title: Aspects of Attention in Rett Syndrome.
abstract: BACKGROUND
  We sought to examine fundamental aspects of attention in children with Rett syndrome, a severely disabling neurodevelopmental disorder caused by spontaneous mutations in the X-linked MECP2 gene. To gauge their attention, we used eye tracking, which bypasses the profound impairments in expressive language and hand use in Rett syndrome. We report two aspects of attention-shifting and sustaining-basic abilities known to drive cognitive growth.
  
  
  METHODS
  Two groups were compared: those with Rett syndrome (N = 20; 3-15 years) and a typically developing comparison group (N = 14; 3-16 years), using a task in which an attractive central stimulus was followed, after a short gap, by a dynamic target presented to one side. Time to shift to the target location (reactive and anticipatory saccades) and time fixating the target were assessed.
  
  
  RESULTS
  Children with Rett syndrome were consistently slower to shift (largely because of fewer anticipations); their reactive saccades were also slower than those of typically developing children, but not significantly so. The Rett syndrome group spent considerable time looking at the target (over 75% of available time), although significantly less so than the typically developing group.
  
  
  CONCLUSIONS
  These findings indicate that children with Rett syndrome could maintain attention on a stimulus and orient relatively quickly to the appearance of a target in the visual field. However, they had difficulty in anticipating predictable events, a difficulty in endogenous attention that is likely to have deleterious implications for executive functioning.
authors.affiliation: Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York. Electronic address: susan.rose@einstein.yu.edu.
authors.forename: Susan A
authors.initials: SA
authors.lastname: Rose
authors.affiliation: Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York; Rett Syndrome Center, Department of Neurology, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York.
authors.forename: Aleksandra
authors.initials: A
authors.lastname: Djukic
authors.affiliation: Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York; Department of Social Sciences, Queensborough Community College/CUNY, Bayside, New York.
authors.forename: Jeffery J
authors.initials: JJ
authors.lastname: Jankowski
authors.affiliation: Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York.
authors.forename: Judith F
authors.initials: JF
authors.lastname: Feldman
authors.affiliation: Touro College, Kew Gardens Hills, New York.
authors.forename: Menachem
authors.initials: M
authors.lastname: Rimler
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D001289:Attention Deficit Disorder with Hyperactivity / Q000175:diagnosis* / Q000209:etiology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D051783:Methyl-CpG-Binding Protein 2 / Q000235:genetics; D009154:Mutation / Q000235:genetics; D010775:Photic Stimulation; D011930:Reaction Time / Q000502:physiology; D015518:Rett Syndrome / Q000150:complications* / Q000235:genetics; D012438:Saccades / Q000502:physiology; D013223:Statistics as Topic
chemical_list: C497392:MECP2 protein, human; D051783:Methyl-CpG-Binding Protein 2
keywords: Rett syndrome; anticipation; executive attention; eye tracking; orienting; shifting attention; sustaining attention