Research Insights on Developmental Epileptic Encephalopathy With Spike Wave Activation in Sleep: Lekha Rao, MD

WATCH TIME: 3 minutes

"Awareness is key—this is a unique entity we can only diagnose with an overnight EEG capturing slow-wave sleep spikes."

Developmental/epileptic encephalopathy with spike wave activation in sleep (D/EE-SWAS), previously known as electrical status epilepticus in slow-wave sleep (ESES), is a rare and severe epilepsy syndrome primarily affecting children. It is characterized by nearly continuous epileptiform discharges during non-REM sleep, accompanied by significant neurocognitive and behavioral impairments. In this patient population, seizure types vary, including absence seizures, tonic-clonic seizures, focal seizures, and atonic seizures; however, in some cases, seizures may not be prominent.

At the 2024 American Epilepsy Society (AES) Annual Meeting, held December 6-10, in Los Angeles, California, a poster presentation focused on the characterization of 30 patients with D/EE-SWAS over a 3-year period in an epilepsy monitoring unit at the University of California Los Angeles (UCLA). The study followed patients for an average of 3.5 years, with D/EE-SWAS onset at an average age of 6 years, 80% of whom had epilepsy starting at an average age of 3.87 years.

During the meeting, NeurologyLive® sat down with study author Lekha Rao, MD, to learn more about the build of the study and some of the main outcomes. Rao, an epileptologist at UCLA Health and associate professor of pediatric neurology, provided insight on the significance of the criteria for the study for this less common patient population. In the discussion, she discussed the challenges in diagnosis, the significance of overnight EEG monitoring, and the potential for EEG features to provide key insights into patient subtypes and disease progression.

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REFERENCE
1. Rao L. Characteristics of Patients with D/EE-SWAS and Their Response to Certain Therapies: Experience at a Single Major Academic Center. Presented at: 2024 AES Annual Meeting; December 6-10; Los Angeles, CA. ABSTRACT 1.304.