An Italian consensus on the management of Lennox-Gastaut syndrome

Seizure. 2022 Oct;101:134-140. doi: 10.1016/j.seizure.2022.07.004. Epub 2022 Jul 15.


Purpose: Although international guidelines exist, the clinical heterogeneity of Lennox-Gastaut syndrome (LGS) and the increasing availability of new and repurposed drugs (e.g., fenfluramine and cannabidiol) requires a practical guide to patient management in the clinical context. We report the results of a consensus survey among 42 Italian experts in the diagnosis and treatment of LGS.

Methods: The consensus procedure followed a modified Delphi approach. Statements were formulated, based on the most recent published evidence and the clinicians' personal experience, then discussed, and agreed upon by the experts through a two-round voting procedure. Approval of a statement was reached with an average score ≥7.

Results: Thirteen statements dealing with three main topics (i.e., clinical diagnosis and prognosis, impact on the Quality of Life (QoL), and treatment strategies) were generated. Six statements achieved a level of agreement sufficient for approval on the first voting round. Following the discussion and a few consequent amendments, most of the statements increased their level of agreement and all 13 were approved.

Conclusions: Overall, the statements draw a slightly more benign picture of this rare and severe disease, highlighting the possibility of remission - albeit modest -, an apparent trend towards lower mortality, and the availability of several effective drugs, to which greater accessibility would be hoped for. Valproate remains a major therapeutic option in LGS patients although lamotrigine, rufinamide, topiramate, cannabidiol, and clobazam are popular therapeutic options in Italy, allowing for a tailor-made antiseizure therapy.

Keywords: Antiseizure medications; Consensus; Lennox-Gastaut syndrome; Pediatric epilepsy.

MeSH terms

  • Anticonvulsants / therapeutic use
  • Cannabidiol* / therapeutic use
  • Clobazam / therapeutic use
  • Consensus
  • Fenfluramine / therapeutic use
  • Humans
  • Lamotrigine
  • Lennox Gastaut Syndrome* / diagnosis
  • Lennox Gastaut Syndrome* / drug therapy
  • Quality of Life
  • Topiramate / therapeutic use
  • Valproic Acid / therapeutic use


  • Anticonvulsants
  • Topiramate
  • Cannabidiol
  • Fenfluramine
  • Clobazam
  • Valproic Acid
  • Lamotrigine