THE LENNOX-GASTAUT SYNDROME
THE LENNOX-GASTAUT SYNDROME
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DOI：
10.1111/j.1528-1157.1993.tb04593.x
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发表时间：
1993-01-01
期刊：
影响因子：
5.6
通讯作者：
NGUYEN, T
中科院分区：
文献类型：
--
作者：
DULAC, O;NGUYEN, T
疾病分类学中最具挑战性的领域之一是幼儿期严重全身性癫痫领域。 Lennox-Gastaut 综合征（LGS）是一种与年龄相关的癫痫性脑病，包括多种类型的全身性癫痫发作，包括强直性癫痫发作、非典型失神癫痫发作和频繁的癫痫持续状态。脑电图显示普遍的慢棘波，随着疾病的进展，认知功能恶化。 LGS 与肌阵挛性癫痫发作和韦斯特综合征一起被列入 1989 年国际抗癫痫联盟的分类中。已经提出了许多变体或非典型形式。因此，鉴别诊断提出了重大挑战，包括特定的全身性癫痫，即代谢性或炎症性癫痫；继发性全身性癫痫，即源自额叶的癫痫；以及严重形式的特发性全身性癫痫，即杜斯综合征。 LGS 的抗癫痫药物 (AED) 治疗效果令人失望。胼胝体前切开术获得的结果是有希望的，但只有少数患者进行了评估。尽管这种综合征很罕见，但 LGS 的严重性和难治性强调了开发特定 AED 的必要性，这将彻底改变这些患者的生活质量。
One of the most challenging areas in nosology is in the field of severe generalized epilepsy of early childhood. This is certainly true in the case of Lennox-Gastaut syndrome (LGS), an age-related epileptogenic encephalopathy which comprises several types of generalized seizures including tonic seizures, atypical absence seizures and frequent status epilepticus. EEG shows generalized slow spike waves, and as the disease progresses, cognitive functions deteriorate. LGS is listed in the 1989 classification of the International League Against Epilepsy alongside epilepsy with myoclonic astatic seizures and West's syndrome. A number of variants or atypical forms have been proposed. As a result, differential diagnosis presents a major challenge and includes specific generalized epilepsies, i.e., metabolic or inflammatory; secondarily generalized epilepsies, i.e., those arising from the frontal lobe; and severe forms of idiopathic generalized epilepsy, i.e., Doose syndrome. Antiepileptic drug (AED) treatment of LGS has been disappointing. Results obtained from anterior callosotomy have been promising, but only a small number of patients have been evaluated. Although the syndrome is rare, the severe nature and intractability of LGS emphasizes the need for the development of specific AEDs which would completely modify the quality of life for these patients.