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

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.

Comprehensive left ventricular mechanics analysis by speckle tracking echocardiography in Chagas disease.

BACKGROUND: Chagas disease (CD) is a frequent cause of dilated cardiomyopathy (CMP) in developing countries, leading to clinical heart failure and worse prognosis. Therefore, the development and evolution of this CMP has always been a major topic in numbers of previous studies. A comprehensive echocardiographic study of left ventricular (LV) mechanics, fully assessing myocardial contraction, has never been done before. This could help characterize and improve the understanding of the evolution of this prevalent CMP. METHODS: A total of 47 chagasic and 84 control patients were included in this study and allocated in groups according to LV ejection fraction. 2D-Echocardiogram was acquired for LV mechanics analysis by speckle tracking echocardiography. RESULTS: Mean age of chagasic individuals was 55y and 16 (34 %) were men. Significant difference was found in global longitudinal velocity analysis, with lower values in indeterminate form. In the group with severe systolic dysfunction, a paradoxical increase in longitudinal and apical radial displacements were demonstrated. In parallel, segmental analyzes highlighted lower values of radial displacement, strain and strain rate into inferior and inferolateral walls, with increase of these values in septal and anterior walls. CONCLUSION: Chagasic CMP has a vicarious pattern of contraction in the course of its evolution, defined by reduced displacement and strain into inferior and posterior walls with paradoxical increase in septal and anterior segments. Also, lower longitudinal velocities were demonstrated in CD indeterminate form, which may indicate an incipient myocardial injury.

Association between educational status and dual-task performance in young adults.

The influence of educational status on perceptual-motor performance has not been investigated. The single- and dual-task performances of 15 Low educated adults (9 men, 6 women; M age=24.1 yr.; 6-9 yr. of education) and 15 Higher educated adults (8 men, 7 women; M age=24.7 yr.; 10-13 yr. of education) were compared. The perceptual task consisted of verbally classifying two figures (equal or different). The motor task consisted of alternating steps from the floor to a stool. Tasks were assessed individually and simultaneously. Two analyses of variance (2 groups×4 blocks) compared the errors and steps. The Low education group committed more errors and had less improvement on the perceptual task than the High education group. During and after the perceptual-motor task performance, errors increased only in the Low education group. Education correlated to perceptual and motor performance. The Low education group showed more errors and less step alternations on the perceptual-motor task compared to the High education group. This difference on the number of errors was also observed after the dual-task, when the perceptual task was performed alone.

Influence of educational status on executive function and functional balance in individuals with Parkinson disease.

OBJECTIVE: This study investigated whether educational status influenced how people with Parkinson disease (PD) performed on Parts A, B, and DELTA of the Trail Making Test (TMT) and on the Berg Balance Scale (BBS). BACKGROUND: Recent studies have shown that educational status may influence cognitive and motor test performance. METHODS: We gave the TMT and the BBS to assess executive function and functional balance in 28 people with PD (Hoehn and Yahr score between 2 and 3) and 30 healthy elderly people. Participants reported their number of years of formal education. We divided each group of participants by educational status: low (4 to 10 years of education) or high (≥11 years). RESULTS: In both the PD (P=0.018) and control (P=0.003) groups, participants with low educational status performed worse on the TMT Part B than did those with high educational status. Within the PD group, the less-educated participants scored worse on the BBS than did the more educated (P<0.001); this difference was not significant between the more- and less-educated controls (P=0.976). CONCLUSIONS: Whether or not they had PD, less-educated people performed worse than more-educated people on the TMT Part B. Educational status affected executive function, but PD status did not. Among individuals with PD, educational status influenced functional balance.

Effect of speech therapy on quality of life in patients with spinocerebelar ataxia type 3.

BACKGROUND: Individuals with spinocerebellar ataxia type 3 (SCA3) present communication and swallowing disorders, and consequent deterioration in quality of life (QOL). OBJECTIVE: To evaluate the impact of a speech therapy rehabilitation program on the QOL of patients with SCA3. METHODS: All participants were randomly assigned to two groups, an intervention group receiving speech therapy (STG) and a control group (CG). The International Cooperative Ataxia Rating Scale scores were 32.4 ± 20.2, and the Scale for the Assessment and Rating of Ataxia scores were 11.8 ± 8.0. The intervention consisted of a 12-session speech therapy rehabilitation program with oral, pharyngeal, and laryngeal strengthening exercises-the so-called ATAXIA-Myofunctional Orofacial and Vocal Therapy (A-MOVT). They all were submitted to pre- and postintervention evaluations using the World Health Organization's Quality of Life (WHOQOL-BREF) assessment, as well as the Living with Dysarthria (LwD), Quality of Life in Swallowing Disorders (SWAL-QOL), and Food Assessment Tool (EAT-10). RESULTS: The study sample consisted of 48 patients with SCA3 (STG = 25; CG = 23), mean age was 47.1 ± 11.4 years; mean age at symptom onset was 36.9 ± 11.3 years; disease duration was 11.9 ± 13.3 years. After the 3-month intervention, there were significant changes in the QOL in the STG compared with the CG, when assessed by the LwD (179.12 ± 62.55 vs. 129.88 ± 51.42, p < 0.001), SWAL-QOL (869.43 ± 153.63 vs. 911.60 ± 130.90, p = 0.010), and EAT-10 (5.16 ± 7.55 vs. 2.08 ± 3.85, p = 0.018). CONCLUSIONS: Patients with SCA3 should receive continuous speech therapy as part of the A-MOVT program, because therapy helps to improve difficulty swallowing and dysarthria.

ANTECEDENTES: Indivíduos com ataxia espinocerebelar tipo 3 (AEC3) apresentam distúrbios da comunicação e deterioração da deglutição e, consequentemente, na qualidade de vida (QV). OBJETIVO: Avaliar o impacto de um programa de reabilitação fonoaudiológica na QV em pacientes com AEC3. MéTODOS: Todos os participantes foram alocados aleatoriamente em dois grupos, um grupo intervenção que recebeu terapia fonoaudiológica (GTF) e um grupo controle (GC). As pontuações das escalas: International Cooperative Ataxia Rating Scale (ICARS) foram 32,4 ± 20,2 e da Scale for the Assessment and Rating of Ataxia (SARA) foram 11,8 ± 8,0. A intervenção consistiu em um programa de reabilitação fonoaudiológica de 12 sessões composto por exercícios de fortalecimento oral, faríngeo e laríngeo - denominados ATAXIA - Terapia Miofuncional Orofacial e Vocal (A-TMOV). Todos foram submetidos a avaliações pré e pós-intervenção por meio dos protocolos World Health Organization's Quality of Life (WHOQOL-BREF), Vivendo com Disartria (VcD), Quality of Life in Swallowing Disorders (SWAL-QOL) e Food Assessment Tool (EAT-10). RESULTADOS: A amostra foi composta por 48 pacientes com AEC3 (25 no GTF e 23 no GC), média de idade 47,1 ± 11,4anos; média de idade de início dos sintomas 36,9 ± 11,3anos; duração da doença 11,9 ± 13,3anos. Após intervenção de três meses, houve mudanças significativas na QV no GTF em comparação com o GC quando avaliado pelo VcD (179,12 ± 62,55 versus129,88 ± 51,42, p < 0,001), SWAL-QOL (869,43 ± 153,63 versus 911,60 ± 130,90, p = 0,010), EAT-10 (5,16 ± 7,55 versus 2,08 ± 3,85, p = 0,018). CONCLUSõES: Pacientes com AEC3 devem receber terapia fonoaudiológica contínua como parte do programa A-TMOV, pois a terapia ajuda a melhorar a dificuldade de deglutição e a disartria.

Relationship of executive function and educational status with functional balance in older adults.

BACKGROUND: The Berg Balance Scale (BBS) is frequently used to assess functional balance in older adults. The relationship of executive function and level of education with the BBS performance has not been described. The aim of this study was to determine whether (1) the performance on a task requiring executive function (part B of the Trail Making Test, TMT-B) influences results of motor and cognitive tests and (2) the number of years of formal education could be related to performance on BBS in older adults. We also explored whether there would be differences, based on performance on TMT-B (high vs low) in motor function (BBS, the timed up and go [TUG]) or cognitive function (TMT-A and TMTDELTA), the Mini Mental State Examination (MMSE), as well as years of education. METHODS: Participants included 101 older adults (age range, 60-80 years) residing in São Paulo, Brazil. Functional balance was assessed using BBS and TUG. Executive function was assessed using the TMT and MMSE. Educational status was determined by self-report of participant's total number of years of formal education. RESULTS: The BBS scores were inversely related to TMT-A time (r = -0.63, r = 0.40, P < .001) and TMT-B time (r = -0.56, r = 0.31, P < .001). There was a similar relationship with TMTDELTA (r = -0.47, r = 0.22, P < .001). The BBS scores were positively correlated to years of formal education (r = 0.48, r = 0.23, P < .001). There was a ceiling effect on the TMT-B, with many individuals reaching maximum score of 300 seconds. Participants with high levels of executive function had higher BBS and MMSE scores, more education, and lower TMT-A, TMTDELTA and TUG scores (P < .001) than the lower functioning group. CONCLUSIONS: Individuals with higher capacity on tasks requiring visuospatial abilities, psychomotor speed, and executive function, such as the TMT, had better performance on BBS. Individuals with a high executive function, measured by TMT-B, also performed better on other motor and cognitive tests.

Translation and cross-cultural adaptation to Brazilian Portuguese of the Modified Tardieu Scale for muscle tone assessment among patients with spinal cord injury.

BACKGROUND: Assessment of muscle tone is of great importance for evaluating people with spinal injuries. OBJECTIVE: To translate and adapt the Modified Tardieu Scale (MTS) to Brazilian Portuguese and validate its use for evaluating patients with spinal cord injury. METHODS: The translation and adaptation of the "Escala de Tardieu Modificada" went through the steps of translation, translation synthesis, back-translation and expert committee meeting. Two evaluators rated the tone of the elbow flexors/extensors, wrist extensors, knee flexors/extensors and ankle plantar flexors of 51 patients with spinal cord injury. These patients were reevaluated after one week. Validation included intra and inter-rater reliability (ICC) and internal and external consistency. The Modified Ashworth Scale (MAS) was used in the evaluations, to investigate the correlations. RESULTS: The Brazilian Portuguese version of the MTS is presented in this study. ICCs ranged from 0.60 to 0.99 (intra and inter-examiner) and there was a moderate to strong correlation with MAS. CONCLUSIONS: The MTS proved to be adequate for assessing the muscle tone of people with spinal cord injury in Brazil.

Effects of resistance training on postural control in Parkinson's disease: a randomized controlled trial.

BACKGROUND: Postural instability affects Parkinson's disease (PD) patients' postural control right from the early stages of the disease. The benefits of resistance training (RT) for balance and functional capacity have been described in the literature, but few studies have been conducted showing its effects on PD patients' postural control. OBJECTIVE: To investigate the effects of a three-month RT intervention on static posturography (SP) measurements and clinical functional balance assessment among PD patients. METHODS: Seventy-four patients were randomly assigned to a three-month RT intervention consisting of using weightlifting machines at a gym (gym group) or RT consisting of using free weights and elastic bands (freew group), or to a control group. The participants were evaluated at baseline, three months and six months. We evaluated changes of SP measurements under eyes-open, eyes-closed and dual-task conditions (primary endpoint), along with motor performance and balance effects by means of clinical scales, dynamic posturography and perceptions of quality of life (secondary endpoints). RESULTS: There were no significant interactions in SP measurements among the groups. Unified Parkinson Disease Rating Scale (UPDRS-III) motor scores decreased in both RT groups (p<0.05). Better perceived quality of life for the mobility domain was reported in the gym group while functional balance scores improved in the freew group, which were maintained at the six-month follow-up (p<0.05). CONCLUSIONS: This study was not able to detect changes in SP measurements following a three-month RT intervention. Both RT groups of PD patients showed improved motor performance, with positive balance effects in the freew group and better perceived quality of life in the gym group.

Timed immersion expiration measures in patients with muscular dystrophies.

INTRODUCTION: Muscular dystrophies (MD) cause muscle weakness, affecting motor and respiratory functions. Aquatic activities maintain strength and ventilatory function and may require immersion expiration control. OBJECTIVES: (1) To describe the evolution of timed immersion expiration in patients with MD in one-year follow-up. (2) to describe motor and respiratory outcomes in one-year follow-up. (3) to investigate possible relationships between timed immersion expiration and age, motor and respiratory functions. METHOD: Fifty-seven patients with MD (12-35 years, Vignos scale 2-8) were evaluated twice, with one-year interval. Immersion expiration control was timed with a chronometer. Motor function was assessed by Motor Function Measure. The respiratory function was evaluated by spirometry. Analysis of variance compared assessments and Pearson tests investigated relationships between variables and age. RESULTS: Motor and respiratory functions decreased (p < 0.001) but timed immersion expiration was maintained. Timed immersion expiration was not correlated to motor and respiratory functions. CONCLUSION: As patients maintained timed immersion expiration in the one-year follow-up, aquatic therapy might be a facilitator for people with MD.

Balance as an Additional Effect of Strength and Flexibility Aquatic Training in Sedentary Lifestyle Elderly Women.

OBJECTIVE: To evaluate the additional effects of on balance an aquatic muscle strengthening and flexibility training program in healthy sedentary lifestyle elderly women. METHOD: This controlled clinical trial included 56 healthy sedentary women, aged from 65 to 70 years, divided into two groups. The aquatic group (AG) underwent aquatic training (45 minutes/session, 32 sessions), and the control group (CG) received no intervention. Data were collected pre- and post-intervention, during a one-week period. Lower limb muscle strength was measured by a force sensor (myometer). Flexibility was measured by biophotogrammetry. Functional balance was evaluated by the Performance Oriented Mobility Assessment (POMA) and the Berg Balance Scale (BBS). RESULTS: Muscle strength, flexibility, and balance improved in AG (p < 0.001), but not in CG. CONCLUSION: The aquatic exercises program, which was originally developed to promote muscle strength and flexibility, also improved functional balance. Aquatic training is an option for physical health promotion for sedentary lifestyle elderly women.

Functional performance and muscular strength in symptomatic female carriers of Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) usually affects men. However, women are also affected in rare instances. Approximately 8% of female DMD carriers have muscle weakness and cardiomyopathy. The early identification of functional and motor impairments can support clinical decision making. OBJECTIVE: To investigate the motor and functional impairments of 10 female patients with dystrophinopathy diagnosed with clinical, pathological, genetic and immunohistochemical studies. METHODS: A descriptive study of a sample of symptomatic female carriers of DMD mutations. The studied variables were muscular strength and functional performance. RESULTS: The prevalence was 10/118 (8.4%) symptomatic female carriers. Deletions were found in seven patients. The age of onset of symptoms in female carriers of DMD was quite variable. Pseudohypertrophy of calf muscles, muscular weakness, compensatory movements and longer timed performance on functional tasks were observed in most of the cases. Differently from males with DMD, seven female patients showed asymmetrical muscular weakness. The asymmetric presentation of muscle weakness was frequent and affected posture and functionality in some cases. The functional performance presents greater number of compensatory movements. Time of execution of activities was not a good biomarker of functionality for this population, because it does not change in the same proportion as the number of movement compensations. CONCLUSION: Clinical manifestation of asymmetrical muscle weakness and compensatory movements, or both can be found in female carriers of DMD mutations, which can adversely affect posture and functional performance of these patients.

Cognitive performance of children with spinal muscular atrophy: A systematic review.

Spinal muscular atrophy (SMA) is genetic and progressive, caused by large bi-allelic deletions in the SMN1 gene, or the association of a large deletion and a null variant. OBJECTIVE: To evaluate the evidence about cognitive outcomes in spinal muscular atrophy (SMA). METHODS: Searches on the PUBMED/Medline, Web of Knowledge and Scielo databases retrieved 26 studies (1989 to 2019, descriptors "spinal muscular atrophy" and "cognition"). Nine studies were selected according to the eligibility criteria: (1) cognition tested in individuals with SMA; (2) written in English or Spanish. The Risk of Bias in Non-Randomized Studies of Interventions was used to describe design, bias, participants, evaluation protocol and main findings. This study was registered on the International prospective register of systematic reviews (PROSPERO). RESULTS: Three studies described normal cognition. In another three studies, cognitive outcomes were above average. Cognitive impairment was found in three studies. Poor cognitive performance was more frequently reported in studies that were recent, included children with SMA type I and that employed visual/auditory attention and executive function tests. Protocols and cognitive domains varied, precluding metanalysis. CONCLUSION: The severity of motor impairment may be related to cognitive outcomes: studies that included a higher number/percentage of children with SMA type I found cognitive impairment. The establishment of gold-standard protocols is necessary. Further studies should compare the cognitive outcomes of subjects with SMA types I to IV.

A atrofia muscular espinhal (SMA) é genética e progressiva, causada por grandes deleções bi-alélicas no gene SMN1, ou pela associação de uma grande deleção e uma variante nula. OBJETIVO: Avaliar as evidências sobre o desempenho cognitivo na atrofia muscular espinhal (AME). MÉTODOS: Pesquisas nas bases de dados PUBMED/ Medline, Web of Knowledge e Scielo localizaram 26 estudos (1989 a 2019, descritores "atrofia muscular espinhal" e "cognição"). Nove estudos foram selecionados de acordo com os critérios de elegibilidade: (1) testaram a cognição em pessoas com AME; (2) escritos em inglês/espanhol. A avaliação do risco de viés em estudos com intervenções não-randomizadas foi utilizada para descrever o desenho experimental, viés, amostra, protocolo de avaliação e principais achados. Este estudo foi aprovado no Registro Internacional Prospectivo de Revisões Sistemáticas (PROSPERO). RESULTADOS: Em três estudos, foi registrado que a cognição estava preservada. Em três estudos, o desempenho cognitivo estava acima da média. O comprometimento cognitivo foi encontrado em três estudos. Desempenho cognitivo pobre foi mais frequentemente relatado em estudos recentes, estudos que incluíram crianças com AME tipo I e estudos que incluíram atenção visual/auditiva e testes de função executiva. Protocolos e domínios cognitivos variaram muito, portanto não foi possível a realização de metanálise. CONCLUSÃO: A gravidade do comprometimento motor pode estar relacionada ao desempenho cognitivo: estudos que incluíram maior número/porcentagem de crianças com AME tipo I encontraram alterações no desempenho cognitivo. O estabelecimento de protocolos padrão-ouro é necessário. Novos estudos devem comparar o desempenho cognitivo de pessoas com AME tipos I a IV, ou seja, com diferenças no prognóstico e no desempenho motor.

Is virtual reality beneficial for dual-task gait training in patients with Parkinson's disease? A systematic review.

This systematic review examined the evidence about the effects of virtual reality (VR) on dual-task gait training in Parkinson's disease (PD). METHODS: this study (PROSPERO registration CRD42019114736) aimed to answer the question: "Is VR beneficial for dual-task gait training in patients with PD?" We searched for studies from 2008 to 2018 on Medline/PubMed and Web of Science/Web of knowledge databases. The keywords were Parkinson AND gait training AND virtual reality OR Parkinson AND gait training AND game. A total of 55 articles were retrieved, of which 11 systematic reviews, 11 opinions, letters to the editor, posters or conferences abstracts and 17 studies not evaluating the effects of VR gait training were excluded. Three further studies addressing VR dual-task gait training in PD (found in references of studies selected) were also included. Therefore, 19 studies were included and analysed. RESULTS: all studies reported gait improvement after VR training. Many clinical scales were used, hampering comparison of the effects of each protocol. CONCLUSION: VR dual-task gait training should be part of rehabilitation protocols for PD. The studies showed that VR training was effective, although specific guidelines have not yet been established.

Essa revisão sistemática examinou as evidências sobre os efeitos da realidade virtual (RV) no treinamento de marcha com tarefa-dupla na doença de Parkinson (DP). MÉTODOS: esse estudo (registro PROSPERO: CRD42019114736) visou responder à questão: "A RV é benéfica no treinamento de marcha com tarefa-dupla em pacientes com DP?" A busca, de 2008 a 2018, foi feita nos bancos de dados Medline/PubMed e Web of Science/Web of Knowledge. Os descritores foram Parkinson, treinamento de marcha, realidade virtual e jogos eletrônicos. Foram encontrados 55 artigos e eliminadas 11 revisões sistemáticas, 11 cartas ao editor, resumos de pôsteres ou conferências e 17 estudos que não avaliaram os efeitos do treinamento da marcha em RV. Três outros estudos (encontrados nas referências dos estudos selecionados) foram incluídos porque também abordaram o treinamento de marcha com dupla tarefa de RV em DP. Portanto, 19 estudos foram incluídos e analisados. RESULTADOS: todos os estudos relataram melhora da marcha após o treinamento com RV. Muitas escalas clínicas foram usadas e foi difícil comparar os efeitos de cada protocolo. CONCLUSÃO: RV deve fazer parte dos protocolos de reabilitação em DP. O treinamento com RV é eficaz, embora recomendações específicas ainda não tenham sido estabelecidas.

Relationship Between Posturography, Clinical Balance and Executive Function in Parkinson´s Disease.

This study aimed to evaluate the relationship between posturography, clinical balance, and executive function tests in Parkinson´s disease (PD). Seventy-one people participated in the study. Static posturography evaluated the center of pressure fluctuations in quiet standing and dynamic posturography assessed sit-to-stand, tandem walk, and step over an obstacle. Functional balance was evaluated by Berg Balance Scale, MiniBESTest, and Timed Up and Go test. Executive function was assessed by Trail Making Test (TMT) and semantic verbal fluency test. Step over obstacle measures (percentage of body weight transfer and movement time) were moderately correlated to Timed Up and Go, part B of TMT and semantic verbal fluency (r > 0.40; p < 0.05 in all relationships). Stepping over an obstacle assesses the responses to internal perturbations. Participants with shorter movement times and higher percentage of body weight transfer (higher lift up index) on this task were also faster in Timed Up and Go, part B of TMT, and semantic verbal fluency. All these tasks require executive function (problem solving, sequencing, shifting attention), which is affected by PD and contribute to postural assessment.

Hip Mobilization at Preterm Age May Accelerate Developmental Dysplasia Recovery.

PURPOSE: Few studies have described mobilization approaches in developmental dysplasia of the hip (DDH). The present study describes the hip mobilization of a preterm infant (born at 33 6/7 weeks of gestational age) diagnosed with DDH. DESIGN AND METHODS: During the 43-day hospital stay, the infant was seen twice a week (ten sessions, 20 minutes each). All sessions included hip approximation maneuvers, with the hip positioned in abduction, lateral rotation and flexion, and lower limbs passive mobilization, which were taught to the mother. Early intervention with auditory, tactile, visual, and vestibular stimulations was also performed. The infant was assessed with hip ultrasound before and after treatment. RESULTS: At 34 2/7 weeks of gestational age, she was classified as Graf IIa (left: alpha: 55°, beta: 68°; right: alpha: 59°, beta: 64°). At 40 5/7 weeks, she was classified as Graf I for left (alpha: 67°; beta: 42°) and right (alpha: 66°; beta: 42°) hips. PRACTICAL IMPLICATIONS: The intervention seemed to accelerate the acquisition of stability of dysplasic hips in a preterm infant. The outcome supports further investigation of hip approximation maneuvers as part of early stimulation in preterm infants with DDH during hospital stay.

A didactic approach to presenting verbal and visual information to children participating in research protocols: the comic book informed assent.

OBJECTIVE: When children participate in research protocols, consent (by a parent or legal guardian) and assent (by the children) must be given. Understanding research protocols can be challenging for an adult and even more difficult for a child. The aim of this study was to describe the development of a comic book created to facilitate children's understanding of informed assent with clear and simple language. METHODS: Five researchers with scores above seven according to the Fehring criteria developed the comic book, avoiding the use of technical terminology. Twenty children between 7 and 12 years old, and enrolled in a larger study, responded using a Likert scale and questions about the clarity of texts and illustrations. The final version met National Health System Resolutions (Resoluções do Conselho Nacional da Saúde - CNS n° 196/1996 and 466/2012). RESULTS: The comic book assent presents a short story containing information about a real study: the invitation to participate, objectives, methods, instruments, procedures, risks, benefits, and the researchers' contact information. Most of the participants answered that they perceived the content of the text to be "Excellent" (40%) and "Very good" (40%), and the illustrations were perceived as "Excellent" (45%) and "Very good" (55%). CONCLUSION: The construction of a simple and clear model of informed assent is possible, and this model should be used in experiments with children.

Reliability, validity and description of timed performance of the Jebsen-Taylor Test in patients with muscular dystrophies.

BACKGROUND: The Jebsen-Taylor Test evaluates upper limb function by measuring timed performance on everyday activities. The test is used to assess and monitor the progression of patients with Parkinson disease, cerebral palsy, stroke and brain injury. OBJECTIVES: To analyze the reliability, internal consistency and validity of the Jebsen-Taylor Test in people with Muscular Dystrophy and to describe and classify upper limb timed performance of people with Muscular Dystrophy. METHODS: Fifty patients with Muscular Dystrophy were assessed. Non-dominant and dominant upper limb performances on the Jebsen-Taylor Test were filmed. Two raters evaluated timed performance for inter-rater reliability analysis. Test-retest reliability was investigated by using intraclass correlation coefficients. Internal consistency was assessed using the Cronbach alpha. Construct validity was conducted by comparing the Jebsen-Taylor Test with the Performance of Upper Limb. RESULTS: The internal consistency of Jebsen-Taylor Test was good (Cronbach's α=0.98). A very high inter-rater reliability (0.903-0.999), except for writing with an Intraclass correlation coefficient of 0.772-1.000. Strong correlations between the Jebsen-Taylor Test and the Performance of Upper Limb Module were found (rho=-0.712). CONCLUSION: The Jebsen-Taylor Test is a reliable and valid measure of timed performance for people with Muscular Dystrophy.

Boys With Duchenne Muscular Dystrophy: 1-Year Locomotor Changes in Relation to a Control Group.

We compared the timed performance and compensatory movements of 32 boys (mean age = 10.0 years) with Duchenne muscular dystrophy (DMD) and 38 healthy boys (mean age = 9.2 years) on 10 -meter walking and four-step stair work (climbing and descending). Dependent measures were digital scores on the Functional Evaluation Scale for DMD obtained by watching the boys on film. Groups were compared initially and after 6 and 12 months, using General Linear Models analysis (for two groups and three assessments). Results showed no significant timed performance differences between groups on 10 -m walking at the initial assessment; but boys with DMD showed longer times after 6 and 12 months, and boys with DMD engaged in more compensatory movements while walking. For stair climbing, boys with DMD were significantly slower than healthy controls when both climbing and descending steps in all assessments.

Cross-cultural adaptation and validation of the International Cooperative Ataxia Rating Scale (ICARS) to Brazilian Portuguese.

OBJECTIVE: The clinical assessment of patients with ataxias requires reliable scales. We aimed to translate, adapt and validate the International Cooperative Ataxia Rating Scale (ICARS) into Brazilian Portuguese. METHODS: The steps of this study were forward translation, translation synthesis, backward translation, expert committee meeting, preliminary pilot testing and final assessment. Thirty patients were enrolled in the preliminary pilot testing and 61 patients were evaluated for construct validity, internal consistency, intra- and inter-rater reliability and external consistency. RESULTS: This study showed good validity of the construct and high internal consistency for the full scale, except for the oculomotor domain (Cronbach's alpha = 0.316, intraclass correlation coefficients intra- = 82.4% and inter- = 79.2%). A high correlation with the Scale for the Assessment and Rating of Ataxia was observed. We found good intra-rater agreement and relative inter-rater disagreement, except in the posture and gait domain. CONCLUSION: The present ICARS version is adapted for the Brazilian culture and can be used to assess our ataxic patients.

Indeterminate form of Chagas disease: is left ventricular torsional mechanics a clue to subclinical myocardial abnormalities?

BACKGROUND: Chagas disease (CD) is highly endemic in Latin America, and has been emerging in developed countries. Early diagnosis of left ventricular (LV) systolic dysfunction, routinely done by echocardiography, is crucial for therapy. Speckle tracking echocardiography allows determination of LV segmental rotations, twist/torsion, and this analysis may disclose an unapparent myocardial contractile deficit in initial stages of CD. Therefore, this study aimed to highlight a possible unapparent myocardial contractile deficit in CD indeterminate form (IF) by a comprehensive torsional mechanics analysis. METHODS: Patients with CDIF diagnosis, initial chagasic cardiomyopathy (CCMP characterized by ECG abnormalities and LVEF > 0.55), and normal controls were prospectively enrolled, and underwent conventional echocardiogram with images acquisition for STE offline analysis. Target parameters were basal and apical rotations, and calculation of twist and torsion. RESULTS: A total of 42 patients were included (CDIF, eight; CCMP, 13; and 21 controls). Chagasic patients had a mean age of 55 years, five (24 %) men, and controls, 50 years, seven (33 %) men. Torsional mechanics analyzes resulted in no significant difference in all parameters studied between groups. Twist in CDIF was 20.8° ± 6.5°; CCMP, 19.0° ± 3.5° and controls, 19.7° ± 3.7° (p = 0.67). Torsion in CDIF was 2.6° ± 0.8°; CCMP, 2.4° ± 0.6° and controls, 2.4° ± 0.5° (p = 0.63). CONCLUSIONS: Patients with Chagas disease have no abnormalities in torsional mechanics during its indeterminate and initial cardiomyopathy stages.