title: Amyotrophic Lateral Sclerosis Survival Score (ALS-SS): A simple scoring system for early prediction of patient survival.
abstract: Our objectives were: (1) to identify independent prognostic factors to determine a survival score for amyotrophic lateral sclerosis (ALS) in a cohort of patients followed in the NEMO Centre (NEuroMuscular Omnicentre); (2) to replicate results in an independent cohort obtained from the Pooled Resource Open Access ALS Clinical Trial Consortium (PRO-ACT) database. Samples were collected from 428 ALS patients from the NEMO database and 2481 patients from the PRO-ACT database. Study design was a retrospective analysis with clinical and biochemical variables, using univariable and multivariable Cox models of analysis. Results showed that, in multivariable analysis, age at diagnosis, diagnostic delay, ALSFRS-R total score, Body Mass Index, aspartate aminotransferase and creatinine level were independently related to survival. These factors were recoded as categorical variables assigning a score from 5 to 15, and the sums of these scores were used to obtain the ALS-Survival Score (ALS-SS). This then allowed to identify three groups having different survival curves. The ALS-SS results were also replicated using data from the PRO-ACT database. In conclusion, considering independent prognostic factors, we were able to give an estimate of survival in our cohort of ALS patients. Whether this ALS-SS may be useful in clinical practice, and potentially in clinical trials, will have to be determined prospectively.
authors.affiliation: a NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Niguarda Ca' Granda Hospital , Milan.
authors.forename: Christian
authors.initials: C
authors.lastname: Lunetta
authors.affiliation: a NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Niguarda Ca' Granda Hospital , Milan.
authors.forename: Andrea
authors.initials: A
authors.lastname: Lizio
authors.affiliation: a NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Niguarda Ca' Granda Hospital , Milan.
authors.forename: Mario Giovanni
authors.initials: MG
authors.lastname: Melazzini
authors.affiliation: a NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Niguarda Ca' Granda Hospital , Milan.
authors.forename: Eleonora
authors.initials: E
authors.lastname: Maestri
authors.affiliation: a NEuroMuscular Omnicentre (NEMO), Fondazione Serena Onlus, Niguarda Ca' Granda Hospital , Milan.|b Department of Biomedical Sciences for Health , University of Milan , Milan , Italy.
authors.forename: Valeria A
authors.initials: VA
authors.lastname: Sansone
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis* / Q000401:mortality*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D011379:Prognosis; D016016:Proportional Hazards Models; D015203:Reproducibility of Results; D018570:Risk Assessment / Q000379:methods*; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index; D016019:Survival Analysis; D055815:Young Adult
keywords: Amyotrophic lateral sclerosis; creatinine; prognosis; survival