Amyotrophic lateral sclerosis as a multi-step process: an Australia population study

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.

Keywords:

• Amyotrophic lateral sclerosis
• incidence
• multi-step process

Disclosure statement

1. Author SV reports receiving honorarium Merck Australia Pty Ltd, Sanofi-Genzyme, CSL.

2. Author HW has nothing to report.

3. Author AAC reports financial relationships with Mitsubishi-Tanabe Pharma, Chronos Therapeutics, OrionPharma, Cytokinetics Inc, and Treeway.

4. Author PT has nothing to report.

5. 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.

6. Author MCK reports being editor in chief of JNNP.

Additional information

Funding

This study was supported by research grant from the Motor Neuron Disease Research Institute of Australia, National Health and Medical Research Council of Australia (Project grant numbers 510233, 1024915, 1055778) and is gratefully acknowledged. This work was also supported by funding to Forefront, a collaborative research group dedicated to the study of frontotemporal dementia and motor neuron disease, from the National Health and Medical Research Council of Australia Program Grant (#1037746). This project is supported through the following funding organizations under the egis of JPND – www.jpnd.eu (United Kingdom, Medical Research Council (MR/L501529/1; MR/R024804/1) and Economic and Social Research Council (ES/L008238/1)) and through the Motor Neurone Disease Association. This study represents independent research part funded by the National Institute for Health Research (NIHR) Biomedical Research Centre at South London and Maudsley NHS Foundation Trust and King’s College London.