Whether longitudinal diffusion tensor MRI imaging (DTI) can capture disease progression in patients with amyotrophic lateral sclerosis (ALS) is unclear. The primary goal of this study was to determine if DTI detects progression of the corticospinal tracts (CST) degeneration in ALS. Seventeen ALS patients and 19 ageand gender-matched healthy controls were scanned with DTI at baseline for cross-sectional analyses. For longitudinal analyses, the ALS patients had repeat DTI scans after eight months. Tractography of the CST was used to guide regions-of-interest (ROI) analysis and complemented by a voxelwise analysis. Cross-sectional study found that baseline FA of the right superior CST was markedly reduced in ALS patients compared to controls. The FA reductions in this region correlated with the disease severity in ALS patients. Longitudinal study found that FA change rate of the right superior CST significantly declined over time. In conclusion, longitudinal DTI study captures progression of upper motor fiber degeneration in ALS. DTI can be useful for monitoring ALS progression and efficacy of treatment interventions.

The dataset available here consists of Fractional Anisotropy (FA), and Mean Diffusivity (MD) images as well as subject-study-specific normalization images (B0, see methods in paper) in Analyze format. There are B0, FA, and MD images for 53 subjects, and a spreadsheet describing each subject's age (at time of scan), gender, and diagnosis (ALS or Control). For ALS subjects, there are two timepoints (duration between timepoints is indicated) as well as additional info including ALS subtype, side at onset, and scores on the Amyotrophic Lateral Sclerosis Functional Rating Scale–revised (ALSFRSr).