Prognostic factors for survival in amyotrophic lateral sclerosis patients treated with riluzole

Abstract

The objective of this study was to identify prognostic factors for survival in amyotrophic lateral sclerosis from a large prospective observational study performed in France. The study included a cohort of 2069 patients fulfilling broad entry criteria treated with riluzole. Over 100 demographic, biological, clinical and quality‐of‐life variables were monitored and assessed for their effect on survival. Patients were randomized post hoc into two groups: one group (two‐thirds of the patients) to generate the prognostic models and one group (one‐third of the patients) to validate the resulting models. Thirteen variables were found to affect survival independently and were used to construct a survival prediction score, RL401. These included age, disease duration, slow vital capacity, intensity of tiredness (visual analogue scale), number of body levels with spasticity, atrophy and/or fasciculations, cough, distal muscle strength, household income, depression and two biological parameters, plasma creatinine levels and neutrophil counts. A simplified score, RL401_S, was constructed, designed to be easy to use and interpret. The predictive powers of the two scores were similar.

• amyotrophic lateral sclerosis
• prognostic factors
• survival analysis