{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T01:40:45Z","timestamp":1781228445907,"version":"3.54.1"},"reference-count":40,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2014,1,31]],"date-time":"2014-01-31T00:00:00Z","timestamp":1391126400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2014,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>To identify the brain networks that are involved in the different electroencephalography (EEG) abnormalities in patients with ring chromosome 20 [r(20)] syndrome. We hypothesize the existence of both distinctive and common brain circuits for the paroxysmal high voltage sharp waves (<jats:styled-content style=\"fixed-case\">hSWs<\/jats:styled-content>), the seizures, and the slow\u2010wave 3\u20137\u00a0Hz rhythm that characterize this condition.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Thirteen patients with [r(20)] syndrome were studied by means of EEG simultaneously recorded with functional magnetic resonance imaging (EEG\u2010<jats:styled-content style=\"fixed-case\">fMRI<\/jats:styled-content>). EEG traces were reviewed in order to detect the pathologic interictal (<jats:styled-content style=\"fixed-case\">hSWs<\/jats:styled-content>) and ictal activities; the 3\u20137\u00a0Hz theta\u2010delta power was derived using a fast Fourier transform. A group\u2010level analysis was performed for each type of EEG abnormality separately using a fixed\u2010effect model and a conjunction analysis. Finally, a second\u2010level random\u2010effect model was applied considering together the different EEG abnormalities, without distinction between <jats:styled-content style=\"fixed-case\">hSW<\/jats:styled-content>, seizures, or theta\u2010delta rhythms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Subcontinuous theta\u2010delta rhythm was recorded in seven patients, seizures in two, and <jats:styled-content style=\"fixed-case\">hSWs<\/jats:styled-content> in three patients. The main results are the following: (1) the slow\u2010wave rhythm was related to blood oxygen level\u2013dependent (BOLD) increases in the premotor, sensory\u2010motor, and temporoparietal cortex, and to BOLD decrements involving the default mode (DMN) and the dorsal attention networks (DANs); (2) the ictal\u2010related BOLD changes showed an early involvement of the prefrontal lobe; (3) increases in BOLD signal over the basal ganglia, either for interictal and ictal activities, were observed; (4) a common pattern of positive BOLD changes in the bilateral perisylvian regions was found across the different EEG abnormalities.<\/jats:p><\/jats:sec><jats:sec><jats:title>Significance<\/jats:title><jats:p>The <jats:styled-content style=\"fixed-case\">BOLD<\/jats:styled-content> increment in the perisylvian network and the decrease of the <jats:styled-content style=\"fixed-case\">DMN<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">DAN<\/jats:styled-content> could be the expression of the [r(20)] syndrome\u2013related cognitive and behavioral deficits. The observed <jats:styled-content style=\"fixed-case\">BOLD<\/jats:styled-content> patterns are similar to the ones detected in other epileptic encephalopathies, suggesting that different epileptic disorders characterized by neurobehavioral regression are associated with dysfunction in similar brain networks.<\/jats:p><jats:p>A PowerPoint slide summarizing this article is available for download in the Supporting Information section <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/epi.12539\/suppinfo\">here<\/jats:ext-link>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/epi.12539","type":"journal-article","created":{"date-parts":[[2014,1,31]],"date-time":"2014-01-31T22:18:59Z","timestamp":1391206739000},"page":"403-413","source":"Crossref","is-referenced-by-count":17,"title":["Epilepsy\u2010related brain networks in ring chromosome 20 syndrome: An EEG\u2010<scp>fMRI<\/scp> study"],"prefix":"10.1111","volume":"55","author":[{"given":"Anna Elisabetta","family":"Vaudano","sequence":"first","affiliation":[{"name":"Department of Biomedical Sciences, Metabolic, and Neuroscience NOCSAE Hospital University of Modena and Reggio Emilia Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Ruggieri","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Metabolic, and Neuroscience NOCSAE Hospital University of Modena and Reggio Emilia Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aglaia","family":"Vignoli","sequence":"additional","affiliation":[{"name":"Department of Health Science Epilepsy Center San Paolo Hospital University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pietro","family":"Avanzini","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Metabolic, and Neuroscience NOCSAE Hospital University of Modena and Reggio Emilia Modena Italy"},{"name":"Department of Neuroscience University of Parma Parma Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Benuzzi","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Metabolic, and Neuroscience NOCSAE Hospital University of Modena and Reggio Emilia Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliana","family":"Gessaroli","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Metabolic, and Neuroscience NOCSAE Hospital University of Modena and Reggio Emilia Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo Frigio","family":"Nichelli","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Metabolic, and Neuroscience NOCSAE Hospital University of Modena and Reggio Emilia Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Darra","sequence":"additional","affiliation":[{"name":"Department of Life and Reproduction Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gaetano","family":"Cantalupo","sequence":"additional","affiliation":[{"name":"Department of Life and Reproduction Sciences University of Verona Verona Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Mastrangelo","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Unit V. 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