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Stridor Is an Independent Risk Factor for Mortality and Disease Progression in Patients with Multiple System Atrophy.

Pauline Dodet1,2, Cecile Proust-Lima3,4, Federico Sirna3,4,5,6

  • 1Sorbonne Université, Paris Brain Institute, Inserm, CNRS, Paris, France.

Movement Disorders : Official Journal of the Movement Disorder Society
|October 7, 2025
PubMed
Summary

Stridor in multiple system atrophy (MSA) significantly increases mortality risk and disease progression. However, continuous positive airway pressure (CPAP) therapy shows a substantial survival benefit for these patients.

Keywords:
multiple system atrophysleepstridor

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Area of Science:

  • Neurology
  • Sleep Medicine
  • Respiratory Medicine

Background:

  • Stridor and sleep apnea syndrome (SAS) are common in multiple system atrophy (MSA), but their prognostic impact remains unclear.
  • Previous retrospective studies have reported conflicting findings on the consequences of stridor and SAS on MSA disease course.

Purpose of the Study:

  • To investigate the prognostic significance of stridor and SAS in MSA.
  • To evaluate the potential survival benefits of continuous positive airway pressure (CPAP) therapy in MSA patients with stridor and SAS.

Main Methods:

  • Retrospective analysis of data from 232 MSA patients.
  • Video-polysomnography was used to diagnose stridor and SAS (Apnea-Hypopnea Index > 15).
  • Survival analyses were performed using a joint modeling approach, adjusting for disease progression, age, sex, MSA subtype, and symptom onset duration.

Main Results:

  • Stridor was independently associated with a 2.44-fold increased mortality risk (HR [1.41;4.22]) and more severe disease progression.
  • Sleep apnea syndrome (SAS) alone did not independently predict mortality or disease progression.
  • Continuous positive airway pressure (CPAP) therapy initiation was linked to a significant reduction in mortality risk (HR = 0.40 [0.19;0.85], P = 0.017).

Conclusions:

  • Stridor is a critical prognostic factor in MSA, correlating with higher mortality and worse disease progression.
  • SAS alone is not associated with altered mortality in MSA.
  • CPAP therapy offers a significant survival advantage for MSA patients experiencing stridor.