TY - JOUR
T1 - The impact of hypsarrhythmia on infantile spasms treatment response
T2 - Observational cohort study from the National Infantile Spasms Consortium
AU - the Pediatric Epilepsy Research Consortium
AU - Demarest, Scott T.
AU - Shellhaas, Renée A.
AU - Gaillard, William D.
AU - Keator, Cynthia
AU - Nickels, Katherine C.
AU - Hussain, Shaun A.
AU - Loddenkemper, Tobias
AU - Patel, Anup D.
AU - Saneto, Russell P.
AU - Wirrell, Elaine
AU - Sánchez Fernández, Iván
AU - Chu, Catherine J.
AU - Grinspan, Zachary
AU - Wusthoff, Courtney J.
AU - Joshi, Sucheta
AU - Mohamed, Ismail S.
AU - Stafstrom, Carl E.
AU - Stack, Cynthia V.
AU - Yozawitz, Elissa
AU - Bluvstein, Judith S.
AU - Singh, Rani K.
AU - Knupp, Kelly G.
N1 - Funding Information:
Funding for this study was received from the American Epilepsy Society and the Pediatric Epilepsy Research Foundation. We would like to give special thanks to Elizabeth Juarez-Colunga, for her assistance with the biostatistical analysis.
Funding Information:
Knupp reports grants from the American Epilepsy Society and Pediatric Epilepsy Research Fund, during the conduct of the study; and grants from Pediatric Epilepsy Research Fund, grants from Zogenix, outside the submitted work. Gaillard reports grants from the Pediatric Epilepsy Research Foundation, during the conduct of the study. Patel reports grants from the Pediatric Epilepsy Research Foundation (PERF), grants from Upsher-Smith, grants and personal fees from LivaNova, grants and personal fees from GW Pharmaceuticals, grants from Brain Sentinal, and personal fees from UCB Pharma, personal fees from Supernus, outside the submitted work. Loddenkemper serves on the Laboratory Accreditation Board for Long Term (Epilepsy and Intensive Care Unit) Monitoring, on the Council (and as 2nd Vice President) of the American Clinical Neurophysiology Society, on the American Board of Clinical Neurophysiology, as an Associate Editor for Seizure, and as an Associate Editor for “Wyllie’s Treatment of Epilepsy, 6th edition.” He is part of pending patent applications to detect and predict seizures and to diagnose epilepsy. He receives research support from the Epilepsy Research Fund, the American Epilepsy Society, the Epilepsy Foundation of America, the Epilepsy Therapy Project, Patient-Centered Outcomes Research Institute, the Pediatric Epilepsy Research Foundation, Citizens United for Research in Epilepsy, HHV-6 Foundation, and received research grants from Lundbeck, Eisai, Upsher-Smith, Acorda, and Pfizer. He serves as a consultant for Zogenix, Upsher Smith, and Lund-beck. All other authors have no conflicts of interest to report. We confirm that we have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines.
PY - 2017/12
Y1 - 2017/12
N2 - Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results: Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4–4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9–5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03–1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06–2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2, 95% CI 2–13.7), prednisolone (OR 8, 95% CI 3.1–20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1–25.8). Significance: First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.
AB - Objective: The multicenter National Infantile Spasms Consortium prospective cohort was used to compare outcomes and phenotypic features of patients with infantile spasms with and without hypsarrhythmia. Methods: Patients aged 2 months to 2 years were enrolled prospectively with new-onset infantile spasms. Treatment choice and categorization of hypsarrhythmia were determined clinically at each site. Response to therapy was defined as resolution of clinical spasms (and hypsarrhythmia if present) without relapse 3 months after initiation. Results: Eighty-two percent of patients had hypsarrhythmia, but this was not associated with gender, mean age, preexisting developmental delay or epilepsy, etiology, or response to first-line therapy. Infants with hypsarrhythmia were more likely to receive standard treatment (adrenocorticotropic hormone, prednisolone, or vigabatrin [odds ratio (OR) 2.6, 95% confidence interval (CI) 1.4–4.7] and preexisting epilepsy reduced the likelihood of standard treatment (OR 3.2, 95% CI 1.9–5.4). Hypsarrhythmia was not a determinant of response to treatment. A logistic regression model demonstrated that later age of onset (OR 1.09 per month, 95% CI 1.03–1.15) and absence of preexisting epilepsy (OR 1.7, 95% CI 1.06–2.81) had a small impact on the likelihood of responding to the first-line treatment. However, receiving standard first-line treatment increased the likelihood of responding dramatically: vigabatrin (OR 5.2, 95% CI 2–13.7), prednisolone (OR 8, 95% CI 3.1–20.6), and adrenocorticotropic hormone (ACTH; OR 10.2, 95% CI 4.1–25.8). Significance: First-line treatment with standard therapy was by far the most important variable in determining likelihood of response to treatment of infantile spasms with or without hypsarrhythmia.
KW - Adrenocorticotropic hormone
KW - Epilepsy
KW - Prednisolone
KW - Vigabatrin
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U2 - 10.1111/epi.13937
DO - 10.1111/epi.13937
M3 - Article
C2 - 29105055
AN - SCOPUS:85032956189
VL - 58
SP - 2098
EP - 2103
JO - Epilepsia
JF - Epilepsia
SN - 0013-9580
IS - 12
ER -