Abstract
Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder that causes severe respiratory dysfunction which is the major cause of death. Early management of respiratory symptoms may improve outcomes and survival.
Aim: Describe survival of patients with ALS and respiratory dysfunction and identify predictive factors of survival.
Methods: Retrospective analysis of patients with ALS evaluated in an outpatient setting. Ventilatory support data was screened. Kaplan-Meier survival analysis was performed and predictive factors were evaluated by Cox multivariate regression.
Results: 60 patients (25 females) with a median age of 64.5 years (range 34-80) were analyzed. At presentation, 33 patients (55%) had slow bulbar-onset and 27 (45%) rapid bulbar-onset. Non-invasive ventilation (NIV) was initiated in 52 patients (86.7%), with a mean vital capacity of 1698.0±768.0 L and 22.0±40.8 months after diagnosis. Mean duration of NIV was 19.6±23.7 months. Mechanical assisted cough was used in 23 patients (38.3%). Gastrostomy was performed in 21 patients (17 rapidly bulbar) and tracheostomy in 10 (9 rapidly bulbar) after a mean time of 13.6 ±17.0 months under NIV. The 5-year survival was 48%. The median overall survival and survival after respiratory muscle aids initiation was significantly higher in slowly bulbar patients compared with rapidly bulbar (p=0.03 and p<0.01, respectively). In multivariate analysis, predictive factors of survival were younger age, slow bulbar-onset, and early NIV initiation.
Conclusion: Survival may be prolonged in patients with ALS and respiratory dysfunction with early NIV initiation. Age and bulbar onset have significant negative impact on survival.
- © 2012 ERS