{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T19:00:33Z","timestamp":1790362833550,"version":"4.1.0"},"reference-count":82,"publisher":"SAGE Publications","issue":"7","license":[{"start":{"date-parts":[[2024,7,1]],"date-time":"2024-07-01T00:00:00Z","timestamp":1719792000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Cephalalgia"],"published-print":{"date-parts":[[2024,7]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Sturge\u2013Weber syndrome (SWS) is a neurocutaneous disorder for which the neurological aspects, particularly headaches, remain poorly understood, despite significantly affecting morbidity. The present study aimed to elucidate the prevalence, characteristics and treatment strategies, as well as explore the pathogenesis of headaches, in SWS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Using Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines, we systematically reviewed observational studies, case reports and series from eight databases (Cochrane Library, EBSCO, Embase, Medline, PubMed, Science Direct, Scopus and Web of Science), published from 1978 to 2023, to investigate the prevalence, characteristics, medication response and pathogenic theories of headaches in SWS.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The review analyzed 48 studies, uncovering headache prevalence between 37% and 71%. Migraine-like headache affected up to 52% of individuals. Prophylactic and acute treatments included non-steroidal anti-inflammatory drugs, triptans and antiepileptic drugs, despite the lack of established guidelines. Life-threatening headaches in SWS are uncommon, typically accompanied by other neurological symptoms. The pathogenesis of headaches in SWS is considered to involve venous congestion and neuronal hyperexcitability linked to leptomeningeal angiomas.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Headaches occur more frequently in individuals with SWS than in the general population. Despite symptoms meeting migraine criteria, these headaches should be considered secondary to vascular conditions. Implementing acute and prophylactic treatment is advised to reduce the impact on patients\u2019 lives.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/03331024241265881","type":"journal-article","created":{"date-parts":[[2024,7,23]],"date-time":"2024-07-23T19:10:57Z","timestamp":1721761857000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":12,"title":["Headache in Sturge\u2013Weber syndrome: A systematic review"],"prefix":"10.1177","volume":"44","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3854-9299","authenticated-orcid":false,"given":"Alessandro","family":"Ferretti","sequence":"first","affiliation":[{"name":"Pediatrics Unit, Neuroscience, Mental Health and Sense Organs (NESMOS) Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, 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University of Rome, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Di Nardo","sequence":"additional","affiliation":[{"name":"Pediatrics Unit, Neuroscience, Mental Health and Sense Organs (NESMOS) Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Solmaz","family":"Abdolrahimzadeh","sequence":"additional","affiliation":[{"name":"Ophthalmology Unit, Neurosciences, Mental Health, and Sense Organs (NESMOS) Department, Sapienza University of Rome, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gabriella","family":"De Marco","sequence":"additional","affiliation":[{"name":"Dermatology Unit, Neuroscience, Mental Health and Sense Organs (NESMOS) Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Orsini","sequence":"additional","affiliation":[{"name":"Pediatric Neurology, Pediatric University Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Pisa, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Foiadelli","sequence":"additional","affiliation":[{"name":"Pediatric Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilaria","family":"Frattale","sequence":"additional","affiliation":[{"name":"Systems Medicine Department, University of Rome Tor Vergata, Rome, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6602-103X","authenticated-orcid":false,"given":"Massimiliano","family":"Valeriani","sequence":"additional","affiliation":[{"name":"Systems Medicine Department, University of Rome Tor Vergata, Rome, Italy"},{"name":"Developmental Neurology, Bambino Ges\u00f9 Children Hospital, IRCCS, Rome, Italy"},{"name":"Center for Sensory-Motor Interaction, Aalborg University, Aalborg, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9042-8120","authenticated-orcid":false,"given":"Pasquale","family":"Parisi","sequence":"additional","affiliation":[{"name":"Pediatrics Unit, Neuroscience, Mental Health and Sense Organs (NESMOS) Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, 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