Literature DB >> 7724315

Specification of the neurobehavioral phenotype in males with fragile X syndrome.

T L Baumgardner1, A L Reiss, L S Freund, M T Abrams.   

Abstract

OBJECTIVE: A controlled clinical study was designed to identify the neurobehavioral profile that is specific to males with fragile X syndrome.
DESIGN: Thirty-one males with fragile X syndrome and 30 age and IQ-matched male controls were evaluated with instruments that assess multiple domains of adaptive functioning and problem behaviors. The Vineland Adaptive Behavior Scales and the Aberrant Behavior Checklist were selected for their dimensional scaling of behavioral ratings.
RESULTS: Parent and Teacher versions of the Aberrant Behavior Checklist demonstrated a profile of behaviors specific to males with fragile X syndrome characterized by significantly higher levels of hyperactivity, stereotypic movements, and unusual speech. The Vineland Adaptive Behavior Scales revealed no fragile X-specific profile of adaptive skills development.
CONCLUSIONS: The distinct pattern of aberrant behavior observed among males with fragile X emphasizes the importance of drawing subtype distinctions within the classification of individuals with mental retardation on the basis of underlying etiology. For clinical research, specifying the fragile X phenotype is a vital part in the effort to elucidate the neurodevelopmental pathways of normal behavior and psychopathology. Understanding the fragile X symptom pattern is essential for designing symptom-specific treatment interventions, as well as for research into the efficacy of interventions strategies.

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Mesh:

Year:  1995        PMID: 7724315

Source DB:  PubMed          Journal:  Pediatrics        ISSN: 0031-4005            Impact factor:   7.124


  61 in total

1.  Narrative Skill in Boys with Fragile X Syndrome with and without Autism Spectrum Disorder.

Authors:  Bruno Estigarribia; Gary E Martin; Joanne E Roberts; Amy Spencer; Agnieszka Gucwa; John Sideris
Journal:  Appl Psycholinguist       Date:  2011

2.  Longitudinal changes in cognitive and adaptive behavior scores in children and adolescents with the fragile X mutation or autism.

Authors:  Gene S Fisch; Richard J Simensen; R J Schroer
Journal:  J Autism Dev Disord       Date:  2002-04

Review 3.  Fragile X: leading the way for targeted treatments in autism.

Authors:  Lulu W Wang; Elizabeth Berry-Kravis; Randi J Hagerman
Journal:  Neurotherapeutics       Date:  2010-07       Impact factor: 7.620

4.  Elevated glycogen synthase kinase-3 activity in Fragile X mice: key metabolic regulator with evidence for treatment potential.

Authors:  Wenzhong William Min; Christopher J Yuskaitis; Qijiang Yan; Christopher Sikorski; Shengqiang Chen; Richard S Jope; Robert P Bauchwitz
Journal:  Neuropharmacology       Date:  2008-10-14       Impact factor: 5.250

5.  Neuroanatomical abnormalities in fragile X syndrome during the adolescent and young adult years.

Authors:  Gisela M Sandoval; Sehoon Shim; David S Hong; Amy S Garrett; Eve-Marie Quintin; Matthew J Marzelli; Swetapadma Patnaik; Amy A Lightbody; Allan L Reiss
Journal:  J Psychiatr Res       Date:  2018-10-25       Impact factor: 4.791

Review 6.  Drug development for neurodevelopmental disorders: lessons learned from fragile X syndrome.

Authors:  Elizabeth M Berry-Kravis; Lothar Lindemann; Aia E Jønch; George Apostol; Mark F Bear; Randall L Carpenter; Jacqueline N Crawley; Aurore Curie; Vincent Des Portes; Farah Hossain; Fabrizio Gasparini; Baltazar Gomez-Mancilla; David Hessl; Eva Loth; Sebastian H Scharf; Paul P Wang; Florian Von Raison; Randi Hagerman; Will Spooren; Sébastien Jacquemont
Journal:  Nat Rev Drug Discov       Date:  2017-12-08       Impact factor: 84.694

7.  Physiological arousal in autism and fragile X syndrome: group comparisons and links with pragmatic language.

Authors:  Jessica Klusek; Gary E Martin; Molly Losh
Journal:  Am J Intellect Dev Disabil       Date:  2013-11

8.  Viewing social scenes: a visual scan-path study comparing fragile X syndrome and Williams syndrome.

Authors:  Tracey A Williams; Melanie A Porter; Robyn Langdon
Journal:  J Autism Dev Disord       Date:  2013-08

Review 9.  Gene, brain, and behavior relationships in fragile X syndrome: evidence from neuroimaging studies.

Authors:  Amy A Lightbody; Allan L Reiss
Journal:  Dev Disabil Res Rev       Date:  2009

10.  Autism spectrum disorder in fragile X syndrome: a longitudinal evaluation.

Authors:  R Nick Hernandez; Rachel L Feinberg; Rebecca Vaurio; Natalie M Passanante; Richard E Thompson; Walter E Kaufmann
Journal:  Am J Med Genet A       Date:  2009-06       Impact factor: 2.802

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