Leveraging Cardiac Magnetic Resonance Imaging to Assess Skeletal Muscle Progression in Duchenne Muscular Dystrophy

dc.contributor.authorKaslow, Jacob A.
dc.contributor.authorSokolow, Andrew G.
dc.contributor.authorDonnelly, Thomas
dc.contributor.authorBuchowski, Maciej S.
dc.contributor.authorDamon, Bruce M.
dc.contributor.authorMarkham, Larry W.
dc.contributor.authorBurnette, W. Bryan
dc.contributor.authorSoslow, Jonathan H.
dc.contributor.departmentPediatrics, School of Medicine
dc.date.accessioned2024-01-04T11:52:32Z
dc.date.available2024-01-04T11:52:32Z
dc.date.issued2022
dc.description.abstractDuchenne Muscular Dystrophy (DMD) is characterized by muscle deterioration and progressive weakness. As a result, patients with DMD have significant cardiopulmonary morbidity and mortality that worsens with age and loss of ambulation. Since most validated muscle assessments require ambulation, new functional measures of DMD progression are needed. Despite several evaluation methods available for monitoring disease progression, the relationship between these measures is unknown. We sought to assess the correlation between imaging metrics obtained from cardiac magnetic resonance imaging (CMR) and functional assessments including quantitative muscle testing (QMT), spirometry, and accelerometry. Forty-nine patients with DMD were enrolled and underwent CMR, accelerometry and QMT at baseline, 1-year and 2-year clinic visits with temporally associated pulmonary function testing obtained from the medical record. Imaging of the upper extremity musculature (triceps and biceps) demonstrated the most robust correlations with accelerometry (p<0.03), QMT (p<0.02) and spirometry (p<0.01). T1-mapping of serratus anterior muscle showed a similar, but slightly weaker relationship with accelerometry and QMT. T2-mapping of serratus anterior demonstrated weak indirect correlation with aspects of accelerometry. These images are either routinely obtained in standard CMR or can be added to a protocol and may allow for a more comprehensive assessment of a patient’s disease progression.
dc.eprint.versionAuthor's manuscript
dc.identifier.citationKaslow JA, Sokolow AG, Donnelly T, et al. Leveraging cardiac magnetic resonance imaging to assess skeletal muscle progression in Duchenne muscular dystrophy. Neuromuscul Disord. 2022;32(5):390-398. doi:10.1016/j.nmd.2022.01.010
dc.identifier.urihttps://hdl.handle.net/1805/37598
dc.language.isoen_US
dc.publisherElsevier
dc.relation.isversionof10.1016/j.nmd.2022.01.010
dc.relation.journalNeuromuscular Disorders
dc.rightsPublisher Policy
dc.sourcePMC
dc.subjectDuchenne Muscular Dystrophy
dc.subjectMRI
dc.subjectSpirometry
dc.subjectAccelerometry
dc.subjectQuantitative muscle testing
dc.titleLeveraging Cardiac Magnetic Resonance Imaging to Assess Skeletal Muscle Progression in Duchenne Muscular Dystrophy
dc.typeArticle
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