Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

Løkken, Nicoline; Hedermann, Gitte; Thomsen, Carsten; Vissing, John

doi:10.1002/ana.24743

Cited by 44 publications

(61 citation statements)

References 22 publications

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“…Additionally, KEMVC in the present study was 25% less than CTRL, compared with 55–58% in previous studies of FSHD . The small differences between the present and previous research can be attributed to the heterogeneity of conditions, participant sex differences (present study all male participants), participants ages, differences in strength assessment, and condition severity in the participant groups [e.g. 47% non‐ambulant (excluding DMD) in the current study], with previous research typically all ambulant .…”

Section: Discussioncontrasting

confidence: 74%

“…Differences from CTRL in the present study, are similar to those reported previously. Of the other MDs measured (BMD, LGMD, FSHD), PFMVC in the present study was 42–49% less than CTRL, compared with 48–61% reported in adults with BMD and LGMD . Additionally, KEMVC in the present study was 25% less than CTRL, compared with 55–58% in previous studies of FSHD .…”

Section: Discussioncontrasting

confidence: 67%

“…The small differences between the present and previous research can be attributed to the heterogeneity of conditions, participant sex differences (present study all male participants), participants ages, differences in strength assessment, and condition severity in the participant groups [e.g. 47% non‐ambulant (excluding DMD) in the current study], with previous research typically all ambulant . The present data are particularly novel in the objective assessment of SB and PA as contributing factors to the differences between groups and participants with MD.…”

Section: Discussioncontrasting

confidence: 60%

“…Of these comparisons, lower isometric maximal voluntary contraction (MVC) strength was attributed to smaller lean mass in the knee extension (KEMVC) muscle group in FSHD . While Løkken et al showed plantar flexors MVC (PFMVC) was associated with cross‐sectional area in adults with LGMD2I. Decreased muscle strength has been shown within children and adolescents with DMD .…”

Section: Introductionmentioning

confidence: 99%

“…The nature of LGMD and FSHD results in great variance with various classifications within each condition, dependent upon the proteins affected . Despite the well‐established molecular and symptomatic differences between these MDs, direct comparisons between muscle strength and size between the conditions, and the potential influence of PA, have not been systematically addressed …”

Section: Introductionmentioning

confidence: 99%

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Relationships between muscle size, strength, and physical activity in adults with muscular dystrophy

Jacques

Onambélé-Pearson

Reeves

et al. 2018

J cachexia sarcopenia muscle

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BackgroundMuscular dystrophy (MD) is characterized by progressive muscle wasting and weakness, yet few comparisons to non‐MD controls (CTRL) of muscle strength and size in this adult population exist. Physical activity (PA) is promoted to maintain health and muscle strength within MD; however, PA reporting in adults with MD is limited to recall data, and its impact on muscle strength is seldom explored.MethodsThis study included 76 participants: 16 non‐MD (CTRL, mean age 35.4), 15 Duchenne MD (DMD, mean age 24.2), 18 Becker's MD (BMD, mean age 42.4), 13 limb‐girdle MD (LGMD, mean age 43.1), and 14 facioscapulohumeral MD (mean age 47.7). Body fat (%) and lean body mass (LBM) were measured using bioelectrical‐impedance. Gastrocnemius medialis (GM) anatomical cross‐sectional area (ACSA) was determined using B‐mode ultrasound. Isometric maximal voluntary contraction (MVC) was assessed during plantar flexion (PFMVC) and knee extension (KEMVC). PA was measured for seven continuous days using triaxial accelerometry and was expressed as daily average minutes being physically active (TPAmins) or average daily percentage of waking hours being sedentary (sedentary behaviour). Additionally, 10 m walk time was assessed.ResultsMuscular dystrophy groups had 34–46% higher body fat (%) than CTRL. DMD showed differences in LBM with 21–28% less LBM than all other groups. PFMVC and KEMVC were 36–75% and 24–92% lower, respectively, in MD groups than CTRL. GM ACSA was 47% and 39% larger in BMD and LGMD, respectively, compared with CTRL. PFMVC was associated with GM ACSA in DMD (P = 0.026, R = 0.429) and CTRL (P = 0.015, R = 0.553). MD groups were 14–38% more sedentary than CTRL groups, while DMD were more sedentary than BMD (14%), LGMD (8%), and facioscapulohumeral MD (14%). Sedentary behaviour was associated with LBM in DMD participants (P = 0.021, R = −0.446). TPAmins was associated with KEMVC (P = 0.020, R = 0.540) in BMD participants, while TPAmins was also the best predictor of 10 m walk time (P < 0.001, R 2 = 0.540) in ambulant MD, revealed by multiple linear regression.ConclusionsQuantified muscle weakness and impaired 10 m walking time is reported in adults with MD. Muscle weakness and 10 m walk time were associated with lower levels of TPA in adults with MD. Higher levels of sedentary behaviour were associated with reduced LBM in DMD. These findings suggest a need for investigations into patterns of PA behaviour, and relevant interventions to reduce sedentary behaviour and encourage PA in adults with MD regardless of impairment severity.

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Section: Discussioncontrasting

confidence: 74%

Section: Discussioncontrasting

confidence: 67%

Section: Discussioncontrasting

confidence: 60%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Relationships between muscle size, strength, and physical activity in adults with muscular dystrophy

Jacques

Onambélé-Pearson

Reeves

et al. 2018

J cachexia sarcopenia muscle

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Natural history of limb girdle muscular dystrophy R9 over 6 years: searching for trial endpoints

Murphy

Morrow

Dahlqvist

et al. 2019

Ann Clin Transl Neurol

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Objective Limb girdle muscular dystrophy type R9 (LGMD R9) is an autosomal recessive muscle disease for which there is currently no causative treatment. The development of putative therapies requires sensitive outcome measures for clinical trials in this slowly progressing condition. This study extends functional assessments and MRI muscle fat fraction measurements in an LGMD R9 cohort across 6 years. Methods Twenty‐three participants with LGMD R9, previously assessed over a 1‐year period, were re‐enrolled at 6 years. Standardized functional assessments were performed including: myometry, timed tests, and spirometry testing. Quantitative MRI was used to measure fat fraction in lower limb skeletal muscle groups. Results At 6 years, all 14 muscle groups assessed demonstrated significant increases in fat fraction, compared to eight groups in the 1‐year follow‐up study. In direct contrast to the 1‐year follow‐up, the 6‐min walk test, 10‐m walk or run, timed up and go, stair ascend, stair descend and chair rise demonstrated significant decline. Among the functional tests, only FVC significantly declined over both the 1‐ and 6‐year studies. Interpretation These results further support fat fraction measurements as a primary outcome measure alongside functional assessments. The most appropriate individual muscles are the vastus lateralis, gracilis, sartorius, and gastrocnemii. Using composite groups of lower leg muscles, thigh muscles, or triceps surae, yielded high standardized response means (SRMs). Over 6 years, quantitative fat fraction assessment demonstrated higher SRM values than seen in functional tests suggesting greater responsiveness to disease progression.

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MRI in Neuromuscular Diseases: An Emerging Diagnostic Tool and Biomarker for Prognosis and Efficacy

Dahlqvist

Widholm

Leinhard

et al. 2020

Annals of Neurology

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There is an unmet need to identify biomarkers sensitive to change in rare, slowly progressive neuromuscular diseases. Quantitative magnetic resonance imaging (MRI) of muscle may offer this opportunity, as it is noninvasive and can be carried out almost independent of patient cooperation and disease severity. Muscle fat content correlates with muscle function in neuromuscular diseases, and changes in fat content precede changes in function, which suggests that muscle MRI is a strong biomarker candidate to predict prognosis and treatment efficacy. In this paper, we review the evidence suggesting that muscle MRI may be an important biomarker for diagnosis and to monitor change in disease severity.

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Contractile properties are disrupted in Becker muscular dystrophy, but not in limb girdle type 2I

Cited by 44 publications

References 22 publications

Relationships between muscle size, strength, and physical activity in adults with muscular dystrophy

Relationships between muscle size, strength, and physical activity in adults with muscular dystrophy

Natural history of limb girdle muscular dystrophy R9 over 6 years: searching for trial endpoints

MRI in Neuromuscular Diseases: An Emerging Diagnostic Tool and Biomarker for Prognosis and Efficacy

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