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| ID | Type | Description | Link |
|---|---|---|---|
| 2017-A00589-44 | Registry Identifier | RCB |
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| Name | Class |
|---|---|
| University College, London | OTHER |
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WIN-HD is a monocentric longitudinal study comparing premanifest Huntingtin (HTT) mutation carriers and non HTT mutation carriers to determine that white-matter atrophy occurs far earlier than clinical onset in HD using Diffusion-weighted Nuclear Magnetic Resonance (N spectroscopy (DWS) and Diffusion Tensor Imaging (DTI).
The investigators will recruit up to 20 premanifest HTT mutation carriers (15 completed) and up to 20 non HTT mutation carriers (15 completed). It is important to have those 2 populations in order to compare our results and determine if there are significant white-matter changes far from the onset of HD. Therefore, non HTT mutation carriers will be age and gender matched to premanifest HTT mutation carriers.
In order to test the hypothesis, the study has 2 visits with a year interval.
This study is based on 4 principal criteria:
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Premanifest HTT mutation carriers | Other |
| |
| non HTT mutation carriers | Other |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Brain imaging | Other | Volume, DWS and DTI |
|
| Measure | Description | Time Frame |
|---|---|---|
| Detection by Diffusion-weighted spectroscopy of abnormal white matter changes prior to the onset of Huntington disease comparing HTT mutation carriers and non HTT mutation carriers over one year | one year |
| Measure | Description | Time Frame |
|---|---|---|
| Detection by Diffusion-weighted spectroscopy of abnormal white matter changes over one year as an intersubject evolution | one year | |
| Detection by Diffusion Tensor Imaging of abnormal white matter changes prior to the onset of Huntington disease comparing HTT mutation carriers and non HTT mutation carriers over one year. |
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Inclusion Criteria:
For presymptomatic individuals:
For controls:
- Genetic test available with CAG repeat length ≤ 36 in HTT gene
Common inclusion criteria for presymptomatic individuals and controls (age-matched and gender-matched with presymptomatic individuals and but without any familial relationship):
Non-Inclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Alexandra DURR, PU-PH | Institut National de la Santé Et de la Recherche Médicale, France | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Brain and Spine Institute | Paris | 750013 | France |
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| ID | Term |
|---|---|
| D006816 | Huntington Disease |
| D049292 | Leukoaraiosis |
| ID | Term |
|---|---|
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
| D002493 | Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
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| ID | Term |
|---|---|
| D059906 | Neuroimaging |
| ID | Term |
|---|---|
| D003952 | Diagnostic Imaging |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
| D003943 | Diagnostic Techniques, Neurological |
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| Neurological assessments | Other | UHDRS |
|
| Psychological assessments | Behavioral | STAI (Spielberger state and Trait Anxiety Inventory) A and B, BDI-II (Beck Depression Inventory), MINI (Mini-International Neuropsychiatric Interview) and MINI-SEA (mini Social cognitive and Emotional Assessment) |
|
| Behavioural assessments | Behavioral | Computerized game |
|
| one year |
| Detection by Diffusion Tensor Imaging white matter changes over one year as an intersubject evolution. | one year |
| Detection of abnormal scores from psychological tests to assess possible early non motor changes and their intersubject evolution over one year. | one year |
| Detection of choice rates and time differences in the behavioral task comparing HTT mutation carriers and non HTT mutation carriers over one year. | one year |
| Detection of time differences in the behavioral task comparing HTT mutation carriers and non HTT mutation carriers over one year. | one year |
| D003704 | Dementia |
| D002819 | Chorea |
| D020820 | Dyskinesias |
| D009069 | Movement Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D003072 | Cognition Disorders |
| D019965 | Neurocognitive Disorders |
| D001523 | Mental Disorders |
| D010335 | Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |
| D008919 | Investigative Techniques |