REST LGS TOOL: real World Use to Screen for LGS and Improve Access to Care
Abstract number :
2.106
Submission category :
4. Clinical Epilepsy / 4B. Clinical Diagnosis
Year :
2022
Submission ID :
2204703
Source :
www.aesnet.org
Presentation date :
12/4/2022 12:00:00 PM
Published date :
Nov 22, 2022, 05:26 AM
Authors :
Steven Wolf, MD – Boston Children’s Health Physicians of NY and CT; New York Medical College; Danielle Boyce, PhD, MPH – Johns Hopkins School of Medicine Department of Neurology; Kate Davis, MD – University of Pennsylvania Perelman School of Medicine, Department of Neurology; Patricia McGoldrick, NP, MPA, MSN – Boston Children’s Health Physicians of NY and CT; New York Medical College; Patricia Peña Artiles, MD – New York Medical College; Jesus Pina-Garza, MD – ew York Medical College The Children's Hospital at TriStar Centennial; Jessica Roland, PhD – Jazz Pharmaceuticals; Bethany Thomas, DNP – University of Pennsylvania Perelman School of Medicine, Department of Neurology; Donika Zogejani, BS – New York Medical College
Rationale: Lennox-Gastaut syndrome (LGS) is frequently undiagnosed in the adult population due to lack of prior history and poor transfer of pediatric testing results to adult providers. Symptoms of LGS evolve over time and the characteristic features may no longer be present in adulthood. The Refractory Epilepsy Screening Tool for LGS patients (REST-LGS) was created to improve identification of patients with LGS. The tool previously underwent a preliminary validation and was reliably used in both epilepsy experts and nonexperts. REST-LGS consists of 8 criteria (4 major, 4 minor) with potential LGS being defined as having 3 major criteria and 2 to 3 minor criteria. The current study aimed to further validate the tool in a real world setting of adults with intractable epilepsy that reside in a residential facility. Identifying adults likely to meet criteria for LGS, but with no formal diagnosis, could improve access to care.
Methods: A retrospective chart review of 100 patients aged 18 and older with DRE (drug resistant epilepsy) and intellectual development disorder (IDD) was conducted. Reviews were performed by two primary care providers that were diagnosis blinded. Reviewers indicated which, if any, of these criteria the patients met.
Results: Of the 74 individuals that did not have a previous LGS diagnosis, REST-LGS identified 42 individuals as having potential LGS. Of the 26 individuals who had a previous LGS diagnosis, 12 were classified as "unlikely LGS.” The majority of these 12 individuals had unavailable data related to the major criteria including EEG results, age of seizure onset, and history of cognitive impairment.
Conclusions: REST-LGS indicated that over half of the individuals without a previous LGS diagnosis should be referred to an epilepsy center for further diagnostic evaluation. Charts reviewed had extensive missing data, which highlights the challenges with diagnosing LGS in an adult population. REST-LGS enables the user to identify previously undiagnosed intractable epilepsy patients with potential LGS, thus opening the door to specialized care.
Funding: Jazz Pharmaceuticals
Clinical Epilepsy