Visual Evoked Potentials as a Readout of Cortical Function in Infants With Tuberous Sclerosis Complex

Tuberous sclerosis complex is an autosomal dominant genetic disorder that confers a high risk for neurodevelopmental disorders, such as autism spectrum disorder and intellectual disability. Studies have demonstrated specific delays in visual reception skills that may predict the development of autis...

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Bibliographic Details
Published in:Journal of child neurology 2016-02, Vol.31 (2), p.195-202
Main Authors: Varcin, Kandice J., Nelson, Charles A., Ko, Jordan, Sahin, Mustafa, Wu, Joyce Y., Jeste, Shafali Spurling
Format: Article
Language:English
Subjects:
Cerebral Cortex - physiopathology
Electroencephalography
Evoked Potentials, Visual
Female
Humans
Infant
Longitudinal Studies
Male
Photic Stimulation
Tuberous Sclerosis - complications
Tuberous Sclerosis - physiopathology
Visual Perception - physiology
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Summary:Tuberous sclerosis complex is an autosomal dominant genetic disorder that confers a high risk for neurodevelopmental disorders, such as autism spectrum disorder and intellectual disability. Studies have demonstrated specific delays in visual reception skills that may predict the development of autism spectrum disorder and intellectual disability. Based on evidence for alterations in the retinogeniculate pathway in animal models of tuberous sclerosis complex, we asked whether children with tuberous sclerosis complex demonstrate alterations in early visual processing that may undermine the development of higher-level visual behaviors. Pattern-reversal visual evoked potentials were recorded in infants with tuberous sclerosis complex (n = 16) and typically developing infants (n = 18) at 12 months of age. Infants with tuberous sclerosis complex demonstrated remarkably intact visual evoked potentials even within the context of intellectual disability and epilepsy. Infants with tuberous sclerosis complex show intact visual cortical processing, suggesting that delays in visually mediated behaviors in tuberous sclerosis complex may not be rooted in early visual processing deficits.
ISSN:0883-0738
1708-8283
DOI:10.1177/0883073815587328