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).
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
OTHER
Masking
NONE
Enrollment
25
High-density surface electromyography
King's College Hospital NHS Foundation Trust
London, United Kingdom
Change in fasciculation frequency over time
To characterise the frequency of fasciculations in patients with motor neurone disease and to determine whether these parameters correlate with the trajectory of disease progression over a 12-month period.
Time frame: 12 months
Change in fasciculation morphology over time
Time frame: 12 months
Change in Functional Rating Scale (FRS) over time
Maximum score of 48; lower scores indicate worse disability
Time frame: 12 months
Change in motor unit number index measurements over time
Time frame: 12 months
Change in MRC power sum score over time
Time frame: 12 months
Change in slow vital capacity over time
Time frame: 12 months
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