Alternative instrument for the evaluation of handgrip strength in Duchenne muscular dystrophy.

BACKGROUND: The commonly used dynamometers can be ineffective in evaluating handgrip in patients with Duchenne muscular dystrophy (DMD), especially children with generalized muscle weakness. The aim of this study was to analyze whether the modified sphygmomanometer is an effective instrument for handgrip strength evaluation in patients with DMD, during different stages of the disease. METHOD: The handgrip strength of 33 patients was evaluated by the Jamar dynamometer and the modified sphygmomanometer. Motor function was evaluated by the Motor Function Measurement (MFM) scale. Four evaluations, with a six-month interval between each, were performed: Evaluation 1 (N = 33), Evaluation 2 (N = 24), Evaluation 3 (N = 15), and Evaluation 4 (N = 8). A linear regression model with mixed effects was used for the longitudinal data and descriptive analysis of strength for all four evaluations. RESULT: The first evaluation data presented very high correlations between the dynamometer and the modified sphygmomanometer (r = 0.977; p < 0.001). The longitudinal analysis showed a significant difference between Evaluation 1 and the other handgrip strength evaluations obtained using the dynamometer (p < 0.05) but not the modified sphygmomanometer (p > 0.05). Null values were obtained only when using the dynamometer device. CONCLUSION: The modified sphygmomanometer seems to be more suitable than the dynamometer for measuring handgrip strength in all stages of DMD.

The use of the gait profile score and gait variable score in individuals with Duchenne Muscular Dystrophy.

Therapeutic gait interventions for individuals with Duchenne Muscular Dystrophy (DMD) should be based on understanding how movement of the individual is affected and whether different clusters of individuals, determined by clinical severity, differ. Gait indexes have been developed to synthesize the data provided by the three-dimensional (3D) gait analysis such as the Gait Deviation Index (GDI) and the Gait Profile Score (GPS) where the gait variable score (GVS) can be calculated. The objective this study was to evaluate the potential use of the GDI and GPS and MAP using data from 3D gait analysis of DMD patients. The dimension 1 score of the Motor Function Measurement defined the groups that composed the cluster analysis. Twenty patients with DMD composed 2 groups according to the cluster analysis (Cluster 1, n = 10; Cluster 2, n = 10). Three-dimensional gait analysis was conducted where GDI, GPS and GVS (pelvic tilt/obliquity; hip flexion-extension/adduction-abduction/rotation; knee flexion-extension; ankle dorsiflexion-plantarflexion, foot progression angle) were calculated. Cluster 1 group presented lower hip flexion-extension and lower pelvic obliquity when compared with Cluster 2 group (p < 0.05). There was no difference between groups for GDI, GPS total and maximum isometric muscle strength of the lower limbs (p > 0.05). This study showed that GVS could detect alterations on the parameters obtained using three-dimensional gait analysis for those DMD patients separated according to motor function regarding pelvic and hip kinematic patterns. The rehabilitation of patients with DMD is recommended from the early stages of the disease (as Cluster 1, with >MFM) with the hip joint being the therapeutic target.

Efeito do uso das órteses no prolongamento da marcha de pacientes com distrofia muscular de Duchenne: revisão da literatura / Effect of using orthoses on prolonging ambulation in patients with Duchenne Muscular Dystrophy: review of literature

A capacidade de marcha em pacientes com distrofia muscular de Duchenne diminui progressivamente devido ao avanço da fraqueza e encurtamento muscular. As órteses para membros inferiores são frequentemente prescritas na tentativa de prolongar a marcha nestes pacientes. Objetivo: Realizar uma revisão da literatura a fim de verificar o efeito do uso das órteses em relação ao prolongamento do tempo de marcha. Método: Foi realizado um levantamento bibliográfico nas bases PUBMED, PEDRO e SCIELO com as palavras-chave orthoses, bracing, gait, gait loss, ambulation, Duchenne muscular dystrophy. Resultados: Nos quatorze artigos selecionados foi identificada a prescrição das órteses do tipo KAFO (também chamadas de órteses longas) e AFO, sempre associada a outra intervenção terapêutica. A maioria dos estudos relatou que o uso do dispositivo prolonga o tempo de marcha. Conclusão: O uso da órtese, independentemente do tipo, prolonga a deambulação, pois retarda o avanço de encurtamentos. Assim, sugere-se o início precoce da AFO a fim de minimizar o prejuízo funcional característico da doença.

The walking ability of patients with Duchenne muscular dystrophy gradually decreases due to advancing weakness and muscle contracture. Lower limb orthoses are often prescribed in an attempt to prolong ambulation in these patients. Objective: To perform a literature review in order to verify the relationship between using orthoses and prolonging ambulation. Method: A literature review was performed in the PUBMED, PEDRO, and SCIELO databases with the keywords orthoses, bracing, gait, gait loss, ambulation, and Duchenne muscular dystrophy. Results: In 14 selected articles the prescription of knee-ankle-foot orthoses (KAFO) (also called long orthoses) and ankle-foot orthoses (AFO) was identified, always associated with another therapeutic intervention. Most studies have reported that the use of such a device prolongs ambulation. Conclusion: The use of orthoses, regardless of type, prolongs ambulation, because it delays the progress of muscle contracture. Thus, the early use of AFO is recommended in order to minimize the functional impairment characteristic of the disease.