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 < 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.
Zoccolella
Stefano
S
Department of Neurological Sciences, University of Bari, Italy.
Beghi
Ettore
E
Palagano
Guerrino
G
Fraddosio
Angela
A
Guerra
Vito
V
Samarelli
Vito
V
Lepore
Vito
V
Simone
Isabella Laura
IL
Lamberti
Paolo
P
Serlenga
Luigi
L
Logroscino
Giancarlo
G
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