Ferroir J P, Milleron B, Ropert A, Billy C, Nahum L, Tcherakian S, Carette M F
Service de Neurologie, Hôpital Tenon, Paris.
Rev Pneumol Clin. 1999 Jun;55(3):168-70.
We report a case of paraneoplastic myasthenic syndrome with clinical features suggesting Lambert Eaton syndrome but without the electromyographic elements required for diagnosis. Anti-calcium channel antibodies were also lacking. The electromyogram evidenced a block and the Tensilon test was positive. The efficacy of anticholinesterases argued in favor of myasthenia but anti-acetylcholine receptor antibodies were negative. The block was more of a mixed nature, involving both presynaptic transmission as in Lambert Eaton syndrome and post-synaptic transmission as in paraneoplastic myasthenia. The primary tumor was identified as a small-cell neuroendocrine lung carcinoma on mediastinal biopsies obtained directly on CT-scan guided puncture of a mediastinal node. Thoracotomy was thus avoided. The Lambert Eaton syndrome is a paraneoplastic manifestation of small-cell lung cancer in 50% of the cases unlike generalized myasthenia which apparently is never associated with small-cell lung cancer. A mixed paraneoplastic neuro-muscle junction disorder with aspects of each can be exceptionally observed.
我们报告一例副肿瘤性肌无力综合征病例,其临床特征提示兰伯特-伊顿综合征,但缺乏诊断所需的肌电图表现,也未检测到抗钙通道抗体。肌电图显示有阻滞,腾喜龙试验呈阳性。抗胆碱酯酶药物有效提示为重症肌无力,但抗乙酰胆碱受体抗体为阴性。该阻滞更具混合性质,既涉及兰伯特-伊顿综合征中的突触前传递,也涉及副肿瘤性肌无力中的突触后传递。通过CT引导下直接穿刺纵隔淋巴结获得的纵隔活检,将原发肿瘤确诊为小细胞神经内分泌肺癌,从而避免了开胸手术。兰伯特-伊顿综合征在50%的病例中是小细胞肺癌的副肿瘤表现,与之不同的是,全身型重症肌无力显然从未与小细胞肺癌相关联。可以罕见地观察到一种兼具两者特征的混合性副肿瘤性神经肌肉接头疾病。