Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Background: Care for people with amyotrophic lateral sclerosis (ALS) has altered at King’s College Hospital over the last 20 years. The clinic has been a multidisciplinary, specialist, tertiary referral centre since 1995 with a large team with integrated palliative and respiratory care since 2006. We hypothesised that these changes would improve survival. Methods: In this retrospective observational study, patients diagnosed with El Escorial definite, probable and possible ALS between 1995– 1998 and 2008–2011 were followed up. The primary outcome measure was a chi-square test for the proportion of each cohort surviving. Kaplan-Meier survival analysis and Cox multivariate regression were secondary analyses. Results: There was low reporting of some interventions. Five hundred and forty-seven people were included. Survival between the cohorts was significantly different (p1⁄4 0.022) with a higher proportion surviving during 2008–2011. Survival time was 21.6 (95% CI 19.2–24.0) months in the 2008–2011 cohort compared to 19.2 years (15.6–21.6) in the 1995–1998 cohort (log rank p1⁄4 0.018). Four hundred and ninety-three cases were included in the Cox regression. Diagnostic cohort was a significant predictor variable (HR 0.79 (0.64–0.97) p1⁄4 0.023). Conclusions: These results support the hypothesis that integrated specialist clinics with multidisciplinary input improve survival in ALS.