Amyotrophic Lateral Sclerosis Survival Score (ALS-SS): A simple scoring system for early prediction of patient survival. Issue 1 (17th February 2016)

• Record Type: Journal Article
• Authors: Lunetta, Christian; Lizio, Andrea; Melazzini, Mario Giovanni; Maestri, Eleonora; Sansone, Valeria A.
• Is Part Of: Amyotrophic lateral sclerosis and frontotemporal degeneration. Volume 17:Issue 1/2(2016)
• Page Start: 93
• Page End: 100
• Publication Date: 2016-02-17
• Subjects: Amyotrophic lateral sclerosis -- survival -- prognosis -- creatinine; 616.839
• DOI: 10.3109/21678421.2015.1083585
• Languages: English
• ISSNs: 2167-8421

Abstract

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.