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

Amyotroph Lateral Scler Other Motor Neuron Disord. 2005 Mar;6(1):37-44. doi: 10.1080/14660820510027035.


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, RL401S, was constructed, designed to be easy to use and interpret. The predictive powers of the two scores were similar.

Publication types

  • Clinical Trial
  • Comparative Study
  • Randomized Controlled Trial

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / drug therapy*
  • Amyotrophic Lateral Sclerosis / epidemiology
  • Amyotrophic Lateral Sclerosis / mortality*
  • Cohort Studies
  • Demography
  • Excitatory Amino Acid Antagonists / therapeutic use*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Models, Statistical
  • Prognosis
  • Retrospective Studies
  • Riluzole / therapeutic use*
  • Survival
  • Survival Analysis
  • Time Factors
  • Treatment Outcome


  • Excitatory Amino Acid Antagonists
  • Riluzole