Lennox–Gastaut Syndrome: Challenges in Diagnosis – Interviews with Two Key Opinion Leaders

Abstract

Lennox–Gastaut syndrome (LGS) is a severe childhood-onset epilepsy syndrome that can be challenging to identify due to its heterogeneous and evolving presentation. During interviews conducted by EMJ in July and August 2023, Karen Keough, a child neurologist specialising in paediatric refractory epilepsy at Texas A&M University School of Medicine, Round Rock, USA, and Rhys Thomas, an adult neurologist specialising in epilepsy (leading on learning disability and genetic testing) at the Royal Victoria Infirmary, Newcastle, UK, and Newcastle University, UK, gave their informed opinions on the challenges of diagnosing LGS. The experts spoke about the characteristic features of LGS, and how its gradual evolution from childhood onset can impede diagnosis. In particular, the issues around diagnosing LGS in adulthood, after considerable disease evolution and against a background of uncertain/incomplete medical history, were discussed. The experts also considered the 2022 International League Against Epilepsy (ILAE) position paper for the definition of epilepsy syndromes in childhood, and its potential impact for directing the diagnosis of LGS. Keough and Thomas concluded by describing how raised awareness, vigilance, and support in diagnosis could help to optimise treatment and readjust the goals for the management of adults and children with LGS.