INS NYC 2024 Program

Symposium	Symposia 2 Program Schedule 02/15/2024 09:00 am - 10:30 am Room: West Side Ballroom - Salon 2 Symposia 2: Therapeutic Strategies for Cognitive and Behavioural Dysfunctions in Patients with rare Neurodevelopmental Disorders Simposium #1 eHealth Interventions for Executive Functioning and Social Cognition in Children and Adults with Noonan Syndrome Spectrum Disorders: Two Pilot Studies Jennifer Kramer, Vincent van Gogh Centre of Excellence for Psychiatry, Venray, Netherlands Renée Roelofs, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands Ellen Wingbermühle, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands Sarah Pieters, Radboud University, Behavioural Science Institute, Nijmegen, Netherlands Jos Egger, Radboud University, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, Netherlands Category: Genetics/Genetic Disorders Keyword 1: RASopathies Keyword 2: executive functions Keyword 3: social cognition Objective: Noonan Syndrome Spectrum Disorders (NSSD) are a group of related genetic disorders, associated with cognitive and psychological vulnerabilities. Especially problems in executive functioning (EF) and social cognition (SC) are frequent. Training of EF and SC is effective in patients with other neurodevelopmental disorders. This pilot study aims to evaluate the feasibility and effectiveness of two eHealth interventions: 1) the computerised EF training Braingame Brian (BGB) for children with NSSD and 2) the online Social-Emotional training for adults with NSSD (eSENS). Participants and Methods: In the BGB study, nine children with NS (age 7-17 years) were randomly allocated to the treatment group (n= 5) or the control group (n= 4). In the eSENS study, 12 adults were included (age 20-41 years). Neuropsychological tests (BGB) and questionnaires (BGB and eSENS) regarding EF and SC were administered pre and post treatment. Adults also completed a weekly questionnaire regarding emotion regulation. Data were analysed using non-parametric tests. Results: In the BGB study, a significant improvement was found in cognitive flexibility (Z= -2.45, p = .016, r = -0.82). No other significant results were found, although the effect size for parent-rated EF was promising (r = -0.5). In the eSENS study, significant improvements were found on self-report questionnaires of alexithymia (Z= -2.75, p= .01), emotion regulation (reappraisal Z= -2.56, p= .01; suppression Z= -2.54, p= .01), anxiety (Z= -2.71, p= .01), and social-emotional self-efficacy (Z= -2.22, p= .03). A weekly assessment of emotion regulation showed an increase in reappraisal (c²= 28.45, p= .03) and a decline in suppression (c²= 54.89, p< .001) during the training. Training partners also reported less problems in alexithymia (Z= -2.51, p= .01), emotion regulation (Z= -2.53, p=.01) and self-evaluation (Z= -2.47, p=.01) in the patients. Conclusions: BGB and eSENS are feasible eHealth interventions for children and adults with NSSD. Although the effectiveness of these interventions have yet to be evaluated in larger and controlled samples, they show encouraging results. The introduction of eHealth interventions of EF and SC seem promising and could greatly improve psychological wellbeing in patients with NSSD.

Symposium

02/15/2024
09:00 am - 10:30 am
Room: West Side Ballroom - Salon 2

Symposia 2: Therapeutic Strategies for Cognitive and Behavioural Dysfunctions in Patients with rare Neurodevelopmental Disorders

Simposium #1

eHealth Interventions for Executive Functioning and Social Cognition in Children and Adults with Noonan Syndrome Spectrum Disorders: Two Pilot Studies

Jennifer Kramer, Vincent van Gogh Centre of Excellence for Psychiatry, Venray, Netherlands
Renée Roelofs, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands
Ellen Wingbermühle, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands
Sarah Pieters, Radboud University, Behavioural Science Institute, Nijmegen, Netherlands
Jos Egger, Radboud University, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, Netherlands

Category: Genetics/Genetic Disorders

Keyword 1: RASopathies
Keyword 2: executive functions
Keyword 3: social cognition

Objective:

Noonan Syndrome Spectrum Disorders (NSSD) are a group of related genetic disorders, associated with cognitive and psychological vulnerabilities. Especially problems in executive functioning (EF) and social cognition (SC) are frequent. Training of EF and SC is effective in patients with other neurodevelopmental disorders. This pilot study aims to evaluate the feasibility and effectiveness of two eHealth interventions: 1) the computerised EF training Braingame Brian (BGB) for children with NSSD and 2) the online Social-Emotional training for adults with NSSD (eSENS).

Participants and Methods:

In the BGB study, nine children with NS (age 7-17 years) were randomly allocated to the treatment group (n= 5) or the control group (n= 4). In the eSENS study, 12 adults were included (age 20-41 years). Neuropsychological tests (BGB) and questionnaires (BGB and eSENS) regarding EF and SC were administered pre and post treatment. Adults also completed a weekly questionnaire regarding emotion regulation. Data were analysed using non-parametric tests.

Results:

In the BGB study, a significant improvement was found in cognitive flexibility (Z= -2.45, p = .016, r = -0.82). No other significant results were found, although the effect size for parent-rated EF was promising (r = -0.5). In the eSENS study, significant improvements were found on self-report questionnaires of alexithymia (Z= -2.75, p= .01), emotion regulation (reappraisal Z= -2.56, p= .01; suppression Z= -2.54, p= .01), anxiety (Z= -2.71, p= .01), and social-emotional self-efficacy (Z= -2.22, p= .03). A weekly assessment of emotion regulation showed an increase in reappraisal (c²= 28.45, p= .03) and a decline in suppression (c²= 54.89, p< .001) during the training. Training partners also reported less problems in alexithymia (Z= -2.51, p= .01), emotion regulation (Z= -2.53, p=.01) and self-evaluation (Z= -2.47, p=.01) in the patients.

Conclusions:

BGB and eSENS are feasible eHealth interventions for children and adults with NSSD. Although the effectiveness of these interventions have yet to be evaluated in larger and controlled samples, they show encouraging results. The introduction of eHealth interventions of EF and SC seem promising and could greatly improve psychological wellbeing in patients with NSSD.