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Background <p>New treatment approaches are needed to manage refractory disease courses of Dravet syndrome (DS). In recent years, several case reports of the successful use of cenobamate (CNB) in DS have been published. The present case series and systematic review aims to evaluate the potential efficacy of CNB as a&#xa0;treatment alternative in DS based on additional treated cases and to critically summarize current data.</p> Methods <p>Two cases, an adolescent and a&#xa0;young adult with treatment-resistant DS, who were treated with CNB are presented in detail. To identify all available studies on the use of CNB in DS, we conducted a&#xa0;systematic literature search covering the period from 2019 to June 2025.</p> Results <p>We present two new case reports on treatment with CNB in <i>SCN1A</i>-associated DS, both of which led to seizure worsening: case&#xa0;1 describes a&#xa0;17-year-old adolescent with status epilepticus and seizure exacerbation while on CNB and case&#xa0;2 a&#xa0;23-year-old woman with increase in myoclonus. In the literature, 13&#xa0;additional cases could be identified. The overall analysis of 15&#xa0;cases shows no response or seizure worsening in 53%, with significant age dependency: the median age of responders was 25&#xa0;years, that of nonresponders 9&#xa0;years (<i>p</i> = 0.01).</p> Conclusion <p>Based on the reported cases, there appears to be an age-dependent response to CNB in patients with DS. Following the initially published successful treatments in DS adults, reports of seizure worsening are increasing, particularly in younger patients. The use of sodium channel blockers in DS is considered contraindicated, and this appears particularly the case in the first two decades of life. CNB should only be considered in DS after careful risk–benefit assessment and, if at all, preferably in adults.</p>

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Anfallszunahme beim SCN1A-assoziierten Dravet-Syndrom unter Cenobamat: Fallserie und Literaturübersicht

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年10月7日

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  • Gerhard Kurlemann,
  • Susanne Schubert-Bast,
  • Felix Rosenow,
  • Felix von Podewils,
  • Leena Kämppi,
  • Ruth Lehbrink,
  • Cecil D. Hahn,
  • Andreas Brunklaus,
  • Adam Strzelczyk

摘要

Background

New treatment approaches are needed to manage refractory disease courses of Dravet syndrome (DS). In recent years, several case reports of the successful use of cenobamate (CNB) in DS have been published. The present case series and systematic review aims to evaluate the potential efficacy of CNB as a treatment alternative in DS based on additional treated cases and to critically summarize current data.

Methods

Two cases, an adolescent and a young adult with treatment-resistant DS, who were treated with CNB are presented in detail. To identify all available studies on the use of CNB in DS, we conducted a systematic literature search covering the period from 2019 to June 2025.

Results

We present two new case reports on treatment with CNB in SCN1A-associated DS, both of which led to seizure worsening: case 1 describes a 17-year-old adolescent with status epilepticus and seizure exacerbation while on CNB and case 2 a 23-year-old woman with increase in myoclonus. In the literature, 13 additional cases could be identified. The overall analysis of 15 cases shows no response or seizure worsening in 53%, with significant age dependency: the median age of responders was 25 years, that of nonresponders 9 years (p = 0.01).

Conclusion

Based on the reported cases, there appears to be an age-dependent response to CNB in patients with DS. Following the initially published successful treatments in DS adults, reports of seizure worsening are increasing, particularly in younger patients. The use of sodium channel blockers in DS is considered contraindicated, and this appears particularly the case in the first two decades of life. CNB should only be considered in DS after careful risk–benefit assessment and, if at all, preferably in adults.