The utility of ALS staging systems in a multi-ethnic patient cohort

Abstract

Objective: To compare two ALS staging systems, King’s clinical staging and Milano-Torino (MiToS) functional staging, using prospective data from a multi-ethnic cohort of ALS patients. Methods: The stages of disease were determined prospectively based on existing definitions. The two systems were compared for timing of stages using box plots, correspondence using chi-square tests and association using Spearman’s rank correlation. Results: The distribution of stages differed between the two systems. The proportions of disease stages of the King’s staging system were more evenly distributed whereas in MiToS, there was greater weight seen at the later stages of disease. At the early stages, patients moved consecutively in the MiToS staging system but not in the King’s staging system where patients tended to skip stages to reach later stages. Both systems had good correlation (Spearman’s rho = 0.869) and the King’s stage 4 most frequently corresponded to MiToS stage 2. Conclusion: We found the King’s staging was helpful in determining the stages of disease burden, whereas both were helpful in determining the time to functional dependence with MiToS further refining the levels of dependence.

Keywords:

• Amyotrophic lateral sclerosis
• Malaysia
• multi-ethnic population
• Asia
• staging system
• motor neurone disease

Acknowledgement

The authors extend their gratitude to patients for their willingness to participate in our research program.

Declaration of interest

The authors report no conflict of interest. The authors are responsible for the content and writing of this article.

Additional information

Funding

NS receives funding support from the Malaysian Ministry of Education Fundamental Research Grant Scheme [FRGS/1/2018/SKK08/UM/01/1], ALS Association [IF008-2019] and Sydney Southeast Asia Center [IF012-2019].