Division of Neurology, National Center for Child Health and Development, Tokyo, Japan.
Department of Laboratory Medicine, University of Tokyo, Tokyo, Japan.
Neuropediatrics. 2022 Aug;53(4):295-298. doi: 10.1055/s-0041-1733983. Epub 2021 Dec 1.
Landau-Kleffner syndrome (LKS) is a rare neurological disorder characterized by acquired aphasia. LKS presents with distinctive electroencephalography (EEG) findings, including diffuse continuous spike and wave complexes (CSW), particularly during sleep. There has been little research on the mechanisms of aphasia and its origin within the brain and how it recovers. We diagnosed LKS in a 4-year-old female with an epileptogenic zone located primarily in the right superior temporal gyrus or STG (nondominant side). In the course of her illness, she had early signs of motor aphasia recovery but was slow to regain language comprehension and recover from hearing loss. We suggest that the findings from our patient's brain imaging and the disparity between her recovery from expressive and receptive aphasias are consistent with the dual-stream model of speech processing in which the nondominant hemisphere also plays a significant role in language comprehension. Unlike aphasia in adults, the right-hemisphere disorder has been reported to cause delays in language comprehension and gestures in early childhood. In the period of language acquisition, it requires a process of understanding what the words mean by integrating and understanding the visual, auditory, and contextual information. It is thought that the right hemisphere works predominantly with respect to its integrating role.
朗-克综合征（LKS）是一种罕见的神经疾病，以获得性失语为特征。LKS 表现出独特的脑电图（EEG）发现，包括弥漫性连续尖波和棘慢波复合波（CSW），特别是在睡眠期间。关于失语症的机制及其在大脑中的起源以及如何恢复的研究很少。我们诊断了一名 4 岁女性患有癫痫发作区主要位于右侧颞上回或 STG（非优势侧）的 LKS。在她的疾病过程中，她有运动性失语症早期恢复的迹象，但语言理解恢复缓慢，听力损失也没有恢复。我们建议，从我们患者的脑成像中发现的，以及她从表达性和接受性失语症中恢复的差异，与言语处理的双通道模型一致，其中非优势半球在语言理解中也起着重要作用。与成人失语症不同，右半球障碍已被报道会导致儿童早期语言理解和手势延迟。在语言习得期间，它需要通过整合和理解视觉、听觉和上下文信息来理解单词含义的过程。人们认为右半球主要与其整合作用有关。
