Related Experiment Video
Updated: Jul 3, 2026
A Method of Trigonometric Modelling of Seasonal Variation Demonstrated with Multiple Sclerosis Relapse Data
Published on: December 9, 2015
Retrospective Analysis of Presymptomatic Treatment In Sturge-Weber Syndrome
Chelsea B Valery1, Isabelle Iannotti2, Eric H Kossoff3
1Department of Neurology, Kennedy Krieger Institute.
Insights
Presymptomatic treatment for Sturge-Weber syndrome (SWS) may prevent seizures in infants. Early intervention with medications and EEG monitoring shows promise for better neurological outcomes in children with SWS.
Area of Science:
- Neurology
- Pediatrics
- Clinical Data Analysis
Background:
- Sturge-Weber syndrome (SWS) with brain involvement frequently leads to early-onset seizures (before age 2), impacting neurological outcomes.
- Presymptomatic treatment, similar to approaches in tuberous sclerosis complex, may delay seizure onset and improve outcomes in SWS.
- Electroencephalogram (EEG) may serve as a predictive biomarker for seizure onset in SWS.
Purpose of the Study:
- To retrospectively analyze clinical data and assess the impact of presymptomatic treatment on seizure onset and neurological outcomes in infants with SWS.
- To investigate the potential of EEG as a biomarker for predicting seizure development in SWS patients.
Main Methods:
- Retrospective analysis of clinical records from two centers for patients with SWS brain involvement.
- Data collected included demographics, seizure onset, brain and port-wine birthmark (PWB) extent, family history, presymptomatic treatment details, neuroscore, and anti-seizure medications.
- Pre-seizure onset EEG reports were analyzed for specific findings.
Main Results:
- Of 92 patients, 32 received presymptomatic treatment. Treated patients were significantly more likely to be seizure-free at 2 years (47% vs. 12%, p<.001).
- Presymptomatically-treated patients had a higher incidence of bilateral brain involvement (38% vs. 17%, p=.026) and better median hemiparesis neuroscores.
- EEG findings (slowing, epileptiform discharges, or seizures) prior to onset were associated with seizure onset by age 2 (p=.001).
Conclusions:
- Presymptomatic treatment shows promise for children with SWS diagnosed before seizure onset.
- Further research, including prospective trials and long-term outcome studies, is needed to validate this approach.
- Prospective EEG analysis is recommended to identify reliable biomarkers for guiding presymptomatic treatment decisions in SWS.
Background:
Ninety percent of infants with Sturge-Weber syndrome (SWS) brain involvement have seizure onset before 2 years of age; this is associated with worse neurologic outcome. Presymptomatic treatment before seizure onset may delay seizure onset and improve outcome, as has been shown in other conditions with a high-risk of developing epilepsy such as tuberous sclerosis complex. Electroencephalogram (EEG) may be a biomarker to predict seizure onset. This retrospective clinical data analysis aims to assess impact of presymptomatic treatment in SWS.
Methods:
This two-centered, IRB-approved, retrospective study analyzed records from patients with SWS brain involvement. Clinical data recorded included demographics, age of seizure onset (if present), brain involvement extent (unilateral versus bilateral), port-wine birthmark (PWB) extent, family history of seizure, presymptomatic treatment if received, neuroscore, and anti-seizure medication. EEG reports prior to seizure onset were analyzed.
Results:
Ninety-two patients were included (48 females), and 32 received presymptomatic treatment outside of a formal protocol (5 aspirin, 16 aspirin and levetiracetam; 9 aspirin and oxcarbazepine, 2 valproic acid). Presymptomatically-treated patients were more likely to be seizure-free at 2 years (15 of 32; 47% versus 7 of 60; 12%; p<.001). A greater percentage of presymptomatically-treated patients had bilateral brain involvement (38% treated versus 17% untreated; p=.026). Median hemiparesis neuroscore at 2 years was better in presymptomatically-treated patients. In EEG reports prior to seizure onset, the presence of slowing, epileptiform discharges, or EEG-identified seizures was associated with seizure onset by 2 (p=.001).
Conclusion:
Presymptomatic treatment is a promising approach to children diagnosed with SWS prior to seizure onset. Further study is needed, including prospective drug trials, long-term neuropsychological outcome, and prospective EEG analysis to assess this approach and determine biomarkers for presymptomatic treatment.
