Evolution of motor and sensory deficits in amyotrophic lateral sclerosis estimated by neurophysiological techniques
Evolution of motor and sensory deficits in amyotrophic lateral sclerosis estimated by neurophysiological techniques
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DOI：
10.1007/s004150050379
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发表时间：
1999-06-01
影响因子：
6
通讯作者：
Robberecht, W
中科院分区：
文献类型：
--
作者：
Al Theys, P;Peeters, E;Robberecht, W
尽管肌萎缩侧索硬化症是一种上下运动神经元的退行性疾病，但有证据表明该疾病可以影响其他系统，包括感觉系统。另一方面，在运动神经元池内，传导速度最快的运动神经元纤维（MNFCV）可能倾向于发生退化过程。我们通过前瞻性评估几种感觉指标并研究脊髓运动神经元损失的选择性，对 50 名患者的疾病的这两个方面进行了研究。在基线时，神经传导研究和体感诱发电位显示外周和中枢感觉通路异常减慢。加热的热阈值升高，但冷却的热阈值正常。超过 60% 的患者至少有一项所研究的感官测试出现异常。然而，除了腓肠神经的感觉神经动作电位振幅显着降低外，这些传入功能障碍在 6 个月的随访期内并未进展，与运动功能的明显恶化形成鲜明对比。应用三种不同的统计模型来评估脱髓鞘的存在、MNFCV 的选择性丧失或快传导和慢传导运动神经元的纯粹随机变性。这些数据表明 MNFCV 选择性丧失，并表明 ALS 中感觉系统的亚临床异常经常存在，但几乎是非进行性的。此外，肌萎缩侧索硬化症疾病过程似乎优先影响 MNFCV。
Although amyotrophic lateral sclerosis is a degenerative disease of the upper and lower motor neurons, there is evidence that the disease can affect other systems, including the sensory system. On the other hand, within the motor neuron pool there is possibly a predilection of the degenerative process for the motor neurons fibers with the fastest conduction velocity (MNFCV). We studied these two aspects of the disease in a group of 50 patients by prospectively assessing several sensory indices and by studying the selectivity of the spinal motor neuron loss. At baseline, nerve conduction studies and somatosensory evoked potentials showed abnormal slowing in the peripheral and central sensory pathways. Thermal thresholds for heating were elevated but were normal for cooling. In more than 60% of the patients at least one of the sensory tests studied was abnormal. However, except for a significant decrease in the amplitude of the sensory nerve action potentials of the sural nerves, these afferent dysfunctions were not progressive over the follow-up period of 6 months, in contrast to the marked deterioration in motor functions. Three different statistical models were applied to evaluate the presence of demyelination, selective loss of MNFCV, or the purely random degeneration of fast- and slow-conducting motor neurons. These data indicate a selective loss of the MNFCV and suggest that subclinical abnormalities of the sensory system in ALS are often present but almost nonprogressive. Furthermore, the amyotrophic lateral sclerosis disease process seems preferentially to affect MNFCV.