Serial Fasciculation Measurements in Motor Neurone Disease

Patients with motor neurone disease (MND) 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. The investigators predict that the site, frequency and shape of fasciculations might provide a sensitive measure of disease progression in an individual. In order to calibrate this technique, the investigators will conduct a 12-month longitudinal study, recruiting 24 patients from the King's College Hospital Motor Nerve Clinic, comprising a mixture of patients with MND and those with benign fasciculation syndrome. Patients in this latter group have fasciculations but do not develop weakness and have normal lifespans. They are therefore an optimal control group. At each visit, the investigators will take resting HDSEMG recordings from all four limbs and perform standard clinical measures of disease progression. The investigators will also monitor the decline in motor unit number using a newly validated neurophysiological technique, called Motor Unit Number Index (MUNIX).