Chaudhry V, Watson D F, Bird S J, Cornblath D R
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD.
Muscle Nerve. 1991 Dec;14(12):1227-30. doi: 10.1002/mus.880141215.
The Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune disorder of neuromuscular transmission. Electrodiagnosis is confirmed by an increase in compound muscle action potential amplitude during high-frequency repetitive nerve stimulation or following brief exercise. We describe the results of stimulated single-fiber electromyography in 4 patients with disorders of neuromuscular transmission: LEMS (2), LEMS/myasthenia gravis (MG) overlap (1), and MG (1). Stimulated SFEMG was performed in the extensor digitorum communis muscle with axonal intramuscular suprathreshold stimulation at low and high rates. In all 4 patients, a rate dependence of jitter was found. In LEMS and LEMS/MG, jitter and blocking improved with high stimulation rates, as compared with the opposite effect in MG. We conclude that stimulated SFEMG is a valuable technique in the diagnosis of LEMS.
兰伯特-伊顿肌无力综合征(LEMS)是一种神经肌肉传递的自身免疫性疾病。高频重复神经刺激期间或短暂运动后复合肌肉动作电位幅度增加可确诊电诊断。我们描述了4例神经肌肉传递障碍患者的刺激单纤维肌电图结果:LEMS(2例)、LEMS/重症肌无力(MG)重叠(1例)和MG(1例)。在指总伸肌进行刺激单纤维肌电图检查,采用轴突肌内阈上刺激,分别以低频率和高频率进行。在所有4例患者中,均发现了颤抖的频率依赖性。与MG中相反的效应相比,在LEMS和LEMS/MG中,高刺激频率时颤抖和阻滞得到改善。我们得出结论,刺激单纤维肌电图是诊断LEMS的一项有价值的技术。