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

Amyotrophic Lateral Sclerosis (ALS) also known as Motor Neurone Disease (MND) is a progressive and ultimately fatal neurodegenerative disease for which there is no cure. People with ALS experience loss of mobility and arm function, breathlessness and chest infections, loss of speech and swallow and in 30-50%, cognitive and behavioural change. Progression of the disease is measured by the ALS Functional Rating Scale-Revised (ALSFRS-R). This scale asks people to rate how they are managing on a scale of 0-4 in speech, swallowing, using their hands, walking and breathing. It is used in clinical trials to measure whether drugs are effective and in clinical practice to measure the rate of decline. However, we know that this scale is not very sensitive to what matters to people in their daily lives, and because of this, it is hard to assess the meaningfulness of some of the changes recorded. This insensitivity of the scale makes it more difficult to bring new drugs to market, as health care providers have no way to relate changes in the ALSFRS-R to meaningful real-life changes for patients (?clinical meaningfulness?). There is an urgent need to provide a new way to translate the ALSFRS-R into measures which show clinical meaningfulness. Technological advances have increased the availability of new sensors and devices that can now be harnessed to address this unmet need. This project will develop a toolkit of sensors and devices to monitor the changes in function experienced in ALS in a sensitive, novel, non-invasive and remote manner. These will be validated, evaluated and incorporated into our multidisciplinary clinic. The validated devices will be suitable for use in future clinical trials to improve the sensitivity to detect clinically meaningful change.

Award Date
28 June 2018
Award Value
Principal Investigator
Dr Deirdre Murray
Host Institution
Trinity College Dublin
MRCG-HRB Joint Funding Scheme