Nakajima M, Eisen A, McCarthy R, Olney R K, Aminoff M J
Neuromuscular Diseases Unit, Vancouver General Hospital, British Columbia, Canada.
Neurology. 1996 Dec;47(6):1555-61. doi: 10.1212/wnl.47.6.1555.
We studied excitatory postsynaptic potentials (EPSPs) arising in single spinal motoneurons (composite EPSPs) induced by Ia afferent and magnetic cortical stimulation in 28 normal subjects ranging in age from 24 to 84 years and 28 patients with amyotrophic lateral sclerosis (ALS) aged 34 to 82 years. The subjects voluntarily recruited single motor units of the first dorsal interosseous muscle. Using peristimulus time histograms, we determined changes in the firing probability of the first dorsal interosseous motor units and measured the magnitude of the EPSP. An early period of increased firing probability (primary peak) occurred at approximately 30 msec after la afferent and 25 msec after cortical stimulation, reflecting underlying EPSPs arising in spinal motoneurons induced by either projection. The latency of the primary peaks for both Ia afferent and cortical stimulation was mildly prolonged in ALS, suggesting a loss of the fastest-conducting spinal motoneurons. Patients with ALS had la afferent-driven EPSPs whose amplitude and rise time were equivalent to those of normal subjects. However, the ratio of cortical to la afferent-driven composite EPSPs in ALS was significantly lower than that for normal subjects. Fourteen of 28 ALS motor units had cortically driven EPSPs that were small or large only because of a prolonged rise time. The findings suggest that in ALS, corticomotoneuronal attrition or dispersion of the descending volley occurs in the presence of normally functioning spinal motoneurons to which they project.
我们研究了28名年龄在24至84岁之间的正常受试者以及28名年龄在34至82岁之间的肌萎缩侧索硬化症(ALS)患者,由Ia传入神经和磁皮层刺激诱发的单个脊髓运动神经元产生的兴奋性突触后电位(EPSP,复合EPSP)。受试者自主募集第一背侧骨间肌的单个运动单位。使用刺激时间直方图,我们确定了第一背侧骨间肌运动单位放电概率的变化,并测量了EPSP的大小。在Ia传入神经刺激后约30毫秒和皮层刺激后25毫秒出现了一个放电概率增加的早期阶段(初级峰值),反映了由任一投射诱发的脊髓运动神经元中潜在的EPSP。在ALS患者中,Ia传入神经和皮层刺激的初级峰值潜伏期均轻度延长,提示传导速度最快的脊髓运动神经元丧失。ALS患者的Ia传入神经驱动的EPSP,其幅度和上升时间与正常受试者相当。然而,ALS患者中皮层驱动与Ia传入神经驱动的复合EPSP的比值显著低于正常受试者。28个ALS运动单位中有14个具有皮层驱动的EPSP,其大小仅因上升时间延长而增大或减小。这些发现表明,在ALS中,皮质运动神经元的损耗或下行冲动的离散发生在其投射的脊髓运动神经元功能正常的情况下。