Related Experiment Video
Updated: Nov 20, 2025
10:30
Simultaneous Recording of Electroretinography and Visual Evoked Potentials in Anesthetized Rats
Published on: July 1, 2016
12.7K
Multisite Study of Evoked Potentials in Rett Syndrome
Joni N Saby1, Timothy A Benke2, Sarika U Peters3
1Division of Radiology Research, Children's Hospital of Philadelphia, Philadelphia, PA.
Annals of Neurology
|January 22, 2021
Summary
Evoked potentials (EPs) show promise as biomarkers for Rett syndrome (RTT) severity. These tests objectively measure cortical function, aiding in treatment assessment and prognosis for RTT patients.
Area of Science:
- Neuroscience
- Biomarkers
- Clinical Trials
Background:
- Rett syndrome (RTT) is a rare neurodevelopmental disorder.
- Disease-modifying therapeutics for RTT are under development.
- Objective biomarkers are needed to assess treatment efficacy in RTT.
Purpose of the Study:
- To evaluate evoked potentials (EPs) as biomarkers of cortical function in RTT.
- To determine the utility of EPs for assessing disease severity and treatment response.
Main Methods:
- Yearly visual evoked potentials (VEPs) and auditory evoked potentials (AEPs) were recorded from individuals with RTT and controls.
- Data from the Rett Syndrome and Related Disorders Natural History Study were analyzed.
- Repeatability and clinical relevance of EP parameters were assessed.
Main Results:
- Individuals with RTT exhibited reduced VEP and AEP amplitude compared to controls.
- Lower EP amplitude correlated with greater RTT symptom severity and older age.
- Year 1 follow-up confirmed findings and demonstrated EP repeatability.
Conclusions:
- Evoked potentials show potential as objective biomarkers for RTT disease severity.
- Multisite EP assessments can aid in disease staging, prognosis, and therapy response evaluation.
