Multiple System Atrophy Presenting with Stridor (Laryngeal Dysfunction)

Clinical Scenario

This protocol addresses patients with Multiple System Atrophy who develop stridor — a strained, high-pitched, harsh respiratory sound that is mainly inspiratory. The symptom arises from laryngeal dysfunction causing narrowing of the rima glottidis, and may occur during sleep or wakefulness.

        Stridor in MSA reflects upper airway compromise secondary to laryngeal dysfunction. Its presence marks a distinct and clinically significant complication that requires targeted management decisions.
      

Approach to Management

Symptomatic treatment of stridor in this setting involves airway intervention. A surgical airway procedure is among the options considered, and the specific approach chosen — including any adjunct ventilatory support — can have meaningful implications for patient survival. The complete structured regimen, including decision criteria and full management steps, is available in the protocol below.

Instant Access to Structured Evidence-Based Regimens

References

Stridor is a strained, high-pitched, harsh respiratory sound, mainly inspiratory, occurring during sleep or wakefulness, and is caused by laryngeal dysfunction leading to narrowing of the rima glottidis.

Continuous positive airway pressure (CPAP) and tracheostomy are options for symptomatic treatment of stridor. Treatment with tracheostomy may contribute to a longer survival when compared with CPAP or no treatment, and tracheostomy with mechanical ventilation may significantly prolong survival compared with tracheostomy alone.

DOI: 10.1212/cont.0000000000001598

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