INS NYC 2024 Program

Poster	Poster Session 10 Program Schedule 02/17/2024 09:00 am - 10:15 am Room: Majestic Complex (Posters 61-120) Poster Session 10: Neurodevelopmental \| Congenital Conditions Final Abstract #89 Contributions Towards Executive Functioning and Behavior Among Children with Duchenne Muscular Dystrophy Sonia Seehra, The Graduate Center, City University of New York, New York, United States Amanda Kenepp, The Graduate Center, City University of New York, New York, United States Rachel Goldman, The Graduate Center, City University of New York, New York, United States Natalie Nagpal, The Graduate Center, City University of New York, New York, United States Robert Fee, Columbia University Irving Medical Center, New York, United States Veronica Hinton, The Graduate Center, City University of New York, New York, United States Category: Medical/Neurological Disorders/Other (Child) Keyword 1: muscular dystrophy Keyword 2: executive functions Keyword 3: adaptive functioning Objective: Duchenne muscular dystrophy (DMD) presents with progressive muscular weakness and with a selective neuropsychological profile. DMD is caused by a mutation in the gene that codes for the protein dystrophin, either “knocking out” dystrophin production or resulting in an isoform with diminished function. Dystrophin is typically found in muscle and brain (as well as other tissues). As a group, children with DMD have been shown to have slightly lower than expected verbal IQ scores, academic skills and verbal span (as measured on tests requiring immediate repetition of verbal information). Boys with DMD also have been found to have increased parent report of executive problems, attention problems and social problems with mixed findings on depressive symptoms. These behavioral problems may be a direct result of the mutation or a secondary reactive response to the disorder. Further, parent ratings of behavior may be colored by parent feelings and bias. The goals of the current study are to examine parent reported behavioral outcomes in greater detail in this population. We examined this both by comparing parent reports of behavior of boys with DMD to their unaffected sibling controls and by examining whether child-derived measures (e.g. mutation position, motor strength, cognitive performance) contribute to the parent reported behavior concerns. Participants and Methods: Parents completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Behavior Assessment System for Children (BASC). Data were analyzed in two different ways. First paired group comparisons between 34 children with DMD and their unaffected siblings (average age: DMD 8.7 + 2.6, controls 9.8 + 3.2) were run. Second, linear regressions were conducted on a different dataset (50 boys with DMD, average age: 11.0 + 3.5) to examine contributions towards parent reported outcomes. Contributors included family income, mutation position, measures of motor function (Brooke and Vignos measures of motor function), and measures of cognitive performance (Comprehensive Test of Nonverbal Intelligence (CTONI), the Woodcock Johnson Tests of Achievement III (WJ), and Digit Span scaled score from WISC-IV. Results: Paired t-tests indicated there were significant differences between the means of scores of children with DMD and their siblings on the BRIEF General Executive composite (t= -4.513, p<0.001), On the BASC, significant between-group differences were seen on the composite internalizing (t= -4.244, p<0.001), behavioral symptoms (t= -3.847, p<0.001) and BASC adaptive skills (t= 4.237, p<0.001), but not on the externalizing skills (t= -1.555, p= 0.129). Linear regressions revealed that family income, mutation position and child- derived motor and cognitive variables of interest did not contribute toward parent reported behavioral outcomes. Conclusions: Findings indicated that parents of children with DMD endorsed more executive, attention, internalizing and adaptive behavior problems than their unaffected siblings, consistent with prior literature. Surprisingly, family income, mutation position and child- derived motor and cognitive variables of interest did not contribute significantly to the variance across behavioral scores.

Poster

02/17/2024
09:00 am - 10:15 am
Room: Majestic Complex (Posters 61-120)

Poster Session 10: Neurodevelopmental | Congenital Conditions

Final Abstract #89

Contributions Towards Executive Functioning and Behavior Among Children with Duchenne Muscular Dystrophy

Sonia Seehra, The Graduate Center, City University of New York, New York, United States
Amanda Kenepp, The Graduate Center, City University of New York, New York, United States
Rachel Goldman, The Graduate Center, City University of New York, New York, United States
Natalie Nagpal, The Graduate Center, City University of New York, New York, United States
Robert Fee, Columbia University Irving Medical Center, New York, United States
Veronica Hinton, The Graduate Center, City University of New York, New York, United States

Category: Medical/Neurological Disorders/Other (Child)

Keyword 1: muscular dystrophy
Keyword 2: executive functions
Keyword 3: adaptive functioning

Objective:

Duchenne muscular dystrophy (DMD) presents with progressive muscular weakness and with a selective neuropsychological profile. DMD is caused by a mutation in the gene that codes for the protein dystrophin, either “knocking out” dystrophin production or resulting in an isoform with diminished function. Dystrophin is typically found in muscle and brain (as well as other tissues). As a group, children with DMD have been shown to have slightly lower than expected verbal IQ scores, academic skills and verbal span (as measured on tests requiring immediate repetition of verbal information). Boys with DMD also have been found to have increased parent report of executive problems, attention problems and social problems with mixed findings on depressive symptoms. These behavioral problems may be a direct result of the mutation or a secondary reactive response to the disorder. Further, parent ratings of behavior may be colored by parent feelings and bias. The goals of the current study are to examine parent reported behavioral outcomes in greater detail in this population. We examined this both by comparing parent reports of behavior of boys with DMD to their unaffected sibling controls and by examining whether child-derived measures (e.g. mutation position, motor strength, cognitive performance) contribute to the parent reported behavior concerns.

Participants and Methods:

Parents completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Behavior Assessment System for Children (BASC). Data were analyzed in two different ways. First paired group comparisons between 34 children with DMD and their unaffected siblings (average age: DMD 8.7 + 2.6, controls 9.8 + 3.2) were run. Second, linear regressions were conducted on a different dataset (50 boys with DMD, average age: 11.0 + 3.5) to examine contributions towards parent reported outcomes. Contributors included family income, mutation position, measures of motor function (Brooke and Vignos measures of motor function), and measures of cognitive performance (Comprehensive Test of Nonverbal Intelligence (CTONI), the Woodcock Johnson Tests of Achievement III (WJ), and Digit Span scaled score from WISC-IV.

Results:

Paired t-tests indicated there were significant differences between the means of scores of children with DMD and their siblings on the BRIEF General Executive composite (t= -4.513, p<0.001), On the BASC, significant between-group differences were seen on the composite internalizing (t= -4.244, p<0.001), behavioral symptoms (t= -3.847, p<0.001) and BASC adaptive skills (t= 4.237, p<0.001), but not on the externalizing skills (t= -1.555, p= 0.129). Linear regressions revealed that family income, mutation position and child- derived motor and cognitive variables of interest did not contribute toward parent reported behavioral outcomes.

Conclusions:

Findings indicated that parents of children with DMD endorsed more executive, attention, internalizing and adaptive behavior problems than their unaffected siblings, consistent with prior literature. Surprisingly, family income, mutation position and child- derived motor and cognitive variables of interest did not contribute significantly to the variance across behavioral scores.