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The objective of the study was to develop and validate a practical prognostic index for patients with amyotrophic lateral scleroses (ALS) using information available at the first clinical consultation. We interrogated datasets generated from two population-based projects (based in the Republic of Ireland and Italy). The Irish patient cohort was divided into Training and Test sub-cohorts. Kaplan–Meier methods and Cox proportional hazards regression were used to identify significant predictors of prognoses in the Training set. Using a weighted grading system, a prognostic index was derived that separated three risk groups. The validity of index was tested in the Irish Test sub-cohort and externally confirmed in the Italian replication cohort. In the Training sub-cohort (n = 117), significant predictors of prognoses were site of disease onset (HR = 1.7, p = 0.012); ALSFRS-R slope prior to first evaluation (HR = 2.8, p < …