{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T15:45:04Z","timestamp":1787240704348,"version":"build-2736575974"},"reference-count":76,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,11,6]],"date-time":"2021-11-06T00:00:00Z","timestamp":1636156800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Focal cortical dysplasia (FCD) Type 1 and its three subtypes have yet not been fully characterized at the clinical, anatomopathological, and molecular level (International League Against Epilepsy [ILAE] FCD classification from 2011). We aimed to describe the clinical phenotype of patients with histopathologically confirmed FCD1A obtained from a single epilepsy center between 2002 and 2016.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Medical records were retrieved from the hospital's archive. Results from electroencephalography (EEG) video recordings, neuroimaging, and histopathology were reevaluated. Magnetic resonance imaging (MRI) post\u2010processing was retrospectively performed in nine patients. DNA methylation studies were carried out from archival surgical brain tissue in 11 patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Nineteen children with a histopathological diagnosis of FCD1A were included. The average onset of epilepsy was 0.9\u00a0years (range 0.2\u201310\u00a0years). All children had severe cognitive impairment and one third had mild motor deficits, yet fine finger movements were preserved in all patients. All patients had daily seizures, being drug resistant from disease onset. Interictal electroencephalography revealed bilateral multi\u2010regional epileptiform discharges. Interictal status epilepticus was observed in 8 and countless subclinical seizures in 11 patients. Regional continuous irregular slow waves were of higher lateralizing and localizing yield than spikes. Posterior background rhythms were normal in 16 of 19 children. Neuroimaging showed unilateral multilobar hypoplasia and increased T2\u2010FLAIR signals of the white matter in 18 of 19 patients. All children underwent tailored multilobar resections, with seizure freedom achieved in 47% (Engel class I). There was no case with frontal involvement without involvement of the posterior quadrant by MRI and histopathology. DNA methylation profiling distinguished FCD1A samples from all other epilepsy specimens and controls.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Significance<\/jats:title>\n                    <jats:p>We identified a cohort of young children with drug resistance from seizure onset, bad EEG with posterior emphasis, lack of any focal neurological deficits but severe cognitive impairment, subtle hypoplasia of the epileptogenic area on MRI, and histopathologically defined and molecularly confirmed by DNA methylation analysis as FCD ILAE Type 1A.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/epi.17114","type":"journal-article","created":{"date-parts":[[2021,11,6]],"date-time":"2021-11-06T04:10:55Z","timestamp":1636171855000},"page":"42-60","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Multilobar unilateral hypoplasia with emphasis on the posterior quadrant and severe epilepsy in children with FCD ILAE Type 1A"],"prefix":"10.1111","volume":"63","author":[{"given":"Hans","family":"Holthausen","sequence":"first","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roland","family":"Coras","sequence":"additional","affiliation":[{"name":"Department of Neuropathology University Hospitals Erlangen  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5813-0308","authenticated-orcid":false,"given":"Yingying","family":"Tang","sequence":"additional","affiliation":[{"name":"Department of Neurology West China Hospital of Sichuan University  Chengdu Sichuan China"},{"name":"Epilepsy Center Cleveland Clinic  Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lily","family":"Bai","sequence":"additional","affiliation":[{"name":"Epilepsy Center Cleveland Clinic  Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3829-5217","authenticated-orcid":false,"given":"Irene","family":"Wang","sequence":"additional","affiliation":[{"name":"Epilepsy Center Cleveland Clinic  Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tom","family":"Pieper","sequence":"additional","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manfred","family":"Kudernatsch","sequence":"additional","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"},{"name":"Paracelsus Private Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Till","family":"Hartlieb","sequence":"additional","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"},{"name":"Paracelsus Private Medical University  Salzburg Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Staudt","sequence":"additional","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Winkler","sequence":"additional","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wiebke","family":"Hofer","sequence":"additional","affiliation":[{"name":"Center for Pediatric Neurology, Neurorehabilitation, and Epileptology Schoen\u2010Clinic  Vogtareuth Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3037-7170","authenticated-orcid":false,"given":"Samir","family":"Jabari","sequence":"additional","affiliation":[{"name":"Department of Neuropathology University Hospitals Erlangen  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0074-2480","authenticated-orcid":false,"given":"Katja","family":"Kobow","sequence":"additional","affiliation":[{"name":"Department of Neuropathology University Hospitals Erlangen  Erlangen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8676-0788","authenticated-orcid":false,"given":"Ingmar","family":"Blumcke","sequence":"additional","affiliation":[{"name":"Department of Neuropathology University Hospitals Erlangen  Erlangen Germany"},{"name":"Epilepsy Center Cleveland Clinic  Cleveland Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,11,6]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/epi4.12366"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(15)00250-1"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41582-020-0395-6"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.16682"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13515"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1703784"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(20)30220-9"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.12347"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2018.07.009"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2020.09.011"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXG.0000000000000294"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40478-020-01085-3"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature26000"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-020-02228-5"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.14934"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02777.x"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-019-02061-5"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13916"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24407"},{"key":"e_1_2_8_21_1","first-page":"553","volume-title":"Surgical Treatment of the Epilepsies","author":"Engel J","year":"1987"},{"key":"e_1_2_8_22_1","volume-title":"McCarthy Scales of Children\u2019s Development","author":"McCarthy D","year":"1972"},{"key":"e_1_2_8_23_1","volume-title":"HamburgWechsler\u2010Intelligenztest f\u00fcr Kinder III (HAWIK\u2010III)","author":"Tewes U","year":"1999"},{"key":"e_1_2_8_24_1","volume-title":"Wechsler Intelligenztest f\u00fcr Erwachsene (WIE)","author":"Aster M","year":"2006"},{"key":"e_1_2_8_25_1","volume-title":"Testbatterie f\u00fcr geistig behinderte Kinder (TBGB)","author":"Bondy C","year":"1975"},{"key":"e_1_2_8_26_1","volume-title":"Bayley Scales of Infant Development","author":"Bayley N","year":"1993"},{"key":"e_1_2_8_27_1","volume-title":"Vineland Adaptive Behavior Scales","author":"Sparrow SS","year":"1984"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2007.01436.x"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1006\/nimg.1998.0395"},{"key":"e_1_2_8_30_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-005-1016-6"},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-011-0920-1"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2014.08.012"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2018.08.010"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.14581\/jer.12010"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-016-3104-4"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1002\/epi4.12289"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2019.03.009"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2014.64"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.yebeh.2015.01.022"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2015.09.004"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003042"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13464"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.seizure.2017.07.008"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2017.08.008"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.4103\/0028-3886.246263"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2017.11.012"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A5877"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1002\/epi4.12312"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13855"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1111\/nan.12462"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.3171\/2014.3.PEDS13560"},{"key":"e_1_2_8_52_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12147"},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1684\/epd.2016.0870"},{"key":"e_1_2_8_54_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12876"},{"key":"e_1_2_8_55_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.12095"},{"key":"e_1_2_8_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2017.01.050"},{"key":"e_1_2_8_57_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2008.01998.x"},{"key":"e_1_2_8_58_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02730.x"},{"key":"e_1_2_8_59_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000035639.54567.5C"},{"key":"e_1_2_8_60_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/115.1.315"},{"key":"e_1_2_8_61_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000114507.30388.7E"},{"key":"e_1_2_8_62_1","doi-asserted-by":"publisher","DOI":"10.5414\/NP301103"},{"key":"e_1_2_8_63_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.16907"},{"key":"e_1_2_8_64_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-019-02052-6"},{"key":"e_1_2_8_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.celrep.2017.11.106"},{"key":"e_1_2_8_66_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2009.05.004"},{"key":"e_1_2_8_67_1","doi-asserted-by":"publisher","DOI":"10.1161\/STROKEAHA.108.521203"},{"key":"e_1_2_8_68_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.1078"},{"key":"e_1_2_8_69_1","doi-asserted-by":"publisher","DOI":"10.1136\/archdischild-2011-300973"},{"key":"e_1_2_8_70_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.293.6.723"},{"key":"e_1_2_8_71_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.13337"},{"key":"e_1_2_8_72_1","doi-asserted-by":"publisher","DOI":"10.1684\/epd.2010.0321"},{"key":"e_1_2_8_73_1","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a006551"},{"key":"e_1_2_8_74_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2012.10.001"},{"key":"e_1_2_8_75_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.devcel.2018.01.023"},{"key":"e_1_2_8_76_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13654"},{"key":"e_1_2_8_77_1","doi-asserted-by":"publisher","DOI":"10.1684\/epd.2014.0648"}],"container-title":["Epilepsia"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.17114","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/epi.17114","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/epi.17114","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,23]],"date-time":"2023-08-23T15:38:03Z","timestamp":1692805083000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/epi.17114"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,11,6]]},"references-count":76,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2022,1]]}},"alternative-id":["10.1111\/epi.17114"],"URL":"https:\/\/doi.org\/10.1111\/epi.17114","archive":["Portico"],"relation":{},"ISSN":["0013-9580","1528-1167"],"issn-type":[{"value":"0013-9580","type":"print"},{"value":"1528-1167","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,11,6]]},"assertion":[{"value":"2021-05-20","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-10-18","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-11-06","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}