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Incorporation of sensor technology to provide clinical meaningfulness for existing standardised measurement scales in Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) (Motor Neurone Disease (MND)) is a rapidly progressive and ultimately fatal neurodegenerative condition characterized by decline in limb, respiratory, speech and swallow function, and in 30-50% cognitive and behavioral impairment. Progression is measured using the ALS Functional Rating Scale-Revised (ALSFRS-R). The ALSFRS-R has 3 defined subscales; spinal, bulbar and respiratory. This is a semi-quantitative ordinal scale with 12 items, each scored from zero to four. The slope of the ALSFRS-R score over time is considered linear and is used as a primary outcome measure in clinical trials of therapeutic interventions. However, analysis of the subscales within the ALSFRS-R demonstrates heterogeneity across patients and important floor and ceiling effects, which limits interpretation of clinical meaningfulness and increases the risk of failure to identify a real effect of a new intervention. We aim to apply novel technological solutions to the limitations of this scale.
This project will address the sensitivity of the ALSFRS-R subscales using custom developed sensor technology in collaboration with biomechanical and chemical engineers from Trinity College Dublin. The views of stakeholders, including patients, regarding the use of digital measurement and monitoring will be incorporated into the study. A series of prototype sensors will be designed incorporating input from stakeholders. The sensors will be used to quantitate ALS associated neurological impairment in a non-invasive and reproducible manner. The psychometric properties of these prototypes will be evaluated, including validity, reliability, interpretability, responsiveness and clinical meaningfulness in a cohort of ALS patients. Patient and healthcare professional interviews will be conducted to evaluate critically meaningful functional thresholds for neurological function and mobility. The sensors will be refined and re-evaluated based on findings, and steps for commercialisation will be initiated. On completion, this study will enhance quantitative assessment in clinical trials, facilitate telemedicine approaches to care and reduce the requirement for attendance at specialist clinics for monitoring.