Stridor Is an Independent Risk Factor for Mortality and Disease Progression in Patients with Multiple System Atrophy
Year: 2025
Type: article
Study design: Observational Study
Abstract: BACKGROUND AND AIM: Stridor and sleep apnea syndrome (SAS) are common in multiple system atrophy (MSA). Retrospective cohort studies have yielded conflicting results regarding the consequences of stridor and SAS on the disease course. This study aimed to assess the prognostic significance of stridor and SAS, as well as the potential survival benefits of continuous positive airway pressure (CPAP) therapy. METHOD: Retrospective data from 232 participants with MSA (mean age 65 years old, 51% male) from the three sites of the French Reference Center for MSA were analyzed. Patients underwent video-polysomnography to confirm the presence of stridor and SAS (AHI >15). Survival analyses adjusted for disease progression, age, sex, MSA subtype, and time since symptom onset were conducted using a joint modeling approach. Disease progression was quantified by the repeated assessments of a six-item composite score and the total Unified MSA Rating Scale I + II. RESULTS: Stridor was independently associated with an increased risk of mortality (hazard ratio [HR] = 2.44 [1.41;4.22]), even after adjusting for disease progression. Stridor was also linked to more severe disease progression over time. In contrast, SAS did not independently predict mortality or disease progression. CPAP therapy initiation was associated with a substantial reduction in mortality risk after adjusting for center and AHI (HR = 0.40 [0.19;0.85], P = 0.017). DISCUSSION: Stridor is a critical prognostic factor in MSA, associated with higher mortality and worse disease progression, whereas SAS alone is not associated with a change in mortality. Patients undergoing CPAP therapy have a much better survival among those with stridor. © 2025 The Author(s). Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
Source: Movement Disorders
DOI: 10.1002/mds.70080
Authors: Pauline Dodet, Cecile Proust‐Lima, Federico Sirna, David Bendetowicz, Giulia Lazzeri, Rachel Debs, Anna Delamarre, Margaux Dunoyer, Margherita Fabbri, Claire Georges, Imad Ghorayeb, David Grabli, Cécile Londner, Anne Pavy‐Le Traon, Maxime Patout, Olivier Rascol, Isabelle Arnulf, Alexandra Foubert‐Samier, Wassilios G. Meissner
Institutions: Centre National de la Recherche Scientifique, Inserm, Sorbonne Université, Pitié-Salpêtrière Hospital, Institut du Cerveau, Université de Bordeaux, Bordeaux Population Health, Centre d'Investigation Clinique Plurithématique de Bordeaux, Centre Hospitalier Universitaire de Bordeaux, Institut des Maladies Neurodégénératives, University of Milan, Hôpital Purpan, Assistance Publique – Hôpitaux de Paris, Centre Hospitalier Universitaire de Toulouse, French Clinical Research Infrastructure Network, Centre d’Investigation Clinique 1436, Université de Toulouse, Institut de Neurosciences Cognitives et Intégratives d’Aquitaine, Neurophysiologie respiratoire expérimentale et clinique, New Zealand Brain Research Institute, University of Otago
Language: English
Subfield: Physiology
Field: Medicine
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $4,480
OpenAlex ID: W4414917855