It has been suggested that patients with Myotonic Dystrophy type 1 have primary altered
ventilatory response to chemical stimuli and chronic hypoventilation is related not always to
muscle weakness. Also, it is known that Non Invasive Mechanical Ventilation can improve
ventilatory response to chemical stimuli, especially to hypercapnia.
This study evaluates the effect of Non Invasive Mechanical Ventilation on ventilatory
response in patients with Type 1 Myotonic Dystrophy, the ventilatory response to chemical
stimuli will be measured before and after mechanical ventilation in patients with myotonic
dystrophy type 1.