OBJECTIVE: To explore the demographic data, clinical characteristics, and predictors of survival in patients with Amyotrophic lateral sclerosis (ALS) registered in the ALS Association Mid-America Chapter.
BACKGROUND: ALS is a fatal neurodegenerative disorder that involves upper and lower motor neurons. Several epidemiological studies have been conducted in the United States; however, there is limited data on patients with ALS in the Midwest.
DESIGN/METHODS: Retrospective cohort study of deceased patients with ALS from January 1, 2010, to September 1, 2020, registered with the ALS Association Mid-America Chapter. Demographic data and clinical variables were collected as routine delivery of care and compared to national demographic data from the National ALS Registry. We evaluated differences in the ALS population based on gender, race, military status, site of onset, clinical interventions (gastrostomy, non-invasive ventilation (NIV), or invasive ventilation), and whether seen in an ALS Association-registered clinic. Survival curves with time from diagnosis to death were compared with log-rank test. Multivariate survival analysis was performed with Cox proportional hazard model.
RESULTS: The database included 1447 individuals with ALS. The Midwestern population was older than the National ALS Registry (median age at diagnosis: 67 years) with a higher frequency of women (43.5 %) and bulbar onset disease (29.4 %). Veterans (N=298) had an older age at onset than non-veterans (mean ± standard deviation: 69.0 ± 10.5 vs 63.9 ± 11.5, p<0.001), but no difference in survival. The median survival was 14.0 months (95% confidence interval: 13.2, 14.8). Bulbar onset disease and age at diagnosis older than 65 years predicted shorter survival, and NIV was associated with better survival.
CONCLUSIONS: We present demographic and survival in a large Midwestern population with ALS. Veterans were found to be older at diagnosis, and bulbar onset, older age at diagnosis, and NIV use were independent prognostic factors.