QUANTITATIVE ELECTROPHYSIOLOGICAL STUDY OF MOTOR NEURON DISEASE
QUANTITATIVE ELECTROPHYSIOLOGICAL STUDY OF MOTOR NEURON DISEASE
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DOI：
10.1136/jnnp.41.9.773
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发表时间：
1978-01-01
影响因子：
11
通讯作者：
BALLANTYNE, JP
中科院分区：
文献类型：
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作者：
HANSEN, S;BALLANTYNE, JP
使用定量电生理学技术对患有运动神经元疾病的患者 (32) 进行了研究。给出了趾短伸肌中幸存运动单位数量的估计以及这些单位的电生理参数的测量值以及运动神经传导速度的值。运动神经元疾病中的神经再支配足以完全补偿供应肌肉的运动神经元池的高达 50% 的损失。神经重新支配的能力大于许多神经病中发现的能力，但神经重新支配的效率随着存活运动单位数量的下降而降低。当 5% 或更少的运动单位仍然存活时，神经支配似乎就会停止。没有电生理学证据表明快速传导轴突优先丧失、病理性传导减慢或影响运动轴突的死亡过程。进行性肌萎缩症和肌萎缩侧索硬化症的电生理参数比较没有显着差异。根据结果讨论了潜在的病理生理机制。
Patients (32) with motor neuron disease were investigated using quantitative electrophysiological techniques. Estimates of the number of surviving motor units in the extensor digitorum brevis muscle and measurements of the electrophysiological parameters of these units are presented along with the values for motor nerve conduction velocities. Reinnervation in motor neuron disease is sufficient to compensate completely for the loss of up to 50% of the motor neuron pool supplying the muscle. The capacity for reinnervation is greater than that found in a number of neuropathies but the efficiency of reinnervation decreases as the number of surviving motor units falls. Reinnervation appears to cease when 5% or less of the motor units remain viable. There is no electrophysiological evidence of a preferential loss of fast conducting axons, of pathological slowing of conduction nor of a dying-back process affecting the motor axon. Comparison of the electrophysiological parameters in progressive muscular atrophy and amyotrophic lateral sclerosis shows no significant differences. The underlying pathophysiological mechanisms are discussed in terms of the results.