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[Efficacy and tolerability of vigabatrin in West syndrome]
Maria Helena P Moraes1, Maria Augusta Montenegro, Renata C Franzon
1Departamento de Neurologia, Faculdade de Ciências Médicas, Universidade Estudual de Campinas, Campinas, SP, Brasil.
Insights
Vigabatrin (VGB) effectively treats West syndrome (WS), a severe childhood epilepsy, with most patients achieving seizure freedom or improvement. While rare, potential adverse events like retinopathy warrant consideration.
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Pharmacology
Context:
- West syndrome (WS) is a severe epileptic encephalopathy in children.
- It is characterized by infantile spasms, developmental regression, and hypsarrhythmia on EEG.
- Current treatment options for WS have limitations.
Purpose:
- To evaluate the safety and efficacy of vigabatrin (VGB) for treating West syndrome.
- To assess seizure control, EEG normalization, and adverse events associated with VGB therapy.
Summary:
- Vigabatrin (VGB) was administered to 23 pediatric patients with West syndrome (WS).
- Seizure freedom was achieved in 69.5% of patients, with partial control in 22%.
- Hypsarrhythmia resolved in all patients, and 50% showed normalized EEGs post-treatment. One patient experienced gabaergic retinopathy.
Impact:
- Vigabatrin (VGB) demonstrates significant efficacy in treating West syndrome (WS), leading to seizure cessation and EEG improvement.
- Early seizure onset (after 6 months) correlated with better outcomes.
- Despite potential adverse events like retinopathy, VGB is a valuable therapeutic option for WS.
Unlabelled:
West syndrome (WS) is a severe epileptic encephalopathy of childhood, characterized by spasms, developmental deterioration and hipsarhythymia.
Objective:
To evaluate the safety and efficacy of vigabatrin (VGB) in the treatment of WS.
Method:
We evaluated every patient diagnosed with WS seen at the pediatric epilepsy clinic and exposed to VGB. Patients were interviewed according to a semistructured questionnaire and we analyzed gender, age, etiology (cryptogenic or symptomatic), associated diseases, age of seizure onset, neuroimaging findings, EEG prior and after VGB, use of other antiepileptic drugs, time for seizure control, electroretinogram, visual complaints, adverse events and family history of epilepsy.
Results:
Twenty-three patients were evaluated, 16 boys, ages ranging from 1.25 years to 11.5 years (mean=5y3m). Sixteen (69.5%) patients were seizure free, five (22%) had partial seizure control and in two (8.5%) there was no improvement. Only one patient presented gabaergic retinopathy. Six (26%) patients presented adverse events: somnolence, aggressivity or retinopathy. Patients with seizure onset after 6 months of age presented better results after VGB introduction (p<0.05). There was no difference in seizure control according to duration of epilepsy before VGB treatment or etiology of the seizures (p>0.05). After VGB, no patient presented hipsarrhythymia and 50% had a normal EEG.
Conclusion:
Although VGB may be associated with serious adverse events such as gabaergic retinopathy, our results show that it should be considered in the treatment of WS.
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