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Abstract Details

Corticosteroid Treatment of Developmental and/or Epileptic Encephalopathy with Spike-wave Activation in Sleep (D/EE-SWAS): A Pediatric Epilepsy Research Consortium (PERC) Survey Study
Epilepsy/Clinical Neurophysiology (EEG)
S36 - Epilepsy Basic Mechanisms, Status Epilepticus, and Mortality (1:36 PM-1:48 PM)
004
Corticosteroids are considered among the most effective therapies to improve developmental outcomes in D/EE-SWAS, based mostly on small retrospective series, however use is limited by potential side effects (SEs) and high relapse rates. Optimal corticosteroid formulation, dosing, taper, and SE surveillance methods have not been defined. 
To define practice preferences, surveillance methods, and perceived barriers for corticosteroid treatment of D/EE-SWAS in the United States.
A conditionally-branching 50-57 item survey was distributed to members of PERC, a multicenter research collaboration spanning >77 US pediatric epilepsy centers. 

70 respondents participated with a 90% completion rate. 98% reported caring for primarily pediatric patients. 97% reported board certification/eligibility in child neurology, 84% in epilepsy, and 48% in clinical neurophysiology. 98% trained in the US. A majority were 5-10 or 10-20 years out of training. 

20% of respondents preferred corticosteroids as first line, while 65% preferred benzodiazepines. When utilizing corticosteroids, 65% preferred daily dosing, 10% preferred pulsed dosing, and 24% preferred a combined daily/pulsed regimen. The preferred daily corticosteroid was prednisone (94%). There was major variability in treatment duration (<1 to 6 months) and taper duration (2 to >12 weeks). Most taper regimens were non-standardized. IV methylprednisolone was the most preferred pulsed corticosteroid (50%), with treatment ranging from <1 to 12 months. All respondents used EEG to monitor treatment response, performed <4 to 12 weeks after treatment initiation. 

Few SEs were surveilled using a formal protocol. Possibility of SEs was the most impactful barrier to corticosteroid use. Respondents reported being more comfortable with managing SEs than their practices reflected.  

There is considerable variability in corticosteroid use for D/EE-SWAS. Daily prednisone and pulsed IV methylprednisolone were the most preferred treatment choices. There was little consensus regarding treatment or taper duration. There is opportunity to better engage neurologists in surveillance and management of SEs through practice standardization. 
Authors/Disclosures
Donald J. Phillips, MD, MPH (CHOC Children's)
PRESENTER
Dr. Phillips has nothing to disclose.
David J. Adams, MD (CHOC Neurology) Dr. Adams has nothing to disclose.
Sonal O. Bhatia, MD (Medical University of South Carolina) Dr. Bhatia has nothing to disclose.
Anthony L. Fine, MD (Mayo Clinic) The institution of Dr. Fine has received research support from Neurocrine Biosciences. The institution of Dr. Fine has received research support from UCB. The institution of Dr. Fine has received research support from American Board of Psychiatry and Neurology.
William D. Gaillard, MD (Children'S National Hospital) The institution of Dr. Gaillard has received research support from all federal or foundation grants, NINDS, NIDCD, NICHD, NSF ,PERF.
Sarah A. Kelley, MD (Johns Hopkins Hopsital) Dr. Kelley has received publishing royalties from a publication relating to health care. Dr. Kelley has received personal compensation in the range of $500-$4,999 for serving as a Author with Elsevier. Dr. Kelley has received personal compensation in the range of $10,000-$49,999 for serving as a Speaker with Med Learning Group.
Suad Y. Khalil, MD Dr. Khalil has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Ucb.
Virginia Liu, MD (UCSF) Dr. Liu has nothing to disclose.
John McLaren, MD Dr. McLaren has nothing to disclose.
Lekha M. Rao, MD Dr. Rao has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Sutton Pierce. Dr. Rao has stock in Epygenix, Inc. The institution of Dr. Rao has received research support from UCB. The institution of Dr. Rao has received research support from Eisai.
Robert C. Stowe, MD (Boston Children's Hospital) Dr. Stowe has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Neurocrine Biosciences. Dr. Stowe has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Takeda Pharmaceuticals. Dr. Stowe has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Kherkher Garcia, LLP.