Verma Sumit, Lin Jenny, Travers Curtis, McCracken Courtney, Shah Durga
Department of Pediatrics, Emory University School of Medicine, Atlanta, Georgia, 30307, USA.
Department of Neurology, Emory University School of Medicine, Atlanta, Georgia, USA.
Muscle Nerve. 2017 Dec;56(6):1168-1171. doi: 10.1002/mus.25678. Epub 2017 May 24.
This study's objective was to evaluate quantitative electromyography (QEMG) using multiple-motor-unit (multi-MUP) analysis in Duchenne muscular dystrophy (DMD).
Ambulatory DMD boys, aged 5-15 years, were evaluated with QEMG at 6-month intervals over 14 months. EMG was performed in the right biceps brachii (BB) and tibialis anterior (TA) muscles. Normative QEMG data were obtained from age-matched healthy boys. Wilcoxon signed-rank tests were performed.
Eighteen DMD subjects were enrolled, with a median age of 7 (interquartile range 7-10) years. Six-month evaluations were performed on 14 subjects. QEMG showed significantly abnormal mean MUP duration in BB and TA muscles, with no significant change over 6 months.
QEMG is a sensitive electrophysiological marker of myopathy in DMD. Preliminary data do not reflect a significant change in MUP parameters over a 6-month interval; long-term follow-up QEMG studies are needed to understand its role as a biomarker for disease progression. Muscle Nerve 56: 1361-1364, 2017.
本研究的目的是在杜氏肌营养不良症(DMD)中使用多运动单位(multi-MUP)分析评估定量肌电图(QEMG)。
对年龄在5至15岁的门诊DMD男孩,在14个月内每隔6个月进行一次QEMG评估。在右侧肱二头肌(BB)和胫前肌(TA)肌肉进行肌电图检查。从年龄匹配的健康男孩中获取QEMG规范数据。进行了Wilcoxon符号秩检验。
招募了18名DMD受试者,中位年龄为7岁(四分位间距7 - 10岁)。对14名受试者进行了为期6个月的评估。QEMG显示BB和TA肌肉的平均运动单位动作电位(MUP)持续时间明显异常,6个月内无显著变化。
QEMG是DMD中肌病的敏感电生理标志物。初步数据未反映6个月间隔内MUP参数的显著变化;需要长期随访QEMG研究以了解其作为疾病进展生物标志物的作用。《肌肉与神经》56: 1361 - 1364, 2017年。