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Vigabatrin as a first-line drug in West syndrome: clinical and electroencephalographic outcome
G Wohlrab1, E Boltshauser, B Schmitt
1Department of Clinical Neurophysiology and Pediatric Neurology, University Children's Hospital, Zürich, Switzerland.
Insights
Vigabatrin monotherapy effectively treats West syndrome, leading to seizure freedom and EEG normalization in many children within two weeks. This treatment
Area of Science:
- Pediatric Neurology
- Epileptology
- Clinical Pharmacology
Background:
- West syndrome is a severe infant epilepsy characterized by infantile spasms and hypsarrhythmia on EEG.
- Successful treatment is defined by the cessation of spasms and normalization of EEG.
- Vigabatrin is an antiepileptic drug used in treating various seizure disorders.
Purpose of the Study:
- To evaluate the efficacy and response time of vigabatrin monotherapy in children with West syndrome.
- To compare vigabatrin's effectiveness with other standard treatments like ACTH.
Main Methods:
- A prospective study involving 28 children diagnosed with West syndrome.
- Children received either 150 mg/kg/day or 65-75 mg/kg/day of vigabatrin monotherapy for at least two weeks.
- Responders were defined as patients with disappearance of infantile spasms and hypsarrhythmia within two weeks.
Main Results:
- Fourteen children (50%) were classified as responders, achieving seizure freedom and EEG normalization within two weeks.
- The responder group maintained seizure freedom during a follow-up period of up to 5.3 years.
- In non-responders, subsequent treatments like ACTH showed efficacy, but relapses occurred after ACTH discontinuation.
Conclusions:
- Vigabatrin monotherapy is an effective and rapid treatment for West syndrome, comparable to ACTH.
- Early response to vigabatrin predicts sustained seizure control and EEG normalization.
- Vigabatrin offers a valuable therapeutic option for managing West syndrome in pediatric patients.
Abstract:
The cessation of infantile spasms and the disappearance of hypsarrhythmia in sleep EEG are the criteria for successful treatment in West syndrome. In a prospective study at the Children's University Hospital, 28 children with West syndrome (17 symptomatic, 7 cryptogenic, 4 idiopathic) were treated with vigabatrin monotherapy for at least 2 weeks. Seven children received vigabatrin 150 mg/kg/day, and 21 children received 65-75 mg/kg/day. Patients were classified as responders, when infantile spasms and hypsarrhythmia in sleep EEG disappeared within 2 weeks. After 2 weeks, 18 patients were seizure-free, 14 without hypsarrhythmia. Fourteen children were responders (8 symptomatic, 3 cryptogenic, 3 idiopathic), and 14 non-responders. The follow-up encompassed 6 months to 5.3 years (mean 20.3 months). In the responder group, 12/14 patients (6 symptomatic, 3 cryptogenic and 3 idopathic) remained seizure-free, no relapse of West syndrome occurred. In the non-responder group, ACTH was efficient in 11, valproic acid in 2 children and clonazepam in 1 child. Relapse occurred in 4 children after discontinuation of ACTH. At last visit EEG was normal in 8/14 responders and 1/14 non-responders. The efficacy of vigabatrin monotherapy was comparable to ACTH and occurred within 2 weeks.