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Objectives: To describe survival of 1226 Scottish adults with amyotrophic lateral sclerosis/motor neurone disease (ALS/MND). Methods: Ten year, prospective, population based disease register. Cox time dependent proportional hazards modelling for multivariate survival analyses. Results: Median survival from onset was 25 months (interquartile range 16–34 months). In multivariate models we found an increased hazard with more recently diagnosed cases—that is, there was an unexpected decline in survival over the 10 year period (hazard ratio (HR) 1.06 (95% CI 1.04 to 1.09). Positive effects on survival were demonstrated for longer time from onset to diagnosis (HR 0.38 (95% CI 0.33 to 0.42), assessment by a neurological specialist (HR 0.56 (95% CI 0.40 to 0.77), and treatment with riluzole (HR 0.24 (95% CI 0.14 to 0.42). Poor prognosis was associated with bulbar onset (HR 1.25 (95% CI 1.09 to 1.46) and a mixed lower and upper motor neurone syndrome (HR 1.23 (95% CI 1.01–1.49) and increasing age. Conclusions: We found an unexpected decline in survival over the 10 year period, despite controlling for potential confounding variables. We would be cautious about overinterpreting these observations and suggest that further research is required to confirm or refute these findings.