Abstract：
Lennox-Gastaut syndrome (LGS) is a severe developmental epileptic encephalopathy associated with numerous neurological signs and symptoms. Altered postural tone and the need for a caregiver-assisted wheelchair are features characterizing patients with LGS. Highly purified cannabidiol (CBD) is a novel antiseizure medication (ASM) recommended for seizure treatment, in combination with clobazam, in patients with LGS. Adding CBD to the previous ASM treatment helps in reducing seizure frequency, specifically drop seizures, in patients with LGS in both clinical trials and real-world studies. However, no data about drug effects on postural tone, motor activity, gait, and stability are available. In this case series, three adult patients diagnosed with LGS were treated with CBD as an add-on. During the follow-up, a slight improvement in seizure frequency was observed. Unexpectedly, an amelioration in postural tone and stability, measured using the validated Gross Motor Function Classification System, was also detected. Our case series suggests that CBD may help in managing patients with LGS regarding seizure control and in improving other aspects of the clinical spectrum of the disease, such as postural tone and stability. The mechanisms at the basis of this improvement may be related, other than seizure reduction, to the drug\'s effect on the brain locomotor centers, as demonstrated in animal model studies.
摘要：
Lennox-Gastaut综合征（LGS）是一种严重的发展性癫痫性脑病，与许多神经系统体征和症状有关。改变的姿势语气和需要护理人员辅助轮椅是LGS患者的特征。高纯度大麻二酚（CBD）是一种推荐用于癫痫治疗的新型抗癫痫药物，与clobazam结合，LGS患者。将CBD添加到以前的抗癫痫药物治疗中有助于减少癫痫发作频率，特别是减少癫痫发作，在临床试验和现实世界研究中，LGS患者。然而,没有关于药物对姿势语气影响的数据，运动活动,步态和稳定性是可用的。在这个系列中,三名诊断为LGS的成年患者接受了CBD作为附加治疗。在后续行动中,观察到癫痫发作频率略有改善。出乎意料的是,姿势语气和稳定性的改善，使用经过验证的粗大运动功能分类系统进行测量，也被发现了。我们的病例系列表明，CBD可能有助于管理LGS患者关于癫痫发作控制和改善疾病临床谱的其他方面，如姿势语气和稳定性。这种改进的基础上的机制可能是相关的，除了减少癫痫发作，药物对大脑运动中心的影响，在动物模型研究中证明。
