Effects of Demographic Factors on Survival Time after a Diagnosis of Amyotrophic Lateral Sclerosis

Abstract

Background: The Agency for Toxic Substances and Disease Registry established surveillance projects to determine the incidence, prevalence, and demographic characteristics of persons with Amyotrophic Lateral Sclerosis (ALS) in defined geographic areas. There is a need to characterize and account for the survival and prognostic factors among a population-based cohort of ALS cases in the United States. Methods: A cohort of incident cases diagnosed from 2009-2011 in New Jersey was followed until death or December 31, 2013, whichever happened first. Survival was assessed using Kaplan-Meier curves and Cox proportional hazards regression was used to identify prognostic factors. Results: Sixty-four percent of incident cases died between 2009 and 2013, 93.7% specifically from ALS. Among the 456 cases studied in the survival analysis, the median survival from diagnosis was 21 months; 46% of cases survived longer than two years from diagnosis. Older age predicted shorter survival. While there is some indication of differences because of sex, race, and ethnicity, these differences were not statistically significant when accounting for age. Conclusions: New Jersey mortality data were queried to determine the vital status of a cohort of incident ALS cases and used to investigate relationships between demographic factors and survival. Results are consistent with other population-based studies. Older age was a strong predictor of shorter survival time. Additional follow-up time is needed to characterize longer-term survival.

Atlanta metropolitan area amyotrophic lateral sclerosis (ALS) surveillance: incidence and prevalence 2009–2011 and survival characteristics through 2015

“…When stratifying the prevalence by race, sex, and ethnicity, the prevalence for males in the Atlanta cohort is significantly higher compared with the prevalence for females, which is similar to existing literature (7,9,10). The survival analysis indicates median survival time for the Atlanta cohort to be 23 months from the time of diagnosis, which is similar to other cohort studies reporting average survival times of 21-26 months (13,14). A study conducted on ancestral origin of ALS cases reveals a median survival time from ALS onset to be highest for those from Central Asia (48 months) and the lowest survival for cases from Northern Europe (25 months) (29).…”

“…The results and findings from the Atlanta metropolitan area are consistent with other similar metropolitan areas participating in the State and Metropolitan Area ALS Surveillance project (11,28,31,32). As in the Atlanta cohort, age was the strongest predictor of survival in other similar metropolitan areas (13)(14)(15)(16).…”

“…However, the age at diagnosis was the only statistically significant factor for survival time, with survival time decreasing as the age of diagnosis increases. Other studies have also concluded that increasing age is a strong predictor of survival time (13,14).…”

“…Those with ALS in the United States are more likely to be older, male, white, and non-Hispanic (6,9,11,12). Median survival time from diagnosis has been reported to be 21-26 months depending on the population studied (13)(14)(15). The strongest predictor of survival time has consistently been age at diagnosis, with older ages having shorter survival time (13)(14)(15)(16).…”

A safety analysis of edaravone (MCI-186) during the first six cycles (24 weeks) of amyotrophic lateral sclerosis (ALS) therapy from the double-blind period in three randomized, placebo-controlled studies

“…Most patients will need assistance with activities of daily living, with subsequent progression leading to respiratory compromise and eventual respiratory failure, which is a leading cause of death in ALS (1). Median survival times are consistently reported as 2-3 years from the diagnosis (or 3-4 years from the first onset of symptom) (2)(3)(4)(5). About 10% of ALS patients live over 10 years (6).…”