Katsuya Nishida, Kento Sakashita, Naonobu Futamura
EN was independently associated with a statistically significant but clinically modest survival advantage, largely by facilitating a comprehensive care pathway including respiratory management. In a small LTS series, the prevention of pneumonia-related death and potential quality-of-life benefits support early structured advance care planning in advanced MSA.
INTRODUCTION: Dysphagia in multiple system atrophy (MSA) leads to pneumonia and malnutrition, impacting survival. This study evaluated the association between enteral nutrition (EN) and survival and described outcomes of laryngotracheal separation (LTS).
METHODS: We retrospectively analyzed 158 patients with probable/definite MSA (2000-2025). Survival from onset was evaluated using Simon-Makuch curves, Mantel-Byar tests with time-dependent EN, and Kaplan-Meier analysis with propensity score matching (PSM) and restricted mean survival time (RMST). Competing risk analyses (Fine-Gray models) assessed the cumulative incidence of death and respiratory interventions (tracheostomy or tracheostomy invasive ventilation [TIV]). Clinical outcomes of LTS (n = 11) were reviewed.
RESULTS: Eighty-seven patients received EN; median survival after EN initiation was 4.3 years. Simon-Makuch analysis showed lower survival in the EN group, reflecting late-stage introduction. After PSM, EN was associated with a modest survival advantage at 7 years (RMST difference 0.68 years, P = 0.005). Significant reduction in sudden death occurred in the EN group (15% vs. 30%, P = 0.001). Competing-risk models showed lower death and higher TIV incidence in the EN group, reflecting proactive respiratory management. In the LTS series, mean survival was 14.5 years with no pneumonia-related deaths and preserved limited oral intake, supporting quality-of-life benefits.
CONCLUSION: EN was independently associated with a statistically significant but clinically modest survival advantage, largely by facilitating a comprehensive care pathway including respiratory management. In a small LTS series, the prevention of pneumonia-related death and potential quality-of-life benefits support early structured advance care planning in advanced MSA.