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Original Article| Volume 57, P22-28, April 2016

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Aspects of Attention in Rett Syndrome

  • Susan A. Rose
    Correspondence
    Communications should be addressed to: Dr. Rose; Department of Pediatrics; Albert Einstein College of Medicine/Children's Hospital at Montefiore; Van Etten Building; 1300 Morris Park Avenue; Bronx, NY 10461.
    Affiliations
    Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York
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  • Aleksandra Djukic
    Affiliations
    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
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  • Jeffery J. Jankowski
    Affiliations
    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
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  • Judith F. Feldman
    Affiliations
    Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine/Children's Hospital at Montefiore, Bronx, New York
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  • Menachem Rimler
    Affiliations
    Touro College, Kew Gardens Hills, New York
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      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.

      Keywords

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      References

        • Rett A.
        On an unusual brain atrophy syndrome in hyperammonemia in childhood.
        Wien Med Wochenschr. 1966; 116: 723-726
        • Chahrour M.
        • Zoghbi H.Y.
        The story of Rett syndrome: from clinic to neurobiology.
        Neuron. 2007; 56: 422-437
        • Neul J.L.
        • Kaufmann W.E.
        • Glaze D.G.
        • et al.
        Rett syndrome: revised diagnostic criteria and nomenclature.
        Ann Neurol. 2010; 68: 944-950
        • Amir R.E.
        • Van den Veyver I.B.
        • Wan M.
        • Tran C.Q.
        • Francke U.
        • Zoghbi H.Y.
        Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein 2.
        Nat Genet. 1999; 23: 185-188
        • Kaufmann W.E.
        • Johnston M.V.
        • Blue M.E.
        MeCP2 expression and function during brain development: implications for Rett syndrome's pathogenesis and clinical evolution.
        Brain Dev. 2005; 27: S77-S87
        • Moretti P.
        • Levenson J.M.
        • Battaglia F.
        • et al.
        Learning and memory and synaptic plasticity are impaired in a mouse model of Rett syndrome.
        J Neurosci. 2006; 26: 319-327
        • Guy J.
        • Gan J.
        • Selfridge J.
        • Cobb S.
        • Bird A.
        Reversal of neurological defects in a mouse model of Rett syndrome.
        Science. 2007; 315: 1143-1147
        • Berger-Sweeney J.
        Cognitive deficits in Rett syndrome: what we know and what we need to know to treat them.
        Neurobiol Learn Mem. 2011; 96: 637-646
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        Attention and recognition memory in the first year of life: A longitudinal study of preterms and full-terms.
        Dev Psychol. 2001; 37: 135-151
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        Processing speed in the 1st year of life: A longitudinal study of preterm and full-term infants.
        Dev Psychol. 2002; 38: 895-902
        • Jacobson S.W.
        • Jacobson J.L.
        • Sokol R.J.
        • Martier S.S.
        • Ager J.W.
        Prenatal alcohol exposure and infant information processing ability.
        Child Dev. 1993; 64: 1706-1721
        • de Haan M.
        • Bauer P.J.
        • Georgieff M.K.
        • Nelson C.A.
        Explicit memory in low-risk infants aged 19 months born between 27 and 42 weeks of gestation.
        Dev Med Child Neurol. 2000; 42: 304-312
        • Fagan J.F.
        Intelligence in infancy.
        in: The Cambridge Handbook of Intelligence [Internet]. Cambridge University Press, New York2011: 130-143
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        A cognitive approach to the development of early language.
        Child Dev. 2009; 80: 134-150
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        Implications of infant cognition for executive functions at age 11.
        Psychol Sci. 2012; 23: 1345-1355
        • Richmond J.
        • Sowerby P.
        • Colombo M.
        • Hayne H.
        The effect of familiarization time, retention interval, and context change on adults' performance in the visual paired-comparison task.
        Dev Psychobiol. 2004; 44: 146-155
        • Djukic A.
        • Valicenti McDermott M.
        Social preferences in Rett syndrome.
        Pediatr Neurol. 2012; 46: 240-242
        • Djukic A.
        • Valicenti McDermott M.
        • Mavrommatis K.
        • Martins C.L.
        Rett syndrome: Basic features of visual processing–a pilot study of eye-tracking.
        Pediatr Neurol. 2012; 47: 25-29
        • Rose S.A.
        • Djukic A.
        • Jankowski J.J.
        • Feldman J.F.
        • Fishman I.
        • Valicenti-Mcdermott M.
        Rett syndrome: an eye-tracking study of attention and recognition memory.
        Dev Med Child Neurol. 2013; 55: 364-371
        • Basson R.
        • Berman J.
        • Burnett A.
        • et al.
        Report of the International Consensus Development Conference on female sexual dysfunction: definitions and classifications.
        J Urol. 2000; 163: 888-893
        • Djukic A.
        • Rose S.A.
        • Jankowski J.J.
        • Feldman J.F.
        Rett syndrome: recognition of facial expression and its relation to scanning patterns.
        Pediatr Neurol. 2014; 51: 650-656
        • Cornish K.
        • Cole V.
        • Longhi E.
        • Karmiloff-Smith A.
        • Scerif G.
        Does attention constrain developmental trajectories in fragile X syndrome? A 3-year prospective longitudinal study.
        Am J Intellect Dev Disabil. 2012; 117: 103-120
        • Scerif G.
        Attention trajectories, mechanisms and outcomes: at the interface between developing cognition and environment.
        Dev Sci. 2010; 13: 805-812
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        • Van Rossem R.
        Pathways from prematurity and infant abilities to later cognition.
        Child Dev. 2005; 76: 1172-1184
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        • Van Rossem R.
        A cognitive cascade in infancy: Pathways from prematurity to later mental development.
        Intelligence. 2008; 36: 367-378
        • Rose S.A.
        • Feldman J.F.
        • Jankowski J.J.
        Information processing in toddlers: continuity from infancy and persistence of preterm deficits.
        Intelligence. 2009; 37: 311-320
        • Fan J.
        • McCandliss B.D.
        • Fossella J.
        • Flombaum J.I.
        • Posner M.I.
        The activation of attentional networks.
        Neuroimage. 2005; 26: 471-479
        • Posner M.I.
        • Petersen S.
        The attention system of the human brain.
        Annu Rev Neurosci. 1990; 13: 25-42
        • Sheese B.E.
        • Rothbart M.K.
        • Posner M.I.
        • White L.K.
        • Fraundorf S.H.
        Executive attention and self-regulation in infancy.
        Infant Behav Dev. 2008; 31: 501-510
        • Rothbart M.K.
        • Ellis L.K.
        • Rueda M.R.
        • Posner M.I.
        Developing mechanisms of temperamental effortful control.
        J Pers. 2003; 71: 1113-1143
        • Sarter M.
        • Givens B.
        • Bruno J.P.
        The cognitive neuroscience of sustained attention: where top-down meets bottom-up.
        Brain Res Brain Res Rev. 2001; 35: 146-160
        • Kovacs A.M.
        • Mehler J.
        Cognitive gains in 7-month-old bilingual infants.
        Proc Natl Acad Sci U S A. 2009; 106: 6556-6560
        • Dougherty T.M.
        • Haith M.M.
        Infant expectations and reaction time as predictors of childhood speed of processing and IQ.
        Dev Psychol. 1997; 33: 146-155
        • Perchet C.
        • Garcia-Larrea L.
        Learning to react: anticipatory mechanisms in children and adults during a visuospatial attention task.
        Clin Neurophysiol. 2005; 116: 1906-1917
        • Kaufmann W.E.
        • Tierney E.
        • Rohde C.A.
        • et al.
        Social impairments in Rett syndrome: characteristics and relationship with clinical severity.
        J Intellect Disabil Res. 2012; 56: 233-247
        • Haith M.M.
        • Hazan C.
        • Goodman G.S.
        Expectation and anticipation of dynamic visual events by 3.5-month-old babies.
        Child Dev. 1988; 59: 467-479
        • Petersen S.E.
        • Posner M.I.
        The attention system of the human brain: 20 years after.
        Annu Rev Neurosci. 2012; 35: 73-89
        • Alvarez J.A.
        • Emory E.
        Executive function and the frontal lobes: a meta-analytic review.
        Neuropsychol Rev. 2006; 16: 17-42