a Department of Neurology, Medical Faculty , BG University Hospital Bergmannsheil, Ruhr University Bochum , Bochum , Germany and.
b Department of Neurology , Outpatient Center for ALS and Other Motor Neuron Disorders, Alfried Krupp Hospital , Essen , Germany.
Amyotroph Lateral Scler Frontotemporal Degener. 2019 May;20(3-4):192-198. doi: 10.1080/21678421.2019.1570270. Epub 2019 Feb 23.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative motoneuron disease. As previous studies reported alterations in motor cortex excitability, we evaluate excitability changes in somatosensory system. We examined 15 ALS patients and 15 healthy controls. Cortical excitability was assessed using paired somatosensory evoked potentials of median nerves. To determine disease severity and functional impairment, we assessed muscle strength and revised ALS-Functional Rating Scale (ALSFRS-R). We found significantly reduced bilateral paired-stimulation inhibition in the ALS-group (both < 0.05). Additionally, paired-stimulation ratios significantly correlated with ALSFRS-R (left somatosensory cortex: = -orte; right somatosensory cortex: = -ort4; both < 0.05) and contralateral muscle strength (left somatosensory cortex: = -orte, = 0.007; right somatosensory cortex: = -ortex = 0.003). The results indicate disinhibition of the somatosensory cortex in ALS. It remains open if central somatosensory disinhibition is a primary characteristic of ALS as one element of a multisystem neurodegenerative disorder or a compensatory up-regulation due to functional motoric impairment. Longitudinal studies are necessary to categorize these findings.
肌萎缩侧索硬化症（ALS）是一种进行性神经退行性运动神经元疾病。正如先前的研究报道的那样，运动皮层兴奋性发生了改变，我们评估了感觉系统的兴奋性变化。我们检查了 15 名 ALS 患者和 15 名健康对照者。使用正中神经的成对体感诱发电位评估皮质兴奋性。为了确定疾病的严重程度和功能障碍，我们评估了肌肉力量和修订后的 ALS 功能评定量表（ALSFRS-R）。我们发现 ALS 组双侧对刺激抑制明显降低（均<0.05）。此外，对刺激比与 ALSFRS-R（左侧体感皮层：=−0.6;右侧体感皮层：=−0.4;均<0.05）和对侧肌肉力量显著相关（左侧体感皮层：=−0.6, =0.007;右侧体感皮层：=−0.4, =0.003）。结果表明 ALS 中体感皮层的抑制解除。中枢感觉抑制是 ALS 作为多系统神经退行性疾病的一个特征还是由于运动功能障碍的代偿性上调，尚不清楚。需要进行纵向研究来对这些发现进行分类。
