Auditory and visual cortical activity during selective attention in fragile X syndrome: a cascade of processing deficiencies.
Abstract
Objective This study examined whether attention deficits in fragile X syndrome (FXS) can be traced back to abnormalities in basic information processing.Method Sixteen males with FXS and 22 age-matched control participants (mean age 29 years) performed a standard oddball task to examine selective attention in both auditory and visual modalities. Five FXS males were excluded from analysis because they performed below chance level on the auditory task. ERPs were recorded to investigate the N1, P2, N2b, and P3b components.Results N1 and N2b components were significantly enhanced in FXS males to both auditory and visual stimuli. Interestingly, in FXS males, the P3b to auditory stimuli was significantly reduced relative to visual stimuli. These modality differences in information processing corresponded to behavioral results, showing more errors on the auditory than on the visual task.Conclusions The current findings suggest that attentional impairments in FXS at the behavioral level can be traced back to abnormalities in event-related cortical activity. These information processing abnormalities in FXS may hinder the allocation of attentional resources needed for optimal processing at higher-levels.Significance These findings demonstrate that auditory information processing in FXS males is critically impaired relative to visual information processing.
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Auditory hypersensitivity is common in humans with FXS as measured by increased sound evoked responses, reduced habituation and expansion of brain regions activated by auditory stimuli.
“Auditory hypersensitivity is common in humans with FXS as measured by increased sound evoked responses, reduced habituation and expansion of brain regions activated by auditory stimuli (Rojas et al., 2001; Castrén et al., 2003; Van der Molen et al., 2012; Ethridge et al., 2016).”
From The Perineuronal ‘Safety’ Net? Perineuronal Net Abnormalities in Neurological Disorders (2018) · cites this paper as Van der Molen et al., 2012
Cites
Updating P300: An integrative theory of P3a and P3b
Polich, J. (2007) · Clinical Neurophysiology · doi:10.1016/j.clinph.2007.04.019 · Cited by 7,919 · see citing works
Peabody Picture Vocabulary Test
Campbell, J. M., & Dommestrup, A. K. (2010) · The Corsini Encyclopedia of Psychology · doi:10.1002/9780470479216.corpsy0649 · Cited by 3,787 · see citing works
Verkerk, A. J. M. H., Pieretti, M., Sutcliffe, J. S., Fu, Y.-H., Kuhl, D. P. A., Pizzuti, A., Reiner, O., Richards, S., Victoria, M. F., Zhang, F., et al. (1991) · Cell · doi:10.1016/0092-8674(91)90397-h · Cited by 3,481 · see citing works
Näätänen, R., & Picton, T. (1987) · Psychophysiology · doi:10.1111/j.1469-8986.1987.tb00311.x · Cited by 3,321 · see citing works
The mismatch negativity (MMN) in basic research of central auditory processing: A review
Näätänen, R., Paavilainen, P., Rinne, T., & Alho, K. (2007) · Clinical Neurophysiology · doi:10.1016/j.clinph.2007.04.026 · Cited by 2,644 · see citing works
Influence of cognitive control and mismatch on the N2 component of the ERP: A review
Folstein, J. R., & Van Petten, C. (2008) · Psychophysiology · doi:10.1111/j.1469-8986.2007.00602.x · Cited by 2,567 · see citing works
Picton, T. W., Bentin, S., Berg, P., Donchin, E., Hillyard, S. A., Johnson, R., Miller, G. A., Ritter, W., Ruchkin, D. S., Rugg, M. D., et al. (2000) · Psychophysiology · doi:10.1111/1469-8986.3720127 · Cited by 2,157 · see citing works
Decision making, the P3, and the locus coeruleus–norepinephrine system
Nieuwenhuis, S., Aston-Jones, G., & Cohen, J. D. (2005) · Psychological Bulletin · doi:10.1037/0033-2909.131.4.510 · Cited by 1,678 · see citing works
The mGluR theory of fragile X mental retardation
Bear, M. F., Huber, K. M., & Warren, S. T. (2004) · Trends in Neurosciences · doi:10.1016/j.tins.2004.04.009 · Cited by 1,580 · see citing works
Absence of expression of the FMR-1 gene in fragile X syndrome
Pieretti, M., Zhang, F., Fu, Y.-H., Warren, S. T., Oostra, B. A., Caskey, C. T., & Nelson, D. L. (1991) · Cell · doi:10.1016/0092-8674(91)90125-i · Cited by 1,476 · see citing works
GABA: A Pioneer Transmitter That Excites Immature Neurons and Generates Primitive Oscillations
Ben-Ari, Y., Gaiarsa, J.-L., Tyzio, R., & Khazipov, R. (2007) · Physiological Reviews · doi:10.1152/physrev.00017.2006 · Cited by 1,304 · see citing works
Duncan, C. C., Barry, R. J., Connolly, J. F., Fischer, C., Michie, P. T., Näätänen, R., Polich, J., Reinvang, I., & Van Petten, C. (2009) · Clinical Neurophysiology · doi:10.1016/j.clinph.2009.07.045 · Cited by 1,191 · see citing works
Neural Mechanisms of Involuntary Attention to Acoustic Novelty and Change
Escera, C., Alho, K., Winkler, I., & Näätänen, R. (1998) · Journal of Cognitive Neuroscience · doi:10.1162/089892998562997 · Cited by 916 · see citing works
A review of the evidence for P2 being an independent component process: Age, sleep and modality
Crowley, K. E., & Colrain, I. M. (2004) · Clinical Neurophysiology · doi:10.1016/j.clinph.2003.11.021 · Cited by 853 · see citing works
Neural mechanisms for detecting and remembering novel events
Ranganath, C., & Rainer, G. (2003) · Nature Reviews Neuroscience · doi:10.1038/nrn1052 · Cited by 779 · see citing works
Involuntary Attention and Distractibility as Evaluated with Event-Related Brain Potentials
Escera, C., Alho, K., Schröger, E., & Winkler, I. (2000) · Audiology and Neurotology · doi:10.1159/000013877 · Cited by 690 · see citing works
Analysis of neocortex in three males with the Fragile X syndrome
Hinton, V. J., Brown, W. T., Wisniewski, K., & Rudelli, R. D. (1991) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320410306 · Cited by 527 · see citing works
Fragile X Syndrome: Diagnosis, Treatment, And Research
Silverman, & Cronister, A. (1991) · Johns Hopkins University Press eBooks · Cited by 481 · see citing works
Näätänen, R., Kujala, T., & Winkler, I. (2010) · Psychophysiology · doi:10.1111/j.1469-8986.2010.01114.x · Cited by 458 · see citing works
Nieuwenhuis, S., de Geus, E. J. C., & Aston‐Jones, G. (2010) · Psychophysiology · doi:10.1111/j.1469-8986.2010.01057.x · Cited by 451 · see citing works
Rho proteins, mental retardation and the cellular basis of cognition
Ramakers, G. J. A. (2002) · Trends in Neurosciences · doi:10.1016/s0166-2236(00)02118-4 · Cited by 374 · see citing works
Self-citationFragile X mice develop sensory hyperreactivity to auditory stimuli
Chen, L., & Toth, M. (2001) · Neuroscience · doi:10.1016/s0306-4522(01)00036-7 · Cited by 345 · see citing works
Fragile X syndrome and autism at the intersection of genetic and neural networks
Belmonte, M. K., & Bourgeron, T. (2006) · Nature Neuroscience · doi:10.1038/nn1765 · Cited by 345 · see citing works
Decreased expression of the GABAA receptor in fragile X syndrome
D'Hulst, C., De Geest, N., Reeve, S. P., Van Dam, D., De Deyn, P. P., Hassan, B. A., & Kooy, R. F. (2006) · Brain Research · doi:10.1016/j.brainres.2006.08.115 · Cited by 318 · see citing works
Synaptic regulation of protein synthesis and the fragile X protein
Greenough, W. T., Klintsova, A. Y., Irwin, S. A., Galvez, R., Bates, K. E., & Weiler, I. J. (2001) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.141145998 · Cited by 317 · see citing works
Sensorimotor gating abnormalities in young males with fragile X syndrome and Fmr1-knockout mice.
Frankland, P. W., Wang, Y., Rosner, B., Shimizu, T., Balleine, B. W., Dykens, E. M., Ornitz, E. M., & Silva, A. J. (2004) · Molecular Psychiatry · doi:10.1038/sj.mp.4001432 · Cited by 275 · see citing works
On the number of trials needed for P300
Cohen, J., & Polich, J. M. (1997) · International Journal of Psychophysiology · doi:10.1016/s0167-8760(96)00743-x · Cited by 255 · see citing works
Escera, C., Yago, E., & Alho, K. (2001) · European Journal of Neuroscience · doi:10.1046/j.0953-816x.2001.01707.x · Cited by 234 · see citing works
Gothelf, D., Furfaro, J. A., Hoeft, F., Eckert, M. A., Hall, S. S., O'Hara, R. M., Erba, H. W., Ringel, J., Hayashi, K. M., Patnaik, S., et al. (2007) · Annals of Neurology · doi:10.1002/ana.21243 · Cited by 204 · see citing works
Dendritic pathology in mental retardation: from molecular genetics to neurobiology
Dierssen, M., & Ramakers, G. J. A. (2006) · Genes Brain & Behavior · doi:10.1111/j.1601-183x.2006.00224.x · Cited by 190 · see citing works
Self-citationA neuropsychological profile of attention deficits in young males with fragile X syndrome
Munir, F., Cornish, K. M., & Wilding, J. P. H. (2000) · Neuropsychologia · doi:10.1016/s0028-3932(00)00036-1 · Cited by 188 · see citing works
Frequency Change Detection in Human Auditory Cortex
May, P., Tiitinen, H., Ilmoniemi, R. J., Nyman, G., Taylor, J. G., & Näätänen, R. (1999) · Journal of Computational Neuroscience · doi:10.1023/a:1008896417606 · Cited by 175 · see citing works
The GABAA receptor: a novel target for treatment of fragile X?
D’Hulst, C., & Kooy, R. F. (2007) · Trends in Neurosciences · doi:10.1016/j.tins.2007.06.003 · Cited by 166 · see citing works
Pfeiffer, B. E., & Huber, K. M. (2007) · The Journal of Neuroscience · doi:10.1523/jneurosci.0054-07.2007 · Cited by 154 · see citing works
Behavioral phenotype of fragile X syndrome: DSM‐III‐R autistic behavior in male children
Reiss, A. L., & Freund, L. S. (1992) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320430106 · Cited by 138 · see citing works
Therapeutic implications of the mGluR theory of fragile X mental retardation
Bear, M. F. (2005) · Genes, Brain and Behavior · doi:10.1111/j.1601-183x.2005.00135.x · Cited by 137 · see citing works
Attention and language in fragile X
Cornish, K. M., Sudhalter, V., & Turk, J. (2004) · Mental Retardation and Developmental Disabilities Research Reviews · doi:10.1002/mrdd.20003 · Cited by 136 · see citing works
Further delineation of the executive deficit in males with fragile-X syndrome
Wilding, J. P. H., Cornish, K. M., & Munir, F. (2002) · Neuropsychologia · doi:10.1016/s0028-3932(01)00212-3 · Cited by 128 · see citing works
Augmentation of auditory N1 in children with fragile X syndrome.
Castrén, M., Pääkkönen, A., Tarkka, I. M., Ryynänen, M., & Partanen, J. (2003) · Brain Topography · doi:10.1023/a:1022606200636 · Cited by 123 · see citing works
Dani, V. S., & Nelson, S. B. (2009) · Journal of Neuroscience · doi:10.1523/jneurosci.1019-09.2009 · Cited by 121 · see citing works
Galvez, R., Gopal, A. R., & Greenough, W. T. (2003) · Brain Research · doi:10.1016/s0006-8993(03)02363-1 · Cited by 118 · see citing works
Strobel, A., Debener, S., Sorger, B., Peters, J. C., Kranczioch, C., Hoechstetter, K., Engel, A. K., Brocke, B., & Goebel, R. (2008) · NeuroImage · doi:10.1016/j.neuroimage.2007.10.065 · Cited by 104 · see citing works
Hanson, J. E., Blank, M., Valenzuela, R. A., Garner, C. C., & Madison, D. V. (2006) · The Journal of Physiology · doi:10.1113/jphysiol.2006.114868 · Cited by 94 · see citing works
Moon, J., Beaudin, A. E., Verosky, S. C., Driscoll, L. L., Weiskopf, M., Levitsky, D. A., Crnic, L. S., & Strupp, B. J. (2006) · Behavioral Neuroscience · doi:10.1037/0735-7044.120.6.1367 · Cited by 88 · see citing works
Profiling Fragile X Syndrome in males: Strengths and weaknesses in cognitive abilities
van der Molen, M. J. W., Huizinga, M., Huizenga, H. M., Ridderinkhof, K. R., van der Molen, M. W., Hamel, B. J. C., Curfs, L., & Ramakers, G. J. A. (2009) · Research in Developmental Disabilities · doi:10.1016/j.ridd.2009.10.013 · Cited by 80 · see citing works
Self-citationPrepulse inhibition in fragile X syndrome: Feasibility, reliability, and implications for treatment
Hessl, D. R., Berry‐Kravis, E., Cordeiro, L., Yuhas, J. M., Ornitz, E. M., Campbell, A., Chruscinski, E., Hervey, C. M., Long, J. M., & Hagerman, R. J. (2008) · American Journal of Medical Genetics Part B Neuropsychiatric Genetics · doi:10.1002/ajmg.b.30858 · Cited by 77 · see citing works
Latent inhibition mediates N1 attenuation to repeating sounds
Sable, J. J., Low, K. A., Maclin, E. L., Fabiani, M., & Gratton, G. (2004) · Psychophysiology · doi:10.1111/j.1469-8986.2004.00192.x · Cited by 77 · see citing works
Scerif, G., Cornish, K. M., Wilding, J. P. H., Driver, J., & Karmiloff‐Smith, A. (2007) · Neuropsychologia · doi:10.1016/j.neuropsychologia.2006.12.005 · Cited by 74 · see citing works
Auditory evoked magnetic fields in adults with fragile X syndrome.
Rojas, D. C., Benkers, T. L., Rogers, S. J., Teale, P. D., Reite, M. L., & Hagerman, R. J. (2001) · Neuroreport · doi:10.1097/00001756-200108080-00056 · Cited by 73 · see citing works
In vivo brain anatomy of adult males with Fragile X syndrome: An MRI study
Hallahan, B. P., Craig, M., Toal, F., Daly, E., Moore, C. J., Ambikapathy, A., Robertson, D., Murphy, K. C., & Murphy, D. (2010) · NeuroImage · doi:10.1016/j.neuroimage.2010.08.015 · Cited by 73 · see citing works
Trajectory of adaptive behavior in males with fragile X syndrome
Dykens, E. M., Hodapp, R. M., Ort, S. I., et al. (1993) · Journal of Autism and Developmental Disorders · doi:10.1007/bf01066423 · Cited by 65 · see citing works
The development of visual- and auditory processing in Rett syndrome: An ERP study
Stauder, J. E. A., Smeets, E., van Mil, S. G. M., & Curfs, L. (2006) · Brain and Development · doi:10.1016/j.braindev.2006.02.011 · Cited by 60 · see citing works
Self-citationMaes, B., Fryns, J. P., Walleghem, M. V., & BERGHE, H. V. D. (1994) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320500211 · Cited by 57 · see citing works
P3 Abnormality in fragile X syndrome
Clair, D. M. S., Blackwood, D., Oliver, C., & Dickens, P. (1987) · Biological Psychiatry · doi:10.1016/0006-3223(87)90148-x · Cited by 56 · see citing works
Sullivan, K. M., Hatton, D. D., Hammer, J., Sideris, J. H., Hooper, S. R., Ornstein, P. A., & Bailey, D. B. (2007) · American Journal of Medical Genetics Part B Neuropsychiatric Genetics · doi:10.1002/ajmg.b.30504 · Cited by 50 · see citing works
Gratton, G., COLES, M. G. H., & Donchin, E. (1989) · Psychophysiology · doi:10.1111/j.1469-8986.1989.tb03160.x · Cited by 47 · see citing works
Multi-modal P3 deflation of event-related brain activity in Prader–Willi syndrome
Stauder, J. E. A., Brinkman, M. J. R., & Curfs, L. (2002) · Neuroscience Letters · doi:10.1016/s0304-3940(02)00377-4 · Cited by 38 · see citing works
Self-citationBertone, A., Hanck, J., Kogan, C. S., Chaudhuri, A., & Cornish, K. M. (2010) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-010-1109-5 · Cited by 19 · see citing works
Bertone, A., Hanck, J., Kogan, C. S., Chaudhuri, A., & Cornish, K. M. (2010) · Journal of Autism and Developmental Disorders · doi:10.1007/s10803-010-1110-z · Cited by 18 · see citing works
Lobar holoprosencephaly in an infant born to a mother with classic phenylketonuria
Keller, K. M., McCune, H., Williams, C. A., & Muenke, M. (2000) · American Journal of Medical Genetics · doi:10.1002/1096-8628(20001113)95:2 · Cited by 12 · see citing works
Mechanisms, models, and mental retardation
Fisch, G. S. (2000) · American Journal of Medical Genetics · doi:10.1002/1096-8628(20001023)94:5 · Cited by 3 · see citing works
Irwin (2001) · doi:10.1002/1096-8628(20010115)98:2
Turner (1996) · doi:10.1002/(sici)1096-8628(19960712)64:1
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- Authors
- Van der Molen, M. J. W., Van der Molen, M. W., Ridderinkhof, K. R., Hamel, B. C. J., Curfs, L. M. G., & Ramakers, G. J. A.
- Affiliations
- University of Amsterdam (NL)
- Radboud University Nijmegen (NL)
- Maastricht University Medical Centre (NL)
- Venue
- Clinical Neurophysiology
- Publisher
- Elsevier BV
- Published
- 2012-04
- Volume
- 123
- Issue
- 4
- Pages
- 720-729
- ISSN
- 1388-2457 (ISSN-L)
- OpenAlex
- W2004021577
- Cited by
- 88
- References
- 63
- Citations by year
- 2026: 1 · 2025: 8 · 2024: 4 · 2023: 6 · 2022: 10 · 2021: 8 · 2020: 2 · 2019: 6 · 2018: 6 · 2017: 6 · 2016: 6 · 2015: 6 · 2014: 7 · 2013: 3 · 2012: 8
- FWCI
- 3.78
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Clinical Neurophysiology (publishedVersion)
- PubMed (publishedVersion)
- UvA-DARE (University of Amsterdam) (publishedVersion)
- Research Publications (Maastricht University) (submittedVersion)
- UvA-DARE (University of Amsterdam) (submittedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Neuroscience › Cognitive Neuroscience › Autism Spectrum Disorder Research
- Life Sciences › Neuroscience › Developmental Neuroscience › Williams Syndrome Research
- Keywords
- fragile X syndrome, selective attention, auditory processing, visual processing, event-related potentials, P3b, N2b, oddball paradigm, modality differences, attention deficits
- MeSH terms
- Acoustic Stimulation, Adolescent, Adult, Aging, Attention, Auditory Cortex, Auditory Perception, Electroencephalography, Evoked Potentials, Evoked Potentials, Auditory, Evoked Potentials, Visual, Fragile X Syndrome, Humans, Male, Middle Aged, Neuropsychological Tests, Photic Stimulation, Psychomotor Performance, Reaction Time, Regression Analysis, Sensation Disorders, Visual Cortex, Visual Perception, Young Adult
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