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.

An Italian consensus on the management of Lennox-Gastaut syndrome / Riva, A.; Coppola, A.; Bonaventura, C. D.; Elia, M.; Ferlazzo, E.; Gobbi, G.; Marini, C.; Meletti, S.; Romeo, A.; Santoro, K.; Verrotti, A.; Capovilla, G.; Striano, P.; Aguglia, U.; Bagnasco, I.; Bartolini, E.; Battaglia, D.; Beccaria, F.; Belcastro, V.; Bernardo, P.; Bonanni, P.; Boniver, C.; Bonuccelli, A.; Briatore, E.; Brigo, F.; Cesaroni, E.; Coa, R.; Costa, C.; D'Aniello, A.; De Giorgis, V.; Gennaro, G. D.; Ferrari, A. R.; Marchese, F.; Matricardi, S.; Messana, T.; Morano, A.; Operto, F. F.; Orsini, A.; Parmeggiani, L.; Peruzzi, C.; Pruna, D.; Puligheddu, M.; Pulitano, P.; Ragona, F.; Romigi, A.; Rosati, A.; Rosati, E.; Russo, A.; Sartori, S.; Spagnoli, C.; Spano, M.; Trabacca, A.; Troisi, S.; Viri, M.; Zucca, C.. - In: SEIZURE. - ISSN 1059-1311. - 101:(2022), pp. 134-140. [10.1016/j.seizure.2022.07.004]

An Italian consensus on the management of Lennox-Gastaut syndrome

Meletti S.;
2022

Abstract

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.
2022
101
134
140
An Italian consensus on the management of Lennox-Gastaut syndrome / Riva, A.; Coppola, A.; Bonaventura, C. D.; Elia, M.; Ferlazzo, E.; Gobbi, G.; Marini, C.; Meletti, S.; Romeo, A.; Santoro, K.; Verrotti, A.; Capovilla, G.; Striano, P.; Aguglia, U.; Bagnasco, I.; Bartolini, E.; Battaglia, D.; Beccaria, F.; Belcastro, V.; Bernardo, P.; Bonanni, P.; Boniver, C.; Bonuccelli, A.; Briatore, E.; Brigo, F.; Cesaroni, E.; Coa, R.; Costa, C.; D'Aniello, A.; De Giorgis, V.; Gennaro, G. D.; Ferrari, A. R.; Marchese, F.; Matricardi, S.; Messana, T.; Morano, A.; Operto, F. F.; Orsini, A.; Parmeggiani, L.; Peruzzi, C.; Pruna, D.; Puligheddu, M.; Pulitano, P.; Ragona, F.; Romigi, A.; Rosati, A.; Rosati, E.; Russo, A.; Sartori, S.; Spagnoli, C.; Spano, M.; Trabacca, A.; Troisi, S.; Viri, M.; Zucca, C.. - In: SEIZURE. - ISSN 1059-1311. - 101:(2022), pp. 134-140. [10.1016/j.seizure.2022.07.004]
Riva, A.; Coppola, A.; Bonaventura, C. D.; Elia, M.; Ferlazzo, E.; Gobbi, G.; Marini, C.; Meletti, S.; Romeo, A.; Santoro, K.; Verrotti, A.; Capovilla, G.; Striano, P.; Aguglia, U.; Bagnasco, I.; Bartolini, E.; Battaglia, D.; Beccaria, F.; Belcastro, V.; Bernardo, P.; Bonanni, P.; Boniver, C.; Bonuccelli, A.; Briatore, E.; Brigo, F.; Cesaroni, E.; Coa, R.; Costa, C.; D'Aniello, A.; De Giorgis, V.; Gennaro, G. D.; Ferrari, A. R.; Marchese, F.; Matricardi, S.; Messana, T.; Morano, A.; Operto, F. F.; Orsini, A.; Parmeggiani, L.; Peruzzi, C.; Pruna, D.; Puligheddu, M.; Pulitano, P.; Ragona, F.; Romigi, A.; Rosati, A.; Rosati, E.; Russo, A.; Sartori, S.; Spagnoli, C.; Spano, M.; Trabacca, A.; Troisi, S.; Viri, M.; Zucca, C.
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