Sleep and breathing in multiple system atrophy

Curr Treat Options Neurol. 2007 Sep;9(5):347-53.


Sleep disorders are common manifestations in multiple system atrophy (MSA) and include reduced and fragmented sleep, excessive daytime sleepiness, rapid eye movement (REM) sleep behavior disorder (RBD), stridor, and sleep-disordered breathing. RBD and nocturnal stridor are considered red flags and may be the first symptoms of the disease. RBD occurs in 90% to 100% of the patients, indicating severe and widespread pathologic impairment in the brain structures that regulate REM sleep. In 50% of the patients, RBD precedes the onset of waking motor symptoms and autonomic failure by several years. Sleep-disordered breathing manifests as 1) central hypoventilation that reflects impaired automatic control of ventilation secondary to degeneration of the pontomedullary respiratory centers; and, more commonly, as 2) stridor and obstructive sleep apnea due to larynx narrowing secondary to combined vocal cord abductor paralysis and excessive adductor activation during inspiration. Nocturnal stridor is a life-threatening condition in MSA associated with respiratory failure and sudden death during sleep. Long-term treatment with continuous positive airway pressure (CPAP) eliminates nocturnal stridor. Tracheostomy is advised when CPAP is not tolerated and stridor occurs during wakefulness.