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

1992 ◽  
Vol 43 (1-2) ◽  
pp. 35-46 ◽  
Author(s):  
Allan L. Reiss ◽  
Lisa Freund
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Behavioral Phenotype ◽  
Autistic Behavior

scholarly journals Heart Activity and Autistic Behavior in Infants and Toddlers With Fragile X Syndrome

2012 ◽  
Vol 117 (2) ◽  
pp. 90-102 ◽  
Author(s):  
Jane E. Roberts ◽  
Bridgette Tonnsen ◽  
Ashley Robinson ◽  
Svetlana V. Shinkareva
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Physiological Arousal ◽  
First Year ◽  
Typically Developing ◽  
Infants And Toddlers ◽  
Autistic Behavior ◽  
Endogenous Factors ◽  
Linear Effect ◽  
Mental Retardation Protein

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.

Heart activity and autistic behavior in infants with fragile X syndrome

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

Validation of Hagerman’s behavioral phenotype for fragile X syndrome among men with intellectual disability

2021 ◽  
Vol ahead-of-print (ahead-of-print) ◽  
Author(s):  
Jacques Bellavance ◽  
Morin Diane ◽  
Catherine Mello
Keyword(s):  
Down Syndrome ◽  
Intellectual Disability ◽  
Fragile X Syndrome ◽  
Design Methodology ◽  
Fragile X ◽  
Behavioral Phenotype ◽  
Care Providers ◽  
Content Type

Purpose The behavioral phenotype of fragile X syndrome (FXS) and intellectual disability (ID) proposed by Hagerman et al. (2009) was primarily based on data from male children and teens. The purpose of this study was to promote a better understanding of how this condition manifests in adults. Design/methodology/approach A total of 18 men of FXS were paired with men with Down syndrome on the basis of age and level of ID. A screening checklist was created on the basis of existing scales and the Hagerman et al. (2009) behavioral phenotype and completed by care providers. Findings Five of the 12 features of the phenotype were significantly more present among men with FXS than in men with Down syndrome. Originality/value This study provides partial confirmation for Hagerman et al.’s (2009) behavioral phenotype of FXS among men with moderate ID and identified some traits that warrant further investigation.

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

2006 ◽  
Vol 140A (17) ◽  
pp. 1804-1813 ◽  
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

Social Approach and Autistic Behavior in Children with Fragile X Syndrome

2006 ◽  
Vol 37 (9) ◽  
pp. 1748-1760 ◽  
Author(s):  
Jane E. Roberts ◽  
Leigh Anne H. Weisenfeld ◽  
Deborah D. Hatton ◽  
Morgan Heath ◽  
Walter E. Kaufmann
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Social Approach ◽  
Autistic Behavior

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.

scholarly journals Evidence of a Distinct Behavioral Phenotype in Young Boys With Fragile X Syndrome and Autism

2012 ◽  
Vol 51 (12) ◽  
pp. 1324-1332 ◽  
Author(s):  
Jason J. Wolff ◽  
James W. Bodfish ◽  
Heather C. Hazlett ◽  
Amy A. Lightbody ◽  
Allan L. Reiss ◽  
...  
Keyword(s):  
Fragile X Syndrome ◽  
Fragile X ◽  
Behavioral Phenotype

Developmental Trajectories of Young Girls With Fragile X Syndrome

2009 ◽  
Vol 114 (3) ◽  
pp. 161-171 ◽  
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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