Problem behavior in boys with fragile X syndrome

Abstract

This study examines problem behavior over time in 59 boys with fragile X syndrome (FXS), aged 4-12 years, using the Child Behavior Checklist (CBCL). Approximately 49% of the boys scored within the borderline or clinical range on total problem behavior, while 56-57% scored in the borderline or clinical range on the attention and thought problems subscales, and 26% scored in this range on the social problems subscale. With a mean of 2.5 assessments per child, behavior problems were stable during the 3-year period of study. Total problem behavior was higher for children who displayed autistic behavior, were rated as low in adaptability, had mothers with higher maternal education levels, and were on medication. Mothers with more education also rated their children as having more attention, thought, and total problems. Children taking medication differed from boys who were not taking medication on social problems, but not on attention and thought problems. Low adaptability and more autistic characteristics predicted thought problems.

Autism spectrum disorder in Fragile X syndrome: Differential contribution of adaptive socialization and social withdrawal

“…Social avoidant behavior has been recognized as a frequent and characteristic abnormality in individuals with FraX [Hagerman, 2002]. Whether hyperarousal, social anxiety, or a different process leading to withdrawal, we Kaufmann et al, 2004] and others [Hatton et al, 2002] have shown that rating scales of problem/aberrant behavior demonstrate markedly high scores on their corresponding social withdrawal subscales in boys with FraX þ ASD. Considering that the continuum of CBCLw and ABCsw scores paralleled the spectrum of severity in autistic behavior , and that delayed adaptive socialization skills is a selective predictor of ASD in FraX, we examined the possibility that severity of social withdrawal was also a correlate of FraX þ ASD.…”

“…Subscales T-scores between 66 and 70 and >70 are considered in the borderline clinical and clinically significant ranges, respectively. This study focused primarily on the CBCLw subscale, which we and others [Hatton et al, 2002] have successfully applied to FraX populations. The ABC-C [Aman and Singh, 1986] is also a widely used parental report that we have applied to the study of ASD in FraX Kaufmann et al, 2004] and Down syndrome [Capone et al, 2005].…”

“…Nonetheless, severity and profile of these autistic behaviors enable a differentiation between individuals with FraX who meet Diagnostic and Statistical Manual of Mental Disorders, 4th edition (DSM-IV) [APA, 1994] criteria for autism spectrum disorder (ASD) and those who do not . The importance of precisely delineating the profile of boys with FraX þ Autism or FraX þ ASD is underscored by the fact that these diagnoses are relatively stable over time [Sabaratnam et al, 2003] and that children with FraX þ ASD display other severe cognitive and behavioral abnormalities [Kau et al, 2000;Bailey et al, 2000Bailey et al, , 2001aHatton et al, 2002Hatton et al, , 2003Sabaratnam et al, 2003;Roberts et al, 2005a].…”

Modeling fragile X syndrome in the <i>Fmr1</i> knockout mouse

“…Individuals with FXS are hyperactive and have difficulties with attention and impulse control (35,(112)(113)(114)(115). Subjects with FXS performed better than learning disabled controls on selective attention, but the subjects with FXS had deficits similar to the learning disabled controls in sustained attention and working memory (116).…”

Disparities in Diagnoses Received Prior to a Diagnosis of Autism Spectrum Disorder

“…Mental retardation is associated with 70-80% of cases of children with autistic disorder (Bonde, 2000;Chakrabarti & Fombonne, 2001). Children with autism often exhibit attention and impulsivity problems (Aman & Langworthy, 2000;Goldstein & Schwebach, 2004) or severe behavior problems (Hatton et al, 2002;Remington, Sloman, Konstantareas, Parker, & Gow, 2002). Criteria from the Diagnostic and Statistical Manual of Mental Disorder IV-TR specify, however, that a diagnosis of autism must be ruled out before diagnosing ADHD or OCD (American Psychiatric Association, 2000).…”