Trojaborg W, Wulff C H
Laboratory of Clinical Neurophysiology, Rigshospitalet (the National Hospital) Copenhagen, Denmark.
Acta Neurol Scand. 1994 Mar;89(3):214-9. doi: 10.1111/j.1600-0404.1994.tb01664.x.
We examined 8 men with X-linked recessive bulbospinal neuronopathy. Quantitative electromyography showed large amplitude motor unit action potentials of prolonged duration and increased polyphasia. There was a pronounced loss of motor units in all but one muscle at maximal volition even in muscles with normal or only mildly decreased force. Denervation activity was present in 53% sampled limb muscles, and fasciculation was recorded in 33% of limb muscles. Nerve conduction studies showed small amplitude sensory action potentials in 6 out of 8 patients. The motor and sensory conduction velocity was normal or borderline slow. The electrophysiologic findings were consistent with chronic partial denervation (motor axonopathy) combined with large fibre sensory axonopathy.
我们研究了8名患有X连锁隐性延髓脊髓神经元病的男性。定量肌电图显示运动单位动作电位波幅大、时限延长且多相波增多。即使在肌力正常或仅轻度减弱的肌肉中，在最大意志收缩时，除一块肌肉外，所有肌肉均有明显的运动单位丢失。53%的采样肢体肌肉出现失神经活动，33%的肢体肌肉记录到肌束震颤。神经传导研究显示，8例患者中有6例感觉动作电位波幅小。运动和感觉传导速度正常或临界减慢。电生理结果符合慢性部分失神经（运动轴索性神经病）合并大纤维感觉轴索性神经病。
