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: 1. Imaging criteria 2. Clinical and neurological criteria 3. Psychological criteria 4. Behavioral criteria
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
OTHER
Masking
NONE
Enrollment
60
Volume, DWS and DTI
UHDRS
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)
Computerized game
Brain and Spine Institute
Paris, France
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
Time frame: one year
Detection by Diffusion-weighted spectroscopy of abnormal white matter changes over one year as an intersubject evolution
Time frame: 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.
Time frame: one year
Detection by Diffusion Tensor Imaging white matter changes over one year as an intersubject evolution.
Time frame: one year
Detection of abnormal scores from psychological tests to assess possible early non motor changes and their intersubject evolution over one year.
Time frame: 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.
Time frame: one year
Detection of time differences in the behavioral task comparing HTT mutation carriers and non HTT mutation carriers over one year.
Time frame: one year
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