Mri As A Tool To Indicate Early Diaphragmatic Weakness In Adult Patients With Pompe Disease

Muscle weakness causing respiratory insufficiency, is one of the typical characteristics in patients with Pompe disease. Early recognition of respiratory muscle weakness is important to allow an early start of treatment. The sensitivity of standard pulmonary function tests to detect diaphragmatic weakness at an early stage is questionable. MRI enables visualization of diaphragmatic motion in detail. The aim of this study was to find early signs of diaphragmatic weakness in Pompe patients.