Abstract
Objective: To model the effects of demographic change under various scenarios of possible future treatment developments in ALS. Methods: Patients diagnosed with ALS at the King’s College Hospital Motor Nerve Clinic between 2004 and 2017, and living within the London boroughs of Lambeth, Southwark, and Lewisham (LSL), were included as incident cases. We also ascertained incident cases from the Canterbury region over the same period. Future incidence of ALS was estimated by applying the calculated age- and sex-specific incidence rates to the UK population projections from 2020 to 2116. The number of prevalent cases for each future year was estimated based on an established method. Assuming constant incidence, we modelled four possible future prevalence scenarios by altering the median disease duration for varying subsets of the population, to represent the impact of new treatments. Results: The total number of people newly diagnosed with ALS per year in the UK is projected to rise from a baseline of 1415 UK cases in 2010 to 1701 in 2020 and 2635 in 2116. Overall prevalence of ALS was predicted to increase from 8.58 per 100,000 persons in 2020 to 9.67 per 100,000 persons in 2116. Halting disease progression in patients with C9orf72 mutations would yield the greatest impact of the modelled treatment scenarios, increasing prevalence in the year 2066 from a baseline of 9.50 per 100,000 persons to 15.68 per 100,000 persons. Conclusions: Future developments in treatment would combine with the effects of demographic change to result in more people living longer with ALS.
Highlights
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease of motor neurons leading to wasting, paralysis, and eventual death from respiratory failure within 3 to 5 years
Our study pooled data from two regions of southeast England to generate robust incidence rate estimates for ALS in the UK, and used these to predict the effects of future demographic and scientific change on the numbers of people living with ALS in the UK up to the year 2116
Demographic change will almost double the number of people newly diagnosed with ALS each year, from a baseline of 1415 cases in 2010 to 2635 cases in 2116
Summary
Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disease of motor neurons leading to wasting, paralysis, and eventual death from respiratory failure within 3 to 5 years. The annual incidence is approximately 2 per 100,000 person-years in European (1) and US populations (2). These incidence rates have remained relatively stable over time (3). The lifetime risk is as high as 1 in 300 (4) with disease burden increasing with age. Across developed and developing countries, the proportion of people in older age groups is predicted to increase significantly over the coming decades (5), secondary to Supplemental data for this article can be accessed here. Correspondence: Ammar Al-Chalabi, Department of Basic and Clinical Neuroscience, King’s College London, Maurice Wohl Clinical Neuroscience Institute, 5 Cutcombe Road, London, SE5 9RX, UK. E-mail: ammar.al-chalabi@kcl.ac.uk (Received 16 October 2018; revised 13 February 2019; accepted 17 February 2019)
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