Autistic behavior in children with fragile X syndrome: Prevalence, stability, and the impact of FMRP

@article{Hatton2006AutisticBI,
  title={Autistic behavior in children with fragile X syndrome: Prevalence, stability, and the impact of FMRP},
  author={Deborah D. Hatton and John H Sideris and Martie L. Skinner and Jean Mankowski and Donald B. Bailey and Jane E Roberts and Penny L. Mirrett},
  journal={American Journal of Medical Genetics Part A},
  year={2006},
  volume={140A}
}
We examined autistic behavior in a cross-sectional sample of 179 children with fragile X syndrome (FXS) and a longitudinal subset of 116 children using the Childhood Autism Rating Scale (CARS) to (a) determine a prevalence of autistic behavior in FXS, (b) examine the stability of autistic ratings over time, and (c) assess the association between the fragile X mental retardation protein (FMRP) and autistic behavior. [...] Key Result Approximately 21% of the sample of 129 children (25.9% of boys) scored at or…Expand
Autism spectrum disorder in fragile X syndrome: A longitudinal evaluation
TLDR
The findings indicate that ASD is a distinctive subphenotype in FXS characterized by deficits in complex social interaction, with similarities to ASD in the general population.
Social Approach and Autistic Behavior in Children with Fragile X Syndrome
TLDR
The Social Approach Scale (SAS) is introduced, an observation scale that includes physical movement, facial expression, and eye contact approach behaviors collected across multiple time points that suggested that social approach behaviors in children with FXS were affected by age, gender, setting, and time spent with an examiner.
Autism Symptomatology in Boys with Fragile X Syndrome: A Cross Sectional Developmental Trajectories Comparison with Nonsyndromic Autism Spectrum Disorder
TLDR
Results suggest that the onset of autism symptoms and their developmental trajectories in males with FXS differ in important ways as a function of CA, nonverbal cognitive ability, and expressive vocabulary relative to males with nonsyndromic ASD.
The emergence and stability of attention deficit hyperactivity disorder in boys with fragile X syndrome.
TLDR
The Surgency facet of temperament at either age does not predict strong relationships of comorbid pathologies of ADHD and ASD in FXS, however, surgency characteristics may serve as informative discriminative factors when studying behavioural outcomes in boys with FXS.
Autism in fragile X syndrome: a category mistake?
TLDR
There are significant differences in the profile of social and communicative symptomatology in FXS compared with individuals diagnosed with idiopathic autism, and the implementation of standard autism interventions for individuals with FXS may not be optimal.
Autistic behavior in boys with fragile X syndrome: social approach and HPA-axis dysfunction
TLDR
Examination of environmental and neuroendocrine factors that convey increased risk for elevated autistic behavior in boys with Fragile X syndrome found blunted cortisol change is associated with increased severity of autistic behaviors only within the FXS and ASD group.
Developmental trajectories of young girls with fragile x syndrome.
TLDR
In this sample, autistic behavior was associated with poorer developmental outcomes, primarily due to interactions of age with autistic behavior, even though autism behavior, measured continuously, was relatively mild.
Rethinking Measurement Standards of Autism Symptomology in Adolescents with Fragile X Syndrome.
TLDR
A singular measure of autism symptomology is insufficient to characterize autism in FXS and three approaches were examined, indicating discrepant classifications.
Heart activity and autistic behavior in infants and toddlers with fragile X syndrome.
TLDR
It is suggested that atypical physiological arousal emerges within the first year and predicts severity of autistic behavior in fragile X syndrome.
Temperament in young children with Fragile X Syndrome
Christina M. Low, Ph.D. Washington State University May 2013 Chair: Maria A Gartstein Fragile X Syndrome (FXS) is an x-linked, genetic disorder that represents the most common hereditary cause of
...
1
2
3
4
5
...

References

SHOWING 1-10 OF 68 REFERENCES
Autistic Behavior, FMR1 Protein, and Developmental Trajectories in Young Males with Fragile X Syndrome
TLDR
FMRP expression accounted for less variance in developmental level than did autistic behavior, and was not significantly related to slope (developmental change over time).
Autistic Behavior in Young Boys with Fragile X Syndrome
TLDR
Within the fraX group, chronological age and socioeconomic status did not correlate with CARS ratings, but severity of delay was strongly related, such that more severely delayed children scored higher (more autistic) on the CARS.
Autism spectrum disorder in fragile X syndrome: Communication, social interaction, and specific behaviors
TLDR
The findings demonstrate that the diagnosis of ASD in FraX reflects, to a large extent, an impairment in social interaction that is expressed with variable severity in young males with FraX.
Behavioral phenotype of fragile X syndrome: DSM-III-R autistic behavior in male children.
TLDR
The investigation supports the contention that fra(X) males manifest a specific subset of behaviors from the autistic spectrum and implications for treatment are discussed.
Fragile X syndrome, DSM-III-R, and autism.
  • A. Reiss, L. Freund
  • Psychology, Medicine
    Journal of the American Academy of Child and Adolescent Psychiatry
  • 1990
TLDR
The criteria most frequently met by fragile X subjects were related to deficits in social interaction with peers, abnormalities in verbal and nonverbal communication, stereotypic motor behavior, and unusual responses to sensory stimuli.
Autistic-like behaviour profile and psychiatric morbidity in Fragile X Syndrome
TLDR
The findings suggest that the autistic-like behaviour pattern is a stable phenotypic feature of FXS, but for increase in resistance to change over time, unlike the general population, which does not increase significantly over time.
Autistic-like behaviour profile and psychiatric morbidity in Fragile X Syndrome
TLDR
The findings suggest that the autistic-like behaviour pattern is a stable phenotypic feature of FXS, but for increase in resistance to change over time, unlike the general population, which does not increase significantly over time.
Autistic Behaviors Among Girls with Fragile X Syndrome
TLDR
Girls with fraX had significantly more autistic behaviors than controls; anxiety and posterior cerebellar area measures had distinct associations with subsets of autistic behaviors; these associations may have important implications for understanding the neurobiology of autism.
Mapping nonverbal IQ in young boys with fragile X syndrome
TLDR
Findings suggest that declines in IQ are the result of steady, but suboptimal intellectual growth, rather than a true deterioration in overall intellectual functioning.
The influence of environmental and genetic factors on behavior problems and autistic symptoms in boys and girls with fragile X syndrome.
TLDR
Results of multiple regression analyses showed that for boys with fragile X, effectiveness of educational and therapeutic services and parental psychological problems predicted internalizing and externalizing types of problems, while the quality of the home environment predicted autistic behavior.
...
1
2
3
4
5
...