title: Executive function in CHARGE syndrome.
abstract: This study addressed the presence of executive dysfunction in children with CHARGE syndrome, a genetic disorder with multiple physical anomalies and severe challenging behaviors. Ninety-eight children were included in the study. More than half received clinically significant scores on the Behavior Rating Inventory of Executive Function (BRIEF; Gioia et al., 2000) scales of Shift, Monitor, and the Behavioral Regulation Index, with additional high scores on Inhibit and the Global Executive Composite. Associations were found with the age the child first walked, scores on the Autism Behavior Checklist (ABC; Krug et al., 1993), and being classified as deafblind. Difficulties with making transitions and flexible problem solving, monitoring their work and their effect on others, and acting on impulse, may be related to the behavioral difficulties exhibited by children with CHARGE. Interventions targeting improved self-regulation may help to manage this challenging behavior.
authors.affiliation: Central Michigan University. Mount Pleasant, MI 48859, USA. harts1ts@cmich.edu
authors.forename: Timothy S
authors.initials: TS
authors.lastname: Hartshorne
authors.forename: Jude
authors.initials: J
authors.lastname: Nicholas
authors.forename: Tina L
authors.initials: TL
authors.lastname: Grialou
authors.forename: Joanna M
authors.initials: JM
authors.lastname: Russ
mesh_terms: D000015:Abnormalities, Multiple / Q000453:epidemiology / Q000523:psychology*; D000293:Adolescent; D001321:Autistic Disorder / Q000453:epidemiology / Q000523:psychology; D001766:Blindness / Q000453:epidemiology / Q000523:psychology; D002648:Child; D002675:Child, Preschool; D002754:Choanal Atresia / Q000453:epidemiology / Q000523:psychology; D003071:Cognition; D003072:Cognition Disorders / Q000175:diagnosis* / Q000453:epidemiology / Q000523:psychology*; D003103:Coloboma / Q000453:epidemiology / Q000523:psychology*; D003638:Deafness / Q000453:epidemiology / Q000523:psychology; D004423:Ear / Q000002:abnormalities; D005260:Female; D005835:Genitalia / Q000002:abnormalities; D006130:Growth Disorders / Q000453:epidemiology / Q000523:psychology*; D006330:Heart Defects, Congenital / Q000453:epidemiology / Q000523:psychology; D006801:Humans; D008297:Male; D010290:Parents / Q000523:psychology; D011795:Surveys and Questionnaires; D013577:Syndrome