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A standard operating procedure for King’s ALS clinical staging

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journal contribution
posted on 18.02.2019, 11:15 by Rubika Balendra, Ahmad Al Khleifat, Ton Fang, Ammar Al-Chalabi

Objective: Clinical stages in amyotrophic lateral sclerosis (ALS) can be measured using a simple system based on the number of CNS regions involved and requirement for gastrostomy or noninvasive ventilation (NIV). We aimed to design a standard operating procedure (SOP) to define the standardized use and application of the King’s staging system. Methods: We designed a SOP for the King’s staging system. We wrote case vignettes representative of ALS patients at different disease stages. During two workshops, we taught health care professionals how to use the SOP, then asked them to stage the vignettes using the SOP. We measured the extent to which SOP staging corresponded with correct clinical stage. Results: The reliability of staging using the SOP was excellent, with a Spearman’s Rank coefficient of 0.95 (p < 0.001), and was high for different groups of health care professionals, and for those with different levels of experience in ALS. The limits of agreement between SOP staging and actual clinical stage lie within a single stage, confirming that there is a clinically acceptable level of agreement between staging using the SOP and actual King’s clinical stage. There were also no systematic biases of the SOP over the range of stages, either for over-staging or under-staging. Conclusions: We have demonstrated that the staging SOP provides a reliable method of calculating clinical stages in ALS patients and can be used prospectively by a range of health care professionals with different levels of experience, as for example may be the case in multicentre clinical trials.


RB is a UCL Leonard Wolfson Clinical Research Training Fellow and is funded by a Wellcome Trust Research Training Fellowship (107196/Z/14/Z). AAC receives salary support from the National Institute for Health Research (NIHR) Maudsley Biomedical Research Centre at South London and Maudsley NHS Foundation Trust and King’s College London. This is work from three EU Joint Programme—Neurodegenerative Disease Research (JPND) projects (STRENGTH, ALS-CarE, and BRAIN-MEND). The 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. The work leading up to this publication was funded by the National Institute for Health Research (NIHR) under its Programme Grants for Applied Research (Grant Reference Number RP-PG-1016-20006) and by the European Community’s Horizon 2020 Programme (H2020-PHC-2014-two-stage; grant agreement number 633413). This was supported by grants from H2020 European Institute of Innovation and Technology.