MRI of motor units in motor neuron disease - version 1.0 14 Aug 2019
Research type
Research Study
Full title
MR imaging of motor unit dynamics as a non-invasive disease biomarker in spinal muscular atrophy and amyotrophic lateral sclerosis
IRAS ID
260922
Contact name
Roger Whittaker
Contact email
Duration of Study in the UK
2 years, 2 months, 31 days
Research summary
Patients with spinal muscular atrophy (SMA) suffer muscle wasting and loss of function caused by damage to the motor nerves which connect the brain, via the spinal cord, to the muscles. Each motor nerve connects with many separate fibres within the same muscle which allows the brain to cause the muscle to contract and move. The collections of fibres connected to each nerve are known as motor units (MU). Damage to the motor nerves in SMA causes changes in the MUs and prevents the muscle from working normally. Therefore, measuring the number and size of MUs is an important diagnostic test for some patients with SMA. This assessment is normally made using electromyography (EMG) which uses either electrodes placed on the skin or a needle electrode inserted into the muscle. EMG cannot show where the MU actually are, can be insensitive and needle measurements can be painful. This project will evaluate a new type of magnetic resonance imaging (MRI) scan called “Motor Unit MRI” (MUMRI) which takes pictures of the MUs. Preliminary investigations in patients with motor neuron disease suggest that MUMRI is very sensitive to motor neuron damage, but we need to conduct a detailed study to fully evaluate and understand the potential of MUMRI to aid diagnosis of motor neuron conditions.
The research will conduct MUMRI imaging in the lower limbs in patients with SMA and in patients with amyotrophic lateral sclerosis (ALS). The MUMRI scans will be compared with the standard tests (EMG and clinical function) to determine how well MRI performs. Patients will be studied on two occasions so we can test whether MUMRI is sensitive to disease progression.
The research will define whether MUMRI is a useful test to aid the follow-up of disease progression and monitor responses to clinical interventions in SMA and ALS patients.REC name
North East - Newcastle & North Tyneside 1 Research Ethics Committee
REC reference
19/NE/0338
Date of REC Opinion
10 Nov 2019
REC opinion
Favourable Opinion