Neurol. praxi. 2025;26(5):415-424 | DOI: 10.36290/neu.2025.069

Lennox-Gastaut syndrome in adulthood

doc. MUDr. Irena Doležalová, Ph.D., prof. MUDr. Milan Brázdil, Ph.D.

Centrum pro epilepsie Brno, člen ERN-EpiCARE, I. neurologická klinika, Fakultní nemocnice u sv. Anny a Masarykovy univerzity, Brno

Lennox-Gastaut syndrome (LGS) represents a syndrome whose characteristics have recently been reviewed by the International League Against Epilepsy (ILAE). LGS is characterized not only by its development in childhood, but also by significant pharmacoresistance that greatly affects the quality of life of patients and their family members. The presence of specific seizures, including tonic seizures and at least one other type of seizure (atypical absences, atonic or myoclonic seizures, tonic-clonic seizures, focal impaired awareness seizures, non-convulsive status epilepticus, and epileptic spasms), is one of the key diagnostic features. An EEG investigation then reveals specific findings that are typical for this syndrome. In our article, we will focus in more detail on all these clinical and neurophysiological characteristics, their variability, and diagnostic specificities. We will also deal with the issue of diagnosing adult patients with LGS, which is an area that often poses a challenge in the clinical practice. In this context, we will introduce a specific screening tool - the REST-LGS questionnaire (Refractory Epilepsy Screening Tool for Lennox-Gastaut syndrome). In conclusion, we will highlight current treatment options that are suitable for patients with LGS, as well as mention novel and innovative therapeutic approaches, such as cannabidiol (CBD) or fenfluramine. The aim of this article is to increase awareness of the neurological community regarding the importance of accurately diagnosing LGS. The article is primarily focused on neurologists in charge of treating adult patients, rather than on paediatric neurologists who - in the vast majority of cases - are well acquainted with this entity.

Keywords: Lennox-Gastaut syndrome, tonic seizures, developmental and epileptic encephalopathies, slow 2.5 Hz spike-and-wave complexes (SW complexes), generalized paroxysmal fast activity (GPFA), fenfluramine, cannabidiol (CBD), REST-LGS (Refractory Epilepsy Screening Tool for Lennox-Gastaut syndrome).

Received: September 29, 2025; Revised: September 29, 2025; Accepted: September 29, 2025; Prepublished online: September 29, 2025; Published: November 6, 2025  Show citation