Unexpected decline in survival from amyotrophic lateral sclerosis/motor neurone disease.
OBJECTIVE
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.
Forbes
R B
RB
Department of Neurology, Ninewells Hospital and Medical School, Dundee, Scotland, UK. Raeburnforbes@aol.com
Colville
S
S
Cran
G W
GW
Swingler
R J
RJ
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000401:mortality* / Q000473:pathology; D015986:Confounding Factors, Epidemiologic; D005260:Female; D006801:Humans; D008017:Life Expectancy; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000401:mortality* / Q000473:pathology; D015999:Multivariate Analysis; D011379:Prognosis; D011446:Prospective Studies; D012606:Scotland / Q000453:epidemiology; D016019:Survival Analysis