RESUMO Antecedentes: Embora haja predomínio de fraqueza muscular facial na distrofia facioescapuloumeral (FSHD), é escassa a literatura sobre aspectos de fala e deglutição. Objetivo: Investigar os padrões de fala e deglutição na FSHD e correlacioná-los com dados clínicos da doença. Métodos: Estudo transversal. Pacientes com confirmação clínica de FSHD e idade acima de 18 anos foram incluídos e pareados por idade e sexo com controles saudáveis. Foram excluídos indivíduos que apresentassem condições neurológicas que pudessem interferir nos resultados dos testes. Aplicaram-se as seguintes avaliações: fala (análise acústica e perceptivo-auditiva); deglutição, por meio do Northwestern Dysphagia Patient Check Sheet (NDPCS), Eat Assessment Tool (EAT-10), Protocolo de Avaliação para Risco de Disfagia (PARD) e Functional Oral Intake Scale (FOIS); estadiamento da doença, por meio da Gardner-Medwin-Walton scale (GMWS); e qualidade de vida, com o Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). Resultados de fala e deglutição foram correlacionados com dados clínicos da doença por teste não paramétrico. Resultados: Foram avaliados 13 indivíduos com FSHD e dez controles saudáveis. Houve diferença significativa entre os grupos nas bases motoras fonação e respiração. Na deglutição, dois (15%) indivíduos apresentaram disfagia leve e sete (53,8%), força reduzida da musculatura da face. Esses resultados não foram correlacionados com tempo de doença, idade de início dos sintomas e qualidade de vida. A disfagia esteve relacionada com a gravidade da doença. Conclusões: Pacientes com FSHD apresentaram disartria e disfagia leves. O monitoramento frequente desses sintomas pode ser uma forma importante de proporcionar reabilitação precoce e melhor qualidade de vida.

ABSTRACT Background: Although facial muscle weakness is common in patients with Facioscapulohumeral Muscular Dystrophy (FSHD), the literature is scarce on the speech and swallowing aspects. Objective: To investigate speech and swallowing patterns in FSHD and assess the correlation with clinical data. Methods: A cross-sectional study was conducted. Patients with clinical confirmation of FSHD and aged above 18 years were included and paired with healthy control individuals by age and gender. Individuals who had neurological conditions that could interfere with test results were excluded. The following assessments were applied: speech tests (acoustic and auditory-perceptual analysis); swallowing tests with the Northwestern Dysphagia Patient Check Sheet (NDPCS), the Eat Assessment Tool (EAT-10), the Speech Therapy Protocol for Dysphagia Risk (PARD), and the Functional Oral Intake Scale (FOIS); disease staging using the modified Gardner-Medwin-Walton scale (GMWS); and quality of life with the Medical Outcomes Study 36-Item Short-Form Health Survey (SF-36). The correlation between test results and clinical data was verified by non-parametric statistics. Results: Thirteen individuals with FSHD and 10 healthy controls were evaluated. The groups presented significant differences in the motor bases of phonation and breathing. Regarding swallowing, two (15%) individuals presented mild dysphagia and seven (53.8%) showed reduced facial muscles strength. These results were not correlated with duration of the disease, age at symptoms onset, and quality of life. Dysphagia was related to worsening disease severity. Conclusions: FSHD patients presented mild dysarthria and dysphagia. Frequent monitoring of these symptoms could be an important way to provide early rehabilitation and better quality of life.

RESUMO Embora seja uma doença predominantemente muscular, alterações no sistema nervoso central em pacientes com Distrofia Facioescapuloumeral (FSHD) têm sido descritas na literatura. Objetivo: Caracterizar o perfil cognitivo de pacientes com FSHD e correlacionar as alterações encontradas com variáveis clínicas e qualidade de vida. Métodos: Estudo transversal, caso-controle que avaliou pacientes com FSHD por meio de uma série de avaliações cognitivas (Mini Exame do Estado Mental — MEEM; Montreal Cognitive Assessment — MoCA; fluência verbal com restrição fonológica — FAS; fluência verbal categórica — FAS-cat; Trail Making Test — TMT; e Rey’s Verbal Auditory Learning Test — RAVLT); uma escala de gravidade neurológica (Gardner-Medwin-Walton — GMWS); e um questionário (Medical Outcomes Study 36-Item Short-Form Health Survey — SF-36). Resultados: A amostra foi composta por 13 indivíduos com FSHD e 26 controles saudáveis, pareados por sexo e idade. A análise comparativa entre os grupos FSHD e controle mostrou diferenças significativas no desempenho cognitivo dos testes MEEM, TMT A e A7 (p≤0.05) e no MoCA (p≤0,001). Verificou-se uma correlação positiva com a idade de início dos sintomas e o prejuízo na memória de longo prazo (r=-0,593, p=0,033). Em relação à qualidade de vida, observou-se uma correlação entre o domínio de limitação emocional e os testes MEEM (r=0,657, p=0,015), TMT A (r=-0,601, p=0,030) e A7 (r=0,617, p=0,025). Conclusões: Pacientes com FSHD apresentaram alterações leves na realização de tarefas que envolvem as funções de atenção, planejamento e memória de longo prazo. Essas alterações não tiveram associação com o tempo de doença nem com sua gravidade neurológica.

ABSTRACT Although it is predominantly a muscular disease, impairments in the central nervous system in patients with facioscapulohumeral muscular dystrophy (FSHD) have been described in the literature. Objective: To describe the cognitive profile of patients with FSHD and to correlate the impairments found with clinical variables and quality of life. Methods: Cross-sectional and case–control study that evaluated FSHD patients using a series of cognitive assessments (Mini-Mental State Examination — MMSE, Montreal Cognitive Assessment — MoCA, verbal fluency with phonological restriction — FAS, categorical verbal fluency — FAS-cat, trail-making test — TMT, and Rey’s Verbal Auditory Learning Test); a neurological severity scale (Gardner–Medwin–Walton — GMWS); and a quality of life measurement tool (Medical Outcomes Study 36-Item Short-Form Health Survey). Results: Individuals with FSHD (13) and healthy controls (26) were paired by gender and age. Significant differences between case and control groups were found in MMSE, TMT A, and A7 (p≤0.05) and MOCA (p≤0.001) performances. A positive correlation was verified in long-term memory impairments and the age in which symptoms appear (r=-0.593, p=0.033). Regarding quality of life assessment, the emotional domain correlated to MEEM (r=0.657, p=0.015), TMT A (r=-0.601, p=0.030), and A7 (r=0.617, p=0.025) performances. Conclusions: Individuals with FSHD presented mild impairments in the performance of tasks that involve attention, planning, and long-term memory functions. Those impairments were associated neither with the disease duration nor with its neurological severity.

Resumo A música tem sido discutida como fator promotor da saúde do idoso. Em um estudo randomizado, uma intervenção baseada em exercícios de percussão e improvisação foi comparada a uma atividade de canto coral. O objetivo foi investigar os efeitos sobre o funcionamento executivo e motor de idosos saudáveis. Um conjunto de instrumentos de avaliação psicológica e motora foi aplicado antes e depois da intervenção. Foram encontradas diferenças no desempenho do grupo de improvisação no Teste de Desenho do Relógio sugerindo possíveis ganhos executivos. Houve ganhos independentes de grupo na parte A do Teste de Trilhas, que avalia a atenção sustentada. Não foram verificadas evidências de efeitos motores. Os resultados indicam que atividades musicais podem contribuir na prevenção do declínio cognitivo decorrente do envelhecimento.

Abstract Music has been debated as a positive factor for the health of elderly people. In a randomized study, the researchers compared an intervention based on percussion and musical improvisation with a choir activity. The objective was to investigate whether improvisation would influence the executive functioning and motor skills of healthy elderly people. A set of instruments for psychological and motor assessment was used before and after the procedure. Differences were found in the performance of the participants of the improvisation group in the Clock Drawing Test suggesting possible gains in executive function. There were gains, regardless of the group, in part A of the Trail Making Test, which indicates a sustained attention. No evidence of motor effects was found in this study. The results suggest that musical activities can contribute to the prevention of cognitive decline caused by aging.

RESUMO Objetivo Investigar os efeitos de dois níveis de ruído sobre tarefas de atenção e de escrita e leitura em estudantes de três níveis de escolaridade. Método 162 sujeitos entre o 3º, 4º e 5º ano do Ensino Fundamental foram alocadas em três grupos conforme a escolaridade: controle (GC), experimental A (GEA) e experimental B (GEB). Todos os grupos foram submetidos aos testes de Atenção Concentrada de Cambraia; Avaliação de Leitura e Palavras Isoladas; Subteste Escrita sob Ditado do International Dyslexia Test. Os grupos GEA e GEB realizaram os testes em ambiente com ruídos de 20 dB e 40dB respectivamente. Os resultados foram analisados com teste de Kruskal-Wallis e correlação de Spearman com significância em 5%. Resultados Foi observado que quanto maior o escore no teste de atenção, menor o tempo gasto na leitura e menor o número de erros no ditado. Não houve diferenças nos três anos de escolaridade para o grupo GEA quanto à influência do ruído sobre a atenção e o desempenho de leitura e escrita. O grupo GEB apresentou decréscimo nos testes de atenção com aumento de erros no ditado. Comparando a escolaridade no teste de leitura, o 4º ano diminuiu o tempo de leitura enquanto os sujeitos dos 3º e 5º ano gastaram mais tempo de leitura. Conclusão A interferência auditiva é capaz de influenciar a capacidade de foco de atenção assim como o desempenho de leitura e de escrita em níveis de ruído mais intenso. Não foram observadas influências da escolaridade sobre os efeitos distratores.

ABSTRACT Purpose This study investigated the effects of two levels of noise on the performance of young students of three educational levels and tested their ability to maintain attentional focus in reading and writing tasks. Methods 162 school children in the third, fourth and fifth grades were placed in three groups according to their educational level: Control Group (CG), Experimental Group A (GEA) and Experimental Group B (GEB). All groups were submitted to a Sustained Attention Test, Reading Assessment and Isolated Words Test and Writing Dictation Sub-test (part of the International Dyslexia Test). The GEA and GEB performed the tests in a noisy environment: 20dB and 40dB, respectively. The CG was assessed in the usual school environmental noise at the same time of the day. The data was submitted to an ANOVA, the Kruskal-Wallis test and Spearman correlation test. Results The higher the score on the Sustained Attention Test, the shorter the time spent reading and fewer errors in the dictation task. There were no differences across the three grades within the GEA (lower levels of noise) with regard to the effect of noise on attention and in the reading and writing task performance. The higher levels of noise for the GEB, however, decreased the attention levels, therefore increasing mistakes on the dictation test. Comparing the performance across educational levels on the reading tasks, the fourth grade presented decreased reading time, while the third and fifth grades spent more time reading. Conclusion Auditory interference can influence the ability to focus attention as well as worsen performance in reading and writing tasks at more intense noise levels.

Resumo Objetivo: analisar o desempenho de memória de participantes com 60 anos ou mais de idade, apresentando ou não sintomas de depressão. Métodos: 199 participantes foram examinados através d. In ventário de Depressão de Beck (BDI) com ponto de corte em 20 pontos para depressão. 22 participantes atingiram o critério para inclusão no grupo de depressão e o restante foi alocado no grupo sem depressão. Os testes utilizados foram: Rey Auditory-Verbal Learning Test (RAVLT) para avaliar aprendizagem verbal, teste de reprodução de figuras I e II da Escala Wechsler Revisada (WMS-R) para memória visual imediata e duradoura e teste de memória lógica I e II da WMS-R para memória verbal. Resultados: os escores médios para aprendizagem verbal no grupo com depressão foram significativamente mais baixos do que no grupo sem depressão (p= 0,001). Não ocorreram diferenças significativas entre os grupos nas escalas para memória visual ou lógica I e II. Conclusões: sintomas de depressão afetam a retenção de informação e a aprendizagem verbal em idosos. Entretanto, não parecem afetar o processamento das memórias visual e lógica. Os resultados sugerem que, além do declínio cognitivo devido ao envelhecimento, a presença de depressão afeta e empobrece o desempenho de memória.

Abstract Objective: To analyze the memory performance of participants aged 60 years and older with and without depressive symptoms. Methods: 199 participants were assessed using the Beck Depressio. In ventory (BDI) with a cut-off point of 20 for depression. Of these, 22 met the criteria for depression group; the remaining participants were allocated to the non-depression group. The Rey Auditory-Verbal Learning Test (RAVLT) was used to assess verbal learning, figures I and II of the Visual Reproduction subtest of the Wechsler Memory Scale Revised (WMS-R) was used to evaluate immediate and delayed visual memory, and the Logical Memory subtests I and II of WMS-R were used to test verbal memory. Results: The average scores for verbal learning in the depression group were significantly lower than those in the non-depression group (p = .001). There were no group differences on visual and logical memory I and II scales. Conclusion: Depressive symptoms affect information retention and verbal learning in the elderly. However, they had no effect on visual and logical memory processing in this sample. The results suggest that, in addition to age-related cognitive decline, depression impairs memory performance considerably.

Resumen Objectivo: Analizar el rendimiento de la memoria de participantes con mas de 60 anos con sintomas de depresion. Metodos: 199 participantes fueron examinados a traves de. In ventario de Depresion de Beck (BDI), tomando en consideracion acima de 20 puntos para la depresion. 22 participantes alcanzaron el criterio de inclusion en el grupo de depresion y lo restante fue colocado en el grupo sin depresion. Las pruebas utilizadas fueron: Rey Auditory-Verbal Learning Test para evaluar el aprendizaje verbal; Prueba de Reproduccion de Figuras I y II de Wechsler Memory Scale (WMS-R) para memoria inmediata y duradera, memoria visual y logica de ensayo I y II de la WMS-R para memoria verbal. Resultados: La aprendizaje verbal en el grupo con depresion fue abajo que en el grupo sin depresion (p = .001). No hubo diferencias significativas entre los grupos en las escalas para memoria I y II visual y logica. Conclusiones: La depresion afecta la retencion de informacion y aprendizaje verbal en los ancianos pero no parece afectar el procesamiento de los recuerdos visuales y logicos. Los resultados sugieren que, ademas de deterioro cognitivo debido al envejecimiento, afecta a la presencia de depresion y el rendimiento de la memoria se vuelve pobre.

Parkinson's disease (PD) is characterized by the manifestation of akinesia, slowness to initiate movement, muscle rigidity, and tremors. However, recent evidence indicates that this pathology also causes alterations in proprioception. Disturbances in proprioceptive mechanisms directly affect postural control and the ability to calculate the velocity and amplitude of movement, suggesting that these alterations are related to the motor symptoms of PD. This article reviews the clinical data on these symptoms and presents evidence of a connection between proprioceptive deficits and the physiology of PD. The identification of proprioceptive impairments in different forms of Parkinsonism can provide valuable clues on the physiopathology of proprioception in idiopathic PD.

Knowledge is currently scarce about what happens in the brain when a decision is made to make a donation. This intriguing donation behavior is often assumed to relate to social relationships. The present study used simulated situations in which participants had to decide how to manage money when possibilities for donation were available. Electroencephalographic signals were recorded while participants were exposed to two cognitive situations, the Dictator's Game and the Prisoner's Dilemma, to simulate money exchange between people. Brain activity was measured to determine whether correlations could be made with decisions to donate. Sixty volunteers were assessed, and stimuli were presented randomly. After the presentation of the cognitive tests, the participants were allocated to two groups for the respective cognitive situations. The data showed significant differences in the left prefrontal cortex between questions with a donation context and questions not related to donations. Participants who heard a question related to donation had higher activation in the left prefrontal cortex. These results are consistent with recent functional magnetic resonance imaging studies, suggesting that greater activation in the prefrontal cortex could be produced by the logical evaluation of dilemmas. These results suggest that logical evaluation occurs when faced with a reasonable donation situation.

The last few years have seen a growing interest in stem cell research. The media has helped to keep attention to the subject making it as popular as therapeutic cloning. A number of studies highlighted the importance of stem cells as prospective therapy for neurodegenerative conditions. The possibility of successful treatment for such pathologies using stem cells has raised enormous hope. Whilst in some countries biotechnologists have realised the commercial potential of the field, human stem cell research and its clinical applications have constantly been subject of ethical and legal debate. The present review brings an overview of recent aspects of stem cell research for central nervous system disorders, with special attention to Parkinson's and Alzheimer's diseases, cerebral ischemic insult and spinal cord injury. Furthermore, the ethical implications regarding the use of embryonic stem cells as a fundamentally more promising cell type are discussed.