Physiotherapy performance in motor and respiratory repercussions of patients with spinal muscular atrophy type I

OBJECTIVES: To verify in the literature the physiotherapeutic resources that have already been used in the motor and respiratory repercussions of patients with spinal muscular atrophy type I. METHODS: This is a literature review carried out in the PubMed, LILACS, SciELO and PEDro databases, in period from June to August 2019, using the keywords “spinal muscular atrophy type I” OR “Werdnig-Hoffmann syndrome” AND “physiotherapy”, as well as the terms in English. RESULTS: 53 articles were found, but after the inclusion criteria, only 5 were relevant to the research. In the respiratory system, Cough Assist®, nasotracheal aspiration, acceleration of expiratory flow and postural drainage were used, both effective for bronchial hygiene. Pulmonary expansion and noninvasive ventilation improved ventilatory mechanics. In the musculoskeletal system, stretching, joint mobilization, kinesiotherapy, and use of orthoses provided gains in functionality and reduced deformities. CONCLUSIONS: Kinesiotherapy provided motor gains such as cervical control, stretching, mobilization and orthoses, helping to maintain functionality. In respiratory repercussions, the use of Cough Assist®, nasotracheal aspiration, acceleration of expiratory flow and postural drainage were able to reduce hospitalizations for pneumonia. The pulmonary expansion and noninvasive ventilation maneuvers reversed microatelectasis, improved hematosis and contributed to increased life expectancy.