Institute for Children and Adolescents with Epilepsy - IDEE, University Hospital of Lyon (HCL) Lyon, France.
Epileptic Disord. 2011 May;13 Suppl 1:S3-13. doi: 10.1684/epd.2011.0422.
Lennox-Gastaut syndrome (LGS) is a severe, chronic, epileptic encephalopathy, primarily with childhood onset, which is characterised by a triad of features: multiple seizure types, including tonic seizures that may appear late in the course of the disorder; abnormal EEG features with slow spike-wave discharges; and cognitive impairment. Recognition of LGS is problematic, since the seizure types and EEG features that characterise it are not pathognomonic and often change over time. Furthermore, although seizures associated with LGS may occur de novo, the appearance of core LGS seizures may be preceded by prolonged periods of other seizure types, including myoclonic seizures, partial seizures, or infantile spasms. This has led some authors to postulate that a continuum between LGS and other types of childhood epilepsy may exist. Accurate diagnosis requires careful assessment of both clinical and EEG features, in order to distinguish LGS from other childhood epilepsy syndromes, such as atypical benign partial epilepsy of childhood, Dravet syndrome or epilepsies with predominantly myoclonic-astatic seizures. Since there is no biological marker that can be used to confirm diagnosis of LGS and because of the multiple aetiologies that could lead to its development, early referral to a specialist team may prove to be crucial for facilitating both diagnosis and management. Such an approach ensures that patients receive the appropriate treatment at the correct time, providing the best opportunity for the clinical course and overall prognosis to be improved. Effective management of LGS requires regular reappraisal of the evolving symptoms and features, and adjustment of the treatment accordingly.
Lennox-Gastaut 综合征(LGS)是一种严重的、慢性的、癫痫性脑病,主要发生在儿童时期,其特征为三联征:多种发作类型,包括可能在疾病过程后期出现的强直发作;具有慢棘慢波放电的异常脑电图特征;以及认知障碍。由于其特征性的发作类型和脑电图特征并非特异性的,且随着时间的推移经常发生变化,因此 LGS 的识别存在问题。此外,尽管与 LGS 相关的发作可能是新发的,但核心 LGS 发作的出现可能先于其他发作类型的长时间,包括肌阵挛发作、部分性发作或婴儿痉挛。这导致一些作者假设 LGS 和其他类型的儿童癫痫之间可能存在连续统。准确的诊断需要仔细评估临床和脑电图特征,以将其与其他儿童癫痫综合征区分开来,例如儿童非典型良性部分性癫痫、Dravet 综合征或主要为肌阵挛-强直发作的癫痫。由于没有可以用于确认 LGS 诊断的生物学标志物,并且由于多种病因可能导致其发展,因此早期向专家团队转诊可能对促进诊断和管理至关重要。这种方法可确保患者在正确的时间接受适当的治疗,为改善临床过程和总体预后提供最佳机会。LGS 的有效管理需要定期重新评估不断变化的症状和特征,并相应调整治疗。