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

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

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 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 AB010. Aberrant behavior profile of fragile X syndrome in medically underserved population

2017 ◽  
Vol 5 (S2) ◽  
pp. AB010-AB010
Author(s):  
Tri Winarni ◽  
Tanjung Sumekar ◽  
Widodo Sardjana ◽  
Hardian Hardian ◽  
Sultana MH Faradz
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Medically Underserved ◽  
Underserved Population ◽  
Aberrant Behavior ◽  
Medically Underserved Population ◽  
Behavior Profile

scholarly journals Intellectual functioning and behavioural features associated with mosaicism in fragile X syndrome

2019 ◽  
Vol 11 (1) ◽  
Author(s):  
Emma K. Baker ◽  
Marta Arpone ◽  
Solange Aliaga Vera ◽  
Lesley Bretherton ◽  
Alexandra Ure ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Cognitive Abilities ◽  
Fragile X ◽  
Autism Diagnostic Observation Schedule ◽  
Intellectual Functioning ◽  
Autism Spectrum ◽  
Aberrant Behavior Checklist ◽  
Age Appropriate ◽  
Maladaptive Behaviours ◽  
Age And Sex

Abstract Background Fragile X syndrome (FXS) is a common cause of intellectual disability and autism spectrum disorder (ASD) usually associated with a CGG expansion, termed full mutation (FM: CGG ≥ 200), increased DNA methylation of the FMR1 promoter and silencing of the gene. Mosaicism for presence of cells with either methylated FM or smaller unmethylated pre-mutation (PM: CGG 55–199) alleles in the same individual have been associated with better cognitive functioning. This study compares age- and sex-matched FM-only and PM/FM mosaic individuals on intellectual functioning, ASD features and maladaptive behaviours. Methods This study comprised a large international cohort of 126 male and female participants with FXS (aged 1.15 to 43.17 years) separated into FM-only and PM/FM mosaic groups (90 males, 77.8% FM-only; 36 females, 77.8% FM-only). Intellectual functioning was assessed with age appropriate developmental or intelligence tests. The Autism Diagnostic Observation Schedule-2nd Edition was used to examine ASD features while the Aberrant Behavior Checklist-Community assessed maladaptive behaviours. Results Comparing males and females (FM-only + PM/FM mosaic), males had poorer intellectual functioning on all domains (p < 0.0001). Although females had less ASD features and less parent-reported maladaptive behaviours, these differences were no longer significant after controlling for intellectual functioning. Participants with PM/FM mosaicism, regardless of sex, presented with better intellectual functioning and less maladaptive behaviours compared with their age- and sex-matched FM-only counterparts (p < 0.05). ASD features were similar between FM-only and PM/FM mosaics within each sex, after controlling for overall intellectual functioning. Conclusions Males with FXS had significantly lower intellectual functioning than females with FXS. However, there were no significant differences in ASD features and maladaptive behaviours, after controlling for intellectual functioning, independent of the presence or absence of mosaicism. This suggests that interventions that primarily target cognitive abilities may in turn reduce the severity of maladaptive behaviours including ASD features in FXS.

scholarly journals Brain circuitry, behavior, and cognition: A randomized placebo-controlled trial of donepezil in fragile X syndrome

2019 ◽  
Vol 33 (8) ◽  
pp. 975-985 ◽  
Author(s):  
Jennifer L Bruno ◽  
SM Hadi Hosseini ◽  
Amy A Lightbody ◽  
Mai K Manchanda ◽  
Allan L Reiss
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Functional Magnetic Resonance Imaging ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Active Group ◽  
Functional Magnetic Resonance ◽  
Resonance Imaging ◽  
Activation Patterns ◽  
Aberrant Behavior Checklist

Background: Fragile X syndrome, the most common inherited cause for intellectual disability, is associated with alterations in cholinergic among other neurotransmitter systems. This study investigated the effects of donepezil hydrochloride, a cholinesterase inhibitor that has potential to correct aberrant cholinergic signaling. Method: Forty-two individuals with fragile X syndrome (mean age=19.61 years) were randomized to receive 2.5–10.0 mg of donepezil ( n=20, seven females) or placebo ( n=22, eight females) per day. One individual in the active group withdrew at week 7. Outcomes included the contingency naming test, the aberrant behavior checklist, and behavior and brain activation patterns during a functional magnetic resonance imaging gaze discrimination task. Results: There were no significant differences between active and placebo groups on cognitive (contingency naming task) or behavioral (total score or subscales of the aberrant behavior checklist) outcomes. At baseline, the active and placebo groups did not differ in functional magnetic resonance imaging activation patterns during the gaze task. After 12 weeks of treatment the active group displayed reduced activation in response to the averted vs direct gaze contrast, relative to the placebo group, in the left superior frontal gyrus. Conclusions: Reduced functional brain activation for the active group may represent less arousal in response to direct eye gaze, relative to the placebo group. Change in functional magnetic resonance imaging activation patterns may serve as a more sensitive metric and predictor of response to treatment when compared to cognitive and behavioral assessments. Our results suggest that donepezil may have an impact on brain functioning, but longer term follow-up and concomitant behavioral intervention may be required to demonstrate improvement in cognition and behavior.

scholarly journals Temperament factor structure in fragile X syndrome: The Children's Behavior Questionnaire

2014 ◽  
Vol 35 (2) ◽  
pp. 563-571 ◽  
Author(s):  
Jane E. Roberts ◽  
Bridgette L. Tonnsen ◽  
Marissa Robinson ◽  
Samuel D. McQuillin ◽  
Deborah D. Hatton
Keyword(s):  
Fragile X Syndrome ◽  
Factor Structure ◽  
Fragile X ◽  
Children's Behavior
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