Reference Centre of Refractory Epilepsy, Cliniques Universitaires St Luc, Université Catholique de Louvain, Avenue Hippocrate, 10, B-1200 Brussels, Belgium.
Neuropsychiatr Dis Treat. 2008 Dec;4(6):1001-19. doi: 10.2147/ndt.s1668.
Lennox-Gastaut syndrome (LGS) is a rare, age-related syndrome, characterized by multiple seizure types, a specific electro-encephalographic pattern, and mental regression. However, published data on the etiology, evolution, and therapeutic approach of LGS are contradictory, partly because the precise definition of LGS used in the literature varies. In the most recent classification, LGS belongs to the epileptic encephalopathies and is highly refractory to all antiepileptic drugs. Numerous treatments, medical and non-medical, have been proposed and results mostly from open studies or case series have been published. Sometimes, patients with LGS are included in a more global group of patients with refractory epilepsy. Only 6 randomized double-blind controlled trials of medical treatments, which included patients with LGS, have been published. Overall, treatment is rarely effective and the final prognosis remains poor in spite of new therapeutic strategies. Co-morbidities need specific treatment. This paper summarizes the definition, diagnosis and therapeutic approach to LGS, including not only recognized antiepileptic drugs, but also "off label" medications, immune therapy, diet, surgery and some perspectives for the future.
Lennox-Gastaut 综合征（LGS）是一种罕见的、与年龄相关的综合征，其特征为多种癫痫发作类型、特定的脑电图模式和智力衰退。然而，关于 LGS 的病因、演变和治疗方法的已发表数据相互矛盾，部分原因是文献中使用的 LGS 的精确定义有所不同。在最近的分类中，LGS 属于癫痫性脑病，对所有抗癫痫药物均高度耐药。已经提出了许多治疗方法，包括医学和非医学方法，并且主要来自开放研究或病例系列研究。有时，LGS 患者被纳入更广泛的难治性癫痫患者群体。只有 6 项关于医学治疗的随机双盲对照试验，其中包括 LGS 患者，已发表。总体而言，尽管有新的治疗策略，但治疗效果很少，最终预后仍然很差。合并症需要特定的治疗。本文总结了 LGS 的定义、诊断和治疗方法，包括公认的抗癫痫药物以及“超适应证”药物、免疫疗法、饮食、手术和一些未来的展望。
