Amyotrophic lateral sclerosis: Prognostic indicators of survival

Abstract

Amyotrophic lateral sclerosis (ALS) has a fatal outcome in about three years, but survival is known to vary considerably, making it difficult to predict disease duration in individual cases. The aim of this study was to investigate possible early prognostic factors of ALS survival. We included 123 probable or definite cases of ALS, with disease onset between 1989 and 1998, and with a follow-up of at least one year. Survival functions were obtained using both the Kaplan-Meier and the actuarial methods. Subgroups, formed on the basis of gender, area of residence, work, and age at and site of onset, were compared using the logrank test and Cox's proportional hazards method (survival functions), and applying the Grizzle, Starmer, Koch (1969), and Koch, Johnson, Tolley (1972) methods (one-year survival probability trends). The survival curves dipped sharply in the first three years, followed by a flattening trend, with 50% of patients dying within 2.5 years, and 89% over seven years. The clinical form with lower limb onset was associated with longer survival than the upper limb onset and bulbar forms (median survival: 39, 27, and 25 months, respectively). Survival was also affected by age at onset (median survival: 34, 27, and 23 months for onset 75 years, respectively), area of residence (median survival: 24 months in mountainous areas, 32 elsewhere), and type of work (median survival: 25 months in agricultural workers, 33.5 in others). Gender did not influence survival, whereas percutaneous endoscopic gastrostomy placement and invasive ventilation did. The estimation of individual ALS survival is important to allow the patient to plan for his future and to make optimal use of medical and community resources. Although age at and site of onset, area of residence, and agricultural work were found to influence survival, there remains an unexplained heterogeneous progression of the disease, suggesting the influence of other, as yet unknown, prognostic factors. The identification of a definite set of prognostic factors may allow physicians to make more reliable survival predictions at diagnosis.