{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T14:32:26Z","timestamp":1781620346855,"version":"3.54.5"},"reference-count":27,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,5,9]],"date-time":"2024-05-09T00:00:00Z","timestamp":1715212800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"DOI":"10.13039\/100009633","name":"Eunice Kennedy Shriver National Institute of Child Health and Human Development","doi-asserted-by":"publisher","award":["5U54HD061222\u201015"],"award-info":[{"award-number":["5U54HD061222\u201015"]}],"id":[{"id":"10.13039\/100009633","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>This study was undertaken to characterize quantitative electroencephalographic (EEG) features in participants from the Natural history study of RTT and Related Disorders and to assess the potential for these features to act as objective measures of cortical function for Rett syndrome (RTT).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>EEG amplitude and power features were derived from the resting EEG of 60 females with RTT (median age\u2009=\u200910.7\u2009years) and 26 neurotypical females (median age\u2009=\u200910.6\u2009years). Analyses focus on group differences and within the RTT group, associations between the EEG parameters and clinical severity. For a subset of participants (n\u2009=\u200920), follow\u2010up data were available for assessing the reproducibility of the results and the stability in the parameters over 1 year.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Compared to neurotypical participants, participants with RTT had greater amplitude variability and greater low\u2010frequency activity as reflected by greater delta power, more negative 1\/f slope, and lower theta\/delta, alpha\/delta, beta\/delta, alpha\/theta, and beta\/theta ratios. Greater delta power, more negative 1\/f slope, and lower power ratios were associated with greater severity. Analyses of year 1 data replicated the associations between 1\/f slope and power ratios and clinical severity and demonstrated good within\u2010subject consistency in these measures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>Overall, group comparisons reflected a greater predominance of lower versus higher frequency activity in participants with RTT, which is consistent with prior clinical interpretations of resting EEG in this population. The observed associations between the EEG power measures and clinical assessments and the repeatability of these measures underscore the potential for EEG to provide an objective measure of cortical function and clinical severity for RTT. ANN NEUROL 2024;96:175\u2013186<\/jats:p><\/jats:sec>","DOI":"10.1002\/ana.26948","type":"journal-article","created":{"date-parts":[[2024,5,9]],"date-time":"2024-05-09T09:14:27Z","timestamp":1715246067000},"page":"175-186","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["<scp>Electroencephalographic<\/scp> Correlates of Clinical Severity in the Natural history study of RTT and Related Disorders"],"prefix":"10.1002","volume":"96","author":[{"given":"Joni N.","family":"Saby","sequence":"first","affiliation":[{"name":"Department of Radiology Children's Hospital of Philadelphia  Philadelphia PA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick J.","family":"Mulcahey","sequence":"additional","affiliation":[{"name":"Duke University School of Medicine  Durham NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy A.","family":"Benke","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Neurology, Pharmacology, and Otolaryngology School of Medicine and Children's Hospital Colorado, University of Colorado  Aurora CO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarika U.","family":"Peters","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Vanderbilt University Medical Center, Vanderbilt Kennedy Center  Nashville TN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shannon M.","family":"Standridge","sequence":"additional","affiliation":[{"name":"Cincinnati Children's Hospital Medical Center Division of Neurology and University of Cincinnati College of Medicine  Cincinnati OH USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David N.","family":"Lieberman","sequence":"additional","affiliation":[{"name":"Department of Neurology Boston Children's Hospital  Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra P.","family":"Key","sequence":"additional","affiliation":[{"name":"Department of Hearing and Speech Sciences Vanderbilt University Medical Center, Vanderbilt Kennedy Center  Nashville TN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan K.","family":"Percy","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Alabama at Birmingham  Birmingham AL USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charles A.","family":"Nelson","sequence":"additional","affiliation":[{"name":"Laboratories of Cognitive Neuroscience, Boston Children's Hospital Department of Pediatrics, Harvard Medical School  Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy P. 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