Autistic behavior in boys with fragile X syndrome: social approach and HPA-axis dysfunction

Abstract The present study contrasted physiological arousal in infants and toddlers with fragile X syndrome to typically developing control participants and examined physiological predictors early in development to autism severity later in development in fragile X syndrome. Thirty-one males with fragile X syndrome (ages 8–40 months) and 25 age-matched control participants were included. The group with fragile X syndrome showed shorter interbeat intervals (IBIs), lower vagal tone (VT), and less modulation of IBI. Data suggested a nonlinear effect with IBI and autistic behavior; however, a linear effect with VT and autistic behavior emerged. These findings suggest that atypical physiological arousal emerges within the first year and predicts severity of autistic behavior in fragile X syndrome. These relationships are complex and dynamic, likely reflecting endogenous factors assumed to reflect atypical brain function secondary to reduced fragile X mental retardation protein. This research has important implications for the early identification and treatment of autistic behaviors in young children with fragile X syndrome.

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Heart activity and autistic behavior in infants with fragile X syndrome

International Journal of Psychophysiology ◽

10.1016/j.ijpsycho.2012.06.155 ◽

2012 ◽

Vol 85 (3) ◽

pp. 348-349

Author(s):

J.E. Roberts ◽

B.L. Tonnsen ◽

A. Robinson ◽

S. Shinkareva

Keyword(s):

Fragile X Syndrome ◽

Fragile X ◽

Heart Activity ◽

Autistic Behavior

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Compulsive, Self-Injurious, and Autistic Behavior in Children and Adolescents With Fragile X Syndrome

American Journal on Mental Retardation ◽

10.1352/0895-8017(2008)113[44:csaabi]2.0.co;2 ◽

2008 ◽

Vol 113 (1) ◽

pp. 44 ◽

Cited By ~ 114

Author(s):

Scott S. Hall ◽

Amy A. Lightbody ◽

Allan L. Reiss

Keyword(s):

Children And Adolescents ◽

Fragile X Syndrome ◽

Fragile X ◽

Autistic Behavior

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Behavioral phenotype of fragile X syndrome: DSM-III-R autistic behavior in male children

American Journal of Medical Genetics ◽

10.1002/ajmg.1320430106 ◽

1992 ◽

Vol 43 (1-2) ◽

pp. 35-46 ◽

Cited By ~ 94

Author(s):

Allan L. Reiss ◽

Lisa Freund

Keyword(s):

Fragile X Syndrome ◽

Fragile X ◽

Behavioral Phenotype ◽

Autistic Behavior

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Social Approach and Emotion Recognition in Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-119.2.133 ◽

2014 ◽

Vol 119 (2) ◽

pp. 133-150 ◽

Cited By ~ 6

Author(s):

Tracey A. Williams ◽

Melanie A. Porter ◽

Robyn Langdon

Keyword(s):

Emotion Recognition ◽

Fragile X Syndrome ◽

Fragile X ◽

Chronological Age ◽

Social Avoidance ◽

Emotional Stimuli ◽

Social Approach ◽

Mental Age ◽

The Social ◽

Social Difficulties

Abstract Evidence is emerging that individuals with Fragile X syndrome (FXS) display emotion recognition deficits, which may contribute to their significant social difficulties. The current study investigated the emotion recognition abilities, and social approachability judgments, of FXS individuals when processing emotional stimuli. Relative to chronological age- (CA-) and mental age- (MA-) matched controls, the FXS group performed significantly more poorly on the emotion recognition tasks, and displayed a bias towards detecting negative emotions. Moreover, after controlling for emotion recognition deficits, the FXS group displayed significantly reduced ratings of social approachability. These findings suggest that a social anxiety pattern, rather than poor socioemotional processing, may best explain the social avoidance observed in FXS.

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

American Journal of Medical Genetics Part A ◽

10.1002/ajmg.a.31286 ◽

2006 ◽

Vol 140A (17) ◽

pp. 1804-1813 ◽

Cited By ~ 255

Author(s):

Deborah D. Hatton ◽

John Sideris ◽

Martie Skinner ◽

Jean Mankowski ◽

Donald B. Bailey ◽

...

Keyword(s):

Fragile X Syndrome ◽

Fragile X ◽

Autistic Behavior ◽

The Impact

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

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-120.5.412 ◽

2015 ◽

Vol 120 (5) ◽

pp. 412-432 ◽

Cited By ~ 13

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.

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Developmental Trajectories of Young Girls With Fragile X Syndrome

American Journal on Intellectual and Developmental Disabilities ◽

10.1352/1944-7558-114.3.161 ◽

2009 ◽

Vol 114 (3) ◽

pp. 161-171 ◽

Cited By ~ 21

Author(s):

Deborah D. Hatton ◽

Anne Wheeler ◽

John Sideris ◽

Kelly Sullivan ◽

Alison Reichardt ◽

...

Keyword(s):

Fragile X Syndrome ◽

Fragile X ◽

Developmental Trajectories ◽

Developmental Outcomes ◽

Small Sample ◽

Considerable Variability ◽

Full Mutation ◽

Autistic Behavior ◽

Young Girls ◽

Autistic Behaviors

Abstract To describe the early phenotype of girls with full mutation fragile X, we used 54 observations of 15 girls between the ages of 6 months and 9 years to examine developmental trajectories as measured by the Battelle Development Inventory. In this sample, autistic behavior was associated with poorer developmental outcomes, primarily due to interactions of age with autistic behavior, even though autistic behavior, measured continuously, was relatively mild. Although this small sample, ascertained primarily through male relatives with fragile X syndrome, limits generalizability, considerable variability in developmental outcome in young girls was documented. In addition, findings support previous research suggesting that even mild autistic behaviors in girls can be associated with developmental outcomes.

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