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MRI correlates of motoneuron loss in SMA
BACKGROUND: Magnetic resonance imaging (MRI) is currently explored as supplemental tool to monitor disease progression and treatment response in various neuromuscular disorders. We here assessed the utility of a multi-parametric magnetic resonance imaging (MRI) protocol including quantitative water...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Springer Berlin Heidelberg
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9813025/ https://www.ncbi.nlm.nih.gov/pubmed/36180649 http://dx.doi.org/10.1007/s00415-022-11326-1 |
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author | Sprenger-Svačina, Alina Haensch, Johannes Weiss, Kilian Große Hokamp, Nils Maintz, David Schlamann, Marc Fink, Gereon R. Schloss, Natalie Laukamp, Kai Wunderlich, Gilbert Lehmann, Helmar C. Lichtenstein, Thorsten |
author_facet | Sprenger-Svačina, Alina Haensch, Johannes Weiss, Kilian Große Hokamp, Nils Maintz, David Schlamann, Marc Fink, Gereon R. Schloss, Natalie Laukamp, Kai Wunderlich, Gilbert Lehmann, Helmar C. Lichtenstein, Thorsten |
author_sort | Sprenger-Svačina, Alina |
collection | PubMed |
description | BACKGROUND: Magnetic resonance imaging (MRI) is currently explored as supplemental tool to monitor disease progression and treatment response in various neuromuscular disorders. We here assessed the utility of a multi-parametric magnetic resonance imaging (MRI) protocol including quantitative water T(2) mapping, Dixon-based proton density fat fraction (PDFF) estimation and diffusion tensor imaging (DTI) to detect loss of spinal motor neurons and subsequent muscle damage in adult SMA patients. METHODS: Sixteen SMA patients and 13 age-matched controls were enrolled in this prospective, longitudinal study. All participants underwent MRI imaging including measurements of Dixon-based PDFF and DTI of the sciatic nerve. SMA patients furthermore underwent measurements of muscle water T(2) (T(2w)) of the biceps femoris muscle (BFM) and quadriceps femoris muscle (QFM). Ten participants returned for a second scan six months later. MRI parameter were correlated with clinical data. All patients were on nusinersen treatment. RESULTS: There were significantly higher intramuscular fat fractions in the BFM and QFM of SMA patients compared to healthy controls at baseline and after 6 months. Furthermore, T2 values significantly correlated positively with intramuscular fat fractions. The Hammersmith functional motor scale significantly correlated with the QFM’s intramuscular fat fractions. DTI scans of the sciatic nerve were not significantly different between the two groups. CONCLUSION: This study demonstrates that, water T(2) mapping and Dixon-based PDFF estimation may distinguish between adult SMA patients and controls, due to massive intramuscular fat accumulation in SMA. More extensive long-term studies are warranted to further evaluate these two modalities as surrogate markers in SMA patients during treatment. |
format | Online Article Text |
id | pubmed-9813025 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Springer Berlin Heidelberg |
record_format | MEDLINE/PubMed |
spelling | pubmed-98130252023-01-06 MRI correlates of motoneuron loss in SMA Sprenger-Svačina, Alina Haensch, Johannes Weiss, Kilian Große Hokamp, Nils Maintz, David Schlamann, Marc Fink, Gereon R. Schloss, Natalie Laukamp, Kai Wunderlich, Gilbert Lehmann, Helmar C. Lichtenstein, Thorsten J Neurol Original Communication BACKGROUND: Magnetic resonance imaging (MRI) is currently explored as supplemental tool to monitor disease progression and treatment response in various neuromuscular disorders. We here assessed the utility of a multi-parametric magnetic resonance imaging (MRI) protocol including quantitative water T(2) mapping, Dixon-based proton density fat fraction (PDFF) estimation and diffusion tensor imaging (DTI) to detect loss of spinal motor neurons and subsequent muscle damage in adult SMA patients. METHODS: Sixteen SMA patients and 13 age-matched controls were enrolled in this prospective, longitudinal study. All participants underwent MRI imaging including measurements of Dixon-based PDFF and DTI of the sciatic nerve. SMA patients furthermore underwent measurements of muscle water T(2) (T(2w)) of the biceps femoris muscle (BFM) and quadriceps femoris muscle (QFM). Ten participants returned for a second scan six months later. MRI parameter were correlated with clinical data. All patients were on nusinersen treatment. RESULTS: There were significantly higher intramuscular fat fractions in the BFM and QFM of SMA patients compared to healthy controls at baseline and after 6 months. Furthermore, T2 values significantly correlated positively with intramuscular fat fractions. The Hammersmith functional motor scale significantly correlated with the QFM’s intramuscular fat fractions. DTI scans of the sciatic nerve were not significantly different between the two groups. CONCLUSION: This study demonstrates that, water T(2) mapping and Dixon-based PDFF estimation may distinguish between adult SMA patients and controls, due to massive intramuscular fat accumulation in SMA. More extensive long-term studies are warranted to further evaluate these two modalities as surrogate markers in SMA patients during treatment. Springer Berlin Heidelberg 2022-10-01 2023 /pmc/articles/PMC9813025/ /pubmed/36180649 http://dx.doi.org/10.1007/s00415-022-11326-1 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . |
spellingShingle | Original Communication Sprenger-Svačina, Alina Haensch, Johannes Weiss, Kilian Große Hokamp, Nils Maintz, David Schlamann, Marc Fink, Gereon R. Schloss, Natalie Laukamp, Kai Wunderlich, Gilbert Lehmann, Helmar C. Lichtenstein, Thorsten MRI correlates of motoneuron loss in SMA |
title | MRI correlates of motoneuron loss in SMA |
title_full | MRI correlates of motoneuron loss in SMA |
title_fullStr | MRI correlates of motoneuron loss in SMA |
title_full_unstemmed | MRI correlates of motoneuron loss in SMA |
title_short | MRI correlates of motoneuron loss in SMA |
title_sort | mri correlates of motoneuron loss in sma |
topic | Original Communication |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9813025/ https://www.ncbi.nlm.nih.gov/pubmed/36180649 http://dx.doi.org/10.1007/s00415-022-11326-1 |
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