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Vigabatrin as First-Line Treatment for Infantile Spasms Not Related to Tuberous Sclerosis Complex
Kevin Jones1, Cristina Go2, Jennifer Boyd2
1The Division of Neurology, Department of Pediatrics, McMaster Children's Hospital, Hamilton, Ontario, Canada.
Insights
Vigabatrin showed limited short-term effectiveness as a first-line treatment for infantile spasms in infants without tuberous sclerosis complex. However, it was most beneficial for infants with normal development at diagnosis.
Area of Science:
- Pediatric Neurology
- Epilepsy Research
- Clinical Therapeutics
Background:
- Infantile spasms are a severe, age-specific infant seizure disorder.
- Current guidelines recommend adrenocorticotropic hormone or vigabatrin for short-term treatment.
Purpose of the Study:
- To evaluate the short-term efficacy of vigabatrin as a first-line therapy for infantile spasms.
- Focus on patients without tuberous sclerosis complex.
Main Methods:
- Retrospective analysis of 61 infants diagnosed with infantile spasms between 2010-2013.
- Minimum 6-month follow-up from treatment initiation.
Main Results:
- 30% of infants responded to vigabatrin within 4 weeks; 11% relapsed.
- 27% were seizure-free at final follow-up.
- Normal development at diagnosis correlated with vigabatrin response.
Conclusions:
- Vigabatrin's first-line efficacy for infantile spasms (non-TSC) was not supported.
- Vigabatrin is most effective in children with normal development at diagnosis.
- Findings aid clinicians in selecting optimal first-line treatment for infantile spasms.
Background:
Infantile spasms are a rare, catastrophic, age-specific seizure disorder of infancy. Adrenocorticotropic hormone or vigabatrin have been recommended for the short-term treatment of infantile spasms by the Child Neurology Society/American Academy of Neurology practice parameter.
Objective:
This retrospective study reviewed the Hospital for Sick Children's experience with the short-term efficacy of vigabatrin as first-line treatment for infantile spasms not related to tuberous sclerosis complex.
Method:
We performed a single-center, retrospective analysis of all cases of newly diagnosed infantile spasms between January 2010 and September 2013. Duration of follow-up was at least 6 months from treatment initiation.
Results:
Eighteen of the 61 infants (30%) had a clinical and electrographic response to vigabatrin therapy within 4 weeks of treatment. Of the vigabatrin responders, 2/18 (11%) relapsed. At final follow-up after initiation of vigabatrin therapy, 17/61 (27%) of the vigabatrin responders were free of all clinical seizure types. Normal development at the time of infantile spasms diagnosis was statistically associated with vigabatrin response.
Conclusion:
These findings do not support our hypothesis that vigabatrin is effective as first-line, short-term treatment of infantile spasms in non-tuberous sclerosis complex patients. However, when used in this setting, vigabatrin is most effective in children with normal development at the time of diagnosis. These findings may assist clinicians in the optimal treatment choice for children at the first presentation of infantile spasms not related to tuberous sclerosis complex.
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