of the gastric cancer. Emphasis was placed on the respiratory failure in Eaton-Lambert syndrome which has rarely been reported.
报告了一名63岁患有伊顿 - 兰伯特综合征并伴有明显呼吸衰竭的男性患者。他于1987年9月开始出现上睑下垂和复视，随后到1988年2月，延髓、颈部和肢体肌肉出现无力及易疲劳症状，并伴有阳痿。1988年8月入院时，发现有Gowers征、腱反射减弱，注射依酚氯铵后肌肉无力症状有所改善。抗乙酰胆碱受体抗体呈阴性。刺激正中神经时，拇短展肌诱发的单肌动作电位幅度异常低；重复神经电刺激研究显示，低频刺激时波幅递减，而高频（30Hz）刺激时波幅递增，提示诊断为伊顿 - 兰伯特综合征。1988年8月经针吸活检标本诊断为早期胃癌（腺癌），但未发现包括胸腺瘤或肺癌在内的其他肿瘤。1988年9月行胃次全切除术后，他陷入呼吸衰竭，需要人工通气7个月。血浆置换以及抗胆碱酯酶、盐酸胍和皮质类固醇等药物对呼吸肌无力的恢复均无效。基于1989年3月的胸部X线检查怀疑有肺癌，1个月后他死于肺炎。尸检时发现有肺小细胞癌，但胃癌无复发及转移。重点强调了伊顿 - 兰伯特综合征中很少被报道的呼吸衰竭情况。
