Patients with motor neurone disease typically experience relentless motor decline and die within three years of symptom onset from respiratory muscle weakness. There are currently no effective therapies and the discovery of novel therapies is hampered by the lack of a sensitive disease biomarker. Consequently, there is a huge drive to discover novel biomarkers, which can reliably track disease progression over time. These can then be incorporated into clinical drug trials to expedite effective drug discovery. Muscle fasciculations represent the hyperexcitability of diseased motor neurons and are almost universally present from the early stages of MND. We predict that the site, frequency and shape of fasciculations might provide a sensitive measure of disease progression in an individual. We have been conducting a 12-month longitudinal study of 25 patients, performing high-density surface EMG every two months. We have validated an automated technique to process these large data sets. Ultrasound is widely used in clinical medicine to assess anatomical structure in a safe and non-invasive way. Dr Emma Hodson-Tole (Manchester Metropolitan University) and her group have been applying this to the analysis of fasciculations in healthy individuals and patients with MND. This collaborative project will explore combining these two techniques simultaneously in patients with motor neuron disease and control subjects. The goal is to explore the nature of electro-mechanical coupling related to fasciculations and to determine whether any of these properties are pathophysiological. This would complement other studies from our two groups, investigating the natural history and potential utility of fasciculations as a biomarker of motor neuron health in MND.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
OTHER
Masking
NONE
Enrollment
25
High-density surface EMG for fasciculation detection
Ultrasound for fasciculation detection
King's College Hospital NHS Foundation Trust
London, United Kingdom
Correlation between automated detection of fasciculations by two methods (high-density surface EMG and ultrasound)
Time frame: Single time-point
To assess the practical feasibility of using high-density surface EMG and ultrasound simultaneously in biceps and gastrocnemius.
Time frame: Single time-point
To highlight potential biomarkers of disease related to the electromechanical coupling of fasciculations (e.g. latency between electrical and mechanical peaks).
Time frame: Single time-point
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