The purpose of this study was to evaluate the quality of life (QoL) of patients with Duchenne muscular dystrophy (DMD) in different stages of the disease, by means of the Life Satisfaction Index for Adolescents (LSI-A). The practicality of this scale was also verified. The LSI-A was applied four times to 95 patients with DMD who were undergoing steroid therapy, at three-month intervals. The patients were divided into four groups according to age. The results from the four applications and the inter and intra-examiner concordance were treated statistically. Comparing the different age groups, patients with DMD did not lose QoL, even with disease progression. We concluded that, in spite of the progressive course of the disease, the QoL in patients with DMD does not get worse. The use of a scale that embraces a great diversity of circumstances in patients' lives, without considering clinical aspects excessively, is a good alternative for assessing the QoL of these patients. Key words: quality of life, Duchenne muscular dystrophy, steroid therapy, questionnaire, Life Satisfaction Index for Adolescents. Distrofia muscular de Duchenne: avaliação da qualidade de vida em 95 pacientes através do Life Satisfaction Index for AdolescentsRESUMO O objetivo deste estudo foi de quantificar a qualidade de vida (QV) em crianças com distrofia muscular de Duchenne (DMD) em diferentes idades através do uso do questionário Life Satisfaction Index for Adolescents (LSI-A). Foi também avaliada a praticidade do questionário. O LSI-A foi aplicado a 95 pacientes com distrofia muscular de Duchenne em corticoterapia, em diferentes idades, e por quatro vezes com intervalos de três meses. Os resultados concernentes às quatro avaliações e a concordância inter e intra-observador foram tratados estatisticamente. Comparando diferentes faixas etárias, mesmo ao longo da progressão da doença, não notamos perda da QV. Concluímos que por não valorizar excessivamente os aspectos clínicos e abranger uma diversidade de circunstâncias cotidianas, O LSI-A é útil na avaliação da QV das crianças com DMD, sendo também de fácil aplicação. Palavras-chave: qualidade de vida, distrofia muscular de Duchenne, corticoterapia, questionário, Life Satisfaction Index for Adolescents.

Objective: To validate the Life Satisfaction Index for Adolescents (LSI-A) scale, parent version and patient version, for Duchenne muscular dystrophy (DMD), spinal muscular atrophy (SMA) and limb-girdle muscular dystrophy (LGMD). Methods: The parent version of the instrument was divided into Groups A, B, C and D; and the patient version, divided into B, C and D. For the statistical calculation, the following tests were used: Cronbach's α, ICC, Pearson and the ROC Curve. Results: The parent and patient versions of the instrument are presented, with the following results in the overall score, respectively: Cronbach's α, 0.87 and 0.89; reliability, r 0.98 and 0.97; reproducibility, ICC 0.69 and 0.80; sensitivity, 0.78 and 0.72; specificity, 0.5 and 0.69; and accuracy, 64% and 70.4%. Conclusion: According to the validity and reproducibility values, the LSI-A Brazil parent and patient versions, are clinically useful to assess quality of life in DMD, SMA or LGMD and may also be useful for other neuromuscular disorders.Keywords: validation studies; quality of life; neuromuscular disorders. RESUMO Objetivo: Validar a escala Life Satisfaction Index for Adolescents (LSI-A) versão pais e versão paciente para doenças neuromusculares.Método: O instrumento versão pais foi dividido nos Grupos A,B, C e D; e paciente, em B, C e D. Para cálculo estatístico utilizou-se os testes α de Cronbach, CIC, Pearson e Curva ROC. Resultados: Valor de Cronbach versão pais e paciente no escore geral, 0.87 e 0.89; confiabilidade,0.98 e 0.97;reprodutibilidade,entre 0.59 e 0.69 e, entre 0.58 e 0.80; sensibilidade, 0.78 e 0.72; especificidade, 0.5 e 0.69; e acurácia, 64% e 70.4% respectivamente. Conclusão: Conforme a validade e reprodutibilidade, o LSI-A Brasil versão pais e paciente é útil clinicamente para avaliar a Qualidade de Vida da Distrofia Muscular de Duchenne, Amiotrofia Espinhal Progressiva ou Distrofia Muscular tipo Cinturas e pode ser usado para outras doenças neuromusculares.Palavras-chave: estudos de validação; qualidade de vida; doenças neuromusculares.The importance of quantifying the quality of life of subjects with neuromuscular disorders (NMD) by use of appropriate instruments is a relevant health issue because of the lack of specific questionnaires. This study dealt with the translation and validation of a questionnaire for NMD patients. In this study, three NMDs were considered: two dystrophies and one atrophy: Duchenne muscular dystrophy (DMD), limb-girdle muscular dystrophy (LGMD) and spinal muscular atrophy (SMA), respectively. The dystrophies are characterized by irreversible degeneration of muscular fibers, which leads to muscular weakness and motor disability, originating from mutations in different genes 1 . The atrophy is characterized by degeneration of the spinal cord motor neurons, i.e., secondary muscular atrophyThere is scientific consensus that the health-related quality of life (HRQOL) involves subjective and multidimensional values, either negative or positive, according to individual perception and ex...

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Objective: To validate the parent-rated Expression and Emotion Scale for Children (EESC) for patients with attention-deficit/hyperactivity disorder (ADHD). Methods: The EESC was applied to parents of children with and without ADHD. The children were divided into age groups: Group A, between six and eight years old; Group B, between nine and 11 years old; and Group C, between 12 and 15 years old. The validation was carried out according to the steps proposed by Guillemin et al. For the statistical calculation, Cronbach's α, Pearson's correlation, the ICC and ROC curve were used. Results: The statistical tests showed satisfactory coefficients: Cronbach's α = 0.76; Pearson's correlation r = 0.91 with CI 95%; replicability ICC = 0.66; sensitivity 0.75; specificity 0.67; accuracy 71%. Conclusion: According to psychometric data on internal and external consistency (reliability, reproducibility), sensitivity, and specificity, the parent-rated EESC for ADHD is useful in assessing emotional expression.Keywords: attention deficit disorder with hyperactivity, child; adolescents. RESUMO Objetivo: Validar o instrumento Expression and Emotion Scale for Children