Long-term survival in amyotrophic lateral sclerosis – data from a population-based registry in Rhineland-Palatinate, Germany

BACKGROUND: Data on long-term survival in amyotrophic lateral sclerosis (ALS) is scarce. In a population-based ALS cohort in Germany we evaluated long-term survival and assessed key factors influencing long-term survival, including demographic and clinical variables that are available at an early stage of the disease. METHODS: Data from patients in the prospective, population-based ALS-registry Rhineland-Palatinate were analyzed. Survival was evaluated separately for time from symptom onset and time from diagnosis. Patient demographic and disease-related variables were analyzed in relation to survival probability using the Kaplan-Meier method and Cox proportional hazards regression. RESULTS: Data from 200 incident ALS patients (106 men and 94 women) were included in the study. The median age of the patients was 68 years (range 23-85 years; mean age 65.8 years; standard deviation 10.5 years). The median survival time was 2.5 years from symptom onset and 1.5 years from diagnosis. 12% of the patients survived for at least ten years from first manifestation of symptoms. Multivariate statistics revealed that younger age, a low progression rate, the absence of frontotemporal dementia and a long interval between symptom onset and diagnosis were predictors of long survival. CONCLUSIONS: 12% of ALS patients are still alive ten years after the onset of symptoms, which is important for advance planning of patient care. In our analysis demographic and clinical variables available at an early stage of the disease have emerged as valuable predictive factors. TRIAL REGISTRATION: ClinicalTrials.gov (NCT01955369; registered retrospectively 28/09/2013).