Definition and natural history of Lennox-Gastaut syndrome
Definition and natural history of Lennox-Gastaut syndrome
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DOI：
10.1111/j.1528-1167.2011.03177.x
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发表时间：
2011-08-01
期刊：
影响因子：
5.6
通讯作者：
Camfield, Peter R.
中科院分区：
文献类型：
--
作者：
Camfield, Peter R.
Lennox-Gastaut 综合征 (LGS) 是一种罕见的癫痫性脑病，发病高峰年龄为 3-5 岁。 LGS 的报告患病率差异很大，占所有儿童癫痫的 1-10%。发病率要低得多。 LGS 的特点是顽固性、多发性、全身性癫痫发作类型，发作间期脑电图显示缓慢的棘波爆发、全身多棘波的阵发性爆发和缓慢的背景。所有患者在睡眠期间都会出现轻微的强直性癫痫发作，而且几乎所有患者都患有难治性的终生癫痫。几乎所有患者都会出现认知停滞和行为问题，并导致生活依赖。鉴别诊断包括其他有症状的全身性癫痫和假伦诺克斯综合征。误诊很常见。患有 LGS 的儿童和成人对其家庭产生巨大影响，改善这些患者的生活质量的努力非常复杂。
Lennox-Gastaut syndrome (LGS) is a rare epileptic encephalopathy with a peak age of onset of 3-5 years of age. Reported prevalence rates for LGS vary widely from 1-10% of all childhood epilepsies. Incidence rates are much lower. LGS is characterized by intractable, multiple, generalized seizure types and an interictal electroencephalogram showing bursts of slow spike-and-wave, paroxysmal bursts of generalized polyspikes, and a slow background. All patients have tonic seizures during sleep that may be subtle, and nearly all have treatment-resistant, lifelong epilepsy. Cognitive stagnation and behavioral problems are seen in almost all patients and lead to a life of dependency. The differential diagnosis includes other symptomatic generalized epilepsies and pseudo-Lennox syndrome. Misdiagnosis is common. Children and adults with LGS have an enormous impact on their families, and efforts to improve the quality of life for these patients are complex.