Functional Imaging of Social Cognition in Premanifest Huntington's Disease

Study Overview
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Brief Summary
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Huntington's disease (HD) is a genetic progressive fatal neurodegenerative disorder. In the western world it affects 5-10 persons per 100000. The main brain changes include the loss of brain cells in subcortical structures. The symptoms of HD include involuntary movements, cognitive deterioration and behavioural disturbances. It has been shown that changes in emotion comprehension occur before the onset of the motor symptoms (preHD). This deficit in perception of emotions has been primarily investigated by means of facial expressions. However, emotions can also be expressed through body language. Here, the investigators propose to investigate whether the emotion comprehension deficit in preHD also includes body language comprehension.

Detailed Description
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The investigators will perform a detailed evaluation of body language comprehension abilities in persons that carry the genetic mutation causing HD, but who do not show any movement symptoms yet. In addition, the associated changes in brain structure and function will be studied. The aim is to perform the study with the same group of participants who participated in a recent study carried on by this research group, in which the functional brain changes associated with increased irritability were investigated. This will allow to relate emotion experience with emotion perception. The results of the study may provide a target to further develop therapeutic interventions and aid caregivers of preHD subjects.

Official Title
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Functional Imaging of Social Cognition in Premanifest Huntington's Disease

Conditions
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Huntington's Disease

Intervention / Treatment
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* Other: functional Imaging


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Huntington's disease positive genotype and non-carrier siblings Exclusion Criteria: visible chorea MRI contra-indications major neurological/psychiatric comorbidity

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 90 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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Accepts Healthy Volunteers

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Basic Science
Allocation: Non-Randomized
Intervention Model: Crossover Assignment
Masking: Single


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: functional Imaging mutation carrier<br>Premanifest mutation carrier, behavioral and neural emotional memory processing | Other: functional Imaging<br>* Brain responses associated with emotional memory will be investigated<br>|
| Active Comparator: functional Imaging non-carrier<br>non-carrier family member, behavioral and neural emotional memory processing | Other: functional Imaging<br>* Brain responses associated with emotional memory will be investigated<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Significant group differences in brain activation for perceiving emotional vs neutral faces by following random effects analyses | Significant group differences in beta-values for the contrast emotional faces vs neutral faces following general linear model parameter estimates | 6 months |