Department of Neurology, Rudolf Magnus Institute of Neuroscience, University Medical Centre Utrecht, Utrecht, The Netherlands.
PLoS One. 2010 Oct 27;5(10):e13664. doi: 10.1371/journal.pone.0013664.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterised by motor neuron degeneration. How this disease affects the central motor network is largely unknown. Here, we combined for the first time structural and functional imaging measures on the motor network in patients with ALS and healthy controls.
METHODOLOGY/PRINCIPAL FINDINGS: Structural measures included whole brain cortical thickness and diffusion tensor imaging (DTI) of crucial motor tracts. These structural measures were combined with functional connectivity analysis of the motor network based on resting state fMRI. Focal cortical thinning was observed in the primary motor area in patients with ALS compared to controls and was found to correlate with disease progression. DTI revealed reduced FA values in the corpus callosum and in the rostral part of the corticospinal tract. Overall functional organisation of the motor network was unchanged in patients with ALS compared to healthy controls, however the level of functional connectedness was significantly correlated with disease progression rate. Patients with increased connectedness appear to have a more progressive disease course.
CONCLUSIONS/SIGNIFICANCE: We demonstrate structural motor network deterioration in ALS with preserved functional connectivity measures. The positive correlation between functional connectedness of the motor network and disease progression rate could suggest spread of disease along functional connections of the motor network.
肌萎缩侧索硬化症（ALS）是一种以运动神经元退化为特征的神经退行性疾病。这种疾病如何影响中枢运动网络在很大程度上尚不清楚。在这里，我们首次结合了 ALS 患者和健康对照组的运动网络的结构和功能成像测量。
方法/主要发现：结构测量包括大脑皮质全层厚度和关键运动束的弥散张量成像（DTI）。这些结构测量与基于静息状态 fMRI 的运动网络功能连接分析相结合。与对照组相比，ALS 患者的初级运动区出现局灶性皮质变薄，且与疾病进展相关。DTI 显示胼胝体和皮质脊髓束的前部 FA 值降低。与健康对照组相比，ALS 患者的运动网络整体功能组织不变，但功能连接水平与疾病进展速度显著相关。具有更高连通性的患者似乎具有更进展性的疾病过程。
结论/意义：我们证明了 ALS 中存在运动网络的结构恶化，同时保留了功能连接测量。运动网络的功能连接与疾病进展速度之间的正相关性表明，疾病可能沿着运动网络的功能连接传播。
