Léger J M, Bachoud-Lévi A C, Eymard B, Théodore C, Bouche P, Pierrot-Deseilligny C
Service de Neurologie et Service d'Explorations Fonctionnelles Neurologie, Clinique Paul Castaigne, Hôpital de la Salpêtrière, Paris.
Rev Neurol (Paris). 1993;149(8-9):485-8.
We report a case of neuromuscular disease overlap between myasthenia gravis and Lambert-Eaton syndrome (LES). Clinical features were those of LES and occurred insidiously in this 68-year old man: proximal weakness predominant in the lower limbs, generalized areflexia, dryness of the mouth and partial right eye palsy. Investigations disclosed a small cell lung cancer. On the other hand, an electrophysiological study showed low amplitude of all motor evoked potentials, and significant decrement in the median nerve at repeated 3 Hz stimulation, but failed to disclose any increment of the motor evoked potential in abductor digiti minimi pedis muscle after both maximal voluntary contraction and repeated 20 Hz stimulation. In addition, the patient improved under anticholinesterase drugs, but failed to respond to guanidine. Titres for both anti-acetylcholine-receptor antibodies and calcium channel antibodies were negative. The relationship between our case and recently reported cases of co-existence of the Lambert-Eaton myasthenic syndrome and myasthenia gravis is discussed.
我们报告一例重症肌无力与兰伯特-伊顿综合征（LES）重叠的神经肌肉疾病病例。该68岁男性临床特征符合LES，起病隐匿：下肢近端无力为主，全身腱反射消失，口干及右眼部分性麻痹。检查发现小细胞肺癌。另一方面，电生理研究显示所有运动诱发电位波幅降低，正中神经在3Hz重复刺激时显著递减，但在小趾展肌最大自主收缩及20Hz重复刺激后运动诱发电位未出现递增。此外，患者对抗胆碱酯酶药物治疗有反应，但对胍无反应。抗乙酰胆碱受体抗体和钙通道抗体滴度均为阴性。本文讨论了我们的病例与最近报道的兰伯特-伊顿肌无力综合征和重症肌无力共存病例之间的关系。
