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
Donald Phillips1, David Adams1, Sonal Bhatia2, Anthony Fine3, William Gaillard4, Sarah Kelley5, Suad Khalil6, Virginia Liu1, John McLaren7, Lekha Rao8, Robert Stowe7
1Children's Hospital Orange County, 2Medical University of South Carolina, 3Mayo Clinic, 4Children's National Hospital, 5Johns Hopkins Hospital, 6Michigan State University, 7Boston Children's Hospital, 8UCLA Mattel Children's Hospital
Objective:
To define practice preferences, surveillance methods, and perceived barriers for corticosteroid treatment of D/EE-SWAS in the United States.
Background:
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.
Design/Methods:
A conditionally-branching 50-57 item survey was distributed to members of PERC, a multicenter research collaboration spanning >77 US pediatric epilepsy centers.
Results:
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.
Conclusions:
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.
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