Related Experiment Video
Updated: May 17, 2026

Electromagnetic Source Imaging in Presurgical Evaluation of Children with Drug-Resistant Epilepsy
Published on: September 20, 2024
Seizure and developmental outcomes following epilepsy surgery for children with Sturge-Weber syndrome
Jonathan Cookson1, Hanna Richardson2, Martin Tisdall3
1UCL Great Ormond Street Institute of Child Health, London, UK; Department of Neurodisability, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Insights
Epilepsy surgery for Sturge-Weber syndrome (SWS) offers good seizure control, particularly in younger patients. While most children maintain or improve cognitive and language skills, motor function can be affected.
Area of Science:
- Neuroscience
- Pediatric Neurology
- Neurosurgery
Background:
- Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder frequently associated with epilepsy in children.
- Epilepsy surgery is a potential treatment, but outcomes regarding seizure control and neurodevelopment are not fully understood.
Purpose of the Study:
- To evaluate seizure control and neurodevelopmental outcomes in children with SWS undergoing epilepsy surgery.
- To analyze the impact of surgery on cognition, language, motor function, and social communication.
Main Methods:
- Retrospective review of case notes for children with SWS who underwent epilepsy surgery.
- Analysis of standardized developmental assessments for cognition, language, and motor function.
Main Results:
- 77.2% of patients achieved good seizure control (Engel 1 outcome).
- Older age at surgery was linked to poorer seizure outcomes.
- Cognitive and language trajectories mostly stabilized or improved; motor skills showed mixed results, with fine motor skills declining in 60%.
Conclusions:
- Epilepsy surgery provides effective seizure control for SWS patients.
- Younger age at surgery appears to correlate with better outcomes.
- Further multicenter studies are necessary to identify factors influencing seizure and developmental outcomes.
Objective:
Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder. Up to 90% of children have epilepsy. Epilepsy surgery is a treatment option. However, the seizure and neurodevelopmental outcomes following this are not well defined. This study aimed to determine the outcomes for children undergoing epilepsy surgery for SWS in relation to seizure control, cognition, language, motor function and social communication.
Methods:
A retrospective case-note review was completed for children with SWS undergoing epilepsy surgery at Great Ormond Street Hospital from 1993 to 2022. Results of standardised developmental assessments for cognition, language and motor function were analysed.
Results:
36 children were operated on (17 hemispherotomies, 13 multilobar surgeries, 6 lobectomy/lesionectomies) with a median follow-up time of 66 months (range 8-198). 77.2% had Engel 1 outcomes (88.2% hemispherotomy, 46.2% multilobar surgery, 83.3% lobectomy/lesionectomy). Older age at surgery (OR for 1-year increase = 1.46, 95% CI 1.08-1.97, p = 0.013), but not type of surgical procedure, was independently associated with worse seizure outcomes. Good seizure control was still achieved in patients who had small residual pial angiomatoses following surgery. Most individual children showed no change or an improvement in their cognitive (67.8%) and language (72%) trajectories, although at group level this was not significant. All children undergoing hemispherotomies had a pre-existing hemiparesis. Following surgery, only 10% of children showed a decline in gross motor functional skills, but 60% had a deterioration in fine motor abilities.
Significance:
This is the second largest SWS epilepsy surgery case series. Epilepsy surgery for SWS resulted in good seizure control, with evidence that younger age is associated with improved outcomes. Most children had stabilisation of their developmental trajectory following surgery. Multicentre studies are needed to better define the factors associated with improved seizure and developmental outcomes.
More Related Videos
06:04Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe
Published on: August 16, 2024
05:54Stereo-Electro-Encephalo-Graphy (SEEG) With Robotic Assistance in the Presurgical Evaluation of Medical Refractory Epilepsy: A Technical Note
Published on: June 13, 2016
Related Concept Videos
Epilepsy and Seizures: Overview
Various factors can trigger epilepsy, including genetic factors, brain damage, metabolic causes, and unknown etiology. Diagnosis of epilepsy involves electroencephalography (EEG), which...
Seizures: Classification
Seizures are typically classified into two main categories: focal and generalized seizures.
Focal Seizures
Focal seizures originate from specific regions of the brain. These seizures are further sub-classified into two types:
Seizures l: Introduction
Epilepsy ll: Types
Seizures ll: Types