Objective

Only few studies investigated voluntary movement abnormalities in patients with motoneuron diseases (MNDs) or their neurophysiological correlates. We aimed to kinematically assess finger tapping abnormalities in patients with amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS), as compared to healthy controls (HCs), and their relationship with motoneuron involvement.

Methods

Fourteen ALS and 5 PLS patients were enrolled. Finger tapping was assessed by a motion analysis system. Patients underwent a central motor conduction time assessment, a motor nerve conduction study, and needle electromyography. Data were compared to those of 79 HCs using non-parametric tests. Possible relationships between clinical, kinematic, and neurophysiological data were assessed in patients.

Results

As a major finding, ALS and PLS patients performed finger tapping slower than HCs. In both conditions, movement slowness correlated with muscle strength. In ALS, movement slowness also correlated with the amplitude of the compound muscle action potential recorded from the muscles involved in the task and with denervation activity. No correlations were found between slowness, measures of upper motoneuron involvement, and other clinical and neurophysiological data.

Conclusions

This study provides novel information on voluntary movement abnormalities in MNDs.

Significance

The results highlight the pathophysiological role of motoneurons in generating movement slowness.

中文翻译：

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运动神经元疾病中的运动迟缓

客观的

只有少数研究调查了运动神经元疾病 (MND) 患者的自主运动异常或其神经生理学相关性。我们的目的是在运动学上评估肌萎缩侧索硬化 (ALS) 和原发性侧索硬化 (PLS) 患者与健康对照 (HC) 相比的手指敲击异常及其与运动神经元受累的关系。

方法

招募了 14 名 ALS 和 5 名 PLS 患者。手指敲击通过运动分析系统进行评估。患者接受了中枢运动传导时间评估、运动神经传导研究和针状肌电图。使用非参数检验将数据与 79 个 HC 的数据进行比较。在患者中评估了临床、运动学和神经生理学数据之间可能的关系。

结果

作为一个主要发现，ALS 和 PLS 患者的手指敲击速度比 HC 慢。在这两种情况下，运动缓慢与肌肉力量相关。在 ALS 中，运动缓慢还与从参与任务的肌肉中记录的复合肌肉动作电位的幅度以及去神经支配活动相关。在缓慢、上运动神经元受累的测量值和其他临床和神经生理学数据之间没有发现相关性。

结论

这项研究提供了有关 MND 中随意运动异常的新信息。

意义

结果突出了运动神经元在产生运动缓慢中的病理生理作用。