Objective: To investigate prognostic factors associated with survival in patients with multiple system atrophy (MSA), with particular focus on surgical interventions.

Background: MSA is a progressive neurodegenerative disorder with poor prognosis. Dysphagia and respiratory complications emerge during disease progression, and surgical interventions such as gastrostomy and tracheostomy are sometimes performed. However, how these interventions relate to survival remains uncertain.

Method: We retrospectively analyzed patients with MSA who had symptom onset between January 2005 and December 2018 and were followed at our institution. Survival time was defined as the interval from symptom onset to death or last follow-up (censoring date: March 31, 2025). Cox proportional hazards regression analysis was used to identify factors associated with mortality. The variables examined were age at onset, sex, MSA subtype, levodopa responsiveness, early clinical features (falls within 3 years of onset, orthostatic hypotension within 3 years of onset, and urinary catheterization within 5 years of onset), REM sleep behavior disorder, stridor, gastrostomy, and tracheostomy. Kaplan–Meier survival curves were generated for significant variables and compared using the log-rank test.

Results: A total of 202 patients were included (mean age at onset, 60.8 ± 9.1 years; 74 men). In multivariable Cox regression analysis, orthostatic hypotension within 3 years of onset was independently associated with higher mortality (HR 2.46, p = 0.01), whereas tracheostomy was associated with lower mortality (HR 0.10, p < 0.001). Gastrostomy was not significantly associated with mortality (HR 0.53, p = 0.10). Kaplan–Meier survival curves showed significantly longer survival in patients who underwent tracheostomy than in those who did not (estimated mean survival time, 212 vs 120 months; log-rank p < 0.001). Patients with orthostatic hypotension within 3 years of onset had significantly shorter survival than those without it (129 vs 153 months; log-rank p = 0.02).

Conclusion: In this cohort, early orthostatic hypotension was associated with poorer survival in MSA. Tracheostomy was associated with longer observed survival, whereas gastrostomy was not independently associated with mortality. However, the association with tracheostomy should be interpreted cautiously because this study does not establish a causal survival benefit.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/prognostic-factors-for-survival-in-multiple-system-atrophy-a-retrospective-cohort-study-with-focus-on-surgical-interventions/