5. Why study event related potentials in Rett Syndrome?
Event related Potentials (ERP) are obtained from scalp EEG's recorded during the first 0.5 s after an event. Evoked Potentials are much earlier: for Auditory Potentials, the first millisecond after a click, reflecting activity in the brainstem. ERP, however, are influenced by cortical activity and are of great interest in conditions with cognitive impairment, like autism. Characteristic ERP abnormalities have been described in autistic Spectrum Disorders (ASD) Rett Syndrome (RS) has many symptoms in common with ASD. Furthermore, being due to a known genetic mutation, RS has a mouse model. ERP abnormalities have been described in mice. ERP from Rett Syndrome patients are somewhat ambiguous, especially in the light of studies of the animal model. ERP have been looked at with MEG in ASD; our study would study both scalp EEG and MEG.
Clinical Neurophysiology