BACKGROUND Sturge-Weber syndrome (SWS) is a congenital neurovascular disorder characterised by capillary-venous malformations involving the skin, brain and eye. Patients experience headache, however little is known about its characteristics or associations. We aimed to estimate prevalence, associations and the impact of headache in children with SWS. MATERIALS AND METHODS Case notes of all patients in a national tertiary paediatric SWS clinic were retrospectively reviewed. Patients were categorised into those with or without a history of headache, with an analysis performed of clinical stigmata of their disease and headache symptomology, associations and treatment. A multivariable logistic regression analysis was undertaken to elicit independent predictors of headache. RESULTS 37% of patients with SWS (n = 84) reported headaches that were variably associated with seizures, a preceding blow to the head and a minority had migraine features. In those reporting headaches, headaches interfered with daily activities of a quarter of the children and 39% experienced headaches frequently (more than 1/month). Headache was associated with glaucoma and aspirin administration while children with monoplegia and hemiplegia were less likely to have headache. CONCLUSIONS Headache is common in children with SWS, often without classical migraine features and affects daily activities. Awareness of headache and its associations in SWS may improve management of this complex population.

中文翻译：

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Sturge-Weber 综合征患儿的头痛 - 患病率、关联和影响

背景 Sturge-Weber 综合征 (SWS) 是一种先天性神经血管疾病，其特征在于涉及皮肤、大脑和眼睛的毛细血管畸形。患者会出现头痛，但对其特征或关联知之甚少。我们旨在估计 SWS 儿童头痛的患病率、关联和影响。材料和方法 对全国三级儿科 SWS 诊所所有患者的病例记录进行了回顾性审查。患者被分为有或没有头痛病史的患者，并对其疾病的临床特征和头痛症状、关联和治疗进行分析。进行多变量逻辑回归分析以得出头痛的独立预测因子。结果 37% 的 SWS 患者 (n = 84) 报告头痛与癫痫发作、先前的头部打击和少数具有偏头痛特征的不同程度相关。在报告头痛的人中，有四分之一的儿童因头痛影响日常活动，39% 的儿童经常头痛（超过 1 次/月）。头痛与青光眼和服用阿司匹林有关，而单瘫和偏瘫儿童头痛的可能性较小。结论 头痛在 SWS 儿童中很常见，通常没有典型的偏头痛特征并影响日常活动。意识到头痛及其在 SWS 中的关联可能会改善对这一复杂人群的管理。头痛干扰了四分之一儿童的日常活动，39% 的儿童经常头痛（超过 1 次/月）。头痛与青光眼和服用阿司匹林有关，而单瘫和偏瘫儿童头痛的可能性较小。结论 头痛在 SWS 儿童中很常见，通常没有典型的偏头痛特征并影响日常活动。意识到头痛及其在 SWS 中的关联可能会改善对这一复杂人群的管理。头痛干扰了四分之一儿童的日常活动，39% 的儿童经常头痛（超过 1 次/月）。头痛与青光眼和服用阿司匹林有关，而单瘫和偏瘫儿童头痛的可能性较小。结论 头痛在 SWS 儿童中很常见，通常没有典型的偏头痛特征并影响日常活动。意识到头痛及其在 SWS 中的关联可能会改善对这一复杂人群的管理。通常没有典型的偏头痛特征并影响日常活动。意识到头痛及其在 SWS 中的关联可能会改善对这一复杂人群的管理。通常没有典型的偏头痛特征并影响日常活动。意识到头痛及其在 SWS 中的关联可能会改善对这一复杂人群的管理。