López-Ibor M I, López-Ibor J J, Hernández Herreros M
Instituto de Investigaciones Neuropsiquiátricas, Madrid.
Actas Luso Esp Neurol Psiquiatr Cienc Afines. 1997 Nov-Dec;25(6):410-6.
The Landau-Kleffner syndrome consists in the association of an aphasia acquired during childhood or adolescence after a period of normal development, accompanied by epileptic fits and, sometimes, psychological disturbances. The appearance of the symptoms may not be simultaneous. The nature of this syndrome has been widely discussed and even the possibility of being a non-unitary syndrome has been considered. It may be possibly due to diverse etiologies, genetic or acquired (infectious). The relation between the aphasic, the psychologic and the convulsive symptoms has also raised controversies. A case of a female is described in which the psychological symptomatology was so severe, she had to be admitted in a psychiatric unit. The symptoms consisted in agitation during the night and severe persistent insomnia so as difficulties for relationship during day. No abnormalities were detected in a TAC nor in a RM but electroencephalographic and neuropsychological abnormalities were detected. The epileptic fits disappeared with a carbamacepine treatment but the rest of symptoms remained even more accentuated. After her admission, a treatment with valproate achieved to control her fits and the aphasic symptoms and neuropsychological deficits were compensated in the course of several months. After a year of treatment the patient's life was normalized, she resumed her studies, the EEG abnormalities disappeared so as the psychological disturbances and the behavior problems with the exception of phobic symptoms, similar to the ones of other family members. The patient has a history of epilepsy on its mother's side. The evolution and treatment response suggests that at least in some cases of the Landau-Kleffner syndrome, the etiology of the aphasia and other neuropsychological deficits and of the behavior disorders are related with some subclinical epileptic discharges and with a "functional inhibition" of some areas of the nervous system.
Landau-Kleffner综合征表现为在儿童期或青春期正常发育一段时间后出现的失语症，伴有癫痫发作，有时还伴有心理障碍。症状的出现可能不是同时发生的。这种综合征的性质已被广泛讨论，甚至有人认为它可能不是单一的综合征。它可能由多种病因引起，包括遗传或后天获得性（感染性）。失语、心理和惊厥症状之间的关系也引发了争议。本文描述了一例女性病例，其心理症状非常严重，不得不入住精神科病房。症状包括夜间烦躁不安和严重的持续性失眠，导致白天人际交往困难。头颅CT和磁共振成像均未发现异常，但脑电图和神经心理学检查发现了异常。卡马西平治疗后癫痫发作消失，但其他症状甚至更加明显。入院后，丙戊酸盐治疗控制了癫痫发作，失语症状和神经心理学缺陷在几个月内得到了改善。经过一年的治疗，患者的生活恢复正常，她重新开始学习，脑电图异常消失，心理障碍和行为问题也随之消失，但仍有恐惧症状，与其他家庭成员相似。患者母亲有癫痫病史。病情演变和治疗反应表明，至少在某些Landau-Kleffner综合征病例中，失语症和其他神经心理学缺陷以及行为障碍的病因与一些亚临床癫痫放电以及神经系统某些区域的“功能性抑制”有关。
