Effects of Emotion on Episodic Memory in Typically Developing Children and Children With Williams-Beuren Syndrome

It's well established in the literature that emotional events are more likely to be remembered than neutral events. Although this Emotional Enhancement of Memory (EEM) has been well demonstrated in the adult literature, little is known about EEM for typically developing children and children with Williams-Beuren syndrome (WBS). In this study, two investigations will be conducted concomitantly: one of EEM in typically developing children and the other of EEM in children with WBS. For this purpose, three behavioral experiments will be established to investigate EEM in this two populations, with the first experiment also including electroencephalography measures. Performances of typically developing children will be compared to young adults, and performances of children with WBS will be compared to control children matched for mental age. Finally, this study aims to bring new knowledge about the interactions between emotion and memory in children, and to better understand cognitive functioning in children with WBS.

The main objective of the present project is to characterize the emotional modulation of episodic memory in typically developing (TD) children and children with Williams-Beuren syndrome (WBS), by using behavioral and encephalography (EEG) measures. Better understanding of interaction between cognition and emotion in TD children and in WBS, and their specificities may increase our knowledge of normal and pathological neurodevelopment and lead to new treatment strategies in a case of WBS. Indeed, it is worth to note that WBS have a strong impact on cognitive, emotional and social aspects of children's behavior in daily life. It was widely shown in animal and human studies that emotionally charged information is better memorized and retrieved than neutral one.This emotional enhancement of memory (EEM) was largely demonstrated with young and older adults independently of the nature of stimuli, the type of memory, the type of memory task and the delay between encoding and retrieval . There is general agreement about emotional regulation of memory in adults, although there are several points currently investigated.

Surprisingly, less is known about EEM in TD children and neurodevelopmental disorders such as WBS. Availability of cognitive resources and emotional regulation is supposed to have an impact on EEM. As both present specific patterns in TD children as compared to adults and in children with WBS , it could be expected that emotional regulation of memory process in TD children and WB presents also some specificities. These specificities are largely unknown, moreover if one considers that neither memory function nor emotional processing are fully developed in TD children.

Few studies that investigated EEM in TD children reported rather contradictory results. Some of them observed EEM and others did not.In addition, most studies used only negative stimuli. Even less is known about EEM in children presenting neurodevelopmental pathologies as WBS, although memory deficit for non-emotional stimuli and deficit in processing of facial expressions, especially negative ones were observed in children with WBS. In addition, a significant dissociation was documented in children with WBS, concerning memory deficit, with auditive-verbal memory rather speared and visuo-spatial memory rather impaired .

Thus, there is an important shortcoming in our understanding of emotional modulation of memory in children, both typically developing and those presenting WBS. With the present project, the investigators propose to fill this gap: two investigations will be conducted concomitantly, one of EEM in typically developing children and the other of EEM in children with WBS. Three behavioral experiments will be established to investigate EEM in this two populations, with the first experiment also including electroencephalography measures. These experiments will address the following specific questions: (1) is the EEM dependent of the stimulus type (images versus words)? - Experiment 1; (2) does the EEM exist in visuospatial episodic memory? - Experiment 2; (3) does the EEM exist for content and source memory - Experiment 3. Performance of typically developing children will be compared to young adults, and performance of children with WBS will be compared to control children matched for mental age.

Eligibility Criteria:
Inclusion Criteria:

Children with William-Beuren syndrome

* Genetic diagnosis (CGH-Array or FISH) of microdeletion 7q11.23
* 8 to 18 years old
* Native French speaker
* Psychotropic medication unchanged during one month preceding inclusion

Typically developing children and healthy adults:

* 6 to 30 years old
* Native French speaker
* Absence of history of neurodevelopmental neurological and psychiatric disease

Exclusion Criteria:

Children with William-Beuren syndrome:

* Diagnosis of severe intellectual deficit (Total IQ lower than 40 evaluated with WPPSI-II or WISC-IV)
* Presence of another genetic anomaly
* Presence of vascular; infectious or neurodegenerative disease
* Medication impacting motor and cognitive abilities
* Non-corrected visual and hearing impairment

Typically developing children and Healthy Adults :

* Diagnosis of severe intellectual deficit (Total IQ lower than 70 evaluated with WPPSI-II or WISC-IV)
* Presence of vascular; infectious or neurodegenerative disease knew by parents or legal representative
* Learning disorders
* History of neurological or psychiatric disease
* Medication impacting motor and cognitive abilities, non-corrected visual and hearing impairment