Instituto de Fisiologia, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Department of Neurosciences and Mental Health, Hospital de Santa Maria, Centro Hospitalar Universitário de Lisboa Norte, Lisbon, Portugal.
Brain and Mind Centre, University of Sydney, and Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia.
Amyotroph Lateral Scler Frontotemporal Degener. 2020 Nov;21(sup1):11-17. doi: 10.1080/21678421.2020.1837174.
Primary lateral sclerosis (PLS) is a motor neuron disease characterized by spinobulbar spasticity, absence of progressive lower motor neuron (LMN) dysfunction and marked by a slow functional decline. Electromyography is essential to exclude significant LMN involvement, particularly in the context of distinguishing PLS from amyotrophic lateral sclerosis (ALS), given that the prognosis is substantially better, and respiratory complications are unusual, in PLS. Nevertheless, minor neurogenic changes and occasional fasciculation potentials can be observed in PLS. The most useful technique for the objective assessment of upper motor neuron (UMN) dysfunction is transcranial magnetic stimulation (TMS), which in PLS is characterized by a high cortical threshold and delayed central conduction times. TMS is sensitive to identify cortical dysfunction in PLS and might have potential for monitoring UMN function in longitudinal studies and in clinical trials. The findings of TMS need to be interpreted in the context of the clinical presentation and phenotype, particularly in the differentiation between PLS and ALS. While other neurophysiological techniques have been investigated, studies to date have tended to involve small patient cohorts and as such, their value in distinguishing PLS from ALS remains unclear.
原发性侧索硬化症（PLS）是一种运动神经元疾病，其特征为脊髓-延髓痉挛性瘫痪，无进行性下运动神经元（LMN）功能障碍，并以缓慢的功能下降为特征。肌电图对于排除明显的 LMN 受累至关重要，特别是在将 PLS 与肌萎缩侧索硬化症（ALS）区分开来时，因为 PLS 的预后要好得多，且呼吸并发症不常见。然而，在 PLS 中可以观察到轻微的神经源性改变和偶尔的肌束震颤电位。对于上运动神经元（UMN）功能障碍的客观评估，最有用的技术是经颅磁刺激（TMS），其在 PLS 中的特征是皮质阈值高和中枢传导时间延迟。TMS 能够灵敏地识别 PLS 中的皮质功能障碍，并且在纵向研究和临床试验中可能具有监测 UMN 功能的潜力。TMS 的发现需要结合临床表现和表型来解释，特别是在 PLS 和 ALS 之间的区分。虽然已经研究了其他神经生理学技术，但迄今为止的研究往往涉及小的患者队列，因此，它们在区分 PLS 和 ALS 中的价值尚不清楚。
