El Mendili Mohamed Mounir, Querin Giorgia, Bede Peter, Pradat Pierre-François
Department of Neurology, Icahn School of Medicine at Mount Sinai, New York, NY, United States.
Biomedical Imaging Laboratory (LIB), Sorbonne University, CNRS, INSERM, Paris, France.
Front Neurol. 2019 Apr 12;10:350. doi: 10.3389/fneur.2019.00350. eCollection 2019.
Amyotrophic lateral sclerosis (ALS) is the most common adult onset motor neuron disease with no effective disease modifying therapies at present. Spinal cord degeneration is a hallmark feature of ALS, highlighted in the earliest descriptions of the disease by Lockhart Clarke and Jean-Martin Charcot. The anterior horns and corticospinal tracts are invariably affected in ALS, but up to recently it has been notoriously challenging to detect and characterize spinal pathology . With recent technological advances, spinal imaging now offers unique opportunities to appraise lower motor neuron degeneration, sensory involvement, metabolic alterations, and interneuron pathology in ALS. Quantitative spinal imaging in ALS has now been used in cross-sectional and longitudinal study designs, applied to presymptomatic mutation carriers, and utilized in machine learning applications. Despite its enormous clinical and academic potential, a number of physiological, technological, and methodological challenges limit the routine use of computational spinal imaging in ALS. In this review, we provide a comprehensive overview of emerging spinal cord imaging methods and discuss their advantages, drawbacks, and biomarker potential in clinical applications, clinical trial settings, monitoring, and prognostic roles.
肌萎缩侧索硬化症(ALS)是最常见的成人起病运动神经元疾病,目前尚无有效的疾病修饰疗法。脊髓变性是ALS的一个标志性特征,Lockhart Clarke和Jean-Martin Charcot在该疾病的最早描述中就强调了这一点。ALS患者的前角和皮质脊髓束总是会受到影响,但直到最近,检测和描述脊髓病变一直极具挑战性。随着最近的技术进步,脊髓成像现在为评估ALS中的下运动神经元变性、感觉受累、代谢改变和中间神经元病变提供了独特的机会。ALS中的定量脊髓成像现已用于横断面和纵向研究设计,应用于症状前突变携带者,并用于机器学习应用。尽管其具有巨大的临床和学术潜力,但一些生理、技术和方法学挑战限制了计算脊髓成像在ALS中的常规使用。在这篇综述中,我们全面概述了新兴的脊髓成像方法,并讨论了它们在临床应用、临床试验环境、监测和预后作用中的优点、缺点和生物标志物潜力。