Epilepsy & behavior : E&B · 2026
Beyond seizures in Dravet and Lennox-Gastaut syndromes - An Italian Expert Consensus on Non-Seizure issues and the role of fenfluramine.
СтатьяConsensus StatementНазвание и аннотация: Перевод в очереди
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Di Gennaro Giancarlo, Battaglia Domenica Immacolata, Bonanni Paolo, Coppola Antonietta, Ferlazzo Edoardo, Gambardella Antonio, Liguori Claudio, Messana Tullio, Pruna Dario, Terrone Gaetano, Trivisano Marina, Di Bonaventura Carlo
Аннотация
BACKGROUND: Dravet syndrome (DS) and Lennox-Gastaut syndrome (LGS) are rare developmental and epileptic encephalopathies (DEEs) in which disability and family burden are driven by seizures and non-seizure issues (NSIs), including cognition, behavior, sleep, communication, motor function, and adaptive abilities.
OBJECTIVE: To develop practical consensus statements on NSIs in DS and LGS, integrating NSI evaluation within a DEE framework and appraising the perceived role of fenfluramine (FFA).
METHODS: An Italian panel of 12 epilepsy specialists spanning pediatric and adult practice developed statements using an Estimate-Talk-Estimate process. Consensus was predefined as ≥ 70% agreement (agree/strongly agree).
RESULTS: Eleven final statements were clustered into three macro-areas, with the first two explored in greater depth: (1) the cross-cutting, lifespan impact of NSIs in DS and LGS; (2) the potential for FFA to provide clinically meaningful benefits across selected NSI domains in DS and, more variably, in LGS, with effects not always fully captured by seizure metrics; and (3) the role of other interventions on NSIs and the need for structured assessment tools.
CONCLUSIONS: Incorporating NSI priorities into shared goal-setting and longitudinal monitoring may improve the clinical relevance of care in DS and LGS. The panel supported an NSI-informed, net-benefit approach to treatment optimization and identified FFA as a relevant option, while recognizing that evidence on NSI outcomes remains heterogeneous and should be strengthened through syndrome-specific, longitudinal assessment.
OBJECTIVE: To develop practical consensus statements on NSIs in DS and LGS, integrating NSI evaluation within a DEE framework and appraising the perceived role of fenfluramine (FFA).
METHODS: An Italian panel of 12 epilepsy specialists spanning pediatric and adult practice developed statements using an Estimate-Talk-Estimate process. Consensus was predefined as ≥ 70% agreement (agree/strongly agree).
RESULTS: Eleven final statements were clustered into three macro-areas, with the first two explored in greater depth: (1) the cross-cutting, lifespan impact of NSIs in DS and LGS; (2) the potential for FFA to provide clinically meaningful benefits across selected NSI domains in DS and, more variably, in LGS, with effects not always fully captured by seizure metrics; and (3) the role of other interventions on NSIs and the need for structured assessment tools.
CONCLUSIONS: Incorporating NSI priorities into shared goal-setting and longitudinal monitoring may improve the clinical relevance of care in DS and LGS. The panel supported an NSI-informed, net-benefit approach to treatment optimization and identified FFA as a relevant option, while recognizing that evidence on NSI outcomes remains heterogeneous and should be strengthened through syndrome-specific, longitudinal assessment.