Abstract
Objective: This study sought to investigate whether a multistep process was also evident in an Australian amyotrophic lateral sclerosis (ALS) population. Methods: Mortality rates for ALS patients (years 2007–2016) were obtained from the Australian Institute of Health and Welfare (AIHW). The log incidence of ALS, as reflected by crude mortality rates, was regressed against log of age disease onset. Results: From a total population of 24 million, 6524 cases of ALS were identified. A linear relation between the log incidence and log age was identified, with r2 value of 0.99, indicating that ALS is a multistep process. The linear slope estimate was 5.0, suggesting that six steps were required for development of ALS. Conclusions: This study established a linear relationship between log incidence and log age onset in an Australia ALS population, consistent with a multistep process. Identification of these steps will likely be of therapeutic benefit in ALS.
Disclosure statement
Author SV reports receiving honorarium Merck Australia Pty Ltd, Sanofi-Genzyme, CSL.
Author HW has nothing to report.
Author AAC reports financial relationships with Mitsubishi-Tanabe Pharma, Chronos Therapeutics, OrionPharma, Cytokinetics Inc, and Treeway.
Author PT has nothing to report.
Author LV reports grants from ALS Foundation Netherlands, The Netherlands Organization for Health Research and Development (Vici scheme, SOPHIA, STRENGTH, ALS-CarE project; the latter three of which are funded through the EU Joint Programme –Neurodegenerative Disease Research), and personal fees from Shire, Biogen, Cytokinetics, and Treeway.
Author MCK reports being editor in chief of JNNP.