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Infantile Spasms and Trisomy 21: Unfavorable Outcomes with First-line Vigabatrin Therapy
Anita N Datta1,2, Jacqueline Crawford2, Peter K H Wong1,2
1Department of Pediatrics, Division of Neurology, BC Children's Hospital, Faculty of Medicine, University of British Columbia, Vancouver, BC, Canada.
Insights
Vigabatrin as a first-line treatment for infantile spasms (IS) in children with trisomy 21 (T21) led to more relapses and subsequent epilepsy compared to controls. Steroids may be a more favorable first-line option for T21 infants with IS.
Area of Science:
- Pediatric Neurology
- Epileptology
- Genetics
Background:
- Infantile spasms (IS) are a severe epilepsy syndrome.
- Children with trisomy 21 (T21) and those with idiopathic IS typically have favorable outcomes.
- Vigabatrin is a common first-line treatment for IS.
Purpose of the Study:
- To compare treatment response, relapse rates, and risk of subsequent epilepsy in children with T21 and IS versus controls with idiopathic IS when treated with vigabatrin.
- To evaluate the efficacy of vigabatrin as a first-line therapy for infantile spasms in these two distinct pediatric populations.
Main Methods:
- Retrospective study comparing two groups: T21 (n=24) and idiopathic IS (n=40).
- Clinical features, treatment response, and epilepsy outcomes were analyzed.
- Statistical analysis was performed to identify significant differences between groups.
Main Results:
- No significant differences in IS onset age, sex, or treatment lag were observed.
- The T21 group required more anti-seizure therapies (3.6 vs. 1.9), experienced more relapses (42% vs. 10%), and had a higher risk of subsequent epilepsy (46% vs. 20%).
- Relapses in the T21 group were often delayed, occurring a mean of 8 months after IS cessation.
Conclusions:
- Vigabatrin as a first-line treatment for IS in T21 may be less favorable than previously suggested by studies using steroids.
- Findings suggest that treatment strategies for IS in T21 may need to be reconsidered.
- Steroids might be a more effective first-line option for T21 infants with IS, offering better control and lower relapse rates.
Introduction:
Among children with infantile spasms (ISs), those with trisomy 21 (T21) and those with normal development at onset and no identifiable etiology (previously referred to as "idiopathic") are expected to have relatively favorable outcomes. The study objective is to determine if differences exist in treatment response, relapse, and subsequent epilepsy between these two groups when vigabatrin is used as first-line treatment.
Methods:
In this retrospective study, patients were classified into the following groups and clinical features were compared: T21 (n = 24) and IS with normal development at onset and no identified etiology (n = 40; control group).
Results:
There was no significant difference in the age of IS onset, sex distribution, or treatment lag between the groups. The T21 compared to the control group required a higher mean number of anti-seizure therapies (3.6 vs. 1.9, p < 0.001), had more relapses [10 (42%) vs. 4 (10%), p < 0.005)], and had higher risk of subsequent epilepsy [11 (46%) vs. 8 (20%), p < 0.003]. Relapses were often delayed in the T21 group, with a mean of 8 months after IS cessation.
Conclusion:
Our results differ from most studies using steroids as first-line treatment where the groups were shown to have similar treatment response and T21 patients had a low risk of relapse and subsequent epilepsy. Therefore, our results suggest that vigabatrin as first-line treatment in T21 with IS may be less favorable than steroids.
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