Predictors of long survival in amyotrophic lateral sclerosis: A population-based study

Background: Although amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder, some ALS cases can survive beyond 10 years. However, the predictors of long survival in ALS patients remain uncertain. Objective: To define clinical predictors of long survival in a cohort of ALS incident cases. Methods: One hundred-thirty incidents cases, diagnosed in 1998-1999 and classified according to the El Escorial criteria (EEC), were enrolled from a prospective population-based registry established in Puglia, Italy. All but two cases were followed-up until death or November 30, 2006. Results: Thirteen patients (high 10% of the survivors) were classified as long survivors (LS), 13 as short survivors (SS) (low 10%), and 102 as average survivors (AS). LS presented a lower frequency of bulbar onset (8% versus 29% of AS and 39% of SS; p = 0.1) and a significantly longer time between symptom onset to diagnosis [(ODI): 13 months versus 10 and 6; p = 0.0005]. In multivariate analysis, predictors of long survival were younger age at diagnosis (>65 compared to <= 45 years: odds ratio (OR): 18.9; 95%CI: 1.8-194.7; p = 0.04), longer interval onset-diagnosis (:5 9 months compared to > 9 months, OR: 7.9; 95%CI: 1.3-47; p = 0.02) and clinical features with predominant upper motor neuron signs (OR: 8.5; 95%CI: 1.1-64.2; p = 0.04). Conclusions: In this population-based study, younger age, longer interval onset to diagnosis, and clinical features with predominance of upper motor signs predicted long survival, while EEC category at diagnosis did not. (C) 2007 Elsevier B.V. All rights reserved.