Motor assessment in patients with Duchenne muscular dystrophy

Abstract

OBJECTIVE: Evaluate muscle force and motor function in patients with Duchenne muscular dystrophy (DMD) in a period of six months. METHOD: Twenty children and adolescents with diagnosis of DMD were evaluated trough: measurement of the strength of the flexors and extensors of the shoulder, elbow, wrist, knee and ankle through the Medical Research Council (MRC), and application of the Motor Function Measure (MFM). The patients were evaluated twice within a six-month interval. RESULTS: Loss of muscle strength was identified in the MRC score for upper proximal members (t=-2.17, p=0.04). In the MFM, it was noted significant loss in the dimension 1 (t=-3.06, p=0.006). Moderate and strong correlations were found between the scores for muscular strength and the MFM dimensions. CONCLUSION: The MFM scale was a useful instrument in the follow up of patients with DMD. Moreover, it is a more comprehensive scale to assess patients and very good for conducting trials to evaluate treatment.

Relationship between muscle strength and motor function in Duchenne muscular dystrophy

“…Moreover, the present study found stronger relationships between muscle strength and MFM than previous studies 13, 15,18,19 . This fact can be attributed to the extensive practice with patients with DMD of all the examiners that participated in this study.…”

“…In 2012, Diniz et al 19 investigated the muscle strength and motor function of 20 patients with DMD. They found moderate to strong correlations between MRC scores and all Dimensions of MFM.…”

“…We found moderate correlations between lower limbs proximal and distal strength and Dimension 1 (r = 0.53, p = 0.031; r = 0.60; p = 0.020, respectively). Diniz et al 19 found a stronger correlation between the lower limbs proximal muscle strength and Dimension 1 (r = 0.90, p < 0.001). They included patients with lower Vignos scores and higher functional performance.…”

“…However, the relationship between muscle strength and motor function in patients with DMD has not been quantified, nor discussed in the literature. The studies that investigated the relationship between muscle strength and motor function included heterogeneous populations with neuromuscular diseases 12,18 , assessed few muscle groups 1,12,19 and/or did not include all the domains of MFM 19 . Besides, the findings have been contradictory.…”

Corticosteroids in Duchenne muscular dystrophy: impact on the motor function measure sensitivity to change and implications for clinical trials

“…7 Some medical teams begin corticosteroid treatment even sooner, between 2 years and 4 years of age, with encouraging results. MFM validity was also established in DMD corticosteroids-naive patients, [16][17][18] as well as in a small number of corticosteroids-treated patients for a short duration of follow-up (<2y). 8 Clinical trials in neuromuscular diseases require a rigorous approach to monitor longitudinal changes in neuromuscular status over time.…”