Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy

Abstract

Objective: During the transitional phase (ambulatory to non-ambulatory), synergies characterize the evolution of Duchenne muscular dystrophy (DMD). This study was performed to describe and quantify compensatory movements while sitting down on/rising from the floor and climbing up/down steps. Method: Eighty videos (5 children × 4 assessments × 4 tasks) were recorded quarterly in the year prior to gait loss. Compensatory movements from the videos were registered based on the Functional Evaluation Scale for DMD. Results: The most frequently observed compensatory movements were upper limb support on lower limbs/floor/handrail during all the tasks and lumbar hyperlordosis, trunk support on handrail, equinus foot, increased base of support, non-alternated descent, and pauses while climbing up/down steps. Conclusion: Climbing up/down steps showed a higher number of compensatory movements than sitting down on/rising from the floor, which seemed to be lost before climbing up/down steps in ambulatory children with DMD.Keywords: Duchenne muscular dystrophy, evaluation, scales. RESUMOObjetivo: Durante a fase de transição (deambulador -não-deambulador), sinergias musculares caracterizam a evolução da distrofia muscular de Duchenne (DMD). Este estudo visou descrever e quantificar os movimentos compensatórios durante o sentar/levantar do solo, subir/descer degraus. Oitenta vídeos (5 crianças × 4 avaliações × 4 tarefas) foram gravados trimestralmente durante o ano que antecedeu a perda da marcha. Método: Os movimentos compensatórios dos vídeos foram registrados utilizando a Escala de Avaliação Funcional para DMD. Resultados: Os movimentos compensatórios mais frequentemente observados foram apoio de membros superiores nos membros inferiores/solo/corrimão durante todas as tarefas funcionais e hiperlordose lombar, apoio de tronco no corrimão, pés equinos, aumento da base de suporte, descida não alternada e pausas ao subir/descer degraus. Subir/descer degraus apresentou um número maior de movimentos compensatórios do que sentar/levantar do solo. Conclusão: Sentar/levantar do solo foram habilidades perdidas antes de subir/descer degraus em crianças com DMD.Palavras-chave: distrofia muscular de Duchenne, avaliação, escalas.

How often should sitting and rising from a chair be evaluated in patients with Duchenne muscular dystrophy?

“…The spontaneous selection of compensatory movements can vary among DMD patients, although some synergies are more frequent than others 6 . The progression of DMD is variable, due to genetic and environmental heterogeneity 23 .…”

“…However, many DMD patients stop performing this activity much earlier than their loss of gait. Therefore, sitting and standing from a chair can be a more accurate biomarker in the analysis of DMD progression prior to gait loss 6 . Sitting and rising from the chair is scored from 0-44 and 0-54, respectively.…”

“…Studies have shown the relevance of motor function follow up to monitor DMD accurately 2,3,4 . Global motor scales should be complemented by functional tasks assessment because, despite the continued decline in muscle strength, children with DMD continue to perform the activities using compensatory movements 5,6 . The analysis of compensatory movements employed during functional activities shows the changes in muscular synergies.…”

“…The analysis of compensatory movements employed during functional activities shows the changes in muscular synergies. Compensatory movements are performed by DMD patients to compensate for muscle weakness, mobility loss, and to deal with task demands 6 .…”

Predicting the loss of ambulation in Duchenne muscular dystrophy

“…9 The authors des cribed and quantified compensatory movements while sitting down/rising from the floor, and while climbing up/down steps. The most frequently observed compensatory move ments were upper limbs support on the lower limbs/floor/handrail during all tasks and lumbar hyperlordosis, trunk support on handrail, equines foot, increased base of support, nonalternated descent, and pauses while climbing up/down steps.…”