PMC

This figure demonstrates steps for inclusion and exclusion of patients. Patients were excluded due to insufficient clinical information, lack of EEG features meeting diagnostic criteria of electrical status epilepticus in sleep or lack of regression and developmental stagnation. Legend: EEG: Electroencephalogram. ESES: Electrical status epilepticus in sleep.

We provide information on patients 1 and 3 in this figure. The information in our whole patient population can be found in . The y-axis displays spike wave percentage on EEG and the x-axis displays the timing of clinical events, including different stages of the condition (as marked by abbrevations for epilepsy onset (EO), electrical status epilepticus in sleep (ESES), regression(R), and seizure freedom (SF) on the bottom of each figure), spike wave percentage as demonstrated by red flags, and antiepileptic treatment choices and respective duration displayed as horizontal bars on top of each figure. The different clinical events are displayed as triangles in the orange timeline. The vertical dotted line represents the time of last follow-up. Treatment with high-dose diazepam reduces epileptiform activity even when it is very elevated and this reduction persists for a period of several months in most cases. Legend: B: Birth. EO: Epilepsy onset. HDDZP: High-dose diazepam. LEV: Levetiracetam. R: Regression/stagnation. SF: Seizure freedom. T HDDZP: Taper high-dose diazepam. VPA: Valproate. ZNS: Zonisamide.