Balance and muscle power of children with Charcot-Marie-Tooth

ABSTRACT | Background: In certain diseases, functional constraints establish a greater relationship with muscle power than muscle strength. However, in hereditary peripheral polyneuropathies, no such relationship was found in the literature. Objective: In children with Charcot-Marie-Tooth (CMT), to identify the impact of muscle strength and range of movement on the static/dynamic balance and standing long jump based on quantitative and functional variables. Method: The study analyzed 19 participants aged between 6 and 16 years, of both genders and with clinical diagnoses of CMT of different subtypes. Anthropometric data, muscle strength of the lower limbs (hand-held dynamometer), ankle and knee range of movement, balance (Pediatric Balance Scale) and standing long jump distance were obtained by standardized procedures. For the statistical analysis, Pearson and Spearman correlation coefficients were used. Results: There was a strong positive correlation between balance and the muscle strength of the right plantar flexors (r=0.61) and dorsiflexors (r=0.59) and a moderate correlation between balance and the muscle strength of inversion (r=0.41) and eversion of the right foot (r=0.44). For the long jump and range of movement, there was a weak positive correlation with right and left plantar flexion (r=0.20 and r=0.12, respectively) and left popliteal angle (r=0.25), and a poor negative correlation with left dorsiflexion (r=-0.15). Conclusions: The data on the patients analyzed suggests that the maintenance of distal muscle strength favors performance during balance tasks, while limitations in the range of movement of the legs seem not to be enough to influence the performance of the horizontal long jump. Keywords: Charcot-Marie-Tooth disease; strength; balance; range of movement; assessment; physical therapy. HOW TO CITE THIS ARTICLE Silva TR, Testa A, Baptista CRJA, Marques Jr W, Mattiello-Sverzut AC. Balance and muscle power of children with CharcotMarie-Tooth. Braz J Phys Ther. http://d

Efeitos morfológicos do retorno da sobrecarga após imobilização em alongamento de músculo esquelético de ratas Morphological effects of resumption of loading after immobilization of skeletal muscles in lengthened position in female rats

CONTEXTUALIZAÇÃO: Na reabilitação, a imobilização em alongamento do músculo esquelético é realizada como contramedida para reverter efeitos de encurtamento muscular severo e em eventos pós-cirúrgicos. Acredita-se que o retorno às atividades funcionais normais estimule mecanotransdutores capazes de reorganizar a citoarquitetura normal muscular, porém a descrição das alterações histopatológicas relacionadas a esses procedimentos são escassas na literatura. OBJETIVOS: Avaliar e quantificar anomalias histológicas induzidas pela imobilização em alongamento do músculo EDL (Extensor Digitorum Longus) e confrontá-las com a livre movimentação do animal após esse procedimento. MÉTODOS: Foram utilizadas 18 ratas Wistar, distribuídas nos grupos: controle (GC); imobilizadas em flexão plantar (EDL em posição alongada) por 14 dias (GI); imobilizadas por 14 dias e liberadas por dez dias (GIL). Fragmentos do EDL foram congelados, seccionados e processados com reações imuno-histoquímica para colágenos I e III e histoquímica para Adenosina Trifosfatase Miofibrilar e Hematoxilina-Eosina. RESULTADOS: Os animais do GI apresentaram discreto aumento da expressão de colágeno I e de fibras em processo degenerativo/necrótico, redução da proporção de fibras tipo (FT) 2A e do diâmetro menor de todos os tipos de fibras, quando comparados com os animais do GC. Para o GIL, observou-se retorno da quantidade de colágeno I às condições controle, além de redução na proporção de FT2D, aumento do número de núcleos centralizados e do diâmetro menor das fibras quando comparadas com o GI, porém a expressão de FT2B e FT2D não atingiu os valores de referência. CONCLUSÕES: Os dados apresentados mostram que a retomada da função durante dez dias foi parcialmente eficiente na recuperação das características do músculo EDL após o período de imobilização e que, se extrapolados os dados à clínica fisioterapêutica, a adoção de procedimentos orientados às disfunções primárias do músculo pode favorecer a resposta morfofuncional do segmento e o seu íntegro restabelecimento.<br>BACKGROUND: In rehabilitation, immobilization of skeletal muscles in the elongated position is performed as a countermeasure in order to reverse the effects of severe muscle shortening and postoperative events. The return to normal functional activities is believed to stimulate mechanotransducers capable of reorganizing the normal muscle cytoarchitecture, but few data describing the histopathological changes relating to these procedures are available in the literature. OBJECTIVES: To assess and quantify histological abnormalities induced by immobilization of the extensor digitorum longus (EDL) muscle in elongation and to compare them with free movement of the animal after this procedure. METHODS: Eighteen female Wistar rats were used, divided into the following groups: Control; Immobilized in plantar flexion (EDL in an elongated position) for 14 days (GI); Immobilized for 14 days and released for 10 days (GIL). EDL fragments were frozen, sectioned and processed through immunohistochemical reactions for collagens I and III and histochemical methods for myofibrillar adenosine triphosphatase using hematoxylin-eosin. RESULTS: GI animals presented slight increases in collagen I and fiber expression in a degenerative/necrotic process, and reductions in the proportion of FT2A fibers and in the diameters of all fiber types, compared with the controls. In GIL, the quantity of collagen I returned to control conditions; the proportion of FT2D decreased; the number of centralized nuclei increased; and the fiber diameter was smaller than in GI. However, FT2B and FT2D expression did not reach the reference values. CONCLUSIONS: The data presented show that the recovery of function over a 10-day period was partially efficient with regard to recuperation of the characteristics of the EDL muscle after the period of immobilization. If the data are extrapolated to physiotherapeutic clinical practice, use of procedures directed towards primary dysfunctions of the muscle may favor a morphofunctional response in the segment and its full recovery

Balance and muscle power of children with Charcot-Marie-Tooth

BACKGROUND: In certain diseases, functional constraints establish a greater relationship with muscle power than muscle strength. However, in hereditary peripheral polyneuropathies, no such relationship was found in the literature. OBJECTIVE: In children with Charcot-Marie-Tooth (CMT), to identify the impact of muscle strength and range of movement on the static/dynamic balance and standing long jump based on quantitative and functional variables. METHOD: The study analyzed 19 participants aged between 6 and 16 years, of both genders and with clinical diagnoses of CMT of different subtypes. Anthropometric data, muscle strength of the lower limbs (hand-held dynamometer), ankle and knee range of movement, balance (Pediatric Balance Scale) and standing long jump distance were obtained by standardized procedures. For the statistical analysis, Pearson and Spearman correlation coefficients were used. RESULTS: There was a strong positive correlation between balance and the muscle strength of the right plantar flexors (r=0.61) and dorsiflexors (r=0.59) and a moderate correlation between balance and the muscle strength of inversion (r=0.41) and eversion of the right foot (r=0.44). For the long jump and range of movement, there was a weak positive correlation with right and left plantar flexion (r=0.20 and r=0.12, respectively) and left popliteal angle (r=0.25), and a poor negative correlation with left dorsiflexion (r=-0.15). CONCLUSIONS: The data on the patients analyzed suggests that the maintenance of distal muscle strength favors performance during balance tasks, while limitations in the range of movement of the legs seem not to be enough to influence the performance of the horizontal long jump

Postural alignment in children with Duchenne muscular dystrophy and its relationship with balance

Background: In Duchenne muscular dystrophy, functional deficits seem to arise from body misalignment, deconditioning, and obesity secondary to weakness and immobility. The question remains about the effects of postural deviations on the functional balance of these children. Objectives: To identify and quantify postural deviations in children with DMD in comparison to non-affected children (eutrophic and overweight/obese), exploring relationships between posture and function. Method: This case-control study evaluated 29 participants aged 6 to 11 years: 10 DMD (DG), 10 eutrophic (EG), and 9 overweight/obese (OG). Digital photogrammetry and SAPo program were used to measure postural alignment and the Pediatric Balance Scale (PBS) was used to measure balance. The Kruskall-Wallis and Dunn post-hoc tests were used for inter-group comparison of posture and balance. Spearman's coefficient tested the correlation between postural and balance variables. Results: The horizontal pelvic alignment data indicated that the anteversion of the DG was similar to that of the OG and twice that of the EG (p<0.05). Compared to the EG, the DG and OG showed an increased forward position of the center of mass (p<0.05). There was a moderate and weak correlation between the PBS score and horizontal pelvic alignment (0.58 and 0.47-left/right). The PBS showed a weak correlation with asymmetries in the sagittal plane (-0.39). The PBS scores for the OG and EG suggest that obesity did not have a deleterious effect on balance. Conclusions: The balance deficit in children with DMD was accompanied by an increased forward position of the center of mass and significant pelvic anteversion that constitutes a compensatory strategy to guarantee similar performance to the children not affected by the disease

Longitudinal assessment of grip strength using bulb dynamometer in Duchenne Muscular Dystrophy

BACKGROUND: Grip strength is used to infer functional status in several pathological conditions, and the hand dynamometer has been used to estimate performance in other areas. However, this relationship is controversial in neuromuscular diseases and studies with the bulb dynamometer comparing healthy children and children with Duchenne Muscular Dystrophy (DMD) are limited. OBJECTIVE: The evolution of grip strength and the magnitude of weakness were examined in boys with DMD compared to healthy boys. The functional data of the DMD boys were correlated with grip strength. METHOD: Grip strength was recorded in 18 ambulant boys with DMD (Duchenne Group, DG) aged 4 to 13 years (mean 7.4&#177;2.1) and 150 healthy volunteers (Control Group, CG) age-matched using a bulb dynamometer (North Coast- NC70154). The follow-up of the DG was 6 to 33 months (3-12 sessions), and functional performance was verified using the Vignos scale. RESULTS: There was no difference between grip strength obtained by the dominant and non-dominant side for both groups. Grip strength increased in the CG with chronological age while the DG remained stable or decreased. The comparison between groups showed significant difference in grip strength, with CG values higher than DG values (confidence interval of 95%). In summary, there was an increment in the differences between the groups with increasing age. Participants with 24 months or more of follow-up showed a progression of weakness as well as maintained Vignos scores. CONCLUSIONS: The amplitude of weakness increased with age in the DG. The bulb dynamometer detected the progression of muscular weakness. Functional performance remained virtually unchanged in spite of the increase in weakness

Propriedades mecânicas do gastrocnêmio eletroestimulado pós-imobilização Mechanical properties of gastrocnemius eletrostimulated after immobilization

INTRODUÇÃO: As propriedades mecânicas (PM) consistem num instrumento de aplicabilidade clínica para profissionais de saúde que atuam no sistema músculo-esquelético. OBJETIVOS: Avaliar dois protocolos de estimulação elétrica neuromuscular (NMES) na potencialização do restabelecimento das PM no complexo músculo-tendíneo após imobilização segmentar de ratas. MATERIAIS E MÉTODOS: Foram utilizados 50 animais distribuídos em: Controle (GC, n=10); Imobilizado (GI, n=10); Imobilizado e remobilizado livre (GIL, n=10), Imobilizado e NMES uma vez ao dia (GIE1, n=10) e Imobilizado e NMES duas vezes ao dia (GIE2, n=10). A imobilização foi realizada por 14 dias. O GIL foi liberado posteriormente por 10 dias. A NMES foi aplicada pós-imobilização por 10 dias, GIE1 aplicado pela manhã (10 minutos) e, GIE2 aplicado pela manhã e à tarde (totalizando 20 minutos). Posteriormente, o músculo gastrocnêmio foi submetido ao ensaio mecânico de tração sendo as PM de rigidez, resiliência, carga e o alongamento no limite máximo avaliadas. RESULTADOS: A imobilização reduziu os valores das propriedades de carga e rigidez (p<0,05). A NMES utilizada duas vezes ao dia determinou resultados menos satisfatórios das PM avaliadas que àqueles obtidos uma vez ao dia e no grupo remobilizado (p>0,05). CONCLUSÃO: O músculo gastrocnênio tornou-se estruturalmente mais organizado frente à aplicação unitária da NMES e na remobilização.<br>INTRODUCTION: Mechanical properties (MP) are clinically applicable tools for healthcare professionals working on the musculoskeletal system. OBJECTIVES: The aim of this study was to evaluate two protocols of neuromuscular electric stimulation (NMES) to improve MP regeneration of the myotendinous complex after segment immobilization in female rats. MATERIALS AND METHODS: Fifty animals were equally distributed into five groups: Control (CG, n=10); Immobilized (IG, n=10); Immobilized and freely remobilized (IFG, n=10); Immobilized and NMES once /day (IEG1, n=10); Immobilized and MNES twice/day (IEG2, n=10). Immobilization was kept for 14 days, and remobilization was subsequently released for 10 days. NMES was applied for 10 days, post-immobilization, every morning for 10 minutes to IEG1 animals and every morning and afternoon (total 20 minutes) to the IEG2 group. After these procedures, the gastrocnemius muscle was submitted to the mechanical traction assay to evaluate stiffness, resilience, load and stretching at maximum limit MPs. RESULTS: Immobilization reduced the MP values concerning load and stiffness (p<0.05). Results for NMES applied twice a day were less satisfactory than the ones obtained with one application or in the remobilized group (p> 0.05). CONCLUSION: It is concluded that the gastrocnemius muscle became structurally better organized through a single NMES application and by remobilization