title: Developmental trajectories in syndromes with intellectual disability, with a focus on Wolf-Hirschhorn and its cognitive-behavioral profile.
abstract: Few studies exist of developmental trajectories in children with intellectual disability, and none for those with subtelomeric deletions. We compared developmental trajectories of children with Wolf-Hirschhorn syndrome to other genetic disorders. We recruited 106 children diagnosed with fragile X, Williams-Beuren syndrome, or Wolf-Hirschhorn syndrome, assessing their intellectual and adaptive behavior abilities. We retested 61 children 2 years later. We compared Time 1 and Time 2 difference scores related to genetic disorder, age, initial IQ, or adaptive behavior composite. Results show genetic disorder and initial IQ score were significant factors for IQ differences, but only genetic disorder affected adaptive behavior differences. Results suggest different gene-brain-behavior pathways likely exist for these genetic disorders. Different developmental trajectories will influence the type and intensity of intervention implemented by caregivers.
authors.affiliation: New York University Colleges of Dentistry and Nursing and Yeshiva University, New York, New York 10003, USA. gene.fish@nyu.edu
authors.forename: Gene S
authors.initials: GS
authors.lastname: Fisch
authors.forename: Nancy
authors.initials: N
authors.lastname: Carpenter
authors.forename: Patricia N
authors.initials: PN
authors.lastname: Howard-Peebles
authors.forename: Jeanette J A
authors.initials: JJ
authors.lastname: Holden
authors.forename: Jack
authors.initials: J
authors.lastname: Tarleton
authors.forename: Richard
authors.initials: R
authors.lastname: Simensen
authors.forename: Agatino
authors.initials: A
authors.lastname: Battaglia
mesh_terms: D000223:Adaptation, Psychological / Q000502:physiology; D000293:Adolescent; D002648:Child; D002652:Child Behavior / Q000502:physiology; D002657:Child Development / Q000502:physiology*; D002675:Child, Preschool; D003071:Cognition / Q000502:physiology; D005260:Female; D005600:Fragile X Syndrome / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D005838:Genotype; D006801:Humans; D008607:Intellectual Disability / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology*; D016014:Linear Models; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D018980:Williams Syndrome / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology; D054877:Wolf-Hirschhorn Syndrome / Q000453:epidemiology* / Q000235:genetics / Q000503:physiopathology*; D055815:Young Adult