Characterization of Patients with Isolated Electrical Status Epilepticus of Slow-wave Sleep (ESES)
Abstract number :
2.163
Submission category :
4. Clinical Epilepsy / 4C. Clinical Treatments
Year :
2023
Submission ID :
748
Source :
www.aesnet.org
Presentation date :
12/3/2023 12:00:00 AM
Published date :
Authors :
Presenting Author: Jennifer Meylor, MD – Medical College of Wisconsin
Pradeep Javarayee, MD – Medical College of Wisconsin; Elham Abushanab, MD – Medical College of Wisconsin; Avantika Singh, MD – Medical College of Wisconsin; Raquel Farias-Moeller, MD – Medical College of Wisconsin; Hema Patel, MD – Medical College of Wisconsin; Shannon Pollock, MD – Medical College of Wisconsin; Jennifer Koop, PhD – Medical College of Wisconsin; Rhonda Werner, MS, APNP – Children's Wisconsin; Rene Andrade-machado, MD, PhD – Medical College of Wisconsin
Rationale: Electrographic Status Epilepticus of Slow Wave Sleep (ESES) is continuous spike-and-wave discharges on electroencephalogram (EEG) during sleep in patients who have epilepsy. ESES can have risk of significant cognitive regression and worsening seizure burden. Treatment for patients with ESES is helpful to prevent further cognitive decline. However, there are a subset of patients with ESES whom, upon diagnosis of ESES, do not show signs of cognitive regression or increased seizure and may benefit from a more conservative approach. Since ESES is rare and treatment is currently the standard of care, it is difficult to study patient outcomes without treatment. Therefore, the goal of this study design is to better characterize this subset of patients who present with isolated ESES by analyzing electrographic and neuropsychological data.
Methods: A total of 124 patients with ESES admitted to the Children’s Hospital of Wisconsin Epilepsy Monitoring Unit between 2013 to present day were included in the study. Electrographic data from continuous EEG was reviewed to analyze the increase in percentage of spike-wave index (SWI) between awake and sleep stage. Ripples (defined as at least four oscillations standing out from the background in the 80-Hz high-pass-filtered signal) were quantified if seen. Neuropsychological data, specifically from the Wechsler Intelligence Scale for Children-V or an abbreviated subset of tests, was obtained at least six months from time of ESES diagnoses.
Results: A total of 23 patients were identified with no signs of cognitive regression or worsening seizure burden at the time of diagnosis with ESES. Patient demographics, including electrographic data, are summarized in Table 1. The median age at diagnosis was six years. A total of 22% of patients (N=16) had an identified lesion on neuroimaging. 39% of patients (N=9) had ripples on EEG. There was no correlation between ripples and neuroimaging, percentage SWI, or activation percentage between sleep and awake state. All patients had Full Scale IQ (FSIQ) < 69 (Intellectually Deficient) at the time of neuropsychologic testing. Six patients had a second neuropsychological test. Two of these patients had cognitive improvement and four patients showed cognitive decline. Cognitive outcome did not appear associated with presence of ripples or percentage SWI. All patients with improved cognitive function were seizure free at the most recent follow-up visit, whereas all patients with worsening cognitive function continued to have refractory seizures. Furthermore, of the patients with worsening cognitive function, three continued to meet the criteria for ESES at most recent follow-up.
Conclusions: Treatment for ESES is currently the standard of care given risk of cognitive decline and worsening seizure burden. However, for the subset of patients who present with isolated ESES there is little information guiding management. Our study starts to answer this question by better characterizing patients with isolated ESES. Limitations of this data include small sample size, further indicating the need for larger prospective studies in this cohort.
Funding: None
Clinical Epilepsy