Background: There is considerable evidence that abnormal zinc homeostasis is related to amyotrophic lateral sclerosis (ALS) pathogenesis, and malnutrition is an independent prognostic factor for worsened survival of ALS patients. Objective: To evaluate the dietary intake and zinc status in patients with ALS, treated in a specialized outpatient facility in Natal, Brazil. Methods: Twenty patients with ALS (case group) and 37 healthy subjects (control group) were included. Clinical and anthropometric assessments were carried out and dietary intake was obtained from two 24-hour recalls. Plasma and urinary zinc concentrations were determined by atomic absorption spectrophotometry. Results: Most of the participants were eutrophic. Mean energy, protein, carbohydrate and fat intake was significantly lower for the case group. There was greater prevalence of inadequate zinc intake in the case group (35%) compared to controls (27%). Mean plasma zinc was significantly lower in the case group than in controls (77.13 ± 22.21 vs 87.84 ± 17.44 µgZn/dl). Urinary zinc did not differ significantly between cases and controls. In the case group, plasma and urinary zinc concentrations were below reference values in 50.0% and 52.6% of patients, respectively. Conclusion: A large portion of patients with ALS exhibited poor dietary intake and changes in body zinc status. The zinc deficiency found in half of the ALS patients may contribute to a worsened prognosis and should be the target of nutritional intervention that aims to correct this deficiency.

Introducción: hay pruebas considerables de que los cambios en la homeostasis del zinc están relacionados con la patogénesis de la esclerosis lateral amiotrófica (ELA) y que la malnutrición es un factor pronóstico capaz de reducir la supervivencia de los pacientes con ELA. Objetivo: evaluar la ingesta dietética y el estado de zinc en pacientes con ELA, tratados en un centro de atención ambulatoria especializado en Natal, Brasil. Métodos: se incluyeron 20 pacientes con ELA (grupo de casos) y 37 sujetos sanos (grupo control). Se realizaron evaluaciones clínicas y antropométricas y se obtuvo la ingesta dietética en dos recordatorios de 24 horas. Las concentraciones plasmáticas y urinarias de zinc se determinaron por espectrofotometría de absorción atómica. Resultados: la mayoría de los participantes fueron eutróficos. El consumo medio de energía, proteínas, carbohidratos y grasas fue significativamente menor en el grupo de casos. Hubo una mayor prevalencia de ingesta inadecuada de zinc en el grupo de casos (35%) en comparación con los controles (27%). El zinc plasmático medio fue significativamente menor en el grupo de casos que en los controles (77,13 ± 22,21 frente a 87,84 ± 17,44 μgZn/dl). El zinc urinario no difirió significativamente entre los casos y los controles. En el grupo de casos, las concentraciones de zinc plasmático y urinario fueron inferiores a los valores de referencia en el 50,0% y 52,6% de los pacientes, respectivamente. Conclusión: gran parte de los pacientes con ELA exhibieron una ingesta dietética pobre y modificación en el estatus de zinc corporal. La deficiencia de zinc encontrada en la mitad de los pacientes con ELA puede contribuir a un empeoramiento del pronóstico y debe ser el objetivo de la intervención nutricional que apunta a corregir esta deficiencia.

INTRODUÇÃO: As doenças neuromusculares infantis são crônicas, degenerativas e determinam alterações funcionais, musculares e nutricionais. OBJETIVOS: Avaliar sistematicamente a força muscular respiratória e o perfil postural e nutricional de crianças com doenças neuromusculares em seguimento multidisciplinar institucional. MATERIAIS E MÉTODOS: Foram estudados pacientes com diferentes doenças neuromusculares por meio da verificação da força muscular respiratória, da avaliação nutricional de massa muscular, do índice de massa corpórea e da porcentagem (%) de gordura corporal, além de avaliação postural e dos padrões de movimento. RESULTADOS: Foram avaliados 41 sujeitos. As crianças do sexo masculino predominaram na população em estudo, sendo 82,9% dela (n = 34), e os outros 17,1% (n = 7) eram do sexo feminino. A média de idade encontrada foi de 9,65 ± 3,11 anos. O principal diagnóstico encontrado foi Distrofia Muscular de Duchenne, 43,9% (n = 18), seguido de Atrofia Muscular Espinhal, 9,75% (n = 4), Distrofia Congênita, 7,31% (n = 3), Distrofia Muscular de Cinturas, Polineuropatia e Miopatia Congênita, todos com 4,9% (n = 2), além de Distrofia Muscular Progressiva, Miastenia Grávis, Charcoot Marie Toot, Emery Dreifuss, encontrados em 2,43% (n = 1). Foi verificada uma diminuição da força muscular respiratória (PImáx = 81 ± 24,3 cmH2O, 91% predito e PEmáx = 70 ± 29,6 cmH2O, 72% predito), mais evidente nos músculos expiratórios. A Hiperlordose lombar foi encontrada em 26 pacientes (64%) e 9 pacientes (22%) já haviam perdido a capacidade de deambular. Em relação ao perfil nutricional, 90% dos pacientes (n = 30) mostraram uma alta incidência de perda de reserva muscular e 52% deles (n = 13) apresentaram a porcentagem de gordura corporal abaixo do aceitável. CONCLUSÃO: A avaliação multidisciplinar das doenças neuromusculares pediátricas podem auxiliar no estabelecimento de tratamento precoce da Fisioterapia para melhorar a qualidade de vida dos pacientes e cuidadores.

INTRODUCTION: Neuromuscular diseases that affect childrens are degenerative and chronic disorders that lead to functional, muscular and nutritional impairments. OBJECTIVE: Assess respiratory strength, postural and nutritional profile in childrens with neuromuscular disorder followed in on ambulatory outpatients clinics. MATERIALS AND METHODS: Patients with neuromuscular diseases were assessed in relation to respiratory muscle strength (PImax and PEmax), postural profile, walk capacity and nutrition assessment that includes body mass index, muscular mass and body fat percentage. RESULTS: Forty-one patients were included in the study. Male children's were predominant in the group, 82,9% (n = 34) of them, while female were 17,1% (n = 7). The mean age was 9,65 ± 3,11 years. The most common disorder was Duchenne Miotonic Dystrophy, found in 43.9% (n = 18) of the children, followed by Spinal Muscular Atrophy, 9,75% (n = 4), Congenital muscular dystrophy, 7,31% (n = 3), Erb's scapulohumeral Dystrophy, Polyneuropathy and Congenital Myopathy, all in 4,9% (n = 2), while Progressive Muscular Dystrophy, Myasthenia Gravis, Charcoot Marie Toot, Emery Dreifuss was found in 2,43% (n = 1) of them. We found a decrease in respiratory muscle strength (PImax = 81 ± 24,3 cmH2O, 91% predict and PEmax = 70 ± 29,6 cmH2O, 72% predict), more evident in expiratory muscles. Lumbar hyperlordosis were found in 26 (64%) of the patients, 9 patients (22%) lost their ability to walk. In relation to nutritional profile, 90% (n = 30) showed one high incidence of muscular reserve loss and 52% (n = 13) of the patient's body fat was bellow acceptable range. CONCLUSION: Multidisciplinary evaluation of children neuromuscular diseases may assist in establishing early Physical Therapy intervention to improve the quality of life of both patients and caregivers.