EEG-NIRS, Language, Phonological Short-Term Memory, Williams Syndrome
Conditions
Keywords
Williams syndrome, Language, Phonological Short-Term Memory, EEG-NIRS
Brief summary
Williams syndrome (WS) is a neurodevelopmental disease characterized by mild to moderate intellectual disability and an extremely heterogeneous cognitive profile. Research has outlined two main features of WS: an atypical social behavior associated with surprising language abilities, and a hyperacusis. The purpose of this project is to investigate the relationships between both these abilities, and particularly the role of the hyperacusis on language abilities. The hyperacusis would be crucial in developing language skills: the apparent strength in the verbal domain could rely on Phonological Short Term Memory (PSTM) more in individuals with WS than in typically developing individuals. In addition, the investigators will compare individuals with WS to individuals with Down syndrome (DS): DS is often associated to strong limitations in the PSTM with poor language abilities. To this end, the investigators will use a highly innovative approach including physiological assessments (EEG-NIRS protocol) and questionnaires. The performance of people with WS will be compared to those of participants with DS and TD participants of same DA and cognitive assessments.
Interventions
OTHEREEG-NIRS protocol
Each participant will be tested individually in a quiet room at the GRAMFC (Groupe de Recherches sur l'Analyse Multimodale de la Fonction Cérébrale, INSERM-UMR1105, Université Picardie Jules Verne and CHU Amiens-Picardie). Duration of subject's participation in the study is 120 minutes
OTHERQuestionnaire
Each participant will be tested individually in a quiet room at the GRAMFC (Groupe de Recherches sur l'Analyse Multimodale de la Fonction Cérébrale, INSERM-UMR1105, Université Picardie Jules Verne and CHU Amiens-Picardie). Duration of subject's participation in the study is 120 minutes
Sponsors
Centre Hospitalier Universitaire, Amiens
Study design
Allocation
NA
Intervention model
PARALLEL
Primary purpose
BASIC_SCIENCE
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
6 Years to No maximum
Healthy volunteers
Yes
Inclusion criteria
* For SW and SD: genetic diagnosis and aged between 6- and 17 years old. * For typical participants: any child, adolescent or young adult of the same developmental or chronological age (between 6 and 17 years of age) * adult group (between 20 and 60 years).
Exclusion criteria
* Autistic Associated Disorders, * West syndrome
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| questionnaire score of patients with WS caracterisation | 2 years | questionnaire score for cognitive profile of patients with WS caracterisation |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Impact of hyperacusis on questionnaire score | 2 years | Impact of hyperacusis on questionnaire score |
Countries
France
Contacts
Primary ContactFabrice Wallois, Pr
Outcome results
None listed