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Abstract
Background and Objectives Standard therapies (adrenocorticotropic hormone [ACTH], oral steroids, or vigabatrin) fail to control infantile spasms in almost half of children. Early identification of nonresponders could enable rapid initiation of sequential therapy. We aimed to determine the time to clinical remission after appropriate infantile spasms treatment initiation and identify predictors of the time to infantile spasms treatment response.
Methods The National Infantile Spasms Consortium prospectively followed children aged 2–24 months with new-onset infantile spasms at 23 US centers (2012–2018). We included children treated with standard therapy (ACTH, oral steroids, or vigabatrin). Sustained treatment response was defined as having the last clinically recognized infantile spasms on or before treatment day 14, absence of hypsarrhythmia on EEG 2–4 weeks after treatment, and persistence of remission to day 30. We analyzed the time to treatment response and assessed clinical characteristics to predict sustained treatment response.
Results Among 395 infants, clinical infantile spasms remission occurred in 43% (n = 171) within the first 2 weeks of treatment, of which 81% (138/171) responded within the first week of treatment. There was no difference in the median time to response across standard therapies (ACTH: median 4 days, interquartile range [IQR] 3–7; oral steroids: median 3 days, IQR 2–5; vigabatrin: median 3 days, IQR 1–6). Individuals without hypsarrhythmia on the pretreatment EEG (i.e., abnormal but not hypsarrhythmia) were more likely to have early treatment response than infants with hypsarrhythmia at infantile spasms onset (hazard ratio 2.23, 95% CI 1.39–3.57). No other clinical factors predicted early responders to therapy.
Discussion Remission after first infantile spasms treatment can be identified by treatment day 7 in most children. Given the importance of early and effective treatment, these data suggest that children who do not respond to standard infantile spasms therapy within 1 week should be reassessed immediately for additional standard treatment. This approach could optimize outcomes by facilitating early sequential therapy for children with infantile spasms.
Glossary
- ACTH=
- adrenocorticotropic hormone;
- AUC=
- area under the curve;
- FDR=
- false discovery rate;
- IQR=
- interquartile range;
- NISC=
- National Infantile Spasms Consortium;
- ROC=
- receiver operating characteristic;
- TSC=
- tuberous sclerosis complex
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Pediatric Epilepsy Research Consortium coinvestigators are listed in the appendix at the end of the article.
↵* These authors are co-senior authors.
Submitted and externally peer reviewed. The handling editor was Barbara Jobst, MD, PhD, FAAN.
CME Course: NPub.org/cmelist
- Received February 10, 2022.
- Accepted in final form July 27, 2022.
- © 2022 American Academy of Neurology
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