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.
Neuromuscular diseases; Respiratory muscles; Posture
