Nieto Barrera M, López Alcaide M I, Candau Fernández-Mensaque R, Ruiz del Portal Bermudo L, Rufo Campos M, Correa Charro A
Sección de Neuropediatría, Virgen del Rocío, Sevilla.
An Esp Pediatr. 1997 Dec;47(6):611-7.
The association of a language disorder with epilepsy is observed in some circumstance, with or without a causal relationship. In Landau-Kleffner syndrome (LKS), it is estimated that the aphasia is directly caused by epileptic discharges in language areas.
Ten children with LKS are studied. The clinical and electroencephalographic characteristics in these ten cases were analyzed.
Aphasia was present between 3 years and 6 years 5 months of age (X: 4 years 8 months) in a progressive form in 8 cases and abruptly in 2 cases. The epileptic seizures present in nine children began between 22 months and 7 years 3 months of age (X: 4 years 1 month). Focal, multifocal and/or generalized discharges, unstable and variable, were frequently noted during awake state EEG records and on EEGs during the sleep state in four children continuous spike waves during 75-80% of slow sleep were observed.
We discuss the importance of the different clinical and EEG findings in the evolution of aphasia which condition the longterm prognosis, emphasizing the value of the discharges on the sleep EEGs. This suggests that LKS could be the severe form of a more widespread age-dependent epileptic syndrome that also includes the CSWS (epilepsy with continuous spike and waves during slow sleep) and the atypical benign partial epilepsy. Three syndromes have cognitive and behavioral manifestations and continuous spike waves during slow sleep.
在某些情况下可观察到语言障碍与癫痫之间的关联，无论是否存在因果关系。在 Landau-Kleffner 综合征（LKS）中，据估计失语是由语言区域的癫痫放电直接引起的。
对 10 例 LKS 患儿进行研究。分析这 10 例病例的临床和脑电图特征。
8 例患儿的失语呈进行性，出现在 3 岁至 6 岁 5 个月之间（平均：4 岁 8 个月），2 例患儿失语突然出现。9 例患儿的癫痫发作始于 22 个月至 7 岁 3 个月之间（平均：4 岁 1 个月）。在清醒状态脑电图记录以及 4 例患儿睡眠状态脑电图中经常观察到局灶性、多灶性和/或全身性放电，不稳定且多变，在 75 - 80%的慢波睡眠期间观察到持续性棘波。
我们讨论了不同临床和脑电图结果在失语演变中的重要性，这些结果影响长期预后，强调睡眠脑电图放电的价值。这表明 LKS 可能是一种更广泛的年龄依赖性癫痫综合征的严重形式，该综合征还包括 CSWS（慢波睡眠期持续性棘慢波癫痫）和非典型良性部分性癫痫。这三种综合征都有认知和行为表现以及慢波睡眠期持续性棘波。
