Development and Validation of the Refractory Epilepsy Screening Tool for Lennox-Gastaut Syndrome (REST-LGS): Improving Detection of LGS (P4.240)

Objective: To develop and validate a clinical screening tool to improve the identification of patients with Lennox-Gastaut Syndrome (LGS). Background: The accurate diagnosis of LGS is complex, and misdiagnosis can result in suboptimal treatment. To improve both identification and treatment of patients with this severe epileptic encephalopathy, the REST-LGS was developed using the Delphi method, and inter-rater reliability was measured as a first step in validating the tool for clinical use. Design/Methods: Using de-identified records of patients >12 years with refractory epilepsy and ≥2 neurology clinic notes, 4 major and 4 minor potential diagnostic criteria were evaluated on individual case report forms by two raters (specialist, non-specialist) at two epilepsy centers. Extent of inter-rater reliability at each site was measured by Cohen’s k. Results: Of 200 records evaluated (100/site), 81%–85% patients met 1–3 major criteria. At Site 1, moderate agreement (k=0.40–0.60) was reached between raters on whether patient records reflected persistent seizures despite trial of ≥2 antiepileptic drugs; at Site 2, both raters agreed that all patients had persistent seizures. Moderate agreement to good agreement (k=0.40–0.80) was reached at each site on the following criteria: ≥2 seizure types; seizure onset ≤12 years; history of EEG with generalized slow spike-and-wave discharges Conclusions: Overall, this first step in validation of the REST-LGS revealed moderate to very good agreement on criteria indicative of LGS among raters at two epilepsy centers. Study Supported by: Lundbeck LLC Disclosure: Dr. Boyce has received personal compensation for activities with Lundbeck LLC and Mallinckrodt as a member of the speakers bureau or as a consultant. Dr. Pina-Garza has received personal compensation for activities with Eisai, Lundbeck LLC, Supernus, and UCB Pharma as a consultant and member of the speakers bureau. Dr. Cantu has received personal compensation for activities with Lundbeck LLC as an employee. Dr. Davis has received personal compensation for activities with Guidepoint Global, GLG Consulting, Schlesinger Associates, and Lundbeck as a consultant. Dr. Gatens has nothing to disclose. Dr. Lai has nothing to disclose. Dr. McGoldrick has received personal compensation for activities with Sunovion, Supernus, and Lundbeck. Dr. Thomas has received personal compensation for activities with Lundbeck LLC as a consultant. Dr. Wolf has received personal compensation for activities with Sunovion, Supernus, Lundbeck LLC, and Eisai.