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Augmentation of auditory N1 in children with fragile X syndrome.
Abstract
We compared the N1 responses of the auditory event-related brain potentials (ERPs) in school-aged children with fragile X syndrome to age-matched controls in order to assess auditory processing. Event-related potentials to non-attended standard and deviant tone stimuli were recorded with EEG electrodes and here the standard tones were analysed. The amplitude of the N1 component to standard tones was significantly larger in children with fragile X syndrome than in control children. In addition, the global field power maximum of ERP corresponding to the N2 component was significantly (p<0.05) larger in fragile X children than in controls. The N2 scalp distribution in children with fragile X syndrome appeared more frontal than that in controls. Furthermore, the fragile X children exhibited no habituation of N1 and an absence of N2 sensitization for repeated tones. Increased responsiveness observed in the N1 evoked potential together with abnormal habituation of auditory responses in childhood may indicate increased sensory sensitivity for auditory stimuli in fragile X syndrome. The data, though very limited, suggest that stimulus processing in the auditory afferent pathways and/or in the corresponding cortical receiving areas is abnormal in children with fragile X syndrome.
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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 Castrén et al., 2003
These phenotypes in the mouse model are remarkably similar to those seen in humans with FXS.
“These phenotypes in the mouse model are remarkably similar to those seen in humans with FXS (Castrén et al., 2003; Ethridge et al., 2016; Wang et al., 2017).”
From The Perineuronal ‘Safety’ Net? Perineuronal Net Abnormalities in Neurological Disorders (2018) · cites this paper as Castrén et al., 2003
Reduced N1 habituation in Fmr1 KO mice resembles human FXS observations, providing an analogous functional sensory outcome measure in mice and humans (Castren et al., 2003; Van der Molen et al., 2012; Schneider et al., 2013).
“The reduced habituation of N1 amplitude in Fmr1 KO mice is similar to observations in humans with FXS providing, for the first time, an analogous functional sensory outcome measure in mice and humans (Castren et al., 2003; Van der Molen et al., 2012; Schneider et al., 2013).”
From Matrix metalloproteinase-9 deletion rescues auditory evoked potential habituation deficit in a mouse model of Fragile X Syndrome (2016) · cites this paper as Castren et al., 2003
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Metadata
- Authors
- Castrén, M., Pääkkönen, A., Tarkka, I. M., Ryynänen, M., & Partanen, J.
- Affiliations
- University of Eastern Finland (FI)
- Kuopio University Hospital (FI)
- Finnish Brain Research and Rehabilitation Center Neuron (FI)
- Oulu University Hospital (FI)
- Venue
- Brain Topography
- Published
- 2003-03
- Volume
- 15
- Issue
- 3
- Pages
- 165-171
- ISSN
- 0896-0267 (ISSN-L)
- 1573-6792
- OpenAlex
- W2293680727
- Cited by
- 123
- References
- 38
- Citations by year
- 2026: 5 · 2025: 9 · 2024: 6 · 2023: 3 · 2022: 11 · 2021: 8 · 2020: 9 · 2019: 9 · 2018: 7 · 2017: 14 · 2016: 6 · 2015: 4 · 2014: 5 · 2013: 3 · 2012: 8
- FWCI
- 0.88
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- Locations
- Brain Topography (publishedVersion)
- PubMed (publishedVersion)
- 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, auditory N1, auditory event-related potentials, auditory processing, N2 component, habituation, sensory sensitivity, children
- MeSH terms
- Acoustic Stimulation, Adolescent, Adult, Brain Mapping, Child, Evoked Potentials, Auditory, Fragile X Syndrome, Habituation, Psychophysiologic, Humans, Male, Reaction Time, Scalp, Case-Control Studies
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