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 over-interpreting these observations and suggest that further research is required to confirm or refute these findings.
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 over-interpreting these observations and suggest that further research is required to confirm or refute these findings.
Authors: Benoît Marin; Philippe Couratier; Simona Arcuti; Massimiliano Copetti; Andrea Fontana; Marie Nicol; Marie Raymondeau; Giancarlo Logroscino; Pierre Marie Preux Journal: J Neurol Date: 2015-10-30 Impact factor: 4.849