MRI of motor units in spinal muscular atrophy - version 1.0

• Research type

  Research Study

• Full title

  Magnetic Resonance Imaging (MRI) of motor unit dynamics as a non-invasive disease biomarker in Spinal Muscular Atrophy (SMA).

• IRAS ID

  326920

• Contact name

  Andrew M Blamire

• Contact email

  andrew.blamire@newcastle.ac.uk

• Sponsor organisation

  Newcastle Hospitals NHS Foundation Trust

• Duration of Study in the UK

  1 years, 0 months, 0 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 spontaneous MU activity called fasciculation and changes in the MUs which prevent the muscle from working normally. Therefore, measuring the spontaneous activity 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 more research studies to fully evaluate and understand the potential of MUMRI to aid diagnosis of other motor neuron conditions.

  The research will conduct MUMRI imaging in the legs, back, arms and tongue in patients with SMA. The MUMRI scans will be compared with the standard EMG tests to determine how well MRI performs. Patients will be studied on one occasion with data compared to scans and EMG in healthy volunteers.
  
  The research will help to define whether MUMRI is a useful test to measure disease activity and body distribution in SMA patients.

• REC name

  Wales REC 2

• REC reference

  23/WA/0295

• Date of REC Opinion

  16 Oct 2023

• REC opinion

  Favourable Opinion