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Ataxia-Telangiectasia: could nerve stimulation help reduce involuntary movements and improve children’s lives?

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Ataxia-telangiectasia (A-T) is a rare genetic condition that affects a child’s nervous system, immune system and other parts of the body. As the condition progresses, children may develop involuntary movements – including muscle spasms, jolts or tremors – which can significantly affect daily activities and quality of life. Professor Rob Dineen at the University of Nottingham is investigating whether a wristwatch-like device that delivers gentle nerve stimulation can help reduce these unwanted movements. If successful, this could ultimately lead to a safe, painless and non-invasive new treatment option that could help improve the everyday lives of teenagers and young people with A-T.

This project is jointly funded by Action Medical Research and Action for A-T.

How are children’s lives affected now?

In children with A-T, nerve cells in part of the brain that controls balance and coordination gradually become damaged and die – causing a range of problems that usually start in early childhood and worsen over time.

“Children may initially appear clumsy or wobbly when sitting, standing and walking. As the condition progresses, they become increasingly unsteady and often need to use a wheelchair by their teenage years,“ says Professor Dineen. “They may also develop involuntary movements – including muscle spasms, sudden jolts or tremors – which can interfere with everyday activities like using a computer, eating and drinking.”

Median nerve stimulation (MNS) is a promising technique being investigated for treating unwanted movements in other conditions. A small, wristwatch-like device delivers tiny electrical pulses to a nerve in the wrist. These signals travel up to the brain, where they may influence the activity of brain circuits involved in controlling movements. 

“This painless, non-invasive technique offers the potential to reduce unwanted movements in children and young people with A-T,” says Professor Dineen.

Our long-term goal is to develop a safe and effective treatment that can help alleviate symptoms and improve the lives of teenagers and young people with A-T.”  

Professor Dineen

How could this research help?

Building on promising results from a pilot study, the team will now test MNS in 20 teenagers and young people with A-T.

“Participants will be filmed while they complete a series of movement tasks – both while they are receiving MNS and when they are not,” says Professor Dineen. “By comparing these recordings, we will find out whether nerve stimulation reduces the number and severity of unwanted movements.”

The researchers will also look for features that could help predict whether an individual will respond to MNS, such as findings from brain scans or tests to measure how well nerves carry electrical signals. They will also gather feedback from participants and their families to help inform the design of a future home-based trial.

If our results are positive, we hope to conduct a larger trial to test the effectiveness of MNS for treating unwanted movements in young adults with A-T during their everyday lives.” 

Professor Dineen.

Research table

Project details

Project Leader Professor Rob Dineen, BMedSci, BMBS, MRCP, FRCR, PhD
Location University of Nottingham
Project Team Dr Caroline Blanchard, MSc PhD
Assistant Professor Alexander Turner, BSc, MSc, PhD, PGCAP
Dr Manish Prasad, MBBS, MRCPCH
Dr Radu Tanasescu, MD PhD FAAN
Dr Clare Bolton, MBBS MRCP PhD
Professor Stephen Jackson, PhD AFHEA
Other Locations Nottingham University Hospitals NHS Trust
Royal Papworth Hospital NHS Foundation Trust, Cambridge
Grant Awarded
Grant Amount £219,916
Duration 30 months
Grant Code (GN number) AT1003

 

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