Initial results from the PHEFREE longitudinal natural history study: Cross-sectional observations in a cohort of individuals with phenylalanine hydroxylase (PAH) deficiency.

Over fifty years have passed since the last large scale longitudinal study of individuals with PAH deficiency in the U.S. Since then, there have been significant changes in terms of treatment recommendations as well as treatment options. The Phenylalanine Families and Researchers Exploring Evidence (PHEFREE) Consortium was recently established to collect a more up-to-date and extensive longitudinal natural history in individuals with phenylketonuria across the lifespan. In the present paper, we describe the structure and methods of the PHEFREE longitudinal study protocol and report cross-sectional data from an initial sample of 73 individuals (5 months to 54 years of age) with PAH deficiency who have enrolled. Looking forward, the study holds the promise for advancing the field on several fronts including the validation of novel neurocognitive tools for assessment in individuals with PKU as well as evaluation of the long-term effects of changes in metabolic control (e.g., effects of Phe-lowering therapies) on outcome.

The individual difference of motor imagery ability evoked by visual stimulus and its personality manifestation.

Motor imagery has been commonly studied as a means of motor rehabilitation but, the individual differences limit its practical application. Visually evoked motor imagery has been widely highlighted by researchers because of its vivid stimulus. However, this modality is still not applicable to all persons. In this study, we studied the different performances of the visually evoked motor imagery between subjects and tried to explore the personality manifestation which can result in this performance. We found that conscientiousness and openness have negative connections with the performance of visually evoked motor imagery. To compare with spontaneous motor imagery, the visually evoked motor imagery reflects less personality difference between subjects with good and bad performances on motor imagery. This indicate that visually stimulus may increase the pervasive application of motor imagery. This study may provide benefits to predict the rehabilitation effect and to rapidly select the suitable motor rehabilitation methods.

Effectiveness of digital home rehabilitation and supervision for stroke survivors: A systematic review and meta-analysis.

Objective: Stroke survivors often experience residual impairments and motor decline post-discharge. While digital home rehabilitation combined with supervision could be a promising approach for reducing human resources, increasing motor ability, and supporting rehabilitation persistence there is a lack of reviews synthesizing the effects. Thus, this systematic review and meta-analysis aimed to synthesize the effect of digital home rehabilitation and supervision in improving motor ability of upper limb, static balance, stroke-related quality of life, and self-reported arm function among stroke survivors. Methods: Six electronic databases, grey literature, ongoing studies, and reference lists were searched for relevant studies. Two investigators independently reviewed titles, abstracts, screened full texts for eligibility and performed data extraction. Meta-analysis of 13 independent studies were grouped into four separate meta-analyses. The Grading of Recommendations, Assessments, Development and Evaluations (GRADE) tool was used for evaluating the overall quality of the evidence. Results: Meta-analyses showed no statistically significant difference between intervention (digital home rehabilitation) and control groups (home training/clinic-based) of all outcomes including motor ability of upper limb, static balance, stroke-related quality of life, and self-reported arm function. In the sub-group analysis digital home rehabilitation was associated with better quality of arm use (standardized mean difference = 0.68, 95% confidence interval: [0.27, 1.09], p = 0.001). Conclusions: This result indicated that digital home rehabilitation has similar effects and could potentially replace home training or clinic-based services. This review highlights better-targeted digital motor interventions to examine the effects of interventions further. The quality of evidence was moderate to high in motor and self-reported arm outcomes, and low for balance and quality of life.

Relationship between Subjective Grip Strength and Physical Functioning among Community-Dwelling Older Women.

This study investigated the relationship between subjective grip strength and physical function in community-dwelling older women. Subjective grip strength was assessed using a questionnaire, and physical function and body composition were compared between groups with strong and weak subjective grip strength. Additionally, the two groups were compared in those with mild cognitive impairment (MCI) and those with normal cognitive function, respectively. The results showed significant differences in grip strength (p < 0.001), 30 s chair-stand (CS-30) test (p = 0.039), timed up-and-go (TUG) test (p = 0.027), maximal gait speed (p = 0.029), and skeletal muscle mass (p < 0.001). Older adults with normal cognitive function showed significant differences in grip strength (p < 0.001), quadriceps muscle strength (p < 0.009), one-leg standing time (p = 0.041), CS-30 (p = 0.002), TUG (p = 0.014), gait speed (p = 0.006), and skeletal muscle mass (p = 0.003). Older adults with low subjective grip strength had lower physical function and skeletal muscle mass. However, no items showed significant differences between groups among older adults with MCI. Thus, subjective grip strength is an indicator of an overall decline in physical function and a reduction in skeletal muscle mass in older adults, and cognitive function should be considered when assessing subjective grip strength in older adults.

Exploring the presence and impact of sensory differences in children with Developmental Coordination Disorder.

BACKGROUND: Children with Developmental Coordination Disorder (DCD) can experience sensory differences. There has been limited exploration of these differences and their impact on children with DCD. AIMS: i) To explore the presence and impact of sensory differences in children with DCD compared to children without DCD; ii) To examine whether sensory differences are related to motor ability, attention deficit hyperactivity disorder (ADHD), or autistic traits. METHOD: Parents of children (8-12 years) with (n = 23) and without (n = 33) DCD used standardised questionnaires to report on their children's sensory differences, autistic traits, and ADHD traits. Motor abilities were assessed through the Movement Assessment Battery for Children-2. Data were explored both categorically (between-groups) and dimensionally. RESULTS: Children with DCD had significantly higher levels of sensory differences than children without DCD. Sensory differences also had a significantly greater impact on daily activities for children with DCD. Higher levels of ADHD and autistic traits, but not motor ability, were significant independent predictors of higher levels of sensory difference. CONCLUSION: Children with DCD experience high levels of sensory differences, which impact on their daily lives. These sensory differences may be a marker for additional neurodivergence in children with DCD. Practitioners should consider the sensory needs of children with DCD. WHAT THIS PAPER ADDS: This paper provides insight into the sensory features of children with DCD and the impact that sensory differences can have on daily living. Using parent-report, we found that children with DCD had increased sensory differences relative to children without DCD. These included increased hyperresponsiveness, increased hyporesponsiveness, and increased sensory interests, repetitions, and seeking behaviours (SIRS). We also found that sensory differences had a greater impact on daily living for children with DCD compared to children without DCD. Across the whole sample, autistic traits predicted hyperresponsivity and hyporesponsivity patterns; whereas traits of hyperactivity and impulsivity predicted SIRS. Motor abilities did not uniquely predict sensory differences, suggesting that other traits of neurodivergence may contribute to the sensory differences in DCD. Taken together, these findings highlight the necessity of considering sensory needs when supporting children with DCD. They also suggest that if sensory differences are identified in children with DCD, it may be due to the presence of co-occurring neurodivergent traits or conditions.

Role of forkhead box protein 2 (FOXP2) in oral-motor abilities of preterm infants: A brief literature review.

Preterm infants, born before the 37-week gestation period, have limited storage for nutrients at birth and are vulnerable to poor feeding, severe nutritional deficits and growth retardation. The immature gastrointestinal system leads preterm infants to experience a delay in initiating enteral nutrition. Inappropriate feeding can cause acute and long-term morbidity, prolonged hospitalization and increased treatment cost. Generally, preterm infants that are born after 32 weeks of gestation without severe comorbidities do not have dysphagia and should start oral feeding soon after birth. Preterm infants should have well-developed sucking-swallowing-breathing coordination by 32-34 weeks of gestational age. However, some infants take days or weeks to master the skill. The oral feeding development involves forkhead box protein 2 (FOXP2)-expressing neurons that are found in the deep layers of the cortex, basal ganglia, parts of the thalamus and Purkinje cells of the cerebellum. In mammals, these areas belong to the brain network circuits working for motor coordination in learning and acquiring sensorimotor skills. This review aimed to describe the role of FOXP2 in oral-motor skills in preterm infants, including oral feeding, sucking-swallowing-breathing coordination and language development. The oral-motor skills development could be an early predictor for language delay in premature infants, representing a vulnerable group susceptible to such delays.

Efectos del programa corporeidad para la adquisición de competencias motrices básicas en escolares de educación primaria

El estudio tuvo por objetivo determinar el efecto de la aplicación del Programa Corporeidad para la adquisición de competencias motrices básicas en escolares de educación primaria, Huanta-Ayacucho, 2023. La investigación fue aplicada, abordó desde el enfoque cuantitativo y diseño cuasi experimental. Participaron 107 estudiantes del 6° grado de primaria en dos grupos; solo al grupo experimental se aplicó el Programa de Corporeidad mediante 15 sesiones en las dimensiones percepción postural y percepción y regulación de móviles. Se aplicó la Batería MOBAK 5-6 midiendo dos competencias motrices básicas: control de objetos y control del cuerpo. Los resultados establecen que ambos grupos (control y experimental) en el pretest no reflejan diferencias significativas en el logro de las competencias motrices básicas (0.170 ≥ 0.05). Asimismo, se evidenció que ambos grupos (control y experimental) en el postest si reflejan diferencias significativas (0.000 < 0.05), encontrándose que el rango promedio del grupo control es 27.51 y del grupo experimental notoriamente más alto con 73.98, dando un valor para la prueba U de Mann-Whitney de 184,500; concluyendo que la aplicación del Programa Corporeidad si tuvo efecto significativo en la adquisición de las competencias motrices básicas en los escolares de educación primaria.

The objective of the study was to determine the effect of the application of the Corporeidad Program for the acquisition of basic motor skills in primary school students, Huanta-Ayacucho, 2023. The research was applied, approached from the quantitative approach and quasi-design experimental. 107 students from the 6th grade of primary school participated in two groups; The Corporeity Program was applied only to the experimental group through 15 sessions in the postural perception and mobile perception and regulation dimensions. The MOBAK Battery 5-6 was applied, measuring two basic motor skills: control of objects and control of the body. The results establish that both groups (control and experimental) in the pretest do not reflect significant differences in the achievement of basic motor skills (0.170 ≥ 0.05). Likewise, it was evidenced that both groups (control and experimental) in the post-test do reflect significant differences (0.000 < 0.05), finding that the average range of the control group is 27.51 and of the experimental group notoriously higher with 73.98, giving a value for the Mann-Whitney U test of 184,500; concluding that the application of the Corporeity Program did have a significant effect on the acquisition of basic motor skills in primary school students.

O objetivo do estudo foi determinar o efeito da aplicação do Programa Corporeidad para a aquisição de habilidades motoras básicas em alunos do ensino fundamental, Huanta-Ayacucho, 2023. A pesquisa foi aplicada, abordada a partir da abordagem quantitativa e quase-desenho experimental. Participaram 107 alunos do 6º ano do ensino fundamental em duas turmas; O Programa de Corporeidade foi aplicado apenas ao grupo experimental através de 15 sessões nas dimensões percepção postural e percepção móvel e regulação. Foi aplicada a Bateria MOBAK 5-6, medindo duas habilidades motoras básicas: controle de objetos e controle do corpo. Os resultados estabelecem que ambos os grupos (controle e experimental) no pré-teste não refletem diferenças significativas na obtenção de habilidades motoras básicas (0,170 ≥ 0,05). Da mesma forma, evidenciouse que ambos os grupos (controle e experimental) no pós-teste refletem diferenças significativas (0,000 < 0,05), verificandose que o intervalo médio do grupo controle é de 27,51 e do grupo experimental notoriamente superior com 73,98, dando um valor para o teste Mann-Whitney U de 184.500; concluindo que a aplicação do Programa Corporeidade teve um efeito significativo na aquisição de habilidades motoras básicas em alunos do ensino fundamental.

Perceptual-motor ability assessment in people with atypical development / Avaliação da maturidade perceptomotora em pessoas com desenvolvimento atípico / Evaluación de la madurez percepto-motriz en personas con desarrollo atípico

The assessment of perceptual-motor maturity has been investigated, and evidence indicates its relevance in the prediction of academic performance, which justifies the relevance of its investigation. This study investigated the psychometric qualities of a screening version of the Bender-Gradual Scoring System (B-SPG in Portuguese) in 273 individuals aged between six and 24 (M = 13.27, SD = 3.63), 166 (60, 8%) males, who were diagnosed with intellectual disabilities. The results of the exploratory factor analysis and regression analysis were satisfactory and demonstrated that it is possible to use only three figures to carry out the evaluation, which facilitates the psychologist's work. The accuracy of the instrument was also estimated, and the results showed adequate indexes. It is concluded that the screening version of the B-SPG proves to be an adequate measure for children with atypical development. However, other studies should be performed.

A avaliação da maturidade perceptomotora tem sido investigada, e evidências indicam sua relevância na predição do desempenho acadêmico, o que justifica a pertinência de sua investigação. O presente estudo investigou as qualidades psicométricas de uma versão de rastreio do Bender-Sistema de Pontuação Gradual (B-SPG) em 273 indivíduos com idades variando de 6 a 24 anos (M = 13,27, DP = 3,63), sendo 166 (60,8%) do sexo masculino, que tinham o diagnóstico de deficiência intelectual. Os resultados da análise fatorial exploratória e da análise de regressão foram satisfatórios e revelaram que é possível utilizar apenas três figuras para realizar a avaliação, o que facilita o trabalho do psicólogo. A precisão também foi estimada, e os resultados mostraram índices adequados. Conclui-se que a versão de rastreio do B-SPG se mostra uma medida adequada para crianças com desenvolvimento atípico, no entanto outros estudos devem ser realizados.

La evaluación del desarrollo percepto-motriz ha sido investigada y existen evidencias de su relevancia para predecir el desempeño académico, lo cual justifica la realización del trabajo. Este estudio investigó las cualidades psicométricas de una versión de sondeo del Bender-Sistema de Pontuação Gradual (B-SPG) en 273 individuos con edades entre 6 y 24 años (M = 13,27, DT = 3,63), siendo 166 (60,8%) del sexo masculino, que poseían diagnóstico de deficiencia intelectual. Los resultados del análisis factorial exploratorio y del análisis de regresión fueron satisfactorios, revelando que es posible utilizar apenas tres figuras para realizar la evaluación, lo cual facilitaría el trabajo del psicólogo. La confiabilidad también fue verificada y los resultados mostraron índices adecuados. Se concluye que la versión de sondeo del B-SPG parece ser una medida adecuada para niños con deficiencia intelectual, pero pese a eso, otros estudios deben ser realizados.

Motor development analysis of three-year-old children born preterm through the Motor Development Scale - Case Report

INTRODUCTION: Prematurity and low birth weight are conditions that may compromise the normal development of a child at different stages of development. Considering that these conditions may cause delay in the acquisition of motor skills, it is important to evaluate, detect and prevent possible changes in motor development. OBJECTIVE: To investigate the influence of prematurity and low birth weight on the motor development of children of three years old relating chronological age to general motor age. METHODS: This is an analysis of five cases of preterm children 32.1 (± 1.82) weeks and underweight 1704 (± 384.41) grams, mean chronological age of 43.2 (± 2.59) months, evaluated through the Motor Development Scale (MDS) in the Laboratory of Electromyography and Kinematics (LAELCIN) of the Federal University of Triângulo Mineiro (UFTM), considering general motor age (GMA) and chronological age (CA) as variables CASE: The five preterm and low birth weight children presented a difference between the mean general motor age (37.6 ± 7.40) months and the mean chronological age (43.2 ± 2.59) months, indicating delayed motor development. CONCLUSION: It was observed the absence of linearity of the variables, indicating delay in the motor development, thus justifying the necessity and importance of the longitudinal monitoring of this population for early detection and intervention.

INTRODUÇÃO: A prematuridade e o baixo peso ao nascimento são condições que podem comprometer o desenvolvimento normal da criança nas diferentes etapas evolutivas. Considerando que estas condições podem acarretar atraso na aquisição de habilidades motoras, é importante avaliar, detectar e prevenir as possíveis alterações no desenvolvimento motor. OBJETIVO: Analisar a influência da prematuridade e do baixo peso ao nascimento no desenvolvimento motor de crianças na faixa etária de três anos de idade, relacionando a idade cronológica com a idade motora geral. MÉTODO: Trata-se de uma análise com cinco casos de crianças nascidas pré-termo 32,1 (±1,82) semanas e com baixo peso 1704 (± 384,41) gramas, idade cronológica média de 43,2 (±2,59) meses, avaliadas por meio da Escala de Desenvolvimento Motor (EDM) no Laboratório de Eletromiografia e Cinemática (LAELCIN) da Universidade Federal do Triângulo Mineiro (UFTM), considerando as variáveis idade motora geral (IMG) e idade cronológica (IC). RELATO: As cinco crianças nascidas pré-termo e com baixo peso apresentaram diferença entre a idade motora geral média (37,6, ±7,40) meses e a idade cronológica média (43,2, ±2,59) meses, indicando atraso no desenvolvimento motor. CONCLUSÃO: Observou-se ausência de linearidade das variáveis, indicando atraso no desenvolvimento motor, justificando assim, a necessidade e importância do acompanhamento longitudinal dessa população para detecção e intervenção precoce.

Teste de Bender: versão de rastreio para a avaliação da maturidade perceptomotora / Bender Test: screening version for evaluation of perceptive-motor maturity / Test de Bender: versión de rastreo para la evaluación de la madurez perceptomotora

Este estudo buscou evidências de validade de conteúdo e critério para a versão de rastreio do Bender - Sistema de Pontuação Gradual. Para isso, 693 crianças, ambos os sexos, com idades entre seis e 10 anos (M = 8,42; DP = 1,38) reproduziram a cópia de três figuras. Os resultados indicaram que a Figura 3 apresentou alto nível de dificuldade, sendo necessário maior nível de maturidade perceptomotora para sua execução. As Figuras 7a e 7b, embora apresentem sobreposição, foram mais fáceis de serem copiadas. Verificou-se que os erros de distorções na reprodução das figuras tendem a diminuir gradualmente conforme aumenta a idade das crianças. Observou-se que as três figuras não apresentam funcionamento diferencial dos itens entre meninas e meninos, além disso também não foram encontradas diferenças de média de desempenho. Esses achados sugerem que a versão de rastreio é uma proposta promissora para ser utilizada na avaliação da maturidade perceptomotora. (AU)

This study sought evidence of content and criteria validity for the screening version of the Bender - Gradual Scoring System. For this, 693 children of both sexes, between 6 and 10 years of age (M = 8.42; SD = 1.38), reproduced three figures. The results indicated that Figure 3 had a greater level of difficulty, with a greater level of perceptive-motor maturity required for its execution. Figures 7a and 7b, although overlapping, were easier to reproduce. It was found that the errors of distortions in the reproduction of figures tended to gradually decrease as the child's age increased. Another point observed was that the three figures did not present differential functioning for the items between girls and boys, nor were any mean performance differences found. These findings suggest that the screening version is a promising proposition for use in the evaluation of perceptive-motor maturity. (AU)

El estudio buscó evidencias de validez de contenido y criterio para la versión de rastreo del Bender - Sistema de Puntuación Gradual. Por esto, participaron 693 niños de ambos sexos con edades entre 6 y 10 años (M= 8,42; DP = 1,38), que reprodujeron la copia de tres figuras. Los resultados indicaron que la Figura 3 presentó alto nivel de dificultad, siendo necesario un mayor nivel de madurez perceptiva para su ejecución. Las Figuras 7a y 7b, aunque presentaban superposición, fueron más fáciles de dibujar. Se verificó que los errores de distorsión en la reproducción de las figuras tienden a disminuir gradualmente según aumenta la edad de los niños. Otro punto observado fue que las tres figuras no presentan funcionamiento diferencial entre las niñas y niños, además tampoco se encontraron diferencias de promedio de rendimiento. Estos hallazgos sugieren que la versión de rastreo es una propuesta oportuna para ser usada en la evaluación de la madurez perceptiva. (AU)

Evolução funcional da distrofia muscular do tipo Cinturas em indivíduos de uma mesma família / Functional evolution of Girdle Muscular Dystrophy in individuals of the same family

A Distrofia Muscular de Cinturas (DMC) possui herança autossômica dominante ou recessiva e caracteriza-se por paresia progressiva que induz à deterioração funcional e dificuldades no desempenho de atividades cotidianas. O objetivo do presente estudo foi analisar a evolução funcional de indivíduos com diagnóstico de DMC de uma mesma família. Nove indivíduos foram avaliados por um questionário para identificação do parentesco, idade de inicio dos primeiros sintomas e queixas principais, e pelas Escalas de Vignos (EV) e Hammersmith (EMFH). O projeto foi aprovado pelo CEP/UFVJM, protocolo nº061/12. A idade média foi de 33 ± 8.1 anos, com seis indivíduos sendo do sexo masculino, a idade de aparecimento dos primeiros sintomas foi aos 9± 2,83 anos e os sintomas iniciais mais frequentes foram dificuldade de correr, quedas e marcha equina. Os sujeitos pontuaram 4, 6 e 7 na EV e no exame físico verificou-se acometimento principalmente dos membros inferiores. Foi encontrado correlação negativa entre idade dos sujeitos e escore na EMFH (r2=-0,839) e entre pontuação na EV e EMFH (r2 =-0,819), e correlação positiva entre EV e uso de dispositivos (r2=0,866). Nossos achados sugerem que a mesma patologia diagnosticada em indivíduos de uma mesma família apresenta repercussões funcionais diferentes. O aspecto ambiente deve ser levado em consideração ao avaliar a funcionalidade desses indivíduos uma vez que, independente do diagnóstico em comum, da idade, e limitações físicas, os indivíduos apresentaram adaptações particulares com objetivo de manutenção do seu deslocamento de acordo com as características do ambiente em que vive.(AU)

Limb Girdle Muscular Dystrophy (LGMD) is an autosomal-dominant or recessive hereditary disease. Progressive muscular weakness leads to functional damage and difficulty to perform activities of daily life. The present study aimed to analyze the functional evolution of LGMD individuals of the same family. Nine individuals were assessed using a questionnaire (to identify their relationship, age of onset of the first signs of the disease and main complaints) and by Vignos and Hammersmith Scales. Projetct was aproved by Ethics Committee of UFVJM, protocol nº061/12. Mean age was 33±8.1 years old, six male, mean age of onset of the first signs was 9± 2,83 years old and the most frequent signs were difficulty to run, falls and gait with ankle on plantar flexion. Subjects scored 4, 6 and 7 on Vignos Scale and on physical exam, lower limbs were the most affected. Negative correlation between age and Hammermith scale (r 2=-0,839), Vignos and Hammersmith Scale (r2=-0,819) were found. Positive correlation between Vignos Scale and use of assistive devices (r2=0,866). Our finds suggest that the same disease in individuals of the same family leads to different functional impairment. The environmental aspect should be considered when assessing functionality of individuals with LGMD once although they present the same diagnosis, they present particular adaptations with the aim of maintain displacement according to characteristics of the environment where they live.(AU)

Habilidad viso-motriz y deficiencia intelectual: estudio de validez para el Bender-SPG / Visual motor ability and intellectual disabilities: validity study of the Bender-SPG / Habilidade viso-motriz e deficiência intelectual: estudo de validade para o Bender-SPG

Este trabajo tuvo por objetivo establecer la evidencia de validez convergente por medio del análisis de la asociación entre el la Prueba Gestáltica Viso-Motriz de Bender-Sistema de Puntuación Gradual (B-SPG), el Dibujo de la Figura Humana-Escala Sisto (DFH) y las Matrices Progresivas Coloridas de Raven (CPM). Participaron en el estudio 397 personas, entre los seis y 24 años de edad, con deficiencia intelectual. Los datos fueron obtenidos en la escuela de los niños y de forma individual. Los resultados indicaron una correlación de magnitud moderada entre los tests, confirmando la evidencia de validez para el B-SPG. Además, al comparar los resultados con los baremos de los tres tests se verificó que el desempeño de los sujetos de esta investigación estuvo muy debajo de lo esperado, una vez que el resultado fue semejante o inferior al de niños de seis años de los baremos que aparecen en los manuales de los tests.

This study aimed to establish evidence of convergent validity through the analysis of the association between the Bender Visual-Motor Gestalt Test -Gradual Scoring System (B-SPG, for its Spanish acronym), the Human Figure Drawing Test - Sisto's Scale (DFH for its Spanish acronym) and the Colorful Raven's Progressive Matrices (CPM).The participants in the study were 397 individuals, aged between 6 and 24 years old, who showed intellectual impairment. Data were gathered on an individual basis at the school these people attended. Results indicated a moderate magnitude correlation between the tests, thus confirming validity evidence for B-SPG. Moreover, when comparing the results with the scales of the three tests it was verified that the performance of the participants in this research was well below expectations, since the scores obtained were similar to or lower than those of children six years old of the scales contained in the testing manuals.

Este trabalho teve como objetivo estabelecer a evidência de validade convergente por meio da análise da associação entre o Teste Gestáltico Visomotor de Bender-Sistema de Pontuação Gradual (B-SPG), o Desenho da Figura Humana-Escala Sisto (DFH) e as Matrizes Progressivas Coloridas de Raven (CPM). Participaram no estudo 397 pessoas, entre seis e vinte e quatro anos de idade, com deficiência intelectual. Os dados foram obtidos na escola das crianças e de forma individual. Os resultados indicaram uma correlação de magnitude moderada entre os testes, confirmando a evidência de validade para o B-SPG. Além disso, ao comparar os resultados com parâmetros dos três testes verificou-se que o desempenho dos sujeitos desta pesquisa esteve muito abaixo do esperado, já que o resultado foi similar ou inferior ao de crianças de seis anos dos parâmetros que aparecem nos manuais dos testes.

Comparison of motor strength and function in patients with Duchenne muscular dystrophy with or without steroid therapy / Comparação da força e função motora em pacientes com distrofia muscular de Duchenne com ou sem corticoterapia

OBJECTIVE: To compare muscle strength (MS) and motor function in patients with Duchenne muscular dystrophy (DMD) receiving steroids for different times against the natural evolution of DMD described by Scott et al. METHOD: 90 patients with DMD (aged 5- 12 years), receiving steroids for one to seven years, were evaluated by Medical Research Council Scale (MRC) and Hammersmith motor ability score. The relation between MS and motor abilities measurement from our data and Scott's ones were ascertained statistically. RESULTS: The relation between patient's age and Hammersmith scores revealed decrease of 0.76 point per year for age against decrease of 2.23 points on Scott's study. The relation between MRC scale and patient's age showed decrease of 0.80 point per year of age against decrease of 3.65 points on Scott's study. CONCLUSION: In patients with DMD aged five to 12 years the progression of the disease is delayed by steroids and the motor function is less reduced than muscular strength.

OBJETIVO: Comparar força muscular e função motora de pacientes com distrofia muscular de Duchenne (DMD) em corticoterapia com a evolução natural da doença descrita por Scott et al. MÉTODO: Noventa pacientes, entre 5 e 12 anos de idade, em corticoterapia por um até sete anos, foram avaliados quanto à força muscular (FM) (escala MRC) e função motora (Hammersmith motor ability score). A relação entre idade, FM e função motora e a comparação com o estudo de Scott et al foram determinadas estatisticamente. RESULTADOS: a relação idade/escore Hammersmith diminuiu 0,76 pontos a cada ano de aumento da idade (2,23 pontos na história natural). A relação idade/MRC decresceu 0,80 pontos a cada ano de aumento da idade (3,65 pontos na história natural). CONCLUSÃO: Nos pacientes em corticoterapia, a progressão da doença é mais lenta que na evolução natural em todas as faixas etárias avaliadas, sendo a FM mais comprometida que a função motora.

A capacidade funcional de idosos fisicamente independentes praticantes de atividade física / Fitness of physically independent and active elderly subjects

O objetivo do presente estudo foi traçar o perfi l funcional de idosos acima de 60 anos de níveis funcionais IV e V em relação às capacidades físico-motoras e capacidade funcional. As variáveis analisadas foram: agilidade, fl exibilidade, equilíbrio, força de membros superiores e inferiores, habilidade em realizar tarefas de coordenação motora fi na, habilidade em calçar meias, levantar-se do solo, subir degraus, sentar, levantar e locomover-se com agilidade e equilíbrio. Os testes foram aplicados em 193 idosos de níveis funcionais IV e V com idade, entre 60 e 90 anos. Para comparação dos resultados foi usada a análise de variância de um fator. Os dados mostraram ausência de diferenças, com exceção para a capacidade motora agilidade e para a capacidade funcional levantar-se do solo. Os resultados obtidos permitem concluir que o perfi l funcional de idosos fi sicamente ativos mantém-se em condições ótimas até idades avançadas, acima da média dos idosos sedentários, quando realizam um programa de atividade física.

The object of this paper is to describe functional and motor abilities of physically independent subjects older than 60 years old. We applied tests for agility; fl exibility; upper and lower limbs strength; fi ne motor coordination; ability to wear socks, to rise from the fl oor; to step up stairs, and balance. The subjects were 193 physically independent elderly subjects, classifi ed at motor-physical and functional abilities levels IV and V. We run one-way ANOVA to compare the results. There were no effects of aging on most of variables, but agility and to rise from the fl oor. We conclude the fi tness of physically active elderly people remains stable across aging, higher than sedentary subjects.

Quantificação da força muscular e habilidades motoras de pacientes com Distrofia Muscular de Duchenne, em corticoterapia por período de 1 a 7 anos / Quantification of muscle strength and motor abilities in patients with Duchenne Muscular Dystrophy on steroid therapy for periods of 1 to 7 years

Os objetivos deste trabalho foram: comparar a força muscular (FM) e as habilidades motoras de pacientes com Distrofia Muscular de Duchenne (DMD) em corticoterapia com a evolução natural da doença (Scott, 1982) e identificar a idade ideal de início da corticoterapia. Noventa pacientes com DMD em seguimento ambulatorial no Hospital das Clínicas da Faculdade de Medicina da USP, submetidos à corticoterapia (deflazacort ou prednisolona) por um período variável de um a sete anos, foram avaliados quanto à FM através da escala MRC e quanto às habilidades motoras através da escala Hammersmith motor ability score. Foram incluídos no estudo todos os pacientes com idade entre cinco e 12 anos, que compreendiam comandos verbais e que não haviam sido submetidos a cirurgias ortopédicas corretivas ou interrompido em algum momento a corticoterapia. A relação entre FM e habilidades motoras, a comparação dos dados com aqueles do estudo de Scott et al. e a análise da influência do tempo de tratamento, da idade de início e da idade na avaliação, sobre os valores obtidos nos testes foram submetidas a tratamento estatístico. Concluiu-se que: a progressão da perda da FM e das habilidades motoras em relação à idade foi mais lenta do que a da evolução natural em todas as faixas etárias avaliadas; quanto maior a idade maior a diferença entre os dois estudos; a perda da FM foi mais intensa do que a perda da funcionalidade; a metodologia utilizada não permitiu estabelecer com clareza a influência da idade de início do tratamento sobre os parâmetros avaliados, porém demonstrou a influência positiva do tempo de tratamento sobre a FM e habilidades motoras.

The aims of this study were: to compare muscle strength (MS) and motor abilities of Duchenne Muscular Dystrophy (DMD) patients in use of steroids, with the natural evolution of the disease (Scott, 1982) and to identify the ideal age for starting on steroid therapy. Ninety patients with DMD followed as outpatients at the Clinics Hospital of the University of São Paulo School of Medicine and submitted to steroid therapy (deflazacort or prednisolone) for a period of one to seven years were assessed for MS using the MRC scale, and for motor abilities with the motor ability score from the Hammersmith scale. All patients aged between five and 12 years who understood verbal commands and who had not been submitted to corrective orthopedic surgery and had no interruption in steroid therapy, were included in the study. Statistical analysis was carried out to assess the relationship between MS and motor abilities and to compare our data against results of Scott's study. The influence of length of treatment, age at disease onset and first assessment, on values obtained in the tests was investigated. We concluded that: the progression in loss of MS and of motor abilities with age was slower than the natural evolution across all age groups studied; the higher the age the greater the difference between the two studies; loss of MS was more intense than loss of functionality; the methodology used was unable to clearly ascertain the influence of age at treatment on the parameters assessed, but a positive influence of length of treatment on both MS and motor abilities was identified.