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Muscle MRI in two SMA patients on nusinersen treatment: A two years follow-up

<p><strong>Introduction:&nbsp;</strong>The effects of nusinersen in adults with SMA rely on neuromotor function scales and qualitative assessments. There are limited clinical or imaging data on muscle changes over time.</p> <p><strong>Methods:&nbsp;</strong>Two adult SMA patients underwent clinical assessments including measures of upper and lower limb function with Revised Upper Limb Module (RULM) and Hammersmith Function Motor Scale Expanded (HFMSE); both patients were also studied with whole-body muscle MRI (T1-weighted and Diffusion Tensor Imaging/DTI sequences), at baseline and after 10 and 24 months from the beginning of treatment with nusinersen.</p> <p><strong>Results:&nbsp;</strong>After two years of treatment, HFMSE and RULM scores were stable in both patients. DTI sequences revealed an increased number, length and organization of muscle fiber tracks, and Fractional Anisotropy (FA) values showed a significant reduction after 10 and 24 months from baseline, in their corresponding maps.</p> <p><strong>Discussion:&nbsp;</strong>Muscle DTI imaging seems to play an interesting role to monitor treatment effects over time in adult SMA patients.</p>

ShareScore

32/100

Overall dataset sharing score

Score breakdown

These five areas show where the dataset supports — or may limit — practical reuse.

Stewardship
4
Harmonization
4
Access
16
Reuse readiness
8
Engagement
0

Dataset details

Repository
zenodo
Published
November 15, 2020
License
cc-by-4.0
DOI
10.5281/zenodo.4275716

Scored with release 0.9.0

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