Mariana C. Voos

Technological Tools for Observational Evaluation - the Experience with the Software for Functional Evaluation Scale for Duchenne Muscular Dystrophy – A Pilot Study Software for Observational Evaluation

Systematic observation is an indispensable tool in clinical neurology evaluation, but data organization and record are extensive and time-consuming, requiring method and training. Reliable scales facilitate this task and technology can be decisive in the implementation of observational data routines. In this study, we aimed to show the experience of a software development to Original Research Article Albuquerque et al.; BJMMR, 16(11): 1-7, 2016; Article no.BJMMR.27482 2 optimize the application of a clinical observational scale. For this proposal it was necessary to consider the needs of the target population, text and image storing and reports generation, using computers with basic configuration, questionnaires and the measurement of time. The software allowed significant reduction in evaluation time and favored the cost-benefit of the task. The proposed methodology was adequate for this type of study.

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.

Hand Function in Muscular Dystrophies: Relationship Between Performance of Upper Limb and Jebsen–Taylor Tests

The aim of this study was to investigate the relationship between Performance of Upper Limb (PUL) and Jebsen–Taylor Test (JTT) to assess and monitor upper limb function progression in patients with muscular dystrophy. Thirty patients diagnosed with Duchenne muscular dystrophy, limb-girdle muscular dystrophy, Becker muscular dystrophy, myotonic dystrophy Type 1, and fascioscapulohumeral dystrophy were submitted to the shoulder, elbow, and wrist domains of PUL, and to JTT subtests. Spearman tests investigated the relationships between PUL and JTT total scores and domains. Correlations were classified as strong (r ≥ 0.70), moderate (0.40 ≤ r < 0.70), or weak (r ≤ 0.40). There were strong correlations between the PUL and JTT total scores (r = −0.706). Although JTT measures time and PUL provides kinesiologic scores, these measures were related. Therefore, muscle synergies, which control the compensatory movements and motor functions involving mainly shoulder, elbow, wrist, and finger movements, are related to timed performance in patients with muscular dystrophies.

Responsiveness of the domain climbing up and going down stairs of the Functional Evaluation scale for Duchenne Muscular Dystrophy: a one-year follow-up

ABSTRACT Objective: To determine the responsiveness of the domain climbing up and going down stairs of the Functional Evaluation Scale for Duchenne Muscular Dystrophy (FES-DMD-D3) in a one-year follow-up study. Method: The study included 26 patients with DMD. Effect Size (ES) and Standardized Response Mean (SRM) described the scale’s responsiveness. Results: For climbing up stairs, ES showed that responsiveness was low in the three-month assessments (0.26; 0.35; 0.13; 0.17), low to moderate in the six-month assessments (0.58; 0.48; 0.33), moderate in the nine-month assessments (0.70; 0.68), and high in the 12-month assessment (0.88). SRM showed that responsiveness was low in the three-month assessments (0.29; 0.38; 0.18; and 0.19), low to moderate in the six-month assessments (0.59; 0.51; 0.36), moderate in the nine-month assessments (0.74 and 0.70), and high in the 12-month assessment (0.89). For going down stairs, ES showed that responsiveness was low in the three- and six-month assessments (0.16; 0.25; 0.09; 0.08 and 0.48; 0.35; 0.18, respectively), low to moderate in the nine-month assessments (0.59; 0.44), and moderate in the 12-month assessment (0.71). SRM showed that responsiveness was low in the three- and six-month assessments (0.25; 0.35; 0.12; 0.09 and 0.47; 0.38; 0.21, respectively), low to moderate in the nine-month assessment (0.62; 0.49), and moderate in the 12-month assessment (0.74). Conclusion: Climbing up stairs should be assessed at intervals of nine months or longer, when responsiveness is moderate to high. Going down stairs should be assessed annually because moderate responsiveness was observed in this period.

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.

Effects of spinal cord stimulation on postural control in Parkinson's disease patients with freezing of gait

Freezing of gait (FoG) in Parkinson’s disease (PD) is an incapacitating transient phenomenon, followed by continuous postural disorders. Spinal cord stimulation (SCS) is a promising intervention for FoG in patients with PD, however, its effects on distinct domains of postural control is not well known. The aim of this study is to assess the effects of SCS on FoG and distinct domains of postural control. Four patients with FoG were implanted with SCS systems in the upper thoracic spine. Anticipatory postural adjustment (APA), reactive postural responses, gait and FoG were biomechanically assessed. In general, the results showed that SCS improved FoG and APA. However, SCS failed to improve reactive postural responses. SCS seems to influence cortical motor circuits, involving the supplementary motor area. On the other hand, reactive posture control to external perturbation that mainly relies on neuronal circuitries involving the brainstem and spinal cord, is less influenced by SCS.

Efeito do treino de isostretching na flexibilidade e na força muscular

Objetivo: Avaliar efeitos do treino de exercicios de isostreching na flexibilidade e forca muscular. Metodo: Trinta e um individuos saudaveis (27 mulheres), de 18 a 28 anos, divididos em 2 grupos: Grupo A, isostretching, submeteu-se a programa de exercicios baseados na tecnica isostretching e Grupo B, padrao, submeteu-se aos mesmos exercicios utilizando principios tecnicos classicos do alongamento, por 12 semanas, duas vezes por semana, uma hora por sessao. Foram avaliadas no pre e pos-teste, flexibilidade por meio de fotogrametria pesquisando a distância punho-chao e a classificacao da postura segundo categorias de encurtamentos musculares descritas por Kendall e, forca muscular por meio de dinamometria. Resultados: Nao houve diferenca estatisticamente significante no teste de flexibilidade nos dois grupos. Analise de significância clinica e melhora pelo Indice de Mudanca Confiavel (IMC) mostrou ganho na flexibilidade atingindo 14 participantes de ambos os grupos. Analise de contorno do corpo do grupo A apresentou atenuacoes nas curvaturas da coluna cervical, lombar e toracica e ângulo de flexao de quadril. O grupo B apresentou atenuacoes na curvatura da coluna cervical e ângulo de flexao de quadril. Em relacao a forca muscular, o grupo A apresentou diferenca estatisticamente significante em alguns grupos musculares especificos, porem sem significância clinica. Conclusao: As duas intervencoes afetam a flexibilidade de forma estatisticamente semelhante, porem com impacto diferente nas curvaturas da coluna. O isostretching afetou clinicamente a flexibilidade de individuos saudaveis, com indicios de que treinamentos mais intensos ou longos possam afetar a forca muscular