Characterization of muscle strength and function in dysferlinopathy in a Brazilian sample
DOI:
https://doi.org/10.33233/fb.v22i6.4919Keywords:
muscular dystrophies limb-girdle; neuromuscular diseases; dysferlin; muscle weakness; motor skills; disability evaluationAbstract
The study of the characterization of muscle strength and function in dysferlinopathy to establish biomarkers of motor skills. Sample of 40 patients with muscle strength with MRC (Medical Research Council), MRC percentage, timed motor performances for walking, Vignos scales, Egen Klassifikation, Functional Assessment for Duchenne muscular dystrophy (FES-DMD) and North Star Ambulatory Assessment Adapted (aNSAA). Prevalence of dysferlinopathy of 25.5% in all dysthophies studied (1340), mean age of 36.5 years, 52.5% of males and 75% of walking. Weak muscles: abdominal, gluteus, iliopsoas, hamstrings, quadriceps femoris, tibialis anterior and medial deltoid. Strong correlation between MRC and time to walk (mean r=0.77) and very strong MRC distal lower limbs with aNSAA (r=0.90). Interaction of MRC of upper limbs and lower limbs between the proximal and distal segments (p<0.001), being more evident in upper limbs than in lower limbs. Variable rate of disease progression, with 60% of patients moderately or severely affected with > 12 years of disease. The findings characterize the pattern of muscular weakness of Brazilians with dysferlinopathy as proximal and distal lower limbs, with associated involvement of the proximal upper limbs, besides elucidating the motor abilities in relation to the locomotion process and cardiorespiratory diseases.
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