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Aspects of Attention in Rett Syndrome
Institution:1. Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children''s Hospital at Montefiore, Bronx, New York;2. Rett Syndrome Center, Department of Neurology, Montefiore Medical Center, Albert Einstein College of Medicine/Children''s Hospital at Montefiore, Bronx, New York;3. Department of Social Sciences, Queensborough Community College/CUNY, Bayside, New York;4. Touro College, Kew Gardens Hills, New York;1. Congenital Cardiology Directorate, Royal Brompton and Harefield NHS Trust, London, United Kingdom;2. Department of Metabolic Medicine, Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom;1. Department of Neuroscience and Cell Biology, Child Health Institute of New Jersey, Rutgers University Robert Wood Johnson Medical School, 89 French Street, Room 3277, New Brunswick NJ 08901, USA;1. Centre for Rett Syndrome, Kennedy Centre, Department of Clinical Genetics, Rigshospitalet, University of Copenhagen, Gl. Landevej 7, 2600 Glostrup, Denmark;2. Molecular Genetic Laboratory, Department of Clinical Genetics, 4062, Rigshospitalet, University of Copenhagen, Blegdamsvej 9, 2100 Copenhagen Ø, Denmark;3. Department of Paediatrics, Roskilde Hospital, University of Copenhagen, Køgevej 7-13, 4000 Roskilde, Denmark;1. Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India;2. Department of Physiology, All India Institute of Medical Sciences, New Delhi, India;3. Department of Biostatistics, All India Institute of Medical Sciences, New Delhi, India;1. Department of Physiotherapy, Faculty of Health Science, University of Málaga, Biomedical Research Institute of Málaga (IBIMA), Málaga, Spain;2. Escola Superior de Saúde Atlantica, Oeiras, Portugal;3. Atlântica- Instituto Universitário, Oeiras, Portugal;4. Research Institute for Medicines (iMed.ULisboa), Faculty of Pharmacy, Universidade de Lisboa, Lisboa, Portugal
Abstract:BackgroundWe 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.MethodsTwo 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.ResultsChildren 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.ConclusionsThese 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.
Keywords:Rett syndrome  shifting attention  sustaining attention  orienting  anticipation  eye tracking  executive attention
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