Wallgren-Pettersson C, Sainio K, Salmi T
Children's Hospital, University of Helsinki, Finland.
Muscle Nerve. 1989 Jul;12(7):587-93. doi: 10.1002/mus.880120710.
To clarify the discrepancies between earlier reports of electromyography (EMG) in congenital nemaline myopathy (CNM), conventional electromyography was done on 13 patients with CNM, and results were compared with those of 18 earlier EMG examinations of the same patients. Fiber density was measured in 10 patients with a computerized method and neuromuscular jitter in 3 with single-fiber EMG. With age, the EMG abnormality progressed, and "neuropathic" EMG features developed in distal muscles. In 9 of 10 patients fiber density was higher than normal. In two of three patients jitter was abnormal. Motor (13 of 13 patients) and sensory (3 of 3 patients) nerve conduction velocities were normal. Our results seem to explain the conflicting reports of EMG in CNM. We conclude that active degeneration and regeneration of muscle fibers takes place in CNM and suggest that the "neuropathic" motor unit potentials seen in our patients may be secondary to myopathic disease activity.
为了澄清先天性杆状体肌病（CNM）早期肌电图（EMG）报告之间的差异，对13例CNM患者进行了常规肌电图检查，并将结果与之前对同一患者进行的18次EMG检查结果进行比较。采用计算机化方法对10例患者测量了纤维密度，采用单纤维肌电图对3例患者测量了神经肌肉抖动。随着年龄增长，肌电图异常进展，远端肌肉出现“神经病变性”肌电图特征。10例患者中有9例纤维密度高于正常。3例患者中有2例抖动异常。运动神经传导速度（13例患者均正常）和感觉神经传导速度（3例患者均正常）均正常。我们的结果似乎解释了CNM中肌电图相互矛盾的报告。我们得出结论，CNM中发生了肌纤维的主动变性和再生，并表明我们患者中所见的“神经病变性”运动单位电位可能继发于肌病活动。
