Neck flexor muscle strength and its relation with functional performance in Duchenne muscular dystrophy

2017 ◽  
Vol 21 (3) ◽  
pp. 494-499
Author(s):  
Sibel Bozgeyik ◽  
İpek Alemdaroğlu ◽  
Numan Bulut ◽  
Öznur Yılmaz ◽  
Ayşe Karaduman
2010 ◽  
Vol 90 (12) ◽  
pp. 1774-1782 ◽  
Author(s):  
Marc Roig ◽  
Janice J. Eng ◽  
Donna L. MacIntyre ◽  
Jeremy D. Road ◽  
W. Darlene Reid

Background The Stair Climb Power Test (SCPT) is a functional test associated with leg muscle power in older people. Objective The purposes of this study were to compare the results of the SCPT in people with chronic obstructive pulmonary disease (COPD) and people who were healthy and to explore associations of the SCPT with muscle strength (force-generating capacity) and functional performance. Design The study was a cross-sectional investigation. Methods Twenty-one people with COPD and a predicted mean (SD) percentage of forced expiratory volume in 1 second of 47.2 (12.9) and 21 people who were healthy and matched for age, sex, and body mass were tested with the SCPT. Knee extensor and flexor muscle torque was assessed with an isokinetic dynamometer. Functional performance was assessed with the Timed “Up & Go” Test (TUG) and the Six-Minute Walk Test (6MWT). Results People with COPD showed lower values on the SCPT (28%) and all torque measures (∼32%), except for eccentric knee flexor muscle torque. In people with COPD, performance on the TUG and 6MWT was lower by 23% and 28%, respectively. In people with COPD, the SCPT was moderately associated with knee extensor muscle isometric and eccentric torque (r≥.46) and strongly associated (r=.68) with the 6MWT. In people who were healthy, the association of the SCPT with knee extensor muscle torque tended to be stronger (r≥.66); however, no significant relationship between the SCPT and measures of functional performance was found. Limitations The observational design of the study and the use of a relatively small convenience sample limit the generalizability of the findings. Conclusions The SCPT is a simple and safe test associated with measures of functional performance in people with COPD. People with COPD show deficits on the SCPT. However, the SCPT is only moderately associated with muscle torque and thus cannot be used as a simple surrogate for muscle strength in people with COPD.


2021 ◽  
pp. 1-14
Author(s):  
Cathleen E. Buckon ◽  
Susan E. Sienko ◽  
Eileen G. Fowler ◽  
Anita M. Bagley ◽  
Loretta A. Staudt ◽  
...  

Background: Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disorder, that is characterized by progressive muscle degeneration and loss of ambulation between 7–13 years of age. Novel pharmacological agents targeting the genetic defects and disease mechanisms are becoming available; however, corticosteroid (CS) therapy remains the standard of care. Objective: The purpose of this longitudinal study was to elucidate the effect of CS therapy on the rate of muscle strength and gross motor skill decline in boys with DMD and assess the sensitivity of selected outcome measures. Methods: Eighty-four ambulatory boys with DMD (49–180 months), 70 on CS, 14 corticosteroid naïve (NCS), participated in this 8-year multi-site study. Outcomes included; isokinetic dynamometry, the Standing (STD) and Walking/Running/jumping (WRJ) dimensions of the Gross Motor Function Measure (GMFM), and Timed Function Tests (TFTs). Nonlinear mixed modeling procedures determined the rate of change with age and the influence of steroids. Results: Despite CS therapy the rate of decline in strength with age was significant in all muscle groups assessed. CS therapy significantly slowed decline in knee extensor strength, as the NCS group declined at 3x the rate of the CS group. Concurrently, WRJ skills declined in the NCS group at twice the rate of the CS group. 4-stair climb and 10 meter walk/run performance was superior in the boys on CS therapy. Conclusion: CS therapy slowed the rate of muscle strength decline and afforded longer retention of select gross motor skills in boys on CS compared to boys who were NCS. Isokinetic dynamometry, Walk/Run/Jump skills, and select TFTs may prove informative in assessing the efficacy of new therapeutics in ambulatory boys with DMD.


2019 ◽  
Vol 28 (16) ◽  
pp. 2686-2695 ◽  
Author(s):  
Pamela Barraza-Flores ◽  
Tatiana M Fontelonga ◽  
Ryan D Wuebbles ◽  
Hailey J Hermann ◽  
Andreia M Nunes ◽  
...  

Abstract Duchenne muscular dystrophy (DMD) is a devastating X-linked disease affecting ~1 in 5000 males. DMD patients exhibit progressive muscle degeneration and weakness, leading to loss of ambulation and premature death from cardiopulmonary failure. We previously reported that mouse Laminin-111 (msLam-111) protein could reduce muscle pathology and improve muscle function in the mdx mouse model for DMD. In this study, we examined the ability of msLam-111 to prevent muscle disease progression in the golden retriever muscular dystrophy (GRMD) dog model of DMD. The msLam-111 protein was injected into the cranial tibial muscle compartment of GRMD dogs and muscle strength and pathology were assessed. The results showed that msLam-111 treatment increased muscle fiber regeneration and repair with improved muscle strength and reduced muscle fibrosis in the GRMD model. Together, these findings support the idea that Laminin-111 could serve as a novel protein therapy for the treatment of DMD.


2014 ◽  
Vol 56 (5) ◽  
pp. 748-752 ◽  
Author(s):  
Shigemi Kimura ◽  
Shiro Ozasa ◽  
Keiko Nomura ◽  
Kowashi Yoshioka ◽  
Fumio Endo

2017 ◽  
Vol 8 (6) ◽  
pp. 72-79
Author(s):  
Vasiliy M. Suslov ◽  
Alexander V. Pozdnyakov ◽  
Dmitry O. Ivanov ◽  
Dmitry I. Rudenko ◽  
Damir A. Malekov ◽  
...  

Because of the necessity of objective instrumental methods for assessing the state of skeletal musculature in patients with Duchenne muscular dystrophy, the aim of our work was to evaluate the correlation between manual methods of assessment of the muscle strength and the results of magnetic resonance imaging (MRI) of the skeletal muscles of lower limbs. We have examined 15 corticosteroid-naive patients with Duchenne muscular dystrophy. Patients were divided into 2 groups: ambulant patients (average age 8.1 years) and non-ambulant patients (mean age 12.7 years). Muscle strength of lower extremities of all patients has been evaluated by Medical Research Council scale and MRI of skeletal muscles of the pelvic girdle, thighs and lower legs has been performed. The following results have been obtained: ambulant patients have been characterized by a high correlation of MRC scale and MRI of the lower extremities in the evaluation of the pelvic girdle and thighs muscles and the total score (pelvic girdle, thighs and lower legs) and salient correlation in the evaluation of the lower legs muscles. Non-ambulant patients have been characterized by moderate correlation in the evaluation of pelvic girdle and thighs muscles, as well as the total score of lower limbs. There was no correlation between MRC and MRI results in the muscles of the lower legs. Magnetic resonance tomography is able to reliably estimate the degree of fatty infiltration in Duchenne muscular dystrophy and is the method of choice in the diagnosis and evaluation of the severity of this disease.


Sign in / Sign up

Export Citation Format

Share Document