EmoDe Study version 1.0
Research type
Research Study
Full title
A study of emotional function in Duchenne muscular dystrophy
IRAS ID
250508
Contact name
Kate Maresh
Contact email
Sponsor organisation
Joint R&D Office GOSH/UCL GOS ICH
Duration of Study in the UK
1 years, 4 months, 31 days
Research summary
Duchenne muscular dystrophy (DMD) is an inherited muscle disease affecting males. It affects the muscles and heart, but the brain is also involved in about half the people with DMD, including learning, behavioural and emotional problems, such as anxiety. In typical people with anxiety the brain’s ‘emotion system’ can be dysfunctional. The brain structures involved in the emotion system are affected in DMD, therefore we propose that the emotion system is also dysfunctional in boys DMD, and this may be why they are predisposed to anxiety.
We will investigate this using a ‘conditioning’ test, which measures the physical responses in the body, such as the heart rate and changes in skin sweating, when exposed to situations that activate the brain’s emotion system. The test doesn’t cause any distress or lasting effects as it is designed to only elicit subtle physical responses. Typical children with anxiety have greater physical responses in these tests, and we think that this may occur in boys with DMD.
We will be testing boys both with and without DMD aged 7-12 years, to enable comparison between patient and control groups. The tests will be done at Great Ormond Street Hospital for Children, London.
In the past, young men with DMD did not live past their teens or 20s, however with current treatments life-expectancy is improving. Mental health problems can significantly impact the lives of people with DMD, particularly as they become older and need to manage their own healthcare. Understanding the underlying basis of these problems will allow improved diagnosis and intervention. Furthermore, future therapeutic developments may allow treatment of the brain in DMD, as has already been done in the brain in DMD mice. This method could provide an accurate way of assessing efficacy in a clinical trial of DMD brain treatment.
REC name
London - London Bridge Research Ethics Committee
REC reference
18/LO/1575
Date of REC Opinion
9 Oct 2018
REC opinion
Favourable Opinion