title: Predictors of long survival in amyotrophic lateral sclerosis: a population-based study.
abstract: 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 < or =45 years: odds ratio (OR):18.9; 95%CI: 1.8-194.7; p=0.04), longer interval onset-diagnosis (< or =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.
authors.affiliation: Department of Neurological Sciences, University of Bari, Italy.
authors.forename: Stefano
authors.initials: S
authors.lastname: Zoccolella
authors.forename: Ettore
authors.initials: E
authors.lastname: Beghi
authors.forename: Guerrino
authors.initials: G
authors.lastname: Palagano
authors.forename: Angela
authors.initials: A
authors.lastname: Fraddosio
authors.forename: Vito
authors.initials: V
authors.lastname: Guerra
authors.forename: Vito
authors.initials: V
authors.lastname: Samarelli
authors.forename: Vito
authors.initials: V
authors.lastname: Lepore
authors.forename: Isabella Laura
authors.initials: IL
authors.lastname: Simone
authors.forename: Paolo
authors.initials: P
authors.lastname: Lamberti
authors.forename: Luigi
authors.initials: L
authors.lastname: Serlenga
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Logroscino
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology* / Q000401:mortality*; D018672:Community Health Planning; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D016019:Survival Analysis