Huynh William, Dharmadasa Thanuja, Vucic Steve, Kiernan Matthew C
Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia.
Prince of Wales Clinical School, University of New South Wales, Sydney, NSW, Australia.
Front Neurol. 2019 Jan 4;9:1141. doi: 10.3389/fneur.2018.01141. eCollection 2018.
The clinical diagnosis of amyotrophic lateral sclerosis (ALS) relies on determination of progressive dysfunction of both cortical as well as spinal and bulbar motor neurons. However, the variable mix of upper and lower motor neuron signs result in the clinical heterogeneity of patients with ALS, resulting frequently in delay of diagnosis as well as difficulty in monitoring disease progression and treatment outcomes particularly in a clinical trial setting. As such, the present review provides an overview of recently developed novel non-invasive electrophysiological techniques that may serve as biomarkers to assess UMN and LMN dysfunction in ALS patients.
肌萎缩侧索硬化症(ALS)的临床诊断依赖于确定皮质以及脊髓和延髓运动神经元的进行性功能障碍。然而,上、下运动神经元体征的不同组合导致了ALS患者的临床异质性,常常导致诊断延迟以及监测疾病进展和治疗结果困难,尤其是在临床试验环境中。因此,本综述概述了最近开发的新型非侵入性电生理技术,这些技术可作为生物标志物来评估ALS患者的上运动神经元和下运动神经元功能障碍。