{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T13:27:02Z","timestamp":1787318822225,"version":"build-2736575974"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T00:00:00Z","timestamp":1779753600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T00:00:00Z","timestamp":1779753600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100007857","name":"Intellectual and Developmental Disabilities Research Center","doi-asserted-by":"publisher","award":["P50 HD 103538"],"award-info":[{"award-number":["P50 HD 103538"]}],"id":[{"id":"10.13039\/100007857","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of the Child Neurology Society"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Sturge\u2013Weber syndrome (SWS) brain involvement has been associated with impairments in the blood\u2013brain barrier (BBB) and microglial activation within involved cortical regions. Acute neurological crises, including seizures, stroke\u2010like episodes, and\/or significant headaches, are common in these patients. This report describes in detail an adolescent with SWS and acute drug\u2010resistant status epilepticus, headache, and stroke\u2010like episode who improved clinically when treated with high\u2010dose steroids. Review of medical records identified two other patients previously treated with steroids for acute neurological symptoms.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case Presentation<\/jats:title>\n                    <jats:p>A 13\u2010year\u2010old boy with SWS brain involvement presented with fever, headache, seizures, and right\u2010sided weakness. Brain magnetic resonance imaging (MRI) revealed characteristic findings of SWS. The patient was placed on continuous electroencephalogram that showed findings consistent with electrographic status epilepticus. Seizures were refractory to multiple anti\u2010seizure medications as well as to an intravenous midazolam drip. A repeat MRI of the brain documented a significant increase in leptomeningeal enhancement with associated gyral edema and sulcal effacement. Steroid therapy with methylprednisolone and prednisone improved his status epilepticus and stroke\u2010like symptoms, including resolution of seizures and marked improvement in his hemiparesis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This patient suggests a potential role for inflammation\u2010targeted therapies in individuals with SWS brain involvement who present with prolonged neurological crises. Two other patients with SWS brain involvement who were treated with high\u2010dose steroids in the context of acute neurological episodes also support the need for future clinical and preclinical research to validate this approach.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/cns3.70068","type":"journal-article","created":{"date-parts":[[2026,5,27]],"date-time":"2026-05-27T05:52:01Z","timestamp":1779861121000},"page":"159-166","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Steroids for Drug\u2010Resistant Seizures and Prolonged Stroke\u2010Like Episode in a Patient With Sturge\u2013Weber Syndrome: A Case Report"],"prefix":"10.1002","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0009-0006-9379-5974","authenticated-orcid":false,"given":"Brenna N.","family":"Keam","sequence":"first","affiliation":[{"name":"Department of Neurology and Developmental Medicine Hugo Moser Kennedy Krieger Research Institute Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-6170-7672","authenticated-orcid":false,"given":"Fabiola A.","family":"Morales\u2010Vias","sequence":"additional","affiliation":[{"name":"Department of Neurology and Developmental Medicine Hugo Moser Kennedy Krieger Research Institute Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siddharth S.","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Neurology and Developmental Medicine Hugo Moser Kennedy Krieger Research Institute Baltimore Maryland USA"},{"name":"Department of Neurology Johns Hopkins School of Medicine Baltimore Maryland USA"},{"name":"Department of Pediatrics Johns Hopkins School of Medicine Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doris D.","family":"Lin","sequence":"additional","affiliation":[{"name":"The Russell H. 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