Caution Should Be Taken When Implementing the 2021 BASED Score and “hypsarrthymia Scoring System” for Individuals with Developmental Epileptic Encephalopathy Without Epileptic Spasms
Abstract number :
1.261
Submission category :
3. Neurophysiology / 3C. Other Clinical EEG
Year :
2024
Submission ID :
1180
Source :
www.aesnet.org
Presentation date :
12/7/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Itay Tokatly Latzer, MD – Boston Children's Hospital
Stephanie Donatelli, MD – Boston Children's Hospital
Catherine Salussolia, MD – Boston Children's Hospital
Christina Briscoe Abath, MD, EdM – Boston Children's Hospital
Ann Bergin, MD – Boston Children's Hospital
Mark Libenson, MD – Boston Children's Hospital
Chellamani Harini, MD – Boston Children's Hospital, Harvard Medical School
Rationale: The 2021 Burden of AmplitudeS and Epileptiform Discharges (BASED) score and the “Hypsarrthymia Scoring System” formulated by Kramer et al. in 1997 are tools used to evaluate the severity of background and interictal EEG findings associated with infantile epileptic spasms syndrome (IESS) and correlate them with outcome. In the setting of infantile spasms, the 2021 BASED score has been shown to reliably predict the presence of ongoing epileptic spasms. We aimed to apply these scoring systems also to EEG tracings of individuals with developmental epileptic encephalopathy (DEE) without epileptic spasms to determine if their scores can distinguish IESS from other DEEs.
Methods: The deidentified pre-treatment EEG tracings of 47 children [25 children with IESS (“IESS Group”), median (IQR) age of 8 (5-11) months and 22 children with DEE and multifocal epileptiform EEG (“DEE Group”), median (IQR) age 15 (11-22) months] were reviewed by four pediatric epileptologists from a single medical center. Five-minute sleep epochs and ten-second epochs containing the most representative and severe segments of these EEG tracings were scored according to the 2021 BASED score and Kramer et al. “Hypsarrthymia Scoring System,” respectively. Inter-rater reliability was determined by a two-way mixed, absolute agreement intraclass correlation coefficient.
Results: The interrater reliability of the 2021 BASED score was 0.93 (CI 0.82-0.98) and of the Kramer et al. Hypsarrthymia Scoring System 0.83 (CI 0.65-0.92). Compared to children from the IESS Group, those from the DEE Group had significantly lower median values of their 2021 BASED scores (p=0.02). However, the number of subjects with high 2021 BASED scores (4 and 5) [n=22 (88%) in the IESS group and n=16 (72%) in the DEE group] did not differ significantly (p=0.18). Likewise, the groups were comparable in the median number of subjects with a high score (above 8), according to the Kramer et al. Hypsarrthymia Scoring System.
Conclusions: The 2021 BASED and more so the Kramer et al. Hypsarrthymia Scoring System may yield high scores in individuals with DEE without epileptic spasms. While these scoring systems are useful for prognosis in established IESS, they should be used with caution in infants with DEE and it is particularly important that the presence of epileptic spasms is confirmed clinically or electrographically in this group, before an IESS diagnosis is made.
Funding: None.
Neurophysiology