| Title: |
Quantification of disease progression in spinal muscular atrophy with muscle MRI-a pilot study |
| Authors: |
Otto, Louise A M; Froeling, Martijn; van Eijk, Ruben P A; Asselman, Fay-Lynn; Wadman, Renske; Cuppen, Inge; Hendrikse, Jeroen; van der Pol, W-Ludo; Projectafdeling SvS; Highfield Research Group; Brain; Circulatory Health; Cancer; Projectafdeling ALS; Verpleegkundig Specialisten; Neurologen; Researchgr. Neuroradiologie; MS Radiologie |
| Publication Year: |
2021 |
| Subject Terms: |
MRI; skeletal muscle; spinal muscular atrophy; Molecular Medicine; Radiology Nuclear Medicine and imaging; Spectroscopy; Journal Article |
| Description: |
Objectives: Quantitative MRI (qMRI) of muscles is a promising tool to measure disease progression or to assess therapeutic effects in neuromuscular diseases. Longitudinal imaging studies are needed to show sensitivity of qMRI in detecting disease progression in spinal muscular atrophy (SMA). In this pilot study we therefore studied one-year changes in quantitative MR parameters in relation to clinical scores. Methods: We repeated quantitative 3 T MR analysis of thigh muscles and clinical testing one year after baseline in 10 treatment-naïve patients with SMA, 5 with Type 2 (21.6 ± 7.0 years) and 5 with Type 3 (33.4 ± 11.9 years). MR protocol consisted of Dixon, T 2 mapping and diffusion tensor imaging (DTI). The temporal relation of parameters was examined with a mixed model. Results: We detected a significant increase in fat fraction (baseline, 38.2% SE 0.6; follow-up, 39.5% SE 0.6; +1.3%, p = 0.001) in all muscles. Muscles with moderate to high fat infiltration at baseline show a larger increase over time (+1.6%, p < 0.001). We did not find any changes in DTI parameters except for low fat-infiltration muscles (m. adductor longus and m. biceps femoris (short head)). The T 2 of muscles decreased from 28.2 ms to 28.0 ms (p = 0.07). Muscle strength and motor function scores were not significantly different between follow-up and baseline. Conclusion: Longitudinal imaging data show slow disease progression in skeletal muscle of the thigh of (young-) adult patients with SMA despite stable strength and motor function scores. Quantitative muscle imaging demonstrates potential as a biomarker for disease activity and monitoring of therapy response. |
| Document Type: |
article in journal/newspaper |
| File Description: |
text/plain |
| Language: |
English |
| ISSN: |
0952-3480 |
| Relation: |
https://dspace.library.uu.nl/handle/1874/442040 |
| Availability: |
https://dspace.library.uu.nl/handle/1874/442040 |
| Rights: |
info:eu-repo/semantics/OpenAccess |
| Accession Number: |
edsbas.2ACC72D |
| Database: |
BASE |