MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1
Abstract Background Myotonic Dystrophy 1 (DM1) causes progressive myopathy of extremity muscles. DM1 may also affect muscles of the trunk. The aim of this study was to investigate fat infiltration and muscle size in trunk muscles in DM1 patients, and in an age and gender matched control group. Furth...
| Main Authors: | , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
BMC
2019-06-01
|
| Series: | BMC Neurology |
| Subjects: | |
| Online Access: | http://link.springer.com/article/10.1186/s12883-019-1357-8 |
| _version_ | 1818519463370162176 |
|---|---|
| author | Gro Solbakken Bård Bjørnarå Eva Kirkhus Bac Nguyen Gunnar Hansen Jan C. Frich Kristin Ørstavik |
| author_facet | Gro Solbakken Bård Bjørnarå Eva Kirkhus Bac Nguyen Gunnar Hansen Jan C. Frich Kristin Ørstavik |
| author_sort | Gro Solbakken |
| collection | DOAJ |
| description | Abstract Background Myotonic Dystrophy 1 (DM1) causes progressive myopathy of extremity muscles. DM1 may also affect muscles of the trunk. The aim of this study was to investigate fat infiltration and muscle size in trunk muscles in DM1 patients, and in an age and gender matched control group. Further, explore how fat infiltration and degree of atrophy in these muscles are associated with motor and respiratory function in DM1 patients. Method We measured fat infiltration and trunk muscle size by MRI in 20 patients with genetically confirmed classic form of DM1, and compared these cases with 20 healthy, age and gender matched controls. In the DM1 group, we investigated correlations between MRI findings and clinical measures of muscle strength, mobility and respiration. We used sum scores for fat infiltration and muscle size in trunk flexors and trunk extensors in the analysis of group differences and correlations. Results Significant differences between cases and controls were present for fat infiltration in trunk flexors (p = 0.001) and trunk extensors (p = < 0.001), and for muscle size in trunk flexors (p = 0.002) and trunk extensors (p = 0.030). Fat infiltration in trunk flexors were significant correlated to back extension strength (rho = − 0.523 p = 0.018), while muscle size in trunk flexors was significantly correlated to trunk flexion strength (rho = 0.506 p = 0.023). Fat infiltration in trunk flexors was significantly correlated with lower general mobility (rho = − 0.628, p = 0.003), reduced balance (rho = 0.630, p < 0.003) and forced vital capacity (rho − 0.487 p = 0.040). Conclusions Trunk muscles in DM1 patients had significant higher levels of fat infiltration and reduced muscle size compared to age and gender matched controls. In DM1 patients, fat infiltration was associated with reduced muscle strength, mobility, balance and lung function, while muscle size was associated with reduced muscle strength and lung function. These findings are of importance for clinical management of the disease and could be useful additional outcome measures in future intervention studies. |
| first_indexed | 2024-12-11T01:24:33Z |
| format | Article |
| id | doaj.art-37ee37043ec041438de8575b06d13939 |
| institution | Directory Open Access Journal |
| issn | 1471-2377 |
| language | English |
| last_indexed | 2024-12-11T01:24:33Z |
| publishDate | 2019-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj.art-37ee37043ec041438de8575b06d139392022-12-22T01:25:34ZengBMCBMC Neurology1471-23772019-06-0119111110.1186/s12883-019-1357-8MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1Gro Solbakken0Bård Bjørnarå1Eva Kirkhus2Bac Nguyen3Gunnar Hansen4Jan C. Frich5Kristin Ørstavik6Department of Neurology, Rheumatology and Rehabilitation, Drammen Hospital Vestre Viken Hospital TrustDepartment of Diagnostic Imaging, Drammen Hospital, Vestre Viken Hospital TrustDivision of Radiology and Nuclear Medicine, Oslo University HospitalDivision of Radiology and Nuclear Medicine, Oslo University HospitalDepartment of Neurology, Rheumatology and Rehabilitation, Drammen Hospital Vestre Viken Hospital TrustFaculty of Medicine, University of OsloDepartment of Neurology, Section for Rare Neuromuscular Disorders, Oslo University HospitalAbstract Background Myotonic Dystrophy 1 (DM1) causes progressive myopathy of extremity muscles. DM1 may also affect muscles of the trunk. The aim of this study was to investigate fat infiltration and muscle size in trunk muscles in DM1 patients, and in an age and gender matched control group. Further, explore how fat infiltration and degree of atrophy in these muscles are associated with motor and respiratory function in DM1 patients. Method We measured fat infiltration and trunk muscle size by MRI in 20 patients with genetically confirmed classic form of DM1, and compared these cases with 20 healthy, age and gender matched controls. In the DM1 group, we investigated correlations between MRI findings and clinical measures of muscle strength, mobility and respiration. We used sum scores for fat infiltration and muscle size in trunk flexors and trunk extensors in the analysis of group differences and correlations. Results Significant differences between cases and controls were present for fat infiltration in trunk flexors (p = 0.001) and trunk extensors (p = < 0.001), and for muscle size in trunk flexors (p = 0.002) and trunk extensors (p = 0.030). Fat infiltration in trunk flexors were significant correlated to back extension strength (rho = − 0.523 p = 0.018), while muscle size in trunk flexors was significantly correlated to trunk flexion strength (rho = 0.506 p = 0.023). Fat infiltration in trunk flexors was significantly correlated with lower general mobility (rho = − 0.628, p = 0.003), reduced balance (rho = 0.630, p < 0.003) and forced vital capacity (rho − 0.487 p = 0.040). Conclusions Trunk muscles in DM1 patients had significant higher levels of fat infiltration and reduced muscle size compared to age and gender matched controls. In DM1 patients, fat infiltration was associated with reduced muscle strength, mobility, balance and lung function, while muscle size was associated with reduced muscle strength and lung function. These findings are of importance for clinical management of the disease and could be useful additional outcome measures in future intervention studies.http://link.springer.com/article/10.1186/s12883-019-1357-8Myotonic dystrophy type 1MRIMuscle-sizeFat-infiltrationTrunk-musclesRespiration |
| spellingShingle | Gro Solbakken Bård Bjørnarå Eva Kirkhus Bac Nguyen Gunnar Hansen Jan C. Frich Kristin Ørstavik MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 BMC Neurology Myotonic dystrophy type 1 MRI Muscle-size Fat-infiltration Trunk-muscles Respiration |
| title | MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 |
| title_full | MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 |
| title_fullStr | MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 |
| title_full_unstemmed | MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 |
| title_short | MRI of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 |
| title_sort | mri of trunk muscles and motor and respiratory function in patients with myotonic dystrophy type 1 |
| topic | Myotonic dystrophy type 1 MRI Muscle-size Fat-infiltration Trunk-muscles Respiration |
| url | http://link.springer.com/article/10.1186/s12883-019-1357-8 |
| work_keys_str_mv | AT grosolbakken mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 AT bardbjørnara mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 AT evakirkhus mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 AT bacnguyen mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 AT gunnarhansen mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 AT jancfrich mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 AT kristinørstavik mrioftrunkmusclesandmotorandrespiratoryfunctioninpatientswithmyotonicdystrophytype1 |