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 #2 Sensory Information Processing, Intellectual Functioning, and Behaviour in Adults with Prader-Willi Syndrome Anja Bos-Roubos, Vincent van Gogh Centres of Excellence for Psychiatry, Venray, Netherlands Ellen Wingbermühle, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands Rosalie Brinke, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands Laura Graaff, Erasmus University Medical Centre, Department of Internal Medicine, Rotterdam, Netherlands Jos Egger, Radboud University, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, Netherlands Category: Genetics/Genetic Disorders Keyword 1: sensory integration Keyword 2: medical disorders/illness Keyword 3: cognitive rehabilitation Objective: Prader-Willi syndrome (PWS) is a neurological developmental disorder caused by a genetic variation on the paternal allele of chromosome 15 (q11-q13) associated with hypothalamic dysfunction and physical problems, intellectual disabilities, and behavioural disorders. Individuals with PWS also show reduced sensitivity to pain and thermal dysregulation, pointing to possible sensory information processing deficits. This study aims to describe comprehensive and in depth profiles of sensory information processing and intellectual functioning in patients with PWS, as well as to establish relationships between sensory information processing and intellectual functioning, in order to find treatment options that promote development and diminish behavioural problems. Participants and Methods: Included were 43 Dutch patients with confirmed PWS aged 17 to 57 years (M = 28, SD = 9.6). Data were obtained using the Sensory Profile proxy version, Dutch translation (SP-NL) and the test Wechsler Adult Intelligence Scale, fourth edition, Dutch translation. To test statistical differences One-Way Repeated Analyses of Variance were carried out. To evaluate if intelligence was predicted by sensory information processing, Multiple Regression Analysis (MRA), using backward entry, was carried out. Different MRA’s were performed with SP-NL scores as predictors (independent variables), and scores of the FSIQ and the four indices of the WAIS-IV-NL as dependent variables. Results: Individuals with PWS showed remarkably low sensory information registration (>2SD), and noticeably more difficulties with modulating endurance/tone, movement, and emotional responses (>2SD). The average full size intelligence quotient (FSIQ; M = 56.17), working memory index (WMI; M = 55.64), were both significantly lower than verbal comprehension (VCI; M = 63.22; p <. 001) and perceptual reasoning (PR; (M = 63.36; p < .001). Also processing speed (PS; M = 55.19) was significantly lower than VCI (p < .003) and PRI (p < .001). In addition, low registration of sensory information predicted lower FSIQ (16.0%) (p = .039) and lower VCI (34.8%) (p = .001). Also, the deviant modulation of endurance/tone predicted FSIQ (19.8%) (p = .012), and VCI (33.1%) (p < .001). Conclusions: Sensory information processing in individuals with PWS appears to be deviant as compared to healthy norm group controls. Individuals with PWS particularly suffer from low registration of sensory information, modulation problems (lack of endurance, low muscle tone, weak body posture, lack of movement), and challenging behaviours. In PWS, specifically low working memory capacity and slow information processing speed explain low overall intelligence levels. Sensory information processing predicts intellectual functioning in adults with PWS. This study identifies the importance of the influence of lower order sensory information processing (‘bottom up input’) on higher order cognitive functioning and behaviour (‘top down control’).

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 #2

Sensory Information Processing, Intellectual Functioning, and Behaviour in Adults with Prader-Willi Syndrome

Anja Bos-Roubos, Vincent van Gogh Centres of Excellence for Psychiatry, Venray, Netherlands
Ellen Wingbermühle, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands
Rosalie Brinke, Vincent van Gogh Centres of Excellence for Neuropsychiatry, Venray, Netherlands
Laura Graaff, Erasmus University Medical Centre, Department of Internal Medicine, Rotterdam, Netherlands
Jos Egger, Radboud University, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, Netherlands

Category: Genetics/Genetic Disorders

Keyword 1: sensory integration
Keyword 2: medical disorders/illness
Keyword 3: cognitive rehabilitation

Objective:

Prader-Willi syndrome (PWS) is a neurological developmental disorder caused by a genetic variation on the paternal allele of chromosome 15 (q11-q13) associated with hypothalamic dysfunction and physical problems, intellectual disabilities, and behavioural disorders. Individuals with PWS also show reduced sensitivity to pain and thermal dysregulation, pointing to possible sensory information processing deficits. This study aims to describe comprehensive and in depth profiles of sensory information processing and intellectual functioning in patients with PWS, as well as to establish relationships between sensory information processing and intellectual functioning, in order to find treatment options that promote development and diminish behavioural problems.

Participants and Methods:

Included were 43 Dutch patients with confirmed PWS aged 17 to 57 years (M = 28, SD = 9.6). Data were obtained using the Sensory Profile proxy version, Dutch translation (SP-NL) and the test Wechsler Adult Intelligence Scale, fourth edition, Dutch translation. To test statistical differences One-Way Repeated Analyses of Variance were carried out. To evaluate if intelligence was predicted by sensory information processing, Multiple Regression Analysis (MRA), using backward entry, was carried out. Different MRA’s were performed with SP-NL scores as predictors (independent variables), and scores of the FSIQ and the four indices of the WAIS-IV-NL as dependent variables.

Results:

Individuals with PWS showed remarkably low sensory information registration (>2SD), and noticeably more difficulties with modulating endurance/tone, movement, and emotional responses (>2SD). The average full size intelligence quotient (FSIQ; M = 56.17), working memory index (WMI; M = 55.64), were both significantly lower than verbal comprehension (VCI; M = 63.22; p <. 001) and perceptual reasoning (PR; (M = 63.36; p < .001). Also processing speed (PS; M = 55.19) was significantly lower than VCI (p < .003) and PRI (p < .001). In addition, low registration of sensory information predicted lower FSIQ (16.0%) (p = .039) and lower VCI (34.8%) (p = .001). Also, the deviant modulation of endurance/tone predicted FSIQ (19.8%) (p = .012), and VCI (33.1%) (p < .001).

Conclusions:

Sensory information processing in individuals with PWS appears to be deviant as compared to healthy norm group controls. Individuals with PWS particularly suffer from low registration of sensory information, modulation problems (lack of endurance, low muscle tone, weak body posture, lack of movement), and challenging behaviours. In PWS, specifically low working memory capacity and slow information processing speed explain low overall intelligence levels. Sensory information processing predicts intellectual functioning in adults with PWS.
This study identifies the importance of the influence of lower order sensory information processing (‘bottom up input’) on higher order cognitive functioning and behaviour (‘top down control’).