Dini Gianluca, Tulli Eleonora, Dell'Isola Giovanni Battista, Mencaroni Elisabetta, Di Cara Giuseppe, Striano Pasquale, Verrotti Alberto
Department of Pediatrics, University of Perugia, Genoa, Italy.
Pediatric Neurology and Muscular Diseases Unit, IRCCS "G. Gaslini" Institute, Genoa, Italy.
Front Pharmacol. 2022 May 20;13:832929. doi: 10.3389/fphar.2022.832929. eCollection 2022.
Epilepsy is among the most common neurological chronic disorders, with a prevalence of 0.5-1%. Despite the introduction of new antiepileptic drugs during recent years, about one third of the epileptic population remain drug-resistant. Hence, especially in the pediatric population limited by different pharmacokinetics and pharmacodynamics and by ethical and regulatory issues it is needed to identify new therapeutic resources. New molecules initially used with other therapeutic indications, such as fenfluramine, are being considered for the treatment of pharmacoresistant epilepsies, including Dravet Syndrome (DS) and Lennox-Gastaut Syndrome (LGS). Drug-refractory seizures are a hallmark of both these conditions and their treatment remains a major challenge. Fenfluramine is an amphetamine derivative that was previously approved as a weight loss drug and later withdrawn when major cardiac adverse events were reported. However, a new role of fenfluramine has emerged in recent years. Indeed, fenfluramine has proved to be a promising antiepileptic drug with a favorable risk-benefit profile for the treatment of DS, LGS and possibly other drug-resistant epileptic syndromes. The mechanism by which fenfluramine provide an antiepileptic action is not fully understood but it seems to go beyond its pro-serotoninergic activity. This review aims to provide a comprehensive analysis of the literature, including ongoing trials, regarding the efficacy and safety of fenfluramine as adjunctive treatment of pharmacoresistant epilepsies.
癫痫是最常见的慢性神经系统疾病之一,患病率为0.5%-1%。尽管近年来引入了新的抗癫痫药物,但约三分之一的癫痫患者仍耐药。因此,特别是在受不同药代动力学和药效学以及伦理和监管问题限制的儿科人群中,需要确定新的治疗资源。最初用于其他治疗适应症的新分子,如芬氟拉明,正被考虑用于治疗耐药性癫痫,包括德雷维特综合征(DS)和伦诺克斯-加斯东综合征(LGS)。药物难治性癫痫发作是这两种疾病的一个标志,其治疗仍然是一项重大挑战。芬氟拉明是一种苯丙胺衍生物,以前被批准作为减肥药,后来在报告了重大心脏不良事件后被撤回。然而,近年来芬氟拉明出现了新的作用。事实上,芬氟拉明已被证明是一种有前景的抗癫痫药物,在治疗DS、LGS以及可能的其他耐药性癫痫综合征方面具有良好的风险效益比。芬氟拉明发挥抗癫痫作用的机制尚未完全了解,但似乎超出了其促5-羟色胺能活性。本综述旨在对有关芬氟拉明作为耐药性癫痫辅助治疗的疗效和安全性的文献进行全面分析,包括正在进行的试验。