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We constructed the voxel-wise CBG networks using the resting-state fMRI data.We found decreased nodal efficiency in the right THA, left CAU, and right preCG in ALS.We found increased nodal efficiency in the left preCG in ALS.The nodal efficiency of the right preCG was associated with disease progression rate in ALS.Evidence suggests that dysfunctional cortical-basal ganglia (CBG) network plays important roles in the motor symptoms in amyotrophic lateral sclerosis (ALS). However, little effort has been made to investigate the functional abnormalities of CBG network in ALS. Here, we constructed voxel-wise CBG networks using the resting-state fMRI data of 20 patients with ALS and 21 normal controls, and characterized the differences of their efficiency parameters between the two groups. Compared to normal controls, patients with ALS exhibited decreased nodal efficiency in the right thalamus (THA), the left caudate (CAU) and the right precentral gyrus (preCG), and increased nodal efficiency in the left preCG. In the patient group, we observed a significant negative correlation between the nodal efficiency of the right preCG and disease progression rate. These results demonstrate that both ineffective information transfer and compensatory mechanisms are involved in the pathophysiological mechanism underlying the motor dysfunctions in patients with ALS. In summary, the present study provides a novel perspective on pathophysiological explanation for the motor symptoms in patients with ALS.