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Headache in children with Sturge-Weber syndrome - Prevalence, associations and impact
L Arkush1, P Prabhakar2, R C Scott3
1Neurosciences Unit, Great Ormond Street Hospital NHS Foundation Trust, UK.
Insights
Headache is common in children with Sturge-Weber syndrome (SWS), affecting daily life for many. Understanding headache patterns and triggers in SWS is crucial for better patient management.
Area of Science:
- Neurology
- Pediatrics
- Genetics
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital neurovascular disorder affecting skin, brain, and eyes.
- Headache is a common symptom in SWS patients, but its characteristics and associations are poorly understood.
- This study investigates the prevalence, associations, and impact of headaches in pediatric SWS cases.
Purpose of the Study:
- To determine the prevalence of headaches in children with SWS.
- To identify clinical associations and symptomology of headaches in this population.
- To understand the impact of headaches on the daily activities of children with SWS.
Main Methods:
- Retrospective review of medical records from a national tertiary pediatric SWS clinic.
- Categorization of patients based on headache history.
- Multivariable logistic regression analysis to identify independent predictors of headache.
Main Results:
- 37% of SWS patients reported headaches, often linked to seizures or head trauma.
- Headaches interfered with daily activities for 25% and occurred frequently ( >1/month) in 39%.
- Headache was associated with glaucoma and aspirin use; monoplegia/hemiplegia correlated with less frequent headaches.
Conclusions:
- Headache is a prevalent issue in pediatric SWS, frequently lacking typical migraine features.
- Headaches significantly impact the daily lives of affected children.
- Increased awareness of headache characteristics and associations in SWS can enhance clinical management.
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.
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