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Neurological function and drug-refractory epilepsy in Sturge-Weber syndrome children: a retrospective analysis
Yu Zhang1, Jiechao Niu1, Jiandong Wang1
1Department of Pediatrics, the First Affiliated Hospital of Zhengzhou University, Henan Province, Zhengzhou, 450052, China.
Insights
Drug-refractory epilepsy (DRE) in children with Sturge-Weber syndrome (SWS) is linked to stroke-like episodes and seizure clusters. Status epilepticus also negatively impacts neurological function in these pediatric patients.
Area of Science:
- Pediatric Neurology
- Neurocutaneous Disorders
Background:
- Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder with a high incidence of seizures.
- Over 75% of children with SWS experience seizures, and 30-57% develop drug-refractory epilepsy (DRE), often leading to poor outcomes.
- Risk factors for DRE in pediatric SWS populations remain largely unclear.
Purpose of the Study:
- To investigate the clinical characteristics of epilepsy in children with SWS.
- To identify risk factors associated with the development of drug-refractory epilepsy (DRE) in pediatric SWS.
- To examine the impact of epilepsy on neurological function in children with SWS.
Main Methods:
- Retrospective analysis of clinical data from 35 children with SWS and epilepsy (January 2013 - October 2022).
- Utilized univariate and multivariate logistic regression to identify factors influencing DRE.
- Assessed neurological function and the occurrence of status epilepticus.
Main Results:
- The study included 35 pediatric SWS patients with epilepsy; 31% developed DRE.
- First seizures occurred within the first year of life in 71% of cases, with focal seizures being most common (77%).
- Stroke-like episodes and seizure clusters were identified as significant risk factors for DRE in SWS children. Status epilepticus was linked to poorer neurological function.
Conclusions:
- Epileptic features in children with SWS were characterized, highlighting key risk factors for DRE.
- Stroke-like episodes and seizure clusters are significant predictors of DRE in pediatric SWS.
- Status epilepticus adversely affects neurological function in SWS children with epilepsy, necessitating long-term monitoring.
Abstract:
Epilepsy in Sturge-Weber syndrome (SWS) is common, but drug-refractory epilepsy (DRE) in SWS has rarely been studied in children. We investigated the characteristics of epilepsy and risk factors for DRE in children with SWS. A retrospective study was conducted to analyze the clinical characteristics of children with SWS with epilepsy in our hospital from January 2013 to October 2022. Univariate and multivariate logistic analyses were performed to investigate the factors influencing DRE in children with SWS. A total of 35 SWS children with epilepsy were included (51% male; mean age of presentation 3.6 ± 0.5 years), 71% of children with SWS had their first seizure within the first year of life, and the most common type of seizure was focal seizure (77%). Eleven (31%) patients developed DRE. The median age of onset for the first seizure was 1.0 years and all these cases were of SWS type I. Multivariate logistic analysis revealed that stroke-like episodes and seizure clusters were risk factors for DRE in SWS children. A poor neurological function group was observed in twenty-five children with SWS. Status epilepticus was a risk factor that affected the neurological function of SWS children with epilepsy. Conclusion: The study explored the epileptic features of children with SWS. The results revealed that stroke-like episodes and seizure clusters are risk factors for DRE in children with SWS. The occurrence of status epilepticus impacts the neurological function of SWS children with epilepsy. Thus, long-term follow-up is necessary to monitor outcomes. What is Known: • Sturge-Weber syndrome (SWS) is a rare neurocutaneous disorder, over 75% of children with SWS experience seizures, and 30-57% develop drug-refractory epilepsy (DRE), which leads to a poor outcome. • Drug-refractory epilepsy in SWS has been rarely studied in children, and the risk factors associated with DRE are unclear. What is New: • Clinical features of SWS children with drug-refractory epilepsy. • In SWS, stroke-like episodes and seizure clusters are risk factors of DRE, the occurrence of status epilepticus impacts the neurological function.
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