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

Abstract

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 such as stridor, obstructive sleep apnea, and central sleep apnea is 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 sleep-disordered breathing 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 patients with MSA and sleep-disordered breathing. Treatment modalities are: continuous positive airway pressure, tracheostomy, tracheostomy-invasive ventilation, noninvasive positive pressure ventilation, adaptive servoventilation, vocal cord surgery, botulinum toxin injections, oral appliance therapy, cervical spinal cord stimulation, selective serotonin reuptake inhibitors.

Conclusions: Conflicting results on survival are found for continuous positive airway pressure therapy. Tracheostomy has a proven survival benefit. Most beneficial outcomes are seen with tracheostomy-invasive ventilation. Continuous positive airway pressure, other types of positive airway pressure therapy and tracheostomy can adequately control symptoms of obstructive sleep apnea. However, continuous positive airway pressure may exacerbate central sleep apnea. There was a lack of sufficient data regarding servoventilation or noninvasive positive pressure ventilation. Some patients exhibit a floppy epiglottis and require a different approach. In conclusion, due to the complex characteristics of sleep-disordered breathing in MSA, an individualized and multidisciplinary approach is mandatory.

Citation: Laga A, Bauters F, Hertegonne K, Tomassen P, Santens P, Kastoer C. A strategic approach of the management of sleep-disordered breathing in multiple system atrophy. J Clin Sleep Med. 2025;21(4):703-711.