Dystonia: boosting the benefits of brain surgery for this serious movement disorder | Children's Charity

Dystonia: boosting the benefits of brain surgery for this serious movement disorder

First published on 21 February 2013

Updated on 3 June 2015

What did the project achieve?

“By exploiting sophisticated brain scans, we’ve shown that it’s possible to gain extra information that could improve the treatment of children with a disabling movement disorder called dystonia,” says Dr Jean-Pierre Lin of Evelina London Children’s Hospital.

Estimates suggest at least 8,000 children and young people in the UK have dystonia.1 They suffer uncontrollable muscle spasms, which can be repetitive and painful, often twisting the body into awkward positions.

“Deep brain stimulation, or DBS, is a revolutionary new way to treat dystonia, which involves surgery to implant electrodes in a very particular area of the brain called the GPi,” says Dr Lin. “We’ve shown that sophisticated MRI scans can reveal different zones of the GPi, which are responsible for different functions. The scans have also enabled us to measure the strength of the connections between the GPi and other parts of the brain.”

“This extra information about the GPi might enable doctors to predict more accurately which children are most likely to benefit from DBS,” continues Dr Lin. “It may also act as a guide for surgeons, enabling them to position electrodes more accurately within the brain, which could improve the outcome of surgery. Our research is continuing in the hope of improving life for children with dystonia in the future.”

References

1. NHS Choices. Dystonia. http://www.nhs.uk/Conditions/Dystonia/Pages/Introduction.aspx Website accessed 17 April 2015.

This research was completed on 3 March 2014

Estimates suggest at least 8,000 children and young people in the UK have dystonia.1 They suffer uncontrollable muscle spasms, which can be repetitive and painful, often twisting the body into awkward postures. A technique called deep brain stimulation (DBS), which involves brain surgery, benefits some children. Dr Jean-Pierre Lin, of Evelina Children’s Hospital, London, is investigating whether a new imaging technique could enable doctors to predict who is likely to benefit from DBS and make treatment more effective for more children.

What is the problem and who does it affect?

“When children have dystonia, their muscles tense up in a way that’s beyond their control,” explains Dr Lin. “Everyday activities such as walking, talking, getting dressed, eating and even just sitting or lying down can be difficult. Sleep is often very fragmented. After a few years, children can develop permanent deformity, with the body becoming literally twisted into awkward, painful shapes.”

“There is no cure for dystonia, but medication sometimes helps, by relaxing muscles, relieving pain and enabling children to sleep,” explains Dr Lin. “Often though, medication doesn’t help very much and even if it does it tends to have unwanted side effects, making children sleepy, causing drooling and interfering with concentration in the classroom.”

“DBS is a revolutionary new way to manage dystonia,” explains Dr Lin. “Some children find DBS dramatically improves life, but the impact of DBS management varies greatly, with others getting much less dramatic benefit.” It’s not possible to predict exactly how each individual child will respond to DBS, especially those with cerebral palsy,2 and the technique involves lengthy brain surgery, with risks of infection and a very small risk of bleeding within the brain, so it can be very difficult for parents, and doctors, to decide whether or not it’s worth trying DBS.

What is the project trying to achieve?

Deep brain stimulation involves surgery to implant small electrodes within the brain. The electrodes are specially positioned to target a very particular area of the brain, the globus pallidus internus (GPi), which is about the size of the fuse in a standard electrical plug and is involved in controlling our movements.

“We are using a new type of MRI scanning technique, which can reveal connections between different parts of the brain,” explains Dr Lin. “We are studying whether these scans enable us to predict how well each child will respond to DBS. This could ensure DBS is offered to the right children and help when deciding whether to go ahead with surgery. We are also investigating whether the new scans could help surgeons to decide how best to position electrodes when they are operating on children with dystonia. We believe this could ensure children get the maximum possible improvement in their symptoms.”

Around 75 children with dystonia, aged four to 16 years are taking part in this study, along with 75 children who do not have the condition.

What are the researchers’ credentials?

Dr Lin and his colleagues are world experts in treating children with dystonia with DBS.3-8 Their groundbreaking use of rechargeable DBS devices in small children, for example, reduces the need for repeat surgery.9 The team has treated children with dystonia from all over the UK and beyond.

Project LeaderDr Jean-Pierre Lin BSc MBCHB MRCP(UK) PhD
Project team
  • Dr Geoffrey Charles-Edwards MPhys MSc PhD MIPEM CSci DipIP
  • Dr Daniel E Lumsden MB/Chir MA Cantab MSc MRCPCH
LocationEvelina Children’s Hospital, Guy's and St Thomas' NHS Foundation Trust, London
Other locations
  • Rayne Institute, King's College London
Duration1 year
Grant awarded21 November 2012
Start date4 March 2013
End date3 March 2014
Grant amount£54,500.00
Grant codeGN2097

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References

  1. NHS Choices. Dystonia. http://www.nhs.uk/conditions/Dystonia/Pages/Introduction.aspx Website accessed 11 March 2013.
  2. Koy A et al. Mov Disord 2013 Feb 13. doi: 10.1002/mds.25339. [Epub ahead of print] PMID: 23408442 [PubMed - as supplied by publisher]
  3. Guy’s and St Thomas’ NHS Foundation Trust. Children’s neuroscience services. Complex paediatric motor disorder assessment and management. http://www.guysandstthomas.nhs.uk/our-services/childrens/services/childr.... Website accessed 24 March 2013.
  4. Gimeno H et al. Eur J Paediatr Neurol 2013; 17:161-8.
  5. Lumsden DE et al. Dev Med Child Neurol 2013 Mar 1. doi: 10.1111/dmcn.12117. [Epub ahead of print]
  6. Lumsden DE et al. Acta Neurochir (Wien). 2013 Feb 21. [Epub ahead of print] PMID: 23430231 [PubMed - as supplied by publisher]
  7. Gimeno H et al. Eur J Paediatr Neurol 2013 Jan 16. doi:pii: S1090-3798(12)00263-2. 10.1016/j.ejpn.2012.12.007. [Epub ahead of print] PMID: 23332134 [PubMed - as supplied by publisher]
  8. Lumsden DE et al. Dev Med Child Neurol 2013 Mar 1. doi: 10.1111/dmcn.12108. [Epub ahead of print] No abstract available. PMID: 23448663 [PubMed - as supplied by publisher]
  9. Kaminska M et al. Stereotact Funct Neurosurg 2012; 90:233-9.
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