Specification of the Neurobehavioral Phenotype in Males with Fragile X Syndrome

PEDIATRICS ◽  
1995 ◽  
Vol 95 (5) ◽  
pp. 744-752 ◽  
Author(s):  
Thomas L. Baumgardner ◽  
Allan L. Reiss ◽  
Lisa S. Freund ◽  
Michael T. Abrams
Keyword(s):  
Fragile X Syndrome ◽  
Adaptive Behavior ◽  
Fragile X ◽  
Specific Treatment ◽  
Distinct Pattern ◽  
Aberrant Behavior ◽  
Vineland Adaptive Behavior Scales ◽  
Aberrant Behavior Checklist ◽  
Symptom Pattern ◽  
Vineland Adaptive Behavior

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 behaviors 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.

scholarly journals Do Children With Fragile X Syndrome Show Declines or Plateaus in Adaptive Behavior?

2015 ◽  
Vol 120 (5) ◽  
pp. 412-432 ◽  
Author(s):  
Laura J. Hahn ◽  
Nancy C. Brady ◽  
Steven F. Warren ◽  
Kandace K. Fleming
Keyword(s):  
Fragile X Syndrome ◽  
Adaptive Behavior ◽  
Cognitive Abilities ◽  
Fragile X ◽  
Vineland Adaptive Behavior Scales ◽  
Autistic Behavior ◽  
Relationship Of ◽  
The Relationship ◽  
Autistic Behaviors ◽  
Vineland Adaptive Behavior

Abstract This study explores if children with fragile X syndrome (FXS) show advances, declines, or plateaus in adaptive behavior over time and the relationship of nonverbal cognitive abilities and autistic behavior on these trajectories. Parents of 55 children with FXS completed the Vineland Adaptive Behavior Scales (Sparrow, Balla, & Cicchetti, 1984; Sparrow, Cicchetti, & Balla, 2005) between 3 and 6 times from 2 to 10 years of age. Using raw scores, results indicate that about half of the sample showed advances in adaptive behavior, whereas the other half showed declines, indicating a regression in skills. Children who were more cognitively advanced and had less autistic behaviors had higher trajectories. Understanding the developmental course of adaptive behavior in FXS has implications for educational planning and intervention, especially for those children showing declines.

Anxiety, attention problems, hyperactivity, and the Aberrant Behavior Checklist in fragile X syndrome

2013 ◽  
Vol 164 (1) ◽  
pp. 141-155 ◽  
Author(s):  
Anne Wheeler ◽  
Melissa Raspa ◽  
Carla Bann ◽  
Ellen Bishop ◽  
David Hessl ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Attention Problems ◽  
Aberrant Behavior ◽  
Aberrant Behavior Checklist

Factor Structure of the Aberrant Behavior Checklist in Individuals with Fragile X Syndrome: Clarifications and Future Guidance

2020 ◽  
Vol 30 (8) ◽  
pp. 512-521
Author(s):  
Michael G. Aman ◽  
Megan Norris ◽  
Aaron J. Kaat ◽  
Howard Andrews ◽  
Tse-Hwei Choo ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Factor Structure ◽  
Fragile X ◽  
Aberrant Behavior ◽  
Aberrant Behavior Checklist

scholarly journals Developing a utility index for the Aberrant Behavior Checklist (ABC-C) for fragile X syndrome

2014 ◽  
Vol 24 (2) ◽  
pp. 305-314 ◽  
Author(s):  
Cicely Kerr ◽  
Katie Breheny ◽  
Andrew Lloyd ◽  
John Brazier ◽  
Donald B. Bailey ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Aberrant Behavior ◽  
Aberrant Behavior Checklist ◽  
Utility Index

scholarly journals Psychometric Study of the Aberrant Behavior Checklist in Fragile X Syndrome and Implications for Targeted Treatment

2011 ◽  
Vol 42 (7) ◽  
pp. 1377-1392 ◽  
Author(s):  
Stephanie M. Sansone ◽  
Keith F. Widaman ◽  
Scott S. Hall ◽  
Allan L. Reiss ◽  
Amy Lightbody ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Targeted Treatment ◽  
Aberrant Behavior ◽  
Psychometric Study ◽  
Aberrant Behavior Checklist

scholarly journals Implicit Discrimination of Basic Facial Expressions of Positive/Negative Emotion in Fragile X Syndrome and Autism Spectrum Disorder

2015 ◽  
Vol 120 (4) ◽  
pp. 328-345 ◽  
Author(s):  
Hayley Crawford ◽  
Joanna Moss ◽  
Giles M. Anderson ◽  
Chris Oliver ◽  
Joseph P. McCleery
Keyword(s):  
Fragile X Syndrome ◽  
Facial Expressions ◽  
Adaptive Behavior ◽  
Negative Emotion ◽  
Fragile X ◽  
Eye Gaze ◽  
Autism Spectrum ◽  
Verbal Abilities ◽  
Spectrum Disorders ◽  
Vineland Adaptive Behavior

Abstract Fragile X syndrome (FXS) and autism spectrum disorders (ASD) are characterized by impaired social functioning. We examined the spontaneous discrimination of happy and disgusted facial expressions, from neutral faces, in individuals with FXS (n  =  13, Mage  =  19.70) and ASD (n  =  15, Mage  =  11.00) matched on adaptive behavior and verbal abilities measured by the Vineland Adaptive Behavior Scale. Eye gaze to the eyes and mouth of neutral faces was also measured. Results suggest individuals with FXS and ASD distinguish facial expressions spontaneously in the same way. Individuals with FXS looked significantly less at the eye region of neutral faces than individuals with ASD. These results provide insight into similarities and differences in face processing in two neurodevelopmental disorders noted for their similarities in social behavior.

Social Communication and Structural Language of Girls With High-Functioning Autism Spectrum Disorder

2020 ◽  
Vol 51 (4) ◽  
pp. 1139-1155
Author(s):  
Jenny M. Burton ◽  
Nancy A. Creaghead ◽  
Noah Silbert ◽  
Allison Breit-Smith ◽  
Amie W. Duncan ◽  
...  
Keyword(s):  
Autism Spectrum Disorder ◽  
Adaptive Behavior ◽  
Social Communication ◽  
High Functioning Autism ◽  
Autism Spectrum ◽  
Vineland Adaptive Behavior Scales ◽  
High Functioning ◽  
Expressive Communication ◽  
Vineland Adaptive Behavior ◽  
Structural Language

Purpose The purpose of this study was to characterize social communication and structural language of school-age girls with high-functioning autism spectrum disorder (HF-ASD) compared to a matched group of girls who are typically developing (TD). Method Participants were 37 girls between 7;5 and 15;2 (years;months)—18 HF-ASD and 19 TD. Children completed the Test of Pragmatic Language–Second Edition (TOPL-2) and Clinical Evaluation of Language Fundamentals–Fifth Edition. Parents completed the Children's Communication Checklist–2 United States Edition (CCC-2) and Receptive and Expressive Communication subdomains of the Vineland Adaptive Behavior Scales–Second Edition. Results In the area of social communication, girls with HF-ASD earned significantly lower scores and were more often classified as having an impairment on the TOPL-2 and the CCC-2. However, 28% and 33% earned average scores on the TOPL-2 and the CCC-2, respectively. In the area of structural language, no significant differences were found between groups on Clinical Evaluation of Language Fundamentals–Fifth Edition indexes. In contrast, girls with HF-ASD earned significantly lower scores and were more often classified as having an impairment on the Vineland Adaptive Behavior Scales–Second Edition. Sixty-one percent and 83% scored below average on the Receptive and Expressive Communication subdomains, respectively. Conclusions It has been argued that girls with HF-ASD, when compared to boys with HF-ASD, may have advantages for social communication and structural language that mask their impairments. However, when compared to girls who are TD, girls with HF-ASD demonstrated impaired social communication and structural language. Clinicians should include and carefully examine multiple sources of information when assessing girls with HF-ASD.

scholarly journals Study of the Interaction between Executive Function and Adaptive Behavior at School in Girls with Fragile X Syndrome

Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1108
Author(s):  
Lorena Joga-Elvira ◽  
Jennifer Martinez-Olmo ◽  
María-Luisa Joga ◽  
Carlos Jacas ◽  
Ana Roche-Martínez ◽  
...  
Keyword(s):  
Executive Function ◽  
Executive Functions ◽  
Fragile X Syndrome ◽  
Adaptive Behavior ◽  
Neuropsychological Tests ◽  
Rating Scale ◽  
Fragile X ◽  
School Setting ◽  
Control Group ◽  
The Relationship

The aim of this research is to analyze the relationship between executive functions and adaptive behavior in girls with Fragile X syndrome (FXS) in the school setting. This study is part of a larger investigation conducted at the Hospital Parc Tauli in Sabadell. The sample consists of a total of 40 girls (26 with FXS and 14 control) aged 7–16 years, who were administered different neuropsychological tests (WISC-V, NEPSY-II, WCST, TOL) and questionnaires answered by teachers (ABAS-II, BRIEF 2, ADHD Rating Scale). The results show that there is a greater interaction between some areas of executive function (cognitive flexibility, auditory attention, and visual abstraction capacity) and certain areas of adaptive behavior (conceptual, practical, social, and total domains) in the FXS group than in the control group. These results suggest that an alteration in the executive functions was affecting the daily functioning of the girls with FXS to a greater extent.

Sign in / Sign up

Export Citation Format

Share Document