Arzimanoglou Alexis, French Jacqueline, Blume Warren T, Cross J Helen, Ernst Jan-Peter, Feucht Martha, Genton Pierre, Guerrini Renzo, Kluger Gerhard, Pellock John M, Perucca Emilio, Wheless James W
Institute for Children and Adolescents with Epilepsy-IDEE, University Hospitals of Lyon and INSERM U821, Lyon, France.
Lancet Neurol. 2009 Jan;8(1):82-93. doi: 10.1016/S1474-4422(08)70292-8.
Lennox-Gastaut syndrome is one of the most severe epileptic encephalopathies of childhood onset. The cause of this syndrome can be symptomatic (ie, secondary to an underlying brain disorder) or cryptogenic (ie, has no known cause). Although Lennox-Gastaut syndrome is commonly characterised by a triad of signs, which include multiple seizure types, slow spike-wave complexes on electroencephalographic (EEG) recordings, and impairment of cognitive function, there is debate with regard to the precise limits, cause, and diagnosis of the syndrome. Tonic seizures, which are thought to be a characteristic sign of Lennox-Gastaut syndrome, are not present at onset and the EEG features are not pathognomonic of the disorder. There are few effective treatment options for the multiple seizures and comorbidities, and the long-term outlook is poor for most patients. Probably as a result of the complexity of the disorder, only a few randomised trials have studied Lennox-Gastaut syndrome, and thus many of the drugs that are more commonly used have little or no supporting evidence base from controlled trials. In this Review, we discuss the main issues with regard to the diagnosis and treatment options available. We also suggest key considerations for future trials and highlight the importance of a comprehensive approach to the assessment and management of this syndrome.
伦诺克斯 - 加斯托综合征是儿童期起病的最严重的癫痫性脑病之一。该综合征的病因可以是症状性的（即继发于潜在的脑部疾病）或隐源性的（即病因不明）。尽管伦诺克斯 - 加斯托综合征通常以三联征为特征，包括多种发作类型、脑电图（EEG）记录上的慢棘慢复合波以及认知功能损害，但关于该综合征的确切界限、病因和诊断仍存在争议。强直发作被认为是伦诺克斯 - 加斯托综合征的特征性表现，但在起病时并不存在，且EEG特征并非该疾病所特有的。对于多种发作和合并症，有效的治疗选择很少，大多数患者的长期预后较差。可能由于该疾病的复杂性，仅有少数随机试验研究了伦诺克斯 - 加斯托综合征，因此许多更常用的药物几乎没有或完全没有来自对照试验的支持证据。在本综述中，我们讨论了有关诊断和现有治疗选择的主要问题。我们还提出了未来试验的关键考虑因素，并强调了对该综合征进行全面评估和管理的重要性。
