Trends in survival of ALS from a population-based registry

Abstract

Objective: To assess survival of ALS patients in general and in selected demographic and clinical subgroups comparing two periods (1998–2000 vs. 2008–2010). Methods: Newly diagnosed adults resident of Lombardy, Northern Italy from a population-based registry were included. Data were collected on age at diagnosis, sex, site of onset, diagnostic delay, and El-Escorial diagnostic category. Patients were followed until death or last observation. Survival was evaluated using Kaplan–Meier curves and Cox’s proportional hazards models. Results: In 2008–2010 (267 patients), median survival was 2.4 years and 1-year, 2-year, 3-year and 5-year survival rates were 79%, 56%, 41% and 24%. Longer survival was associated with male sex, younger age, spinal onset, and longer diagnostic delay. Multivariable analysis confirmed higher death in 65–69yr (HR 2.8; 95% CI 1.4-5.6), 70–74yr (HR 3.2; 95% CI 1.6-6.3) and 75 + yr (HR 6.9; 95% CI 3.5-13.8) categories, compared to ≤49yr, in females (HR 1.4; 95% CI 1.02-1.8), compared to males, and in patients diagnosed after 6–12 months (HR 1.9; 95% CI 1.4-2.7), compared with longer diagnostic delay. In 1998–2000 (235 patients), median survival was 2.2 years. The 1-year, 2-year, 3-year and 5-year survival rates were 77%, 53%, 38% and 20%. When adjusting for demographic and clinical variables, the HR for death in 2008–2010 versus 1998–2000 was 0.80 (95% CI 0.66–0.98). A significant increase of survival in 2008–2010 was found only in patients aged 50–59yr and 70–74yr at diagnosis. Conclusions: Survival of ALS has increased over time in the last decades, especially in middle aged and elderly patients. The benefits of comprehensive care in selected age groups might explain our findings.

Keywords:

• Survival
• epidemiology
• amyotrophic lateral sclerosis
• population-based
• registry

Acknowledgements

The authors are grateful to all municipal registry officers of Lombardy for the collaboration to data collection activities. Susanna Franceschi is also acknowledged for typing and helping with the submission of the manuscript.

Ethical approval

The study was conducted ethically in accordance with the World Medical Association Declaration of Helsinki. The study was undertaken in respect of the General Data Protection Regulation (EU) 2016/679 (GDPR) after approval by the ethics committee of the Mario Negri Institute.

Declaration of interest

Dr. Beghi reports grants from Italian Ministry of Health, grants from SOBI, personal fees from Arvelle Therapeutics, grants from American ALS Association, outside the submitted work.

All the other authors have nothing to declare.

Data availability statement

Anonymized data can be shared by request from qualified investigators upon approval of the local investigators.

Additional information

Funding

The study was supported by the Istituto di Ricerche Farmacologiche Mario Negri IRCCS.