The Lennox Gastaut Center of Excellence is open to patients
Cynthia Keator, M.D., and Jerico Pimentel, APRN have begun seeing patients at the Lennox Gastaut Center of Excellence, part of the Jane and John Justin Institute for Mind Health.
Recently, the International League Against Epilepsy defined Lennox Gastaut Syndrome (LGS), a severe developmental and epileptic encephalopathy, based on the following criteria:
- Onset before 18 years old
- Tonic seizures with at least one additional seizure type
- Mild to profound intellectual disability
- Drug-resistant epilepsy
- Diffuse slow spike-and-wave complexes (SSW) at ≤2.5 Hz
- Generalized paroxysmal fast activity (GPFA) in sleep on EEG
The center aims to be a standardized care model of how our clinical team will follow patients with LGS, to collect clinical data, gather natural history and identify high-risk populations to then investigate biomarkers. To date, no center of this nature exists in the country.
Our nationally ranked neurology program is a major referral center for patients with LGS. The program has more than 300 annual referrals for this condition, providing ample patients for study. With around 49,000 children in Texas diagnosed with epilepsy, approximately 1,000 cases of LGS in our state may benefit from these services. We will align our work with that of other institutions including the Pediatric Epilepsy Research Consortium, LGS Special Interest Group and the LGS Foundation, maximizing the likelihood of meaningful outcomes from this endeavor.
Dr. Keator, an Endowed Chair at Cook Children’s, has an international review board for patients with LGS, and the patients are seen in conjunction with Dr. Keator’s research team, gathering specific details for her natural history study. The goal is to see patients once a year in the Lennox Gastaut Center of Excellence. Their neurology provider will continue to see them for their ongoing neurological care.
The center will gather the history of the syndrome looking at multiple factors, like age of onset, medications, imaging, EEGs and genetic testing. Part of the program is also addressing and identifying other comorbidities related to LGS such as development, sleep, cognition and behavior.
All patient data lives in the patient’s electronic medical record and will be used to analyze specific questions and identify patterns of patients with LGS. Using a team treatment approach, the center will develop and improve treatment algorithms using iterative expertise, while collecting robust, standardized clinical data that will both answer key natural history research questions while forming the foundation of our search for biomarkers.