3D figure of epilepsy syndromes

Abstract

We propose an instructive figure that summarized the classification of epilepsy syndromes according to the 2022 report of the ILAE Task Force on Nosology and Definitions. Our aim is to present on the same figure different concepts such as the names of epilepsy syndromes, their extreme and classical ages of onset, their epilepsy types (generalized, focal, or generalized and focal) but also their membership in groups of epilepsy syndromes as for self-limited or developmental and epileptic encephalopathies. With this figure, we provide an interactive tool, as supplementary data, helping to present this classification and link it to electro-clinical mandatory, alerts, and exclusionary criteria of each syndrome, in accordance with the ILAE position papers on syndromes classification and nosology. This report may be used as an illustrative tool for teaching epilepsy syndromes and as a practical and comprehensive aid for the classification of epilepsy individuals' syndromes.

Keywords: developmental and epileptic encephalopathy; epilepsy syndromes; interactive classification; self-limited epilepsies; teaching tool.

FIGURE 1

The epilepsy syndromes according to the 2017 epilepsy syndrome classification and the 2022 report of the ILAE Task Force on Nosology and Definitions,, , , , using the full names of the different epilepsy syndromes (A) or their specific abbreviations (B). The different epilepsy syndromes are divided into different colored areas according to their type of seizure: generalized in yellow, focal in blue, or combined focal and generalized in green. The concentric dotted circles indicated the age of onset of the syndrome. For each epilepsy syndrome, the dotted line represented the extreme age of onset and the thick line the classical age. The main groups of epilepsy syndromes are depicted in specific colors: self‐limiting epilepsies in turquoise, idiopathic generalized epilepsies in orange, developmental and/or epileptic encephalopathies and syndromes with progressive neurological deterioration in red and genetic generalized epilepsies in purple. Note that idiopathic generalized epilepsies are a subcategory of genetic generalized epilepsies. Developmental and Epileptic Encephalopathy (DEEs) are a group of epilepsy syndromes where impairments (encephalopathy) are due to both the underlying etiology of the epilepsy and frequent seizures or epileptiform discharges, whereas in epileptic encephalopathy (EE), the encephalopathy is due to frequent seizures or epileptiform discharges. A horizontal axis was used to separate self‐limited and/or pharmacoresponsive epilepsies (white diagonal hatching) from commonly drug‐resistant epilepsies. The letters used for the abbreviations of the different epilepsy syndromes have been bolded. It is important to bear in mind that this figure reflects the current organization of epilepsy syndromes as defined in the 2022 report of the ILAE Working Group on Nosology and Definitions, but it gives scant information on the underlying etiologies.