Visual evoked potentials detect cortical processing deficits in Rett syndrome.

ABSTRACT: OBJECTIVE:Rett syndrome (RTT) is a neurodevelopmental disorder caused by mutation of the X-linked MECP2 gene and characterized by developmental regression during the first few years of life. The objective of this study was to investigate if the visual evoked potential (VEP) could be used as an unbiased, quantitative biomarker to monitor brain function in RTT. METHODS:We recorded pattern-reversal VEPs in Mecp2 heterozygous female mice and 34 girls with RTT. The amplitudes and latencies of VEP waveform components were quantified, and were related to disease stage, clinical severity, and MECP2 mutation type in patients. Visual acuity was also assessed in both mice and patients by modulating the spatial frequency of the stimuli. RESULTS:Mecp2 heterozygous female mice and RTT patients exhibited

SUBMITTER: LeBlanc JJ 

PROVIDER: S-EPMC7374762 | biostudies-literature | 2015 Nov

REPOSITORIES: biostudies-literature