Surgical treatment of scoliosis in spinal muscular atrophy. Em 12 pacientes foi realizada artrodese via posterior com instrumental de Luque-Galveston, e em 2 com instrumental de Cotrel-Dubousset. METHOD: Clinical data and radiographic imaging from 14 SMA patients with surgical treatment of scoliosis were reviewed, and all were reassessed clinically with new spinal radiographs and a questionnaire. The mean follow-up were 22 months.

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Amiotrofia Espinhal Progressiva Artigo

Respiratory complications are the main causes of morbidity and mortality in patients with neuromuscular disease NMD. The objectives of this study were to determine the effects that routine daily home air-stacking maneuvers have on pulmonary function in patients with spinal muscular atrophy SMA and in patients with congenital muscular dystrophy CMD , as well as to identify associations between spinal deformities and the effects of the maneuvers. Eighteen NMD patients ten with CMD and eight with SMA were submitted to routine daily air-stacking maneuvers at home with manual resuscitators for four to six months, undergoing pulmonary function tests before and after that period. In the patients without scoliosis, there was also a significant increase in FVC. Neuromuscular diseases NMDs are acquired or inherited conditions that affect parts of the neuromuscular system, such as skeletal muscles, peripheral motor nerves, neuromuscular junction, and motor neurons in the spinal cord. Spinal muscular atrophy SMA is an autosomal recessive neurodegenerative disease of childhood characterized by degeneration and loss of lower motor neurons in the anterior horn cells of the spinal cord, causing progressive proximal weakness and atrophy of skeletal muscles. Some of the most common inherited muscle diseases include progressive muscular dystrophies e.


Management of neuromuscular diseases and spinal muscular atrophy in Latin America

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