Kaga M
Department of Developmental Disorders, National Institute of Mental Health, National Center of Neurology and Psychiatry, Chiba, Japan.
J Child Neurol. 1999 Feb;14(2):118-22. doi: 10.1177/088307389901400210.
In the present long-term study, we analyzed language disorders in four patients with Landau-Kleffner syndrome. Their common first symptoms were disability in understanding spoken words, followed by inarticulation and a decreased amount of speech. All patients showed auditory verbal agnosia to some degree at some stage of their illness. However, one patient showed typical sensory aphasia as the first symptom, and another patient showed nonverbal auditory agnosia followed by pure word deafness. Thus, patients with Landau-Kleffner syndrome show sequential and sometimes hierarchical language disorders beginning with sensory aphasia, followed by auditory agnosia, and finally word deafness during their disease process. During long-term follow-up (20 to 30 years), all patients showed marked recovery in language without any intellectual handicap, but with some disability in spoken language, auditory verbal perception, and a discrepancy between Wechsler Verbal and Performance IQ scores.
在这项长期研究中，我们分析了4例Landau-Kleffner综合征患者的语言障碍情况。他们常见的首发症状是理解口语能力丧失，随后是发音不清和言语量减少。所有患者在病程的某些阶段均出现了一定程度的听觉性言语失认。然而，1例患者以典型的感觉性失语作为首发症状，另1例患者则先出现非言语听觉失认，随后发展为纯词聋。因此，Landau-Kleffner综合征患者在疾病过程中表现出从感觉性失语开始，依次为听觉失认，最终发展为纯词聋的连续性、有时甚至是分级性的语言障碍。在长期随访（20至30年）期间，所有患者的语言功能均有显著恢复，未出现任何智力障碍，但在口语、听觉言语感知以及韦氏语言智商和操作智商评分之间存在差异。
