Abstract：
Background. Lennox-Gastaut syndrome (LGS) is a developmental and epileptic encephalopathy (DEE) in which drug resistance to antiepileptic drugs (AEDs) is common. Focal-onset seizures (FOS) are among the seizure types characterizing LGS. Cenobamate (CNB) is a new AED indicated for the treatment of FOS and it has shown promising results in terms of seizure frequency reduction in both clinical trials and real-world experience. To date, the use of CNB in patients with DEEs is limited to Dravet syndrome. Methods: This was a retrospective study aimed to determine the 12-month effectiveness and tolerability of CNB in patients with LGS following real-world practice. Results: Four patients with LGS receiving CNB treatment were identified. At 12 months from starting CNB, the reduction in baseline seizure frequency ranged from 25 to 74%, with two patients achieving ≥50% seizure reduction. CNB was generally well tolerated and adjustments in doses of concomitant AEDs were required. Conclusions: CNB may represent a promising therapeutic option in patients with drug-resistant epilepsy associated with LGS. Further research is needed to confirm this preliminary evidence.
摘要：
背景。Lennox-Gastaut综合征（LGS）是一种发展性和癫痫性脑病（DEE），对抗癫痫药（AEDs）的耐药性很常见。局灶性发作性癫痫（FOS）是表征LGS的癫痫发作类型之一。Cenobamate（CNB）是一种用于治疗FOS的新型AED，在临床试验和实际经验中，它在减少癫痫发作频率方面都显示出了有希望的结果。迄今为止,在DEE患者中使用CNB仅限于Dravet综合征.方法：这是一项回顾性研究，旨在确定CNB在现实实践中对LGS患者的12个月有效性和耐受性。结果：确定了4例接受CNB治疗的LGS患者。从CNB开始12个月时，基线癫痫发作频率的降低范围为25％至74％，两名患者癫痫发作减少≥50%。CNB通常具有良好的耐受性,并且需要调整伴随的AED的剂量。结论：对于与LGS相关的耐药性癫痫患者，CNB可能是一种有希望的治疗选择。需要进一步的研究来证实这一初步证据。
