Respiratory and sleep-related complications of multiple system atrophy

The purpose of this article is to provide a contemporary review of sleep issues affecting patients with multiple system atrophy (MSA).

Prodromal symptoms of MSA may occur years prior to diagnosis, including autonomic dysfunction such as orthostatic hypotension, urogenital dysfunction, rapid eye movement (REM) sleep behavior disorder (RBD), and stridor. Patients may also develop sleep-related respiratory disorders such as obstructive sleep apnea (OSA), central sleep apnea (CSA), and stridor. The development of stridor is associated with a shortened lifespan and sudden death, which may be further accelerated by autonomic instability. MSA appears to follow a 'prion-like' disease progression.

MSA is a rapidly progressive neurodegenerative disease characterized by a combination of autonomic failure and motor symptoms. MSA is often misdiagnosed as the initial presentation mimics other neurodegenerative disorders. There are diagnostic criteria to identify possible, probable, and definite MSA. Prodromal symptoms may occur years prior to diagnosis, including autonomic dysfunction such as orthostatic hypotension, urogenital dysfunction, REM RBD, and stridor. In previous years, treatment consisted of tracheostomy but did not address the component of CSA, which commonly coexisted or developed later because of destruction of medullary chemoreceptors. Positive airway pressure may be as effective as tracheostomy alone in ameliorating obstruction at the vocal cord level.