Department of Advanced Medical and Surgical Sciences; MRI Research Center SUN-FISM, Università degli Studi della Campania "Luigi Vanvitelli", Naples, Italy.
Department of Psychology, Università degli Studi della Campania "Luigi Vanvitelli", Caserta, Italy.
CNS Spectr. 2021 Jun;26(3):258-267. doi: 10.1017/S109285292000005X. Epub 2020 Feb 24.
Advanced neuroimaging techniques may offer the potential to monitor disease progression in amyotrophic lateral sclerosis (ALS), a neurodegenerative, multisystem disease that still lacks therapeutic outcome measures. We aim to investigate longitudinal functional and structural magnetic resonance imaging (MRI) changes in a cohort of patients with ALS monitored for one year after diagnosis.
Resting state functional MRI, diffusion tensor imaging (DTI), and voxel-based morphometry analyses were performed in 22 patients with ALS examined by six-monthly MRI scans over one year.
During the follow-up period, patients with ALS showed reduced functional connectivity only in some extramotor areas, such as the middle temporal gyrus in the left frontoparietal network after six months and in the left middle frontal gyrus in the default mode network after one year without showing longitudinal changes of cognitive functions. Moreover, after six months, we reported in the ALS group a decreased fractional anisotropy (P = .003, Bonferroni corrected) in the right uncinate fasciculus. Conversely, we did not reveal significant longitudinal changes of functional connectivity in the sensorimotor network, as well as of gray matter (GM) atrophy or of DTI metrics in motor areas, although clinical measures of motor disability showed significant decline throughout the three time points.
Our findings highlighted that progressive impairment of extramotor frontotemporal networks may precede the appearance of executive and language dysfunctions and GM changes in ALS. Functional connectivity changes in cognitive resting state networks might represent candidate radiological markers of disease progression.
高级神经影像学技术可能为监测肌萎缩侧索硬化症（ALS）的疾病进展提供潜力，ALS 是一种神经退行性多系统疾病，目前仍缺乏治疗效果的衡量标准。我们旨在研究经诊断后一年内连续监测的 ALS 患者的功能和结构磁共振成像（MRI）的纵向变化。
对 22 例 ALS 患者进行静息态功能 MRI、弥散张量成像（DTI）和基于体素的形态测量分析，在一年中进行了 6 个月的 MRI 扫描。
在随访期间，ALS 患者仅在一些运动外区域显示出功能连接的减少，例如左额顶叶网络中的中颞叶在 6 个月后，默认模式网络中的左额中回在 1 年后，而认知功能没有显示出纵向变化。此外，在 6 个月时，我们在 ALS 组中报告了右侧钩束的各向异性分数（FA）降低（P =.003，经 Bonferroni 校正）。相反，我们没有发现运动网络的功能连接、灰质（GM）萎缩或运动区的 DTI 指标有明显的纵向变化，尽管运动功能障碍的临床评估在三个时间点都显示出明显的下降。
我们的发现强调了运动前扣带皮质网络的进行性损伤可能先于 ALS 中执行功能和语言功能障碍以及 GM 变化的出现。认知静息状态网络的功能连接变化可能是疾病进展的候选影像学标志物。
