Abstracts

Rationale: The care of children with increased activation of epileptiform discharges in sleep has been a contested subject in child neurology. Prior studies found variable definitions for the terms “electrical status epilepticus in sleep” (ESES) and “continuous spike and wave in slow wave sleep” (CSWS), and lack of agreement regarding treatment (Sanchez-Fernandez 2013, 2014). Since then, the scope of current practice has not been evaluated. The goal of this project was to assess practice patterns in the treatment of sleep potentiated discharges and ESES, including definitions, diagnosis, and treatment amongst practicing child neurology and pediatric epilepsy providers.

Methods: A 22-item survey was distributed to members of the Child Neurology Society and the American Epilepsy Society. Respondents were queried on subspecialty training, clinical practice, definitions of ESES and CSWS, and were presented with vignettes describing children with sleep-potentiated spikes and a spectrum of clinical presentations.

Results: 270 respondents participated with completion rates of 70-94% per item. The majority (158/255/n, 62%) were board certified. 62% (158/255) were board certified in clinical neurophysiology and/or epilepsy, with 58% (148/255/n) primarily practicing epilepsy. Nearly all completed their highest level of training in the United States.

Regarding diagnostic criteria, 99% (221/223) and 97% (216/223) selected that excessive spike waves on EEG were required in ESES and CSWS respectively. On EEG criteria, 56% (127/228) required spikes to occupy at least 85% of sleep, 37% (86/228) required at least 50% of sleep, and 7% (15/228) had no formal cut-off. The most frequent method for evaluation of ESES was the spike-wave index (SWI, percentage of 1-second bins containing spikes in NREM sleep) (64%) followed by subjective visual diagnosis (18%), then spike counts (12%).

For the vignettes, all respondents would treat a child with regression and almost all (93%) would treat a child with developmental stagnation, while fewer considered treatment based on diagnoses of autism (79%) or ADHD (56%). The spike burden required to initiate treatment varied depending on clinical severity; 46% would treat if regression was present even if spikes occupied less than 50% of sleep, whereas only 4% would treat a child with ADHD and a similar spike burden. Benzodiazepines were the preferred treatment (range 42-52% between scenarios) followed by antiseizure medication (range 22-41%); steroids were considered primarily only for children presenting with regression (21% vs 7-8% between scenarios).

Conclusions: Variability in the diagnosis and treatment of ESES-related syndromes remains high, similar to findings reported nearly a decade ago. Benzodiazepines were the preferred treatment choice, in contrast to steroids typically used by our European colleagues (van den Munckhof 2015). Ongoing multicenter collaboration, future prospective studies, and the need for expert consensus are needed for the standardized classification, diagnosis, and treatment for children with ESES-related disorders.

Funding: Please list any funding that was received in support of this abstract.: N/A.