Abstract Details

Title Resting-State EEG Abnormalities in Children with Rett Syndrome

Topic Child Neurology and Developmental Neurology

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 7-035

Objective To examine baseline patterns of neural activity in children with Rett syndrome.

Background Rett syndrome (RTT) is a form of developmental encephalopathy characterized by regression, epilepsy, and other signs of global brain dysfunction. Scalp electroencephalography (EEG) has been used to study the neurophysiological underpinnings of various RTT symptom domains. This study examined resting-state neural activity in children with RTT syndrome in relation to clinical severity.

Design/Methods Dense-array scalp EEG was collected from 58 children with RTT syndrome and 20 age-matched, typically-developing (TD) controls. In this preliminary analysis, 20 children (15 RTT, 5 TD) were studied. Resting-state EEG (~15 minutes/participant) was acquired at 512 Hz and processed using automated techniques. The power spectrum was obtained from 10-20 electrodes using fast Fourier transformation. Group comparisons (RTT vs. TD) were performed for standard frequency bands and the derived alpha/delta power ratio (ADR). Clinical severity was measured using the Clinical Severity Score (CSS).

Results Relative delta power (0-4 Hz) was diffusely increased in RTT compared to TD participants, maximally over the bi-temporal scalp region (RTT: 0.48 ± 0.09, TD: 0.37 ± 0.03, p<0.01). Widespread decrements in relative alpha power (8-13 Hz) were observed between RTT and TD participants, prominently over the centro-parietal midline (RTT: 0.17 ± 0.04, TD: 0.23 ± 0.07, p=0.04) and posterior scalp regions. The ADR was significantly reduced among RTT participants at the whole-scalp level (RTT: 0.36 ± 0.03, TD: 0.56 ± 0.10, p<0.001) and within all sub-regions, excluding the frontal scalp. Significant negative correlations were observed between CSS and alpha power in the centro-parietal midline (r=-0.59, p=0.02), and bi-temporal (r=-0.58,p=0.02), and posterior (r=-0.51, p<0.05) scalp regions.

Conclusions

Children with RTT syndrome exhibited widespread EEG slowing over bi-temporal and posterior scalp regions compared to TD controls. Posterior relative alpha power may represent a biomarker for clinical severity among children with RTT syndrome.

Authors/Disclosures
Samuel Tomlinson (University of Rochester School of Medicine and Dentistry) PRESENTER	No disclosure on file
	No disclosure on file
Clare Cutri-French	No disclosure on file
Dallas Armstrong, MD (CHOP)	The institution of Dr. Armstrong has received research support from Taysha.
Eric D. Marsh, MD, PhD (Children's Hospital of Philadlephia)	Dr. Marsh has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia Pharmacuticals. Dr. Marsh has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Stoke Therapeutics. Dr. Marsh has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Acadia Pharmaceuticals. The institution of Dr. Marsh has received research support from NIH. The institution of Dr. Marsh has received research support from Rett Syndrome Research Trust. The institution of Dr. Marsh has received research support from International Rett Syndrome Foundation. The institution of Dr. Marsh has received research support from Eagles Autism Challenge. The institution of Dr. Marsh has received research support from LouLou Foundation. The institution of Dr. Marsh has received research support from International CDKL5 Resarch Foundation. The institution of Dr. Marsh has received research support from Acadia Pharmaceuticals. The institution of Dr. Marsh has received research support from Marinus. The institution of Dr. Marsh has received research support from Stoke Therapeutics. The institution of Dr. Marsh has received research support from Takeda Pharmaceuticals. Dr. Marsh has received personal compensation in the range of $500-$4,999 for serving as a Grant Review with NIH. Dr. Marsh has received personal compensation in the range of $5,000-$9,999 for serving as a Expert Witness with Department of Human Services. Dr. Marsh has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Medscape.

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