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.

This pilot version of FES-DMD-GD showed reliability and correlated to age and timed motor performance.

Rising from the floor, climbing up, and climbing down steps have some components in common, such as the demand for muscle strength and the recruitment of compensatory muscle synergies, as DMD progresses. To sit down on the floor, some children let themselves fall, resulting in a faster performance, but more compensatory movements.

The authors developed an evaluation scale for sit-stand from the ground for children with Duchenne muscular dystrophy (DMD) and tested its reliability. The construction occurred in stages: (a) the characterization of the movement in healthy children, (b) the characterization of the movement in children with DMD, (c) the elaboration of the 1st version of the scale and the manual, (d) the evaluation by experts and readjustments, and (e) the analysis of inter- and intraexaminer reliability and correlation with the Vignos Scale, age, and time for the execution of the activity. The scale comprehended 3 phases for sitting and 5 for the standing. A very good repeatability of the measures of sitting and standing (ICC = 0.89 and 0.84, respectively) and excellent reproducibility (ICC = 0.93 and 0.92, respectively) was demonstrated. The Kappa coefficient for the 8 phases in the interexaminer analysis varied from 0.77 to 1.00 (excellent reliability), and in the intraexaminer analysis varied from 0.80 to 1.00 (excellent reliability). Good correlation was found between the variables on the Vignos Scale (age: r = 0.58; stand: r = 0.56). The scale is a reliability instrument that allows evaluation of the activity of sitting and standing in children with DMD.

The authors aimed to (a) develop a scale to evaluate non-wheelchair-dependent children with Duchenne muscular dystrophy (DMD) while sitting and standing from the chair, (b) test its reliability, and (c) correlate the scores of this scale with the time, the age and the Vignos. Thirty DMD boys performed sit-to-stand and stand-to-sit from the chair 4 times. Scale development was based on a previous movement characterization in healthy children and in DMD children and on suggestions by physical therapists with expertise in DMD. The final version of the scale was submitted to the analysis of reliability. The sitting evaluation consists of phases: flexion, contact of the hip with the chair, extension. The standing evaluation comprehends the phases: flexion; transference; extension. Sitting and standing phases presented an excellent reliability (intraclass correlation coefficient [ICC] ≥ 0.91) and a good reproducibility (ICC ≥ 0.89). The scores generated by sitting on the chair correlated to the time taken to perform the tasks (r = .69) and to the age of the patient (r = .44) and the score of standing from the chair also correlated to the time of performance (r = .66). The sit-to-stand functional evaluation scale DMD is a reliable assessment tool that allows the description and quantification of the functional performance of DMD children.

Measuring muscle strength and motor function is part of Duchenne muscular dystrophy (DMD) assessment. However, the relationship between these variables is controversial. Objective To investigate the relationship between muscle strength and motor function and between these variables and age. Method Muscle strength was measured by Medical Research Council (MRC) scale and motor function, by Motor Function Measure (MFM), in 40 non-ambulatory patients. Spearman tests investigated the relationships between muscle strength, motor function and age. Results Total MRC and MFM scores were strongly related to each other (r = 0.94; p < 0.001), but not to age (r = -0.19, r = -0.31, respectively; p > 0.05). Strong and moderate relationships between partial muscle strength and motor function scores were found. Higher correlation coefficients were found between total scores and Dimensions 2 (axial/ proximal control) and 3 (distal control) of MFM. Conclusion Muscle strength and motor function are strongly correlated and seem to decrease proportionally in DMD.

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Objective: To determine how often sitting/rising from a chair should be assessed in Duchenne muscular dystrophy (DMD) patients to avoid redundant/missing data. Methods: Sitting/rising from a chair was evaluated in 26 DMD children (5-12 yrs), in three-month intervals, over twelve months, with the Functional Evaluation Scale (domain sitting/rising from a chair). Scores were compared by effect sizes (ES) and standardized response means (SRM) (responsiveness analysis). Results: Sit-to-stand showed low-to-moderate responsiveness in three-month intervals