Relationship between the climbing up and climbing down stairs domain scores on the FES-DMD, the score on the Vignos Scale, age and timed performance of functional activities in boys with Duchenne muscular dystrophy

Abstract

BACKGROUND: Knowing the potential for and limitations of information generated using different evaluation instruments favors the development of more accurate functional diagnoses and therapeutic decision-making. OBJECTIVE: To investigate the relationship between the number of compensatory movements when climbing up and going down stairs, age, functional classification and time taken to perform a tested activity (TA) of going up and down stairs in boys with Duchenne muscular dystrophy (DMD). METHOD: A bank of movies featuring 30 boys with DMD performing functional activities was evaluated. Compensatory movements were assessed using the climbing up and going down stairs domain of the Functional Evaluation Scale for Duchenne Muscular Dystrophy (FES-DMD); age in years; functional classification using the Vignos Scale (VS), and TA using a timer. Statistical analyses were performed using the Spearman correlation test. RESULTS: There is a moderate relationship between the climbing up stairs domain of the FES-DMD and age (r=0.53, p=0.004) and strong relationships with VS (r=0.72, p=0.001) and TA for this task (r=0.83, p<0.001). There were weak relationships between the going down stairs domain of the FES-DMD-going down stairs with age (r=0.40, p=0.032), VS (r=0.65, p=0.002) and TA for this task (r=0.40, p=0.034). CONCLUSION: These findings indicate that the evaluation of compensatory movements used when climbing up stairs can provide more relevant information about the evolution of the disease, although the activity of going down stairs should be investigated, with the aim of enriching guidance and strengthening accident prevention. Data from the FES-DMD, age, VS and TA can be used in a complementary way to formulate functional diagnoses. Longitudinal studies and with broader age groups may supplement this information.

Boys With Duchenne Muscular Dystrophy: 1-Year Locomotor Changes in Relation to a Control Group

“…In the present study, in which patients with Vignos 1-3 (Vignos et al, 1963) participated, the timed performance of the DMD and control groups did not differ in the first assessment. However, timed performance can be longer (and less efficient) in patients with higher Vignos classifications, as observed in past studies (Fernandes et al, 2014;Martini et al, 2015) and in the present study (timed performance got longer as DMD progressed).…”

“…Both Carvalho et al (2015) and Martini et al (2015) found correlations between the number of compensatory movements and the timed performance on 10 -m walking. Fernandes et al (2014) found that timed performance correlated with the number of compensatory movements on climbing up and down steps. These studies show that for samples of ambulatory children with DMD with higher clinical variability (Vignos 1-6) timed performance and compensatory movements are correlated.…”

“…However, when less severe patients are included, there may be high variability in compensatory movements in locomotion tasks, without the same variability in performance time, as seen in the present study. Fernandes et al (2014) investigated the correlation between timed performance and compensatory movements in climbing steps in younger children with DMD (mean age ¼ 7 years). On climbing up steps, timed performance was strongly correlated to compensatory movements (r ¼ .83); but, on climbing down steps, the correlation was weaker (r ¼ .40).…”

Validação da versão brasileira da escala "Medida da Função Motora - Versão Reduzida (MFM-20)" para doenças neuromusculares em crianças de dois a sete anos de idade

“…Adicionalmente, visto que o declínio das atividades motoras durante o curso da doença é inevitável, o uso de escalas de avaliação funcional se faz necessário para acompanhamento clínico e guia de condutas terapêuticas. Na prática clínica, a observação de atividades funcionais é um exame simples e acessível 25 30,[32][33][34][35]37 .…”

“…Quanto maior a pontuação obtida, maior o comprometimento e a dependência funcional apresentada pelo paciente 25,39,40 46,47,48,49,50,51 .…”