Michele Emy Hukuda

Elaboração e confiabilidade da escala funcional do subir e do descer escada para Distrofia Muscular de Duchenne

CONTEXTUALIZACAO: Os instrumentos de avaliacao funcional utilizados para pacientes com distrofia muscular de Duchenne (DMD), citados na literatura, sao limitados e escassos, dificultando a tomada de decisao clinica fisioterapeutica. OBJETIVOS: Descrever o processo de criacao de uma escala de avaliacao funcional do subir e do descer escadas, especifica para criancas com diagnostico de DMD, e examinar sua confiabilidade inter e intraexaminadores. METODOS: A construcao da escala seguiu cinco etapas, a saber, elaboracao de um roteiro para observacao dirigida com base na literatura; observacao do subir e do descer em 120 registros filmados de 30 criancas com DMD (5 a 11 anos); elaboracao da escala, considerando o grau crescente de dificuldade de execucao dos movimentos; criacao do manual de preenchimento e submissao da escala e do manual a 10 examinadores, seguida de reajustes para criacao da versao final. A confiabilidade foi testada pelo pesquisador (repetibilidade) e dois examinadores independentes (reprodutibilidade). Utilizou-se o Indice de Correlacao Intra-Classe (ICC) e a Correlacao de Kappa Ponderado. RESULTADOS: A escala elaborada abrange cinco fases para o subir e quatro fases para o descer escadas. Encontrou-se excelente confiabilidade intra/interexaminadores, com valores da Correlacao de Kappa Ponderado > 0,78 em todas as fases e ICCs > 0,89, com p<0,05 entre todos os escores. CONCLUSAO: A escala proposta mostrou excelente repetibilidade e reprodutibilidade, sendo necessaria a continuidade do estudo com os objetivos de avaliar sua acuracia e validade e de construir uma ferramenta digital para otimizar a coleta de dados.

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

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.

Evaluation Scale Development, Reliability for Sitting and Standing From the Chair for Duchenne Muscular Dystrophy

ABSTRACT 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.

Development of an Evaluation Scale for Sitting and Standing From the Ground for Children With Duchenne Muscular Dystrophy

ABSTRACT 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.

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.

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.

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.

Relationship between muscle strength and motor function in Duchenne muscular dystrophy.

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.

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

ABSTRACT 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.