Kelly G Knupp1, Jason Coryell2, Katherine C Nickels3, Nicole Ryan4, Erin Leister5, Tobias Loddenkemper6, Zachary Grinspan7, Adam L Hartman8, Eric H Kossoff8, William D Gaillard9, John R Mytinger10, Sucheta Joshi11, Renée A Shellhaas11, Joseph Sullivan12, Dennis Dlugos4, Lorie Hamikawa13, Anne T Berg14, John Millichap14, Douglas R Nordli14, Elaine Wirrell3. 1. Departments of Pediatrics and Neurology, School of Medicine, University of Colorado Anschutz Medical Campus, Aurora, CO. 2. Departments of Pediatrics and Neurology, School of Medicine, Oregon Health & Sciences University, Portland, OR. 3. Departments of Neurology and Pediatrics, Mayo Clinic, Rochester, MN. 4. Division of Neurology, Children's Hospital of Philadelphia and Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA. 5. Colorado School of Public Health, Department of Biostatistics and Informatics, University of Colorado Anschutz Medical Campus, Aurora, CO. 6. Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Boston Children's Hospital, Boston, MA. 7. Weill Cornell Medical Center, New York, NY. 8. Departments of Neurology and Pediatrics, Johns Hopkins Hospital, Baltimore, MD. 9. Center for Neuroscience, Children's National Health System, Dallas, TX. 10. Department of Pediatrics, Division of Pediatric Neurology, Ohio State University, Nationwide Children's Hospital, Columbus, OH. 11. Department of Pediatrics & Communicable Diseases (Division of Pediatric Neurology), University of Michigan, Ann Arbor, MI. 12. Departments of Pediatrics and Neurology, University of San Francisco, San Francisco, CA. 13. Department of Neurology, University of Washington, Seattle, WA. 14. Ann & Robert H. Lurie Children's Hospital of Chicago and Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL.
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.
OBJECTIVE:Infantile spasms are seizures associated with a severe epilepticencephalopathy 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.
Authors: Heather E Olson; Scott T Demarest; Elia M Pestana-Knight; Lindsay C Swanson; Sumaiya Iqbal; Dennis Lal; Helen Leonard; J Helen Cross; Orrin Devinsky; Tim A Benke Journal: Pediatr Neurol Date: 2019-02-23 Impact factor: 3.372
Authors: Renée A Shellhaas; Anne T Berg; Zachary M Grinspan; Courtney J Wusthoff; John J Millichap; Tobias Loddenkemper; Jason Coryell; Russell P Saneto; Catherine J Chu; Sucheta M Joshi; Joseph E Sullivan; Kelly G Knupp; Eric H Kossoff; Cynthia Keator; Elaine C Wirrell; John R Mytinger; Ignacio Valencia; Shavonne Massey; William D Gaillard Journal: Pediatr Neurol Date: 2017-06-27 Impact factor: 3.372
Authors: Juma S Mbwana; Zachary M Grinspan; Russell Bailey; Madison Berl; Jeffrey Buchhalter; Adrian Bumbut; Zach Danner; Tracy Glauser; Angie Glotstein; Howard Goodkin; Brian Jacobs; Lisa Jones; Barbara Kroner; Gardiner Lapham; Tobias Loddenkemper; Demetrius M Maraganore; Doug Nordli; William D Gaillard Journal: Neurol Clin Pract Date: 2019-02
Authors: Kelly G Knupp; Erin Leister; Jason Coryell; Katherine C Nickels; Nicole Ryan; Elizabeth Juarez-Colunga; William D Gaillard; John R Mytinger; Anne T Berg; John Millichap; Douglas R Nordli; Sucheta Joshi; Renée A Shellhaas; Tobias Loddenkemper; Dennis Dlugos; Elaine Wirrell; Joseph Sullivan; Adam L Hartman; Eric H Kossoff; Zachary M Grinspan; Lorie Hamikawa Journal: Epilepsia Date: 2016-09-12 Impact factor: 5.864