Child Neurology Section, Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States.
Epilepsy Behav. 2021 Jan;114(Pt A):107612. doi: 10.1016/j.yebeh.2020.107612. Epub 2020 Nov 24.
Lennox-Gastaut syndrome (LGS) is a childhood-onset epileptic encephalopathy characterized by multiple types of medically intractable seizures, cognitive impairment, and generalized slow spike-wave discharges in electroencephalography (EEG). Although the onset of this epileptic syndrome occurs typically before eight years of age with a peak age between 3 and 5 years, lifelong persistence of the syndrome is usual. The evolution of clinical features, EEG findings, and paucity of knowledge about LGS among adult health care providers can make LGS significantly underdiagnosed in the adult population. Management of LGS remains problematic beyond childhood due to intractable seizures, the difficult transition from pediatric to adult neurologists, challenging behaviors, impaired cognition, poor quality of life, and disabled social life. In focusing on the management of LGS beyond childhood, this narrative review describes medical and surgical management of epilepsy, the transition from pediatric to adult care, and management of other common comorbidities associated with LGS. Several antiepileptic drugs (AEDs) such as lamotrigine, topiramate, felbamate, rufinamide, clobazam, and Epidiolex (pure pharmaceutical grade cannabidiol (CBD) oil) have been noted to be effective in well-designed, randomized controlled trials. Other non-pharmacological therapies, such as vagus nerve stimulation, ketogenic diet, and epilepsy surgery, have been frequently utilized in the management of intractable seizures associated with LGS. However, effective management of LGS requires a broader perspective to not only control seizures but improve the quality of life by addressing cognitive and behavioral problems, sleep disturbances, physical disability, social disability, and educational and employment challenges.
Lennox-Gastaut 综合征（LGS）是一种儿童期起病的癫痫性脑病，其特征为多种药物难治性癫痫发作、认知障碍和脑电图（EEG）中的广泛慢棘慢波放电。尽管这种癫痫综合征的发病通常在 8 岁之前，高峰年龄在 3 至 5 岁之间，但该综合征通常会终生持续。由于成年医护人员对该癫痫综合征认识不足、临床表现、脑电图表现的演变以及缺乏关于 LGS 的知识，LGS 在成年人群中可能会被严重漏诊。由于难治性癫痫、从儿科到成人神经科医生的过渡困难、行为问题、认知障碍、生活质量差和社交生活受限，LGS 在儿童期后仍难以管理。在关注儿童期后 LGS 的管理时，本综述描述了癫痫的药物和手术治疗、从儿科到成人护理的过渡以及与 LGS 相关的其他常见合并症的管理。几种抗癫痫药物（AEDs），如拉莫三嗪、托吡酯、氨己烯酸、鲁非酰胺、氯巴占和 Epidiolex（纯制药级大麻二酚（CBD）油），已在设计良好的随机对照试验中被证明是有效的。其他非药物治疗方法，如迷走神经刺激、生酮饮食和癫痫手术，在 LGS 相关难治性癫痫的治疗中经常被应用。然而，要有效管理 LGS，不仅需要控制癫痫发作，还需要从认知和行为问题、睡眠障碍、身体残疾、社交残疾以及教育和就业挑战等方面来改善生活质量，需要有更广泛的视角。
