title: 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, RL401S, was constructed, designed to be easy to use and interpret. The predictive powers of the two scores were similar.
authors.affiliation: Service de Pharmacologie Clinique, Hôpital de la Pitié-Salpêtrière, Paris, France.
authors.forename: C
authors.initials: C
authors.lastname: Paillisse
authors.forename: L
authors.initials: L
authors.lastname: Lacomblez
authors.forename: M
authors.initials: M
authors.lastname: Dib
authors.forename: G
authors.initials: G
authors.lastname: Bensimon
authors.forename: S
authors.initials: S
authors.lastname: Garcia-Acosta
authors.forename: V
authors.initials: V
authors.lastname: Meininger
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000188:drug therapy* / Q000453:epidemiology / Q000401:mortality*; D015331:Cohort Studies; D003710:Demography; D018691:Excitatory Amino Acid Antagonists / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015233:Models, Statistical; D011379:Prognosis; D012189:Retrospective Studies; D019782:Riluzole / Q000627:therapeutic use*; D013534:Survival; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D018691:Excitatory Amino Acid Antagonists; D019782:Riluzole