{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T23:29:57Z","timestamp":1780356597425,"version":"3.54.1"},"reference-count":19,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,6,12]],"date-time":"2024-06-12T00:00:00Z","timestamp":1718150400000},"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":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Sturge\u2013Weber syndrome (SWS) is a rare neurocutaneous syndrome, frequently associated with pharmaco\u2010resistant, early\u2010onset epilepsy. Optimal seizure control is paramount to maximize neurodevelopment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Method<\/jats:title><jats:p>A single\u2010centre case series of 49 infants explored early SWS care. Ninety\u2010two per cent of children developed seizures aged 0 to 3\u2009years; 55% of cases were before diagnostic magnetic resonance imaging (MRI) or tertiary referral. Delay in SWS diagnosis affected 31% of infants because of a lack of gadolinium enhancement for initial MRI. First seizures were frequently prolonged, with phenytoin administration necessary in 46%. Presymptomatic antiseizure medication prophylaxis (<jats:italic>n<\/jats:italic>\u2009=\u20098\/49) decreased seizure burden. No patients on antiseizure medication prophylaxis suffered status epilepticus for longer than 30\u2009minutes, and half of them (<jats:italic>n<\/jats:italic>\u2009=\u20094) had not developed seizures at last follow\u2010up (aged 2\u201310\u2009years).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A parental survey enabled further service evaluation. Eighty\u2010three per cent of parents considered local clinicians' understanding of SWS inadequate: 61% felt insufficiently informed about SWS and 81% received no epilepsy education before seizures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>To overcome the identified shortfalls, guidelines towards improving and standardizing SWS management are proposed.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.15983","type":"journal-article","created":{"date-parts":[[2024,6,13]],"date-time":"2024-06-13T05:13:43Z","timestamp":1718255623000},"page":"111-118","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["Diagnostic pathway and management of first seizures in infants with Sturge\u2013Weber syndrome"],"prefix":"10.1111","volume":"67","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6885-8738","authenticated-orcid":false,"given":"Finola Lauren","family":"Mankel","sequence":"first","affiliation":[{"name":"Neurosciences Department Great Ormond Street Hospital for Children NHS Foundation Trust  London UK"},{"name":"University College London Medical School  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5093-6075","authenticated-orcid":false,"given":"Apostolos","family":"Papandreou","sequence":"additional","affiliation":[{"name":"Neurosciences Department Great Ormond Street Hospital for Children NHS Foundation Trust  London UK"},{"name":"Developmental Neurosciences University College London, Great Ormond Street Institute of Child Health  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5979-9337","authenticated-orcid":false,"given":"Kshitij","family":"Mankad","sequence":"additional","affiliation":[{"name":"Neuroradiology Department Great Ormond Street Hospital for Children NHS Foundation Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanna","family":"Richardson","sequence":"additional","affiliation":[{"name":"Neurosciences Department Great Ormond Street Hospital for Children NHS Foundation Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah E.","family":"Aylett","sequence":"additional","affiliation":[{"name":"Neurosciences Department Great Ormond Street Hospital for Children NHS Foundation Trust  London UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,6,12]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.13203"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpn.2021.07.005"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2018.12.002"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/jmri.20627"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ad.2016.09.022"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.13121"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2018.04.009"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1053\/seiz.2001.0629"},{"key":"e_1_2_8_10_1","unstructured":"Sturge Weber Syndrome [Internet].NORD (National Organization for Rare Disorders). 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