Honbolygó Ferenc, Csépe Valéria, Fekésházy Attila, Emri Miklós, Márián Teréz, Sárközy Gergely, Kálmánchey Rozália
Research Institute for Psychology, Hungarian Academy of Sciences, Department of Psychophysiology, Research Group of Developmental Psychophysiology, H-1068, Szondi u. 83-85, Budapest, Hungary.
Clin Neurophysiol. 2006 Feb;117(2):295-305. doi: 10.1016/j.clinph.2005.10.016. Epub 2006 Jan 4.
To assess the linguistic abilities of a boy having Landau-Kleffner Syndrome, and relate the focal disturbance of brain activity due to epilepsy to the cognitive and linguistic deficits.
Several kinds of assessments were carried out, including epileptic source analysis using electronic source localization methods and PET, neuropsychological assessment of cognitive functions, and assessment of speech perception skills (discrimination of phonetic and stress cues) using ERPs.
The source of epileptic activity was localized in the left superior temporal lobe. The neuropsychological assessment showed dissociation between verbal and nonverbal functions, and the performance in former was bellow the normal range. ERPs obtained to the processing of phonetic and stress speech cues indicated that the two cues were processed asymmetrically: the mismatch negativity component (MMN) was obtained for the phoneme difference, but not for the stress pattern difference.
Our data converged as it showed that the patient presented a selective impairment of the language system, and the verbal working memory system appeared to be especially defective. It is suggested that the language deficit is at least partly due to the focal disturbance of those neural networks that underlie the functioning of the working memory system.
LKS is a childhood language disorder that might serve as a model in studying what happens to the language system if, in the course of development, the essential neural circuits are severely disturbed.
评估一名患有 Landau-Kleffner 综合征男孩的语言能力，并将癫痫导致的大脑活动局灶性紊乱与认知和语言缺陷联系起来。
进行了多种评估，包括使用电子源定位方法和正电子发射断层扫描（PET）进行癫痫源分析、对认知功能进行神经心理学评估，以及使用事件相关电位（ERP）评估语音感知技能（语音和重音线索辨别）。
癫痫活动源定位于左侧颞上叶。神经心理学评估显示言语和非言语功能之间存在分离，且前者的表现低于正常范围。对语音和重音语音线索处理所获得的 ERP 表明，这两种线索的处理是不对称的：对于音素差异可获得失匹配负波成分（MMN），但对于重音模式差异则未获得。
我们的数据表明，该患者存在语言系统的选择性损伤，且言语工作记忆系统似乎尤其受损。提示语言缺陷至少部分归因于那些构成工作记忆系统功能基础的神经网络的局灶性紊乱。
Landau-Kleffner 综合征是一种儿童期语言障碍，在研究如果在发育过程中基本神经回路受到严重干扰，语言系统会发生什么情况时，它可能作为一个模型。
