Sustained attention and response inhibition in boys with fragile X syndrome: Measures of continuous performance

Abstract

Sustained attention and response inhibition were examined in boys with full mutation fragile X syndrome (FXS) using adapted visual and auditory continuous performance tests (CPTs). Only 61% of 56 boys with visual CPT data and 54% of 52 boys with auditory data were able to demonstrate sufficient understanding to complete the visual and auditory CPTs, respectively. Mental age (MA) predicted whether boys with FXS were able to demonstrate understanding of the CPTs. The performance of boys with FXS who were able to complete the CPTs was compared to a sample of boys without disabilities matched on MA. Boys with FXS demonstrated similar or smaller declines in sustained attention over task time than their MA-matched peers on the visual and auditory CPTs, respectively, but consistently demonstrated greater declines in response inhibition over task time than their MA-matched peers. There were no differences between groups for response time of hits. Higher MAs consistently predicted better sustained attention and response inhibition over task time on the visual and auditory CPTs. Furthermore, boys taking psychotropic medication performed better at the beginning of most tasks, although their performance deteriorated at a faster rate over time, and boys rated as meeting diagnostic criteria for ADHD-hyperactive type had more difficulty over task time with response inhibition on the auditory CPT. For both boys with FXS and their MA matches, performance was better on the visual CPT than on the auditory CPT though this effect may be attributable to a number of factors other than the modality.

Fragile X Syndrome as a Multilevel Model for Understanding Behaviorally Defined Disorders

“…Furthermore, scientific research has revealed that already in infancy, those with FXS demonstrate poor response inhibition (Scerif, Cornish, Wilding, Driver, & Karmiloff-Smith, 2004, poor saccadic eye-movement control (Scerif, Karmiloff-Smith, Campos, Elsabbagh, Driver, & Cornish, 2005), and prolonged visual attention to objects or what is known as sticky fixation (Roberts, Hatton, Long, Anello, & Colombo, 2012). In subsequent FXS development, school children and adolescents also display poor response inhibition (Sullivan, Hatton, & Hammer, 2007) and atypical patterns of visual attention (Hooper, Hatton, & Baranek, 2000;Munir, Cornish, & Wilding, 2000a, 2000b. Finally, executive function is clearly deficient in FXS in childhood, with concomitant memory impairments (Lanfranchi, Cornoldi, & Drigo, 2009).…”

El síndrome del cromosoma x frágil: fenotipo conductual y dificultades de aprendizaje

“…Los síntomas suelen aparecer a partir de los 2 años de edad, tienden a mejorar después de la pubertad y suelen ser los rasgos más importantes en niños con un funcionamiento elevado (inteligencia normal). En los casos de premutación pueden presentar síntomas de hiperactividad (Calvani et al, 2001;Sullivan et al, 2006;Sullivan et al, 2007;Beltrán et al, 2011).…”

Parent Ratings of Ability to Consent for Clinical Trials in Fragile X Syndrome

“…Studies of FXS have shown particular deficits in sustained attention, response inhibition, working memory, and other executive functions associated with decisional capacity (Baker et al, 2011; Hooper et al, 2008; Ornstein et al, 2008; Sullivan et al, 2007). Reading is a challenge for males (Roberts et al, 2005); a national survey reported that although 44% of adult males with FXS could read basic picture books or simple stories, only 19% could read books that contain new words or concepts (Bailey, Raspa, Holiday, Bishop, & Olmsted, 2009).…”