{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T03:27:55Z","timestamp":1761017275094},"reference-count":15,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,10,31]],"date-time":"2017-10-31T00:00:00Z","timestamp":1509408000000},"content-version":"vor","delay-in-days":60,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Epileptic Disorders"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Epileptic encephalopathies with continuous spike\u2010and\u2010waves during sleep (CSWS) are characterized by cognitive or language impairment, and are occasionally associated with pathogenic variants of the <jats:italic>GRIN2A<\/jats:italic> gene. In these disorders, speech dysfunction could be either related to cerebral dysfunction caused by the <jats:italic>GRIN2A<\/jats:italic> deleterious variant or intense interictal epileptic activity.<\/jats:p><jats:p>Here, we present a patient with apraxia of speech, clearly linked to severity of epilepsy, carrying a <jats:italic>GRIN2A<\/jats:italic> variant. A 6\u2010year\u2010old boy developed acute regression of expressive language following epileptic seizures, leading to complete mutism, at which time EEG revealed CSWS. MEG showed bilateral superior parietal and opercular independent CSWS onsets and PET with fluorodeoxyglucose demonstrated significant increase in relative glucose metabolism in bilateral superior parietal regions. Corticosteroids induced a regression of CSWS together with impressive improvement in speech abilities.<\/jats:p><jats:p>This case supports the hypothesis of a triggering role for epileptic discharges in speech deterioration observed in children carrying a deleterious variant of <jats:italic>GRIN2A<\/jats:italic>. When classic antiepileptic drugs fail to control epileptic activity, corticosteroids should be considered. Multimodal functional neuroimaging suggests a role for opercular and superior parietal areas in acquired epileptic opercular syndrome. [<jats:italic>Published with video sequences on<\/jats:italic> <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.epilepticdisorders.com\">www.epilepticdisorders.com<\/jats:ext-link>]<\/jats:p>","DOI":"10.1684\/epd.2017.0931","type":"journal-article","created":{"date-parts":[[2019,8,2]],"date-time":"2019-08-02T13:24:29Z","timestamp":1564752269000},"page":"345-350","source":"Crossref","is-referenced-by-count":6,"title":["Acquired epileptic opercular syndrome related to a heterozygous deleterious substitution in <i>GRIN2A<\/i>"],"prefix":"10.1002","volume":"19","author":[{"given":"Claudine","family":"Sculier","sequence":"first","affiliation":[{"name":"Department of Pediatric Neurology, H\u00f4pital Erasme Universit\u00e9 libre de Bruxelles (ULB) Brussels"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anne\u2010Sophie","family":"Tilmant","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, H\u00f4pital Erasme Universit\u00e9 libre de Bruxelles (ULB) Brussels"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xavier","family":"De Ti\u00e8ge","sequence":"additional","affiliation":[{"name":"Laboratoire de Cartographie Fonctionnelle du Cerveau, H\u00f4pital Erasme Universit\u00e9 libre de Bruxelles (ULB) Brussels"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sanda","family":"Giurgea","sequence":"additional","affiliation":[{"name":"Department of Neurology H\u00f4pital de Tivoli La Louvi\u00e8re"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Philippe","family":"Paquier","sequence":"additional","affiliation":[{"name":"Department of Neurology, H\u00f4pital Erasme Universit\u00e9 libre de Bruxelles (ULB) Brussels"},{"name":"Unit of Clinical and Experimental Neurolinguistics, Center for Linguistics (CLIN) Vrije Universiteit Brussel (VUB) Brussels"},{"name":"Unit of Translational Neurosciences, School of Medicine and Health Sciences Universiteit Antwerpen (UA) Antwerp Belgium"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabrielle","family":"Rudolf","sequence":"additional","affiliation":[{"name":"IGBMC, CNRS UMR7104, INSERM U964 Strasbourg University Strasbourg"},{"name":"Department of Neurology Strasbourg University Hospital Strasbourg"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gaetan","family":"Lesca","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Groupement Hospitalier Est, and ERN EpiCARE University Hospitals of Lyon (HCL) Lyon"},{"name":"Lyon Neuroscience Research Centre CNRS UMR5292 INSERM U1028 Lyon"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrick","family":"Van Bogaert","sequence":"additional","affiliation":[{"name":"Laboratoire de Cartographie Fonctionnelle du Cerveau, H\u00f4pital Erasme Universit\u00e9 libre de Bruxelles (ULB) Brussels"},{"name":"Department of Pediatric Neurology Centre Hospitalier 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