A strategic approach of the management of sleep-disordered breathing in multiple system atrophy.

Study objectives: Multiple system atrophy (MSA) is a rare neurodegenerative disorder characterized by autonomic dysfunction associated with a combination of cerebellar, parkinsonian, or pyramidal signs. Sleep-disordered breathing (SDB) such as stridor, obstructive sleep apnea (OSA) and central sleep apnea (CSA) are common in MSA and can impact survival. Several studies have evaluated treatment modalities. However, the optimal strategy often remains unclear in these patients. This review aims to provide an overview of the current evidence on treatment of SDB in MSA.

Methods: Systematic review of the current literature through combined keyword search in PubMed, Embase, the Cochrane Library and cited references: multiple system atrophy, stridor, sleep apnea syndrome, sleep-disordered breathing, Shy Drager syndrome.

Results: Twenty-nine papers were included, with a total of 681 MSA patients with SDB. Treatment modalities are: continuous positive airway pressure (CPAP); tracheostomy; tracheostomy invasive ventilation (TIV); non-invasive positive pressure ventilation (NPPV); adaptive servoventilation (ASV); vocal cord surgery; botulinum toxin injections; oral appliance therapy; cervical spinal cord stimulation; selective serotonin reuptake inhibitors (SSRIs).

Conclusions: Conflicting results on survival are found for CPAP therapy. Tracheostomy has a proven survival benefit. Most beneficial outcomes are seen with TIV. CPAP, other types of PAP and tracheostomy can adequately control symptoms of OSA. However, CPAP may exacerbate central sleep apnea. There was a lack of sufficient data regarding ASV or NPPV. Some patients exhibit a floppy epiglottis and require a different approach. In conclusion, due to the complex characteristics of SDB in MSA, an individualized and multidisciplinary approach is mandatory.