{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T12:02:22Z","timestamp":1790424142588,"version":"4.1.0"},"reference-count":261,"publisher":"MDPI AG","issue":"10","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006769","name":"Russian Science Foundation","doi-asserted-by":"publisher","award":["20-68-46042"],"award-info":[{"award-number":["20-68-46042"]}],"id":[{"id":"10.13039\/501100006769","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Rett syndrome (RTT) is a rare neurodevelopmental disorder that is usually caused by mutations of the MECP2 gene. Patients with RTT suffer from severe deficits in motor, perceptual and cognitive domains. Electroencephalogram (EEG) has provided useful information to clinicians and scientists, from the very first descriptions of RTT, and yet no reliable neurophysiological biomarkers related to the pathophysiology of the disorder or symptom severity have been identified to date. To identify consistently observed and potentially informative EEG characteristics of RTT pathophysiology, and ascertain areas most worthy of further systematic investigation, here we review the literature for EEG abnormalities reported in patients with RTT and in its disease models. While pointing to some promising potential EEG biomarkers of RTT, our review identify areas of need to realize the potential of EEG including (1) quantitative investigation of promising clinical-EEG observations in RTT, e.g., shift of mu rhythm frequency and EEG during sleep; (2) closer alignment of approaches between patients with RTT and its animal models to strengthen the translational significance of the work (e.g., EEG measurements and behavioral states); (3) establishment of large-scale consortium research, to provide adequate Ns to investigate age and genotype effects.<\/jats:p>","DOI":"10.3390\/ijms22105308","type":"journal-article","created":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T12:17:16Z","timestamp":1621340236000},"page":"5308","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Reviewing Evidence for the Relationship of EEG Abnormalities and RTT Phenotype Paralleled by Insights from Animal Studies"],"prefix":"10.3390","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0384-647X","authenticated-orcid":false,"given":"Kirill","family":"Smirnov","sequence":"first","affiliation":[{"name":"Institute of Higher Nervous Activity and Neurophysiology, Russian Academy of Science, 117485 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tatiana","family":"Stroganova","sequence":"additional","affiliation":[{"name":"Center for Neurocognitive Research (MEG-Center), Moscow University of Psychology and Education, 123290 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Molholm","sequence":"additional","affiliation":[{"name":"The Cognitive Neurophysiology Laboratory, Ernest J. Del Monte Institute for Neuroscience, Department of Neuroscience, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA"},{"name":"The Cognitive Neurophysiology Laboratory, Departments of Pediatrics and Neuroscience, Albert Einstein College of Medicine &amp; Montefiore Medical Center, Bronx, NY 10461, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olga","family":"Sysoeva","sequence":"additional","affiliation":[{"name":"Institute of Higher Nervous Activity and Neurophysiology, Russian Academy of Science, 117485 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1016\/S0387-7604(85)80048-6","article-title":"Rett Syndrome: Criteria for Inclusion and Exclusion","volume":"7","author":"Hagberg","year":"1985","journal-title":"Brain Dev."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"445","DOI":"10.1542\/peds.91.2.445","article-title":"Epidemiology of Rett Syndrome: A Population-Based Registry","volume":"91","author":"Kozinetz","year":"1993","journal-title":"Pediatrics"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1016\/j.jpeds.2005.10.037","article-title":"Rett Syndrome in Australia: A Review of the Epidemiology","volume":"148","author":"Laurvick","year":"2006","journal-title":"J. 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