Neuropsychological Assessment of Children and Adolescents With Turner Syndrome
- Conditions
- Social CognitionTurner SyndromePediatricsIsolated Growth Hormone DeficiencyCognitive Functions
- Interventions
- Behavioral: psychological evaluation
- Registration Number
- NCT03812913
- Lead Sponsor
- University Hospital, Angers
- Brief Summary
Turner syndrome (TS) is a rare chromosomal disorder characterized by partial or complete loss of one of the X chromosomes that affects about one in every 2000 female babies born. These young patients described difficulties making friends, understanding others' emotions and intentions, and controlling their own emotions. Difficulties in these domains could led to social withdrawal, to reduced social skills and could have a significant impact on self esteem and mental health as well as on long-term academic and social functioning in affected individuals. The purpose of this project is to identify functional and dysfunctional cognitive and socio-cognitive abilities in these young patients which could account social difficulties described by some of them and their family. To this end, 35 girls with TS and 35 girls with isolated growth hormone deficiency and normal cerebral MRI will be recruited. Subjects will be 7 to 16 years and 11 months of age. Socio-cognitive and cognitive functions will be assessed with neuropsychological and experimental tasks. Questionnaires completed by patient, parents or teacher, will evaluate social and behavioral functioning.
- Detailed Description
Cross-sectional study (2 groups : Turner syndrome, isolated growth hormone deficiency), not randomized, controlled, associating several specialized centers in endocrine diseases.
The pediatric endocrinologist of the participating centers will contact parents of each patient or voluntary child/adolescent in order to inform them of the study.
The visit of inclusion will take place in the participant center during a routine monitoring visit.
The first visit (V1) will take place immediately after the visit of inclusion in order to avoid a specific displacement for the needs for the study. The second visit (V2) will take place in participant's home. Each visit (V1 and V2) will last half-day. The period between V1 and V2 will be three months maximum.
The visits V1 and V2 will be carried out by neuropsychologists of the Laboratoire de Psychologie des Pays de la Loire (LPPL - EA 4638).
Recruitment & Eligibility
- Status
- RECRUITING
- Sex
- Female
- Target Recruitment
- 70
Not provided
Not provided
Study & Design
- Study Type
- OBSERVATIONAL
- Study Design
- Not specified
- Arm && Interventions
Group Intervention Description isolated GHD psychological evaluation * 35 girls with isolated Growth Hormone Deficiency (GHD). * age : 7 years -16 years and 11 months. * Psychological evaluation of cognition, social cognition and affective cognition Turner Syndrome psychological evaluation * 35 girls with Turner syndrome (except patients with part of a Y chromosome in their karyotype and r(X) cases). * age : 7 years -16 years and 11 months. * Psychological evaluation of cognition, social cognition and affective cognition
- Primary Outcome Measures
Name Time Method Empathy 3 months Interpersonal Reactivity Index (IRI)
Executive abilities-Inhibition 3 3 months non-verbal cancellation task
Cognitive theory of mind second order 3 months second-order theory of mind tasks Perner \& Wimmer, 1983)
Wechsler Intelligence Scale for Children (WISC-V) 3 months intelligence scale.
Cognitive theory of mind first order 3 months First order theory of mind tasks (Baron-Cohen et al., 1985)
Cognitive theory of mind advanced 3 months advanced theory of mind tasks (Happé, 1994)
Cognitive theory of mind : humor 3 months Humor understanding task
Affective theory of mind : faux pas 3 months Faux pas recognition test (Baron Cohen et al., 1999)
Affective theory of mind : first order 3 months First-order affective theory of mind tasks
Affective theory of mind : second order 3 months Second-order affective theory of mind tasks
Affective theory of mind : emotional inference 3 months Emotional inference for context test
Emotional valence recognition : valence 3 months Emotional valence rating from visual scenes
Facial emotion recogniton 3 months Facial emotion recognition task
Vocal emotion recognition 3 months Emotional prosody recognition task
Emotional regulation 3 months Emotion Regulation Questionnaire for Children and Adolescents (ERQ-CA)
Alexithymia 3 months Questionnaire d'alexithymie chez l'enfant (QAE)
Oral Language comprehension abilities 1 3 months Comprehension of Instructions (NEPSY-II, Korkman et al., 2012)
Oral Language comprehension abilities 2 3 months Verbal comprehension index (from WISC-V)
Visual and visual-spatial abilities:1 3 months Arrows subtest (NEPSY-II - Korkman et al., 2012),
Visual and visual-spatial abilities:2 3 months Picture Puzzles subtest (NEPSY-II - Korkman et al., 2012)
Visual and visual-spatial abilities:3 3 months Benton Facial Recognition Test (Benton et al., 1983)
Executive abilities-Verbal Working memory 1 3 months Verbal information updating task
Executive abilities-Visuo-Spatial Working memory 2 3 months Visuo-spatial information updating task
Executive abilities-Inhibition 1 3 months Stroop test.
Executive abilities-Inhibition 2 3 months Tapping test
Executive abilities-Cognitive Flexibility 1 3 months Verbal fluency task (NEPSY-II Korkman et al., 2012)
Executive abilities-Cognitive Flexibility 2 3 months Modified Wisconsin Card Sorting Test for children
Executive abilities-Cognitive Flexibility 3 3 months Trail Making Test
Executive abilities-Higher-order executive functions 1 3 months script generation task
Executive abilities-Higher-order executive functions 2 3 months Rey Complex Figure Test : Copy
Executive abilities in daily life 3 months Behavioral Rating Inventory of Executive Functions
Self Esteem 3 months Rosenberg self-esteem scale (1965).
Depression inventory 3 months Depression: Children's Depression Inventory (Kovacks \& Beck, 1977).
Anxiety inventory 3 months Revised Children's Manifest Anxiety Scale (Reynolds \& Richmond, 1999).
Social desirability 3 months Children's Social Desirability Scale (Crandall et al., 1965)
social adjustment 1 3 months Conners' Parent Rating Scale-Revised: Short Form (Conners, 1997)
social adjustment 2 3 months Conners' Teacher Rating Scale-Revised: Short Form (Conners, 1997)
behavioral adjustment 3 months Child Behavior Checklist (Achenbach, 1991).
- Secondary Outcome Measures
Name Time Method
Trial Locations
- Locations (3)
CHU Rennes
🇫🇷Rennes, Ile Et Vilaine, France
CHU Nantes
🇫🇷Nantes, Loire Atlantique, France
CHU Angers
🇫🇷Angers, Maine Et Loire, France