Responsiveness of the Gait Domain of the Functional Evaluation Scale for Children with Duchenne Muscular Dystrophy.

AIM: To determine the responsiveness of functional gait assessment of children with Duchenne muscular dystrophy (DMD). METHOD: A total of 160 films of 32 children (mean age: 9.5 ± 2.7) with DMD were scored by the gait domain of the Functional Evaluation Scale - DMD. Children were recorded every 3 months for 1 year (0, 3, 6, 9, and 12 months). Responsiveness was analyzed by the effect sizes (ES) and standardized response means (SRM). RESULTS: Responsiveness was low to moderate at the 3-month interval (ES 0.12 to 0.34; SRM 0.27 to 0.80); low to high at the 6-month interval (ES 0.36 to 0.72; SRM 0.37 to 1.10); moderate to high at the 9-month interval (ES 0.70 to 1.0; SRM 0.50 to 1.43), and it was high at the 12-month interval (ES 0.74 to 1.34; SRM 0.88 to 1.53). CONCLUSION: Functional gait assessment of children with DMD was responsive since 3-month intervals. Responsiveness increased as reassessment intervals got longer. The highest responsiveness was observed when children were reevaluated after 12 months. The use of the gait domain of the Functional Evaluation Scale - DMD is recommended in 6- to 12-month intervals, which showed moderate to high responsiveness.

Development and Reliability of the Functional Evaluation Scale for Duchenne Muscular Dystrophy, Gait Domain: A Pilot Study.

BACKGROUND AND PURPOSE: The progression of Duchenne muscular dystrophy (DMD) results in the emergence of multiple and varied synergies to compensate muscle weakness and to deal with the demands of the functional tasks (e.g. gait). No functional evaluation instrument for individuals with DMD allows the detailed description (subjective qualitative evaluation) and compensatory movement scoring (objective quantitative evaluation) exclusively of gait. For this reason, clinicians and therapists face difficulties in assessment and decision-making of this functional activity. This study aimed to elaborate the gait domain of the Functional Evaluation Scale for DMD (FES-DMD-GD) and test its intra-rater and inter-rater reliabilities and its relationship with age and timed motor performance. METHOD: We listed all the compensatory movements observed in 102 10-m gait videos of 51 children with DMD. Based on this report, the FES-DMD-GD was created and submitted to the review of 10 experts. After incorporating the experts suggestions, three examiners scored the videos using the FES-DMD-GD. The intra-rater and inter-rater reliabilities was calculated. Spearman correlation tests investigated the relationships between FES-DMD-GD and age and timed motor performance (p < 0.05). RESULTS: The FES-DMD-GD was composed of three phases and had 14 items to quantify compensatory movements on gait. Intra-class correlation coefficients ranged from acceptable (0.74) to excellent (0.99). FES-DMD-GD correlated to age and timed motor performance. CONCLUSION: This pilot version of FES-DMD-GD showed reliability and correlated to age and timed motor performance.

Evaluation scale development, reliability for sitting and standing from the chair for Duchenne muscular dystrophy.

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.