Cynthia Keator M.D., receives endowed chair to establish Lennox-Gastaut syndrome center of excellence

News and Announcements, Innovative Care | May 15, 2024 | Jane and John Justin Institute for Mind Health

Cynthia Keator, M.D., medical director of Neurology at the Jane and John Justin Institute for Mind Health, was recently named an Endowed Chair awardee from the W.I. Cook Foundation for her proposal to establish a Lennox-Gastaut Syndrome Center of Excellence. Lennox-Gastaut Syndrome (LGS) is a severe developmental and epileptic encephalopathy with onset in childhood, characterized by multiple seizure types (e.g. tonic, atonic, atypical absence, and tonic clonic) and characteristic electroencephalogram (EEG) findings (e.g. generalized slow spike wave and paroxysmal fast activity). The underlying etiologies vary considerably and the majority of patients will develop drug-resistant epilepsy (DRE) and suffer from significant comorbid cognitive, behavioral and developmental conditions as a result of this developmental epileptic encephalopathy. While multiple risk factors for developing LGS have been identified, early biomarkers indicating risk of progressing to slow spike wave pattern and LGS diagnosis remain elusive. Likewise, the natural history of LGS is not completely elucidated, making development of disease-modifying therapies with the potential to alter the course of this severe childhood epilepsy difficult. Furthermore, best practices for the diagnosis and treatment of comorbid cognitive and behavioral symptoms of this condition are lacking.

The LGS Center of Excellence aims to be a standardized care model for patients with LGS collecting clinical data and natural history to identify high-risk populations and biomarkers. In addition, such a clinical model provides opportunity to develop best practices for the diagnosis and treatment of LGS and its various comorbid developmental/behavioral conditions while providing a level of patient care and satisfaction unparalleled in other major epilepsy centers. To date, no center of this nature exists in the country.

The Jane and John Justin Institute for Mind Health provides the ideal treatment environment for such a clinical endeavor, capitalizing on the collaborative expertise of nine neuroscience specialties (i.e., neurology, neurosurgery, neuropsychology, psychology, psychiatry, developmental pediatrics, developmental psychology, physiatry, pain management) in addition to a robust team of research scientists within the Neurosciences Research Center. Through the Endowed Chair program, the LGS Center of Excellence will be developed utilizing the network of providers within the Justin Institute. By using this interconnected institute model, as opposed to a true multidisciplinary clinic, we'll be able to address the needs of this unique pediatric population in a more efficient manner. Using the team treatment approach, we'll 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. Our nationally ranked neurology program is a major referral center for patients with LGS with over 300 annual referrals for this condition providing ample patients for study. In addition, with approximately 49,000 children in Texas diagnosed with epilepsy, approximately 1,000 cases of LGS in our state may benefit from these services. We'll align our work with that of other institutions including the Pediatric Epilepsy Research Consortium (PERC) LGS Special Interest Group (SIG) and the LGS Foundation, maximizing the likelihood of meaningful outcomes from this endeavor.

The specific aims for this new program include:

Aim 1: Create and maintain a clinical team of specialists dedicated to the care of children with LGS, using quality improvement techniques to create standardized diagnosis and treatment algorithms to improve care in this vulnerable population.

Aim 2: Using standardized template(s) to document medical history in the health record, we'll create a natural history of LGS through the lifespan while identifying patient characteristics that confer highest risk to evolving to slow spike wave and LGS diagnosis.

Aim 3: Having identified the patients at highest risk for developing LGS, we'll identify novel biomarkers that predict LGS emergence through longitudinal multimodal imaging to allow early recognition and treatment to alter the course of this disease compared to natural history.

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