Background: Sturge-Weber 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. Methods: 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. Results: 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. Conclusions: 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' lives.

Headache in Sturge–Weber syndrome. a systematic review / Ferretti, Alessandro; Muscianese, Marta; Fanfoni, Claudia; Bellone, Giulia; Mennini, Maurizio; Di Nardo, Giovanni; Abdolrahimzadeh, Solmaz; De Marco, Gabriella; Orsini, Alessandro; Foiadelli, Thomas; Frattale, Ilaria; Valeriani, Massimiliano; Parisi, Pasquale. - In: CEPHALALGIA. - ISSN 0333-1024. - 44:7(2024). [10.1177/03331024241265881]

Headache in Sturge–Weber syndrome. a systematic review

Ferretti, Alessandro
Primo
;
Muscianese, Marta;Fanfoni, Claudia;Bellone, Giulia;Mennini, Maurizio;Di Nardo, Giovanni;Abdolrahimzadeh, Solmaz;De Marco, Gabriella;Orsini, Alessandro;Parisi, Pasquale
Ultimo
2024

Abstract

Background: Sturge-Weber 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. Methods: 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. Results: 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. Conclusions: 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' lives.
2024
sturge–weber syndrome; headache; migraine; secondary headache; systematic review
01 Pubblicazione su rivista::01g Articolo di rassegna (Review)
Headache in Sturge–Weber syndrome. a systematic review / Ferretti, Alessandro; Muscianese, Marta; Fanfoni, Claudia; Bellone, Giulia; Mennini, Maurizio; Di Nardo, Giovanni; Abdolrahimzadeh, Solmaz; De Marco, Gabriella; Orsini, Alessandro; Foiadelli, Thomas; Frattale, Ilaria; Valeriani, Massimiliano; Parisi, Pasquale. - In: CEPHALALGIA. - ISSN 0333-1024. - 44:7(2024). [10.1177/03331024241265881]
File allegati a questo prodotto
File Dimensione Formato  
Ferretti_Headache-sturge-weber_2024.pdf

accesso aperto

Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Creative commons
Dimensione 1.4 MB
Formato Adobe PDF
1.4 MB Adobe PDF

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1716567
Citazioni
  • ???jsp.display-item.citation.pmc??? 1
  • Scopus 0
  • ???jsp.display-item.citation.isi??? 0
social impact