In a polysomnography study of 32 neuromuscular patients - 22 with a form of muscular dystrophy, 3 with a form of congenital myopathy, 4 with a form of spinal muscular atrophy, 1 with a recurrent form of polymyositis and 1 with osteogenesis imperfecta syndrome - of which 21 were nonambulatory, we observed sleep related respiratory disturbances represented by: drops in oxygen saturation (SaO2), cardiac arrythmia, sleep disruption, apneas, tachypnea, tachycardia and snoring. Nine out of the cohort of 32 patients presented with significant desaturations periods. These patients presented with an associated restrictive syndrome and thoracic deformities, some with tachypnea and/or SaO2 below 90% during wakefulness. In this group, snoring was observed in those patients with a form of muscular dystrophy while tachypnea was observed in patients who presented the highest desaturations levels. Sleep quantification revealed an increase of stage 1 sleep coupled with a decrease or even total absence of REM sleep. This is, we believe, a likely consequence of episodic desaturations that may accompany sleep hypoventilation which is potentialised during REM sleep stage.
polysomnography; neuromuscular diseases; sleep related respiratory disturbances