Wirtz P W, Kuks J B, Wintzen A R, Verschuuren J J
Leids Universitair Medisch Centrum, afd. Neurologie, Postbus 9600, 2300 RC Leiden.
Ned Tijdschr Geneeskd. 2001 Jan 13;145(2):57-61.
Three patients with Lambert-Eaton myasthenic syndrome (LEMS), two men aged 61 and 64 and a woman aged 55 years, all developed proximal weakness, depressed tendon reflexes and autonomic dysfunction. Although this clinical triad is highly suggestive for LEMS, the disorder had not been recognized initially. The woman had a small-cell bronchial carcinoma, treated successfully by chemotherapy, whereafter the LEMS symptoms gradually disappeared. The first man was treated with 3,4-diaminopyridine and azathioprine, whereupon his symptoms diminished. The other man had only slight complaints and refused drug treatment. The three cases illustrate that presentation and course of LEMS can vary between patients. Furthermore, clinical and electrophysiological features can suggest myasthenia gravis, myopathy or axonal polyneuropathy. Therapeutic options and the risk of underlying malignancy make early diagnosis important. In conclusion, in every patient presenting with unexplained proximal weakness, LEMS should be considered, especially if depressed tendon reflexes and autonomic dysfunction are found as well.
三名患有兰伯特-伊顿肌无力综合征（LEMS）的患者，两名男性，年龄分别为61岁和64岁，一名女性，年龄55岁，均出现近端肌无力、腱反射减弱和自主神经功能障碍。虽然这一临床三联征高度提示LEMS，但该疾病最初未被识别。该女性患有小细胞支气管癌，经化疗成功治疗，此后LEMS症状逐渐消失。第一名男性接受了3,4-二氨基吡啶和硫唑嘌呤治疗，症状随之减轻。另一名男性只有轻微症状，拒绝药物治疗。这三个病例表明，LEMS在不同患者中的表现和病程可能有所不同。此外，临床和电生理特征可能提示重症肌无力、肌病或轴索性多发性神经病。治疗选择和潜在恶性肿瘤的风险使得早期诊断很重要。总之，对于每一位出现不明原因近端肌无力的患者，都应考虑LEMS，尤其是如果同时发现腱反射减弱和自主神经功能障碍。
