Response to treatment in a prospective national infantile spasms cohort

Kelly G. Knupp, Jason Coryell, Katherine C. Nickels, Nicole Ryan, Erin Leister, Tobias Loddenkemper, Zachary Grinspan, Adam L. Hartman, Eric H. Kossoff, William D. Gaillard, John R. Mytinger, Sucheta Joshi, Renée A. Shellhaas, Joseph Sullivan, Dennis Dlugos, Lorie Hamikawa, Anne T. Berg, John Millichap, Douglas R. Nordli, Elaine Wirrell

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Abstract

Objective Infantile spasms are seizures associated with a severe epileptic encephalopathy presenting in the first 2 years of life, and optimal treatment continues to be debated. This study evaluates early and sustained response to initial treatments and addresses both clinical remission and electrographic resolution of hypsarrhythmia. Secondarily, it assesses whether response to treatment differs by etiology or developmental status. Methods The National Infantile Spasms Consortium established a multicenter, prospective database enrolling infants with new diagnosis of infantile spasms. Children were considered responders if there was clinical remission and resolution of hypsarrhythmia that was sustained at 3 months after first treatment initiation. Standard treatments of adrenocorticotropic hormone (ACTH), oral corticosteroids, and vigabatrin were considered individually, and all other nonstandard therapies were analyzed collectively. Developmental status and etiology were assessed. We compared response rates by treatment group using chi-square tests and multivariate logistic regression models. Results Two hundred thirty infants were enrolled from 22 centers. Overall, 46% of children receiving standard therapy responded, compared to only 9% who responded to nonstandard therapy (p < 0.001). Fifty-five percent of infants receiving ACTH as initial treatment responded, compared to 39% for oral corticosteroids, 36% for vigabatrin, and 9% for other (p < 0.001). Neither etiology nor development significantly modified the response pattern by treatment group. Interpretation Response rate varies by treatment choice. Standard therapies should be considered as initial treatment for infantile spasms, including those with impaired development or known structural or genetic/metabolic etiology. ACTH appeared to be more effective than other standard therapies.

Original languageEnglish (US)
Pages (from-to)475-484
Number of pages10
JournalAnnals of neurology
Volume79
Issue number3
DOIs
StatePublished - Mar 1 2016

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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    Knupp, K. G., Coryell, J., Nickels, K. C., Ryan, N., Leister, E., Loddenkemper, T., Grinspan, Z., Hartman, A. L., Kossoff, E. H., Gaillard, W. D., Mytinger, J. R., Joshi, S., Shellhaas, R. A., Sullivan, J., Dlugos, D., Hamikawa, L., Berg, A. T., Millichap, J., Nordli, D. R., & Wirrell, E. (2016). Response to treatment in a prospective national infantile spasms cohort. Annals of neurology, 79(3), 475-484. https://doi.org/10.1002/ana.24594