Not provided
Not provided
| ID | Type | Description | Link |
|---|---|---|---|
| 302390 | Other Identifier | IRAS |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Sarepta Therapeutics, Inc. | INDUSTRY |
Not provided
Not provided
Not provided
Study aims to develop and to evaluate the neurophysiological and physiological response to a classical conditioning task.To better understand how Duchenne Muscular Dystrophy (DMD) and Becker Muscular Dystrophy (BMD) impacts mental health and how to assess it. Participants invited to complete questionnaires about behaviour, cognitive function and social interactions, complete computer tasks and have an optional MRI brain scan,
The investigation aims to develop and to evaluate the neurophysiological and physiological response to a classical conditioning task, which is comparable to findings that have been made in the mdx dystrophic mouse (deficient in Dp427). The investigators will assess correlations between the specific DMD/BMD genotype and susceptibility to conditioning, as well as the relationship between conditioning and behavioural/emotional characteristics of the syndrome. At the end of the study, the objective is to deliver a comprehensive test battery that is suitable for use in a trial of AON delivery to improve brain function.
Not provided
Not provided
Not provided
Not provided
Not provided
| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Cohort |
|
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Classical conditioning task | Behavioral | To evaluate the neurophysiological and physiological response to a classical conditioning task, which is comparable to findings that have been made in the mdx dystrophic mouse (deficient in Dp427). The investigators will assess correlations between the specific DMD/BMD genotype and susceptibility to conditioning, as well as the relationship between conditioning and behavioural/emotional characteristics of the syndrome. |
| Measure | Description | Time Frame |
|---|---|---|
| Group differences between DMD, BMD and controls in the initial aversive unconditioned stimulus. | Following an emotional response task, an interim analysis will be done after the first 30 patients have been tested (10 DMD, 10 BMD, 10 controls). A favourable outcome will demonstrate a difference in the emotional response of these groups. Groups will complete questionnaires, an emotional response task, and a fine motor assessment. | through study completion, an average of 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| To observe any difference between and within BMD, DMD and control groups in regard to learning, habituation and extinction | This will be measured by analysis measuring any difference between and within groups (10 BMD, 10 DMD and 10 control). Physiological responses generated by the task will be measured, along with neural imaging. | through study completion, an average of 2 years |
Not provided
Inclusion Criteria:
DMD patients:
BMD patients:
Control participants:
Exclusion Criteria:
DMD & BMD patients:
Control participants:
General exclusion criteria for MRI:
Male
40 DMD, 30 BMD patients and 30 age-matched controls.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Anna Kolesnik, Dr | Contact | 44 (0) 20 7905 2600 | braindmd@ucl.ac.uk | |
| Natasha Aslam, MSc | Contact | 44 (0) 20 7905 2600 | natasha.aslam@ucl.ac.uk |
| Name | Affiliation | Role |
|---|---|---|
| Francesco Muntoni, Professor | University College, London | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| UCL GOS Institute of Child Health | Recruiting | London | WC1N 1EH | United Kingdom |
Not provided
| Label | URL |
|---|---|
| Duchenne clinical trial listing | View source |
| Health Research Authority research summary | View source |
Not provided
The results of the study will be published in peer-reviewed scientific journal(s), presented at relevant national and international meetings, and reported as part of submissions to regulatory and funding bodies (Joint Research & Development Office, GOSH & ICH; NHS Research Ethics Committee; GOSH Charity). Participants will be asked if they wish to be contacted when the results are published, and if in agreement they will be informed by letter/email of any resulting publications.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
|