Pediatrics Unit, Neuroscience, Mental Health and Sense Organs (NESMOS) Department, Faculty of Medicine and Psychology, Sapienza University of Rome, Rome, Italy.
Private Practice, Rome, Italy.
Cephalalgia. 2024 Jul;44(7):3331024241265881. doi: 10.1177/03331024241265881.
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.
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.
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.
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.
Sturge-Weber 综合征（SWS）是一种神经皮肤疾病，尽管对发病率有重大影响，但神经系统方面，尤其是头痛，仍了解甚少。本研究旨在阐明 SWS 头痛的患病率、特征和治疗策略，并探讨其发病机制。
我们使用系统评价和荟萃分析的首选报告项目（PRISMA）指南，系统地检索了从 1978 年至 2023 年在八个数据库（Cochrane 图书馆、EBSCO、Embase、Medline、PubMed、Science Direct、Scopus 和 Web of Science）发表的观察性研究、病例报告和系列研究，以调查 SWS 中头痛的患病率、特征、药物反应和发病理论。
综述分析了 48 项研究，发现头痛的患病率在 37%至 71%之间。偏头痛样头痛影响了高达 52%的个体。预防性和急性治疗包括非甾体抗炎药、曲坦类药物和抗癫痫药，尽管缺乏既定的指南。SWS 中危及生命的头痛并不常见，通常伴有其他神经症状。SWS 头痛的发病机制被认为涉及静脉充血和与软脑膜血管畸形相关的神经元过度兴奋。
SWS 患者头痛的发生率高于普通人群。尽管这些头痛符合偏头痛的标准，但应将其视为血管疾病的继发症状。建议采用急性和预防性治疗，以减轻对患者生活的影响。
