Respiratory Management of Myotonic Dystrophy

Abstract

Patients with myotonic dystrophy can avoid episodes of respiratory failure by using up to continuous noninvasive ventilatory support (CNVS). Of 154 patients consecutively presenting to a neuromuscular disease clinic, 143 had types 1 or 2 and 11 had congenital myotonic dystrophy. Of the 76 who came multiple times, 41 were prescribed and used at least sleep NVS to relieve symptoms of fatigue and hypersomnolence.