INTERVENÇÃO DA FISIOTERAPIA COM O MÉTODO SELF-HEALING E EXERCÍCIOS DE EMPILHAMENTO DE AR NA MIOPATIA DE BETHLEM: relato de caso

Abstract

Introduction: Bethlem myopathy is characterized by muscle loss and weakness, contractures involving multiple joints and respiratory dysfunction, and is designated as an autosomal dominant myopathy. Goal: To report the intervention of physiotherapy with the selfhealing method and the performance of breathing exercises of air stacking with a patient diagnosed with Bethlem myopathy. Participant and methods: A case study was conducted with a 36-year-old female patient with a medical diagnosis of Bethlem myopathy, with a mutation in the COL-6A2 gene. The motor function was evaluated through the Motor Function Measurement Scale (MFM) and the perimetry of the thigh; the respiratory function through the manuvacuometry (PiMax; PeMax) and peak flow. Motor physical therapy was performed by applying the self-healing method and respiratory physiotherapy with the exercises of air stacking maneuver. Evaluations were performed before (D1) and after eight sessions (D2). Results: The MFM presented a D1 score of 54 points and D2 60 points, and the higher the score, the better the motor function performance. In perimetry there was no loss of muscle mass. In the manuvacuometry the PiMax was in D1 -40 cmH2O and D2 -43 cmH2O, PeMax D1 40 cmH2O and D2 50 cmH2O and peak expiratory flow in D1 270 cmH2O and D2 350 cmH2O. Conclusion: The results indicate that self - healing intervention and air - stacking exercises may be an alternative treatment for patients with Bethlem myopathy.