Rondepierre P, Furby A, Godefroy O, Hurtevent J F, Destée A, Fourrier F, Leys D
Service de Neurologie B, Centre hospitalo-universitaire de Lille.
Rev Neurol (Paris). 1992;148(3):193-9.
We report a new case of neuromuscular block overlap between Myasthenia Gravis and Eaton-Lambert syndrome. A 64-year-old man with a 4-months history of gait disturbance was admitted for ophthalmoplegia worsening during exercise and decreasing at rest. Clinical examination after exercise, revealed limbs weakness and areflexia, palsy of the left eye abduction and a left ptosis. The level of anti-acetylcholin-receptor antibodies was high. Electrophysiological explorations revealed a decrement at 3 Hz and a increment at 30 Hz, with a reduction in amplitude of the initial motor potential. This patient improved under a combination of guanidine and anticholinesterase drugs. From this case and 9 previously reported cases, we propose 4 criteria for the diagnosis of such neuromuscular blocks: 1) exercising symptoms and signs, including areflexia, 2) presence of anti-acetylcholin-receptor antibodies, 3) reduction of the amplitude of the initial motor potential, with a decrement at 3 Hz, and an increment at 30 Hz, and 4) clinical and electrophysiological improvement under guanidine and anticholinesterasic drugs therapy.
我们报告了一例重症肌无力和伊顿-兰伯特综合征之间神经肌肉阻滞重叠的新病例。一名64岁男性，有4个月步态障碍病史，因运动时眼肌麻痹加重、休息时减轻而入院。运动后的临床检查发现肢体无力、腱反射消失、左眼外展麻痹和左侧上睑下垂。抗乙酰胆碱受体抗体水平较高。电生理检查显示3Hz时波幅递减、30Hz时波幅递增，初始运动电位波幅降低。该患者在胍和抗胆碱酯酶药物联合治疗下病情改善。结合该病例及之前报道的9例病例，我们提出了诊断此类神经肌肉阻滞的4条标准：1）运动时的症状和体征，包括腱反射消失；2）存在抗乙酰胆碱受体抗体；3）初始运动电位波幅降低，3Hz时波幅递减，30Hz时波幅递增；4）在胍和抗胆碱酯酶药物治疗下临床和电生理表现改善。
