Brain Topography

, Volume 15, Issue 3, pp 165–171 | Cite as

Augmentation of Auditory N1 in Children with Fragile X Syndrome

  • Maija Castrén
  • Ari Pääkkönen
  • Ina M. Tarkka
  • Markku Ryynänen
  • Juhani Partanen


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.

Fragile X FMRP Event-related potentials N1 Mental retardation 


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Copyright information

© Human Sciences Press, Inc. 2003

Authors and Affiliations

  • Maija Castrén
    • 1
  • Ari Pääkkönen
    • 2
  • Ina M. Tarkka
    • 3
  • Markku Ryynänen
    • 4
  • Juhani Partanen
    • 2
  1. 1.A.I.Virtanen Institute,University of Kuopio,Finland
  2. 2.Department of Clinical Neurophysiology,Kuopio University Hospital and University of Kuopio,Finland
  3. 3.Brain Research and Rehabilitation Center Neuron,Kuopio,Finland
  4. 4.Department of Gynaecology,University Hospital of Oulu,Finland

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