Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy
Subjects with Duchenne Muscular Dystrophy (DMD) suffer from progressive muscle damage leading to diaphragmatic weakness that ultimately requires ventilation. Emerging treatments have generated interest in better characterizing the natural history of respiratory impairment in DMD and responses to the...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2018-01-01
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| Series: | Frontiers in Neurology |
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| Online Access: | http://journal.frontiersin.org/article/10.3389/fneur.2018.00009/full |
| _version_ | 1811200363072585728 |
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| author | Courtney A. Bishop Valeria Ricotti Christopher D. J. Sinclair Christopher D. J. Sinclair Matthew R. B. Evans Matthew R. B. Evans Jordan W. Butler Jasper M. Morrow Jasper M. Morrow Michael G. Hanna Michael G. Hanna Paul M. Matthews Tarek A. Yousry Tarek A. Yousry Francesco Muntoni John S. Thornton John S. Thornton Rexford D. Newbould Robert L. Janiczek |
| author_facet | Courtney A. Bishop Valeria Ricotti Christopher D. J. Sinclair Christopher D. J. Sinclair Matthew R. B. Evans Matthew R. B. Evans Jordan W. Butler Jasper M. Morrow Jasper M. Morrow Michael G. Hanna Michael G. Hanna Paul M. Matthews Tarek A. Yousry Tarek A. Yousry Francesco Muntoni John S. Thornton John S. Thornton Rexford D. Newbould Robert L. Janiczek |
| author_sort | Courtney A. Bishop |
| collection | DOAJ |
| description | Subjects with Duchenne Muscular Dystrophy (DMD) suffer from progressive muscle damage leading to diaphragmatic weakness that ultimately requires ventilation. Emerging treatments have generated interest in better characterizing the natural history of respiratory impairment in DMD and responses to therapy. Dynamic (cine) Magnetic Resonance Imaging (MRI) may provide a more sensitive measure of diaphragm function in DMD than the commonly used spirometry. This study presents an analysis pipeline for measuring parameters of diaphragmatic motion from dynamic MRI and its application to investigate MRI measures of respiratory function in both healthy controls and non-ambulant DMD boys. We scanned 13 non-ambulant DMD boys and 10 age-matched healthy male volunteers at baseline, with a subset (n = 10, 10, 8) of the DMD subjects also assessed 3, 6, and 12 months later. Spirometry-derived metrics including forced vital capacity were recorded. The MRI-derived measures included the lung cross-sectional area (CSA), the anterior, central, and posterior lung lengths in the sagittal imaging plane, and the diaphragm length over the time-course of the dynamic MRI. Regression analyses demonstrated strong linear correlations between lung CSA and the length measures over the respiratory cycle, with a reduction of these correlations in DMD, and diaphragmatic motions that contribute less efficiently to changing lung capacity in DMD. MRI measures of pulmonary function were reduced in DMD, controlling for height differences between the groups: at maximal inhalation, the maximum CSA and the total distance of motion of the diaphragm were 45% and 37% smaller. MRI measures of pulmonary function were correlated with spirometry data and showed relationships with disease progression surrogates of age and months non-ambulatory, suggesting that they provide clinically meaningful information. Changes in the MRI measures over 12 months were consistent with weakening of diaphragmatic and inter-costal muscles and progressive diaphragm dysfunction. In contrast, longitudinal changes were not seen in conventional spirometry measures during the same period. Dynamic MRI measures of thoracic muscle and pulmonary function are, therefore, believed to detect meaningful differences between healthy controls and DMD and may be sensitive to changes in function over relatively short periods of follow-up in non-ambulant boys with DMD. |
| first_indexed | 2024-04-12T02:03:36Z |
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| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-12T02:03:36Z |
| publishDate | 2018-01-01 |
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| spelling | doaj.art-637ffdccfc304b66b7187a719eee9dc02022-12-22T03:52:36ZengFrontiers Media S.A.Frontiers in Neurology1664-22952018-01-01910.3389/fneur.2018.00009303620Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular DystrophyCourtney A. Bishop0Valeria Ricotti1Christopher D. J. Sinclair2Christopher D. J. Sinclair3Matthew R. B. Evans4Matthew R. B. Evans5Jordan W. Butler6Jasper M. Morrow7Jasper M. Morrow8Michael G. Hanna9Michael G. Hanna10Paul M. Matthews11Tarek A. Yousry12Tarek A. Yousry13Francesco Muntoni14John S. Thornton15John S. Thornton16Rexford D. Newbould17Robert L. Janiczek18Imanova Limited, Hammersmith Hospital, London, United KingdomDubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London, United KingdomMRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United KingdomNeuroradiological Academic Unit, UCL Institute of Neurology, London, United KingdomMRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United KingdomNeuroradiological Academic Unit, UCL Institute of Neurology, London, United KingdomDubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London, United KingdomMRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United KingdomNeuroradiological Academic Unit, UCL Institute of Neurology, London, United KingdomMRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United KingdomNeuroradiological Academic Unit, UCL Institute of Neurology, London, United KingdomDivision of Brain Sciences, Centre for Neurotechnology, UK Dementia Research Institute, Imperial College London, London, United KingdomMRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United KingdomNeuroradiological Academic Unit, UCL Institute of Neurology, London, United KingdomDubowitz Neuromuscular Centre, UCL Great Ormond Street Institute of Child Health, London, United KingdomMRC Centre for Neuromuscular Diseases, UCL Institute of Neurology, London, United KingdomNeuroradiological Academic Unit, UCL Institute of Neurology, London, United KingdomImanova Limited, Hammersmith Hospital, London, United KingdomGlaxoSmithKline plc., Stevenage, United KingdomSubjects with Duchenne Muscular Dystrophy (DMD) suffer from progressive muscle damage leading to diaphragmatic weakness that ultimately requires ventilation. Emerging treatments have generated interest in better characterizing the natural history of respiratory impairment in DMD and responses to therapy. Dynamic (cine) Magnetic Resonance Imaging (MRI) may provide a more sensitive measure of diaphragm function in DMD than the commonly used spirometry. This study presents an analysis pipeline for measuring parameters of diaphragmatic motion from dynamic MRI and its application to investigate MRI measures of respiratory function in both healthy controls and non-ambulant DMD boys. We scanned 13 non-ambulant DMD boys and 10 age-matched healthy male volunteers at baseline, with a subset (n = 10, 10, 8) of the DMD subjects also assessed 3, 6, and 12 months later. Spirometry-derived metrics including forced vital capacity were recorded. The MRI-derived measures included the lung cross-sectional area (CSA), the anterior, central, and posterior lung lengths in the sagittal imaging plane, and the diaphragm length over the time-course of the dynamic MRI. Regression analyses demonstrated strong linear correlations between lung CSA and the length measures over the respiratory cycle, with a reduction of these correlations in DMD, and diaphragmatic motions that contribute less efficiently to changing lung capacity in DMD. MRI measures of pulmonary function were reduced in DMD, controlling for height differences between the groups: at maximal inhalation, the maximum CSA and the total distance of motion of the diaphragm were 45% and 37% smaller. MRI measures of pulmonary function were correlated with spirometry data and showed relationships with disease progression surrogates of age and months non-ambulatory, suggesting that they provide clinically meaningful information. Changes in the MRI measures over 12 months were consistent with weakening of diaphragmatic and inter-costal muscles and progressive diaphragm dysfunction. In contrast, longitudinal changes were not seen in conventional spirometry measures during the same period. Dynamic MRI measures of thoracic muscle and pulmonary function are, therefore, believed to detect meaningful differences between healthy controls and DMD and may be sensitive to changes in function over relatively short periods of follow-up in non-ambulant boys with DMD.http://journal.frontiersin.org/article/10.3389/fneur.2018.00009/fullDuchenneDuchenne muscular dystrophyMagnetic Resonance Imagingdynamicrespirationdiaphragm |
| spellingShingle | Courtney A. Bishop Valeria Ricotti Christopher D. J. Sinclair Christopher D. J. Sinclair Matthew R. B. Evans Matthew R. B. Evans Jordan W. Butler Jasper M. Morrow Jasper M. Morrow Michael G. Hanna Michael G. Hanna Paul M. Matthews Tarek A. Yousry Tarek A. Yousry Francesco Muntoni John S. Thornton John S. Thornton Rexford D. Newbould Robert L. Janiczek Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy Frontiers in Neurology Duchenne Duchenne muscular dystrophy Magnetic Resonance Imaging dynamic respiration diaphragm |
| title | Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy |
| title_full | Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy |
| title_fullStr | Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy |
| title_full_unstemmed | Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy |
| title_short | Semi-Automated Analysis of Diaphragmatic Motion with Dynamic Magnetic Resonance Imaging in Healthy Controls and Non-Ambulant Subjects with Duchenne Muscular Dystrophy |
| title_sort | semi automated analysis of diaphragmatic motion with dynamic magnetic resonance imaging in healthy controls and non ambulant subjects with duchenne muscular dystrophy |
| topic | Duchenne Duchenne muscular dystrophy Magnetic Resonance Imaging dynamic respiration diaphragm |
| url | http://journal.frontiersin.org/article/10.3389/fneur.2018.00009/full |
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