A fisioterapia na amiotrofia espinhal progressiva tipo I: Uma revisão de literatura

Abstract

The spinal muscular atrophy type I is the most severe form of muscular atrophies, starting at about six months of life, leading to death in childhood. Objective. To evaluate the role of physiotherapy, through a literature review, in preventing and treating signs and symptoms of spinal muscular atrophy type I, minimizing motor and respiratory complications and improving quality of life. Method. Electronic research databases in the Cochrane Library, IBECS, Lilacs, Medline, PEDro and Scielo, from 1999 to 2012, in English and Portuguese, with the descriptors: neuromuscular diseases, spinal muscular atrophy, Werdning Hoffman, physiotherapy, respiratory disorders, muscle weakness. Results. Of the 43 items surveyed, 20 articles were used. Of these, only six analyzed the importance of physical therapy in the treatment of spinal muscular atrophy. Conclusions. Physical therapy is essential and compulsory for children with spinal muscular atrophy, in order to delay or prevent complications that appear early, through prophylactic and therapeutic management of respiratory infections, contractures and deformities, making the child as functional as possible, and promoting proper ventilatory assistance and improving survival and quality of life.