Development of syndrome severity scores for pediatric epilepsy

Purpose: A severity rating for each of the pediatric epilepsy syndromes can contribute to a more comprehensive understanding of seizure condition severity. We describe the development of the Epilepsy Syndrome Severity Scores-Child (ESSS-C). Methods: The Delphi Technique was used to establish severity scores for pediatric epileptic syndromes as defined by the International League Against Epilepsy (ILAE). Pediatric neurologists with expertise in epilepsy were asked to rate the severity of each syndrome on a scale of 1 to 10, considering: (a) response to medical treatment, (b) seizure severity, and (c) long-term prognosis. Syndrome severity ratings took place in four different rounds. Experts provided initial scores in rounds 1 and 2. Results: Of the 18 experts who agreed to participate in the development of the scale, 12 completed all four rounds. The median served as the syndrome severity score if the mean and median agreed within 0.5. Otherwise, whichever of these two numbers was closest to the mode was selected. Syndromes that were rated with high severity scores (9 or 10) or low severity scores (1 or 2) had unanimous or near unanimous ratings. The syndromes with scores in the middle range (4 to 8) had a wider range of ratings. Conclusions: If further studies provide empirical support for the validity of these epileptic syndrome severity scores, then the ESSS-C has potential for use both clinically and in future research in the prediction of outcomes of treatments.