Objectives: To determine how continuous spike and wave during slow wave sleep (CSWS) is currently managed and to compare the effectiveness of current treatment strategies using a database from 11 pediatric epilepsy centers in the US. Study design: This retrospective study gathered information on baseline clinical characteristics, CSWS etiology, and treatment(s) in consecutive patients seen between 2014 and 2016 at 11 epilepsy referral centers. Treatments were categorized as benzodiazepines, steroids, other antiseizure medications (ASMs), or other therapies. Two measures of treatment response (clinical improvement as noted by the treating physician; and electroencephalography improvement) were compared across therapies, controlling for baseline variables. Results: Eighty-one children underwent 153 treatment trials during the study period (68 trials of benzodiazepines, 25 of steroids, 45 of ASMs, 14 of other therapies). Children most frequently received benzodiazepines (62%) or ASMs (27%) as first line therapy. Treatment choice did not differ based on baseline clinical variables, nor did these variables correlate with outcome. After adjusting for baseline variables, children had a greater odds of clinical improvement with benzodiazepines (OR 3.32, 95%CI 1.57-7.04, P = .002) or steroids (OR 4.04, 95%CI 1.41-11.59, P = .01) than with ASMs and a greater odds of electroencephalography improvement after steroids (OR 3.36, 95% CI 1.09-10.33, P = .03) than after ASMs. Conclusions: Benzodiazepines and ASMs are the most frequent initial therapy prescribed for CSWS in the US. Our data suggests that ASMs are inferior to benzodiazepines and steroids and support earlier use of these therapies. Multicenter prospective studies that rigorously assess treatment protocols and outcomes are needed.
Bibliographical noteFunding Information:
Supported by the National Institutes of Health (1K23NS116110 [to F.B.]; R21NS109669, R01DC016902, & U54HD090257 [to W.G.]; RO1HL147261 & RO1NS111166 [to R.S.]), the National Science Foundation (1532061 [to W.G.]), the Pediatric Epilepsy Research Foundation (to W.G., A.O., and R.S.), PCORI (to R.S.), PCORnet (to W.G.), and the University of Michigan (to R.S.). R.S. serves as a consultant for the Epilepsy Study Consortium, receives honoraria from UpToDate, serves as an Associate Editor for Neurology, and served on the Editorial Board for The Journal of Pediatrics. W.G. is the current president of the American Epilepsy Society. The other authors declare no conflicts of interest.T.L. discloses that he serves on the Council of the American Clinical Neurophysiology Society, as founder and consortium PI of the pediatric status epilepticus research group (pSERG), as an Associate Editor for Wyllie's Treatment of Epilepsy 6th edition and 7th editions (Wolters Kluwer), and as a member of the NORSE Institute, and CCEMRC. He served as Associate Editor of Seizure (Elsevier), and served on the Laboratory Accreditation Board for Long Term (Epilepsy and Intensive Care Unit) Monitoring, and American Board of Clinical Neurophysiology in the past. He has several patent applications for detection and prediction of clinical outcomes and seizures and is a co-inventor of the TriVox Health technology, for which he may be compensated in the future. He received research support from the Epilepsy Research Fund, NIH, CIMIT/DOD, PCORI, the Epilepsy Foundation of America, the American Epilepsy Society, the Epilepsy Therapy Project, the Pediatric Epilepsy Research Foundation, the Danny Did Foundation, Cure, the HHV-6 Foundation, and received research grants from Lundbeck, Eisai, Upsher-Smith, Mallinckrodt, Sunovion, SAgE, Empatica, Acorda, and Pfizer, including past device donations from various companies, including Empatica, SmartWatch, and Neuro-electrics. In the past, he served as a consultant for Eisai, Lundbeck, UCB, Amzell, Sunovion, Upsher Smith, and Zogenix. He has received speaker honorariums/Grand Round travel support from national/international societies and national/international academic centers and several trainees received salary support from international foundations/societies and academic centers while working in his laboratory.
© 2021 Elsevier Inc.
- Landau-Kleffner Syndrome
- developmental regression
- electrical status epilepticus of sleep