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Vigabatrin therapy in infantile spasms
Pongkiat Kankirawatana1, Nattee Raksadawan, Kampanad Balangkura
1Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Insights
Vigabatrin (VGB) effectively treats infantile spasms (IS) with a high response rate and good tolerability. Most children achieving a year spasm-free can be weaned off VGB, but careful monitoring for side effects is crucial.
Area of Science:
- Pediatric Neurology
- Clinical Pharmacology
- Epileptology
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome in infants.
- Vigabatrin (VGB) is a medication used for IS treatment.
- Understanding VGB's clinical outcomes and side effects is essential.
Purpose of the Study:
- To evaluate the clinical effectiveness of vigabatrin (VGB) in treating infantile spasms (IS).
- To assess the short-term and long-term side effects of VGB therapy in children with IS.
- To determine criteria for discontinuing VGB therapy after successful IS control.
Main Methods:
- A cohort of 36 children diagnosed with IS and treated with VGB was studied.
- Clinical data collected included age of IS onset, duration before treatment, seizure recurrence, and adverse events.
- Long-term follow-up assessed outcomes, epilepsy development, and VGB discontinuation.
Main Results:
- VGB showed a high response rate (66.67%) in controlling IS within a mean of 2.95 days.
- Recurrent IS occurred in 3 patients, and 5 developed long-term epilepsy.
- 10 patients were successfully weaned off VGB after a mean of 22.5 months spasm-free; transient drowsiness and irritability were noted side effects.
Conclusions:
- Vigabatrin (VGB) demonstrates a high response rate and is generally well-tolerated for infantile spasms (IS).
- Successful weaning from VGB is possible for patients who remain spasm-free for over a year.
- Due to monitoring challenges for serious side effects like visual field abnormalities, VGB withdrawal or switching to alternative antiepileptic drugs (AEDs) is suggested after a prolonged spasm-free period.
Rationale:
To determine the clinical outcome and side effects of vigabatrin (VGB) in the treatment of infantile spasms (IS) and its long-term outcome.
Method:
All children with IS treated with vigabatrin were studied. Clinical data regarding age of onset, duration of IS before therapy started, recurrence of IS, types of seizures that relapse, clinical outcome and side effects were monitored.
Results:
36 children (17 girls, 19 boys) with IS participated in the study. The mean age of onset of IS was 115.55 +/- 67.3 days old (range, 15 to 300 days). Six were cryptogenic IS and 30 were symptomatic IS. The etiologies of symptomatic IS in this study were tuberous sclerosis, hypoxic ischemic encephalopathy (HIE)/periventricular leukomalacia, porencephaly, partial agenesis of corpus callosum, hemimegalencephaly, cortical dysplasia, and microcephaly. 66.67 per cent (24 of 36) of patients responded to VGB within a mean 2.95 +/- 2.25 days (range, 1 to 7 days). In those who responded to VGB, 3 patients developed recurrent IS within 69.3 +/- 46.7 days (range, 30 to 121 days). Five patients developed epilepsy with different types of seizure during long-term follow-up. The mean duration of subsequent epilepsy after cessation of IS was 16.4 months (range, 5 months to 3 years 10 months). The mean duration of follow-up was 2.74 years (range, 1.09 years to 5.76 years). 10 patients were successfully weaned off VGB after a mean IS free period of 22.5 +/- 5.5 months (range, 12 to 27 months). Transient drowsiness was seen in 4 patients. Three patients had transient abnormal sleep patterns and irritability. Visual field abnormalities were not found but difficult to assess fully in this study.
Conclusion:
VGB therapy has a high response rate for the control of IS and is well tolerated in most children. All patients who responded to VGB and were spasm free for more than one year were successfully weaned off VGB therapy. Because serious side effects such as visual field abnormalities are difficult to monitor, the authors propose that VGB could be withdrawn or switched to another AED after a spasm-free period of more than one year.