Tankisi H, Pugdahl K, Johnsen B, Camdessanché J P, de Carvalho M, Fawcett P, Labarre-Vila A, Liguori R, Nix W, Schofield I, Fuglsang-Frederiksen A
Department of Clinical Neurophysiology, Aarhus University Hospital, Aarhus, Denmark.
Department of Neurology, Saint-Etienne University Hospital, Saint-Etienne, France.
Clin Neurophysiol Pract. 2025 Mar 7;10:79-83. doi: 10.1016/j.cnp.2025.02.011. eCollection 2025.
Electrodiagnostic testing plays an important role in diagnosing disorders of neuromuscular transmission (NMT), especially in seronegative myasthenia gravis. However, electrodiagnostic criteria for the diagnosis are sparse. This study aimed at inferring evidence-based recommendations for the electrodiagnostic examination of NMT disorders.
A total of 164 cases with a consensus diagnosis of NMT disorder obtained by peer review by eight experienced neurophysiologists were analysed for differences in examination strategy, the sensitivity of different tests, and inferring minimal criteria. The diagnostic performance of the suggested criteria was validated on 24 MG patients and 50 patients with neuropathy (17), myopathy (15), or fatigue (18).
We recommend as minimal electrodiagnostic criteria for NMT disorders, either (a) 2 abnormal repetitive nerve stimulation (RNS), (b) 1 abnormal RNS and 1 abnormal single fiber electromyography (SFEMG) or (c) 2 abnormal SFEMG. These showed a good diagnostic performance with a sensitivity of 87.5 % and a specificity of 100 %.
Recommendations with high diagnostic sensitivity and specificity for the minimum number of RNS and SFEMG studies to diagnose NMT disorders developed by an international consensus group are suggested.
The suggested electrodiagnostic recommendations for diagnosing NMT disorders are reliable and suitable for use at different centres.
电诊断测试在神经肌肉传递障碍（NMT）的诊断中起着重要作用，尤其是在血清阴性重症肌无力中。然而，诊断的电诊断标准却很稀少。本研究旨在推断出基于证据的NMT障碍电诊断检查建议。
对164例经8位经验丰富的神经生理学家同行评审达成共识诊断为NMT障碍的病例进行分析，以探讨检查策略的差异、不同测试的敏感性，并推断出最低标准。在24例重症肌无力患者和50例患有神经病变（17例）、肌病（15例）或疲劳（18例）的患者中验证了所建议标准的诊断性能。
我们建议将以下情况作为NMT障碍的最低电诊断标准：（a）2次重复神经刺激（RNS）异常，（b）1次RNS异常和1次单纤维肌电图（SFEMG）异常，或（c）2次SFEMG异常。这些标准显示出良好的诊断性能，敏感性为87.5%，特异性为100%。
建议采用国际共识小组制定的关于诊断NMT障碍所需的最低RNS和SFEMG研究次数的具有高诊断敏感性和特异性的建议。
所建议的用于诊断NMT障碍的电诊断建议是可靠的，适用于不同中心。
