This study aimed to investigate possible asymmetries and relationships between performance of dominant and non-dominant upper limbs (UL) in patients with Duchenne and Becker muscular dystrophies (DMD/BMD), to compare UL performance of patients and healthy subjects and to investigate the relationship between timed performance of UL and age, motor function and muscle strength in DMD/BMD patients. Sixteen patients with DMD and 3 with BMD were evaluated with Jebsen-Taylor Test (timed performance), Vignos scale and Dimension 3 of Motor Function Measure (motor function), and Medical Research Council scale (muscle strength) on a single session. ANOVA showed no asymmetry between dominant and non-dominant UL, except in the writing subtest, in patients and in healthy controls. There were relationships between dominant and non-dominant UL performances. Correlations between timed performance, motor function and muscle strength were found, but age was not correlated with these variables. These findings may reduce the assessment time, prevent fatigue and provide more accurate clinical reasoning involving UL in DMD/BMD treatment.
The aim of this study was to investigate the relationship between Performance of Upper Limb (PUL) and Jebsen-Taylor Test (JTT) to assess and monitor upper limb function progression in patients with muscular dystrophy. Thirty patients diagnosed with Duchenne muscular dystrophy, limb-girdle muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy Type 1, and fascioscapulohumeral dystrophy were submitted to the shoulder, elbow, and wrist domains of PUL, and to JTT subtests. Spearman tests investigated the relationships between PUL and JTT total scores and domains. Correlations were classified as strong ( r ≥ 0.70), moderate (0.40 ≤ r < 0.70), or weak ( r ≤ 0.40). There were strong correlations between the PUL and JTT total scores ( r = -0.706). Although JTT measures time and PUL provides kinesiologic scores, these measures were related. Therefore, muscle synergies, which control the compensatory movements and motor functions involving mainly shoulder, elbow, wrist, and finger movements, are related to timed performance in patients with muscular dystrophies.
Hubo gran variabilidad en el desempeño funcional de los niños con mielomeningocele, detectada por los campos autocuidado y función social de la PEDI. Los niños presentaron gran daño en el dominio de la movilidad.
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The main objective of this study was to evaluate the effects of interactive video games on functional balance and mobility in poststroke individuals. The Health Science databases accessed included Medline via PubMed, LILACS, SciELO, and PEDro. The inclusion criteria were as follows: clinical studies evaluating the use of interactive video games as a treatment to improve functional balance and mobility in individuals poststroke and studies published in the Brazilian Portuguese, English, or Spanish language between 2005 and April 2016. PEDro Scale was used to analyze the methodological quality of the studies. The Berg Balance Scale and Timed Up and Go Test (TUGT) data were evaluated using a meta-analysis, the publication bias was assessed by funnel plots, and the heterogeneity of the studies by I statistic. Eleven studies were included in the final analysis. Functional balance improved in individuals treated using interactive video games (mean difference = 2.24, 95% confidence interval [0.45, 4.04], p = .01), but no improvement was observed in mobility as measured by TUGT. The studies presented low heterogeneity (24%). The mean score on the PEDro Scale was 6.2 ± 1.9. Interactive video games were effective in improving functional balance but did not influence the mobility of individuals poststroke.
The purpose of this longitudinal study was to identify and verify the compensatory motor strategies for upper limb functioning of individuals with Duchenne Muscular Dystrophy (DMD). We evaluated 32 patients diagnosed with DMD (aged 6-19 years) for cognitive and motor functioning using the Mini Mental State Examination (MMSE), Vignos Scale Jebsen Taylor Test (JTT) and Functional Skill Scale (FSS) at baseline testing and over retest intervals of six and 12 months. We used the MMSE to screen participants for capacity to engage in the research, and we analyzed absolute and percentile changes in the frequency distribution of motor strategies participants used on each JTT subtest. We also used analysis of variance with repeated measures and Bonferroni post-hoc testing of multiple comparisons to identify disease progression through FSS scores. We observed an increased frequency of compensatory motor strategies over six months. We recommend the associated use of the JTT and FSS to assess patients with DMD, since we observed worsened movement quality over a time interval of six months even while essential motor competence was maintained.
Background and ObjectivesNemaline myopathy (NM) is a genetically heterogeneous inherited myopathy related with at least 12 genes, whereas pathogenic variants inNEBgene are the most common genetic cause. The clinical spectrum of NM caused by NEB pathogenic variants (NM-NEB) is very broad, ranging from mild to severe presentations manifesting with generalized weakness, as well as respiratory and bulbar involvement. There is currently not enough data regarding the progression of the disease. In this study, we present a genotypic and phenotypic spectrum of 33 patients with NM caused by NEB variants (NM-NEB) classified according to age groups and the use of ventilatory support. We focused on interventional support, genotype-phenotype correlation, and association between respiratory, bulbar, and motor systems in groups of patients stratified by age and by the use of ventilatory support (VS).MethodsClinical and genetic data from patients with NM-NEB followed up in one specialized center were collected through regular consultations. Patients were evaluated regarding motor, bulbar, and respiratory functions.ResultsThirty-three patients with NM-NEB were evaluated consisting of 15 females and 18 males with an average age of 18 (±12) years and a median of 17 (±11) years. 32% of patients with NM-NEB used a G tube, 35% were not able to walk without support, and 55% needed VS. Scoliosis and dysphagia were more common among patients who used VS. Described for the first time, half of the patients presented tongue atrophy in a triple furrow pattern, and the presence of the atrophy was associated with dysphagia. Comparing the patients grouped by age, we found that, proportionally, older patients had more scoliosis and respiratory dysfunction than younger groups, suggesting the progression of the disease in these domains. In addition to that, we showed that VS use was associated with scoliosis and dysphagia.DiscussionNM-NEB is a very debilitating disease. There is an association between scoliosis and respiratory dysfunction while patients using VS have more often scoliosis than the no-VS group. Triple furrow tongue atrophy is a novel and frequent finding, which is directly associated with dysphagia. Grouping patients by age suggested disease stability in motor and swallow function, but a progression in respiratory dysfunction and skeletal deformities. All observations are relevant in the management care of patients with NM.
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