Department of Clinical Neurophysiology, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands.
Department of Neurology, Erasmus MC, University Medical Centre Rotterdam, Rotterdam, The Netherlands.
J Neurol Neurosurg Psychiatry. 2015 Nov;86(11):1234-9. doi: 10.1136/jnnp-2014-308589. Epub 2014 Dec 24.
To determine and compare the diagnostic accuracy of electrically elicited multiplet discharges (MDs) and fasciculation potentials (FPs) in motor neuron disease (MND).
Patients were eligible when they had MND in their differential diagnosis and were referred for electromyogram (EMG). Stimulated high-density surface EMG of the thenar muscles was performed on the same day as standard EMG examination. High-density recordings were analysed for presence of MDs and needle EMG of any muscle investigated in the cervical region for presence of FPs.
Of the 61 patients enrolled in this diagnostic study, 24 patients were clinically diagnosed with amyotrophic lateral sclerosis (ALS) and 11 patients with progressive muscular atrophy (PMA). Another diagnosis was made in 26 patients. Sixteen patients in whom MDs were detected were diagnosed with either ALS (n = 11) or PMA (n = 5; sensitivity = 47.1%, PPV = 94.1%). MDs were detected in only one patient initially diagnosed with PMA, but in whom later on, multifocal motor neuropathy could not be excluded (specificity = 96.2%). Electrically elicited MDs had a higher specificity than FPs (96.2% vs 53.9%, p < 0.001, n = 26) and lower sensitivity (47.1% vs 85.3%, p = 0.002, n = 34). When considering presence of MDs in MND as neurogenic EMG abnormality, lower motor neuron involvement of ≥ 1 EMG region increased from 50% to 73.5% (p = 0.008, n = 34).
Electrically evoked MDs are highly specific for ALS and PMA and are an early sign of lower motor neuron dysfunction.
确定并比较电诱发多相放电（MDs）和肌束震颤电位（FPs）在运动神经元病（MND）中的诊断准确性。
患者在鉴别诊断中存在 MND 且被转介进行肌电图（EMG）检查时符合入组条件。同日进行鱼际肌的受刺激高密度表面 EMG 检查，并对所有接受颈椎针极肌电图检查的肌肉进行 FPs 检查。
在这项诊断研究中，共有 61 名患者入组，其中 24 名患者临床诊断为肌萎缩侧索硬化症（ALS），11 名患者为进行性肌萎缩症（PMA）。另外 26 名患者的诊断结果不同。在 16 名检测到 MDs 的患者中，11 名被诊断为 ALS 或 PMA（敏感性=47.1%，PPV=94.1%）。MDs 仅在最初诊断为 PMA 的 1 名患者中被检测到，但后来不能排除多灶性运动神经病（特异性=96.2%）。与 FPs 相比，电诱发 MDs 的特异性更高（96.2% vs 53.9%，p<0.001，n=26），而敏感性更低（47.1% vs 85.3%，p=0.002，n=34）。当将 MND 中的 MDs 视为神经源性 EMG 异常时，≥1 个 EMG 区域的下运动神经元受累从 50%增加到 73.5%（p=0.008，n=34）。
电诱发 MDs 对 ALS 和 PMA 具有高度特异性，是下运动神经元功能障碍的早期征象。
