Blijham Paul J, Schelhaas H Jurgen, Ter Laak Henk J, van Engelen Baziel G M, Zwarts Machiel J
Department of Clinical Neurophysiology, Institute of Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
J Neurol Sci. 2007 Dec 15;263(1-2):154-7. doi: 10.1016/j.jns.2007.07.008. Epub 2007 Jul 25.
We prospectively investigated whether early diagnosis of amyotrophic lateral sclerosis (ALS) could be facilitated by demonstrating signs of denervation in a muscle of a clinical and electromyographical unaffected region. Muscle fibre conduction velocity (MFCV) was determined in 18 patients in whom the diagnosis ALS was considered but not established beyond a level of clinically possible ALS according to the revised El Escorial criteria. A muscle biopsy was obtained from the same muscle, to demonstrate neurogenic changes. The study followed the guidelines from the STARD initiative.
Results were analysed with respect to the final diagnosis. After a mean follow-up of 16 months, 9 patients developed probable or definite ALS. Sensitivity of abnormal MFCV for developing ALS was 89%. Muscle biopsy confirmed that denervation was the cause of abnormal MFCV. We concluded that MFCV can be used to detect denervation in muscles that show no clinical or electromyographical signs of lower motor neuron disease, and thus may contribute to early diagnosis of probable laboratory-supported ALS.
我们前瞻性地研究了通过在临床和肌电图未受影响区域的肌肉中显示失神经支配迹象，是否能够促进肌萎缩侧索硬化症（ALS）的早期诊断。在18例根据修订的埃尔埃斯科里亚尔标准被怀疑患有ALS但尚未确诊至临床可能的ALS程度的患者中，测定了肌纤维传导速度（MFCV）。从同一肌肉获取肌肉活检样本，以显示神经源性改变。本研究遵循了STARD倡议的指南。
根据最终诊断对结果进行分析。平均随访16个月后，9例患者发展为可能或确诊的ALS。MFCV异常对发展为ALS的敏感性为89%。肌肉活检证实失神经支配是MFCV异常的原因。我们得出结论，MFCV可用于检测未显示下运动神经元疾病临床或肌电图体征的肌肉中的失神经支配，因此可能有助于早期诊断可能的实验室支持的ALS。
