Authors :
Presenting Author: Elia Pestana Knight, MD – Cleveland Clinic Epilepsy Center
Author: Deepak Lachhwani, MD – Cleveland Clinic Epilepsy Center
Xiaoming Zhang, BS – Cleveland Clinic Epilepsy Center
Ahsan Naduvil Valappil, MD – Cleveland Clinic Epilepsy Center
Ajay Gupta, MD – Cleveland Clinic Epilepsy Center
Prakash Kotagal, MD – Cleveland Clinic Epilepsy Center
Rationale:
ILAE adopted the name of Developmental/Epileptic Encephalopathy with Spike Wave Activation in Sleep (DEE-SWAS) to group conditions with cognitive, language, behavioral and motor regression associated with significant spike and wave activation during sleep (SWAS). The EEG pattern in SWAS is the electrographic status epilepticus of sleep (ESES). We retrospectively reviewed the electroclinical presentation and changes in management and treatment response for children who had ESES defined as a spike wave index of more than 50%Methods:
After IRB approval, the authors identified patients with ESES from the EEG database of Cleveland Clinic from 2012-2022. SWI was calculated by counting number of seconds with at least 1 spike in a 10-minute epoch. Outcome for this study included only EEG changes seen at follow up. Response to treatment was Effective when SWI reduced < 50% or Ineffective when SWI was unchanged or increased. Benzodiazepines counted as antiseizure medications (ASMs)Results:
49 patients had ESES; 31 males (63.2%) and 18 females (36.7%). Results summarized in Table 1. The median age was 11.5y, (mean 12.1y). The median age of epilepsy onset was 2.9 ± 2.6y, and diagnosis of ESES at a median age of 6.8 ± 2.3y. All patients experienced seizures prior to ESES diagnosis, with focal epilepsy in 29 patients, generalized epilepsy in 17, and combined focal and generalized epilepsy in 2 patients. Seventeen patients (34.7%) had the diagnosis of self-limited epilepsy of childhood (SeLFE). Developmental regression of speech and language, cognition, behavior or motor and auditory agnosia, occurred in 69.4% of patients. Etiology of epilepsy was post-infectious in 2 patients, genetic in 4 patients, structural in 12 patients, and unknown in 31 patients.
At the time of ESES diagnosis, 42 patients (90%) were receiving ASMs. Treatment modifications following the ESES diagnosis (Figure 1) included adding another ASM (25 patients), increasing the dose of the current ASM (10 patients), initiating steroid therapy (12 patients), and combining steroids with intravenous therapy in 2 patients. Six patients (12.2%) went on to have epilepsy surgery. ESES treatment outcome was effective in 31 patients (63.3%), ineffective in 17 patients (34.7%) and unknown in 1 patient (2.0%). For the group of patients with SelFE, new ASM was added in 11/17, higher dose of ASM in 3/17 and steroids in 3/17. SeLFE group treatment outcome was effective 13/17 and ineffective 4/17Conclusions:
This single center cohort of patients with h/o seizures followed by EEG evidence of ESES was due to unknown etiology in more than 50% of the patients. Like other US studies (Baumer FM et al, 2021), ASM (a new ASM or a higher dose of the existing ASM) was the most common treatment change after identification of ESES. One third of the patients received steroids and 12.25% ultimately had epilepsy surgery. Patients with SeLFE received similar medical management. The follow up EEG was obtained at a variable time to monitor effect of intervention. Study of this cohort highlights the need for a more systematic definition, diagnosis, treatment and follow up approach for patients with ESESFunding: None