Luat Aimee F, Chugani Harry T, Asano Eishi, Juhász Csaba, Trock Gary, Rothermel Robert
Carman and Ann Adams Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit, MI, USA.
Brain Dev. 2006 Oct;28(9):592-6. doi: 10.1016/j.braindev.2006.03.006. Epub 2006 May 2.
We report a four-year-old boy with Landau-Kleffner Syndrome (LKS) characterized by episodic receptive aphasia lasting for few weeks followed by gradual recovery of baseline language functions. Neuropsychological evaluation during an episode showed severe impairment in verbal skills and comprehension, but relative preservation of non-verbal skills. Although he could carry on a conversation during remission, neuropsychological evaluation demonstrated findings reflective of receptive language deficits. Prolonged EEG disclosed frequent sharp-wave activity in the left and right temporal regions but no electrographic seizures. Glucose metabolism PET scan during the fourth episode of aphasia revealed intense hypermetabolism in the left temporal neocortex. Awake EEG during the PET tracer uptake period showed 6.0 spikes/min in the left temporal region and 4.0 spikes/min in the right. A repeat PET scan during remission showed hypometabolism in the left temporal cortex. Awake EEG during the FDG uptake period showed 16.0 spikes/min in the right temporal region and 0.3 spikes/min in the left. During his fifth aphasic episode, EEG (without PET) showed 0.16 spikes/min in the right temporal region and none in the left. Intermittent short episodes of predominantly receptive aphasia with near-total recovery between episodes can be one of the clinical presentations of LKS. This case illustrates the dynamic changes of glucose metabolism in the temporal lobe during episodes of aphasia and remission in a case with LKS. The clinical course of our patient with transient EEG and PET findings suggest that glucose metabolism in LKS cannot be attributed solely to interictal epileptiform activities on scalp EEG.
我们报告了一名患有 Landau-Kleffner 综合征（LKS）的4岁男孩，其特征为发作性感受性失语持续数周，随后基线语言功能逐渐恢复。发作期间的神经心理学评估显示言语技能和理解能力严重受损，但非言语技能相对保留。尽管他在缓解期能够进行对话，但神经心理学评估显示出反映感受性语言缺陷的结果。长时间脑电图显示左、右颞叶区域频繁出现尖波活动，但无脑电图癫痫发作。在失语症第四次发作期间进行的葡萄糖代谢正电子发射断层扫描（PET）显示左侧颞叶新皮质强烈代谢亢进。PET 示踪剂摄取期间的清醒脑电图显示左侧颞叶区域有 6.0 次/分钟的尖波，右侧有 4.0 次/分钟。缓解期重复进行的 PET 扫描显示左侧颞叶皮质代谢减退。FDG 摄取期间的清醒脑电图显示右侧颞叶区域有 16.0 次/分钟的尖波，左侧有 0.3 次/分钟。在他第五次失语发作期间，脑电图（无 PET）显示右侧颞叶区域有 0.16 次/分钟的尖波，左侧无尖波。间歇性短暂发作的主要为感受性失语且发作间期近乎完全恢复，可能是 LKS 的临床表现之一。本病例说明了 LKS 患者失语发作期和缓解期颞叶葡萄糖代谢的动态变化。我们这位患者短暂脑电图和 PET 检查结果的临床病程表明，LKS 中的葡萄糖代谢不能仅归因于头皮脑电图上的发作间期癫痫样活动。
