Lou H C, Brandt S, Bruhn P
Acta Neurol Scand. 1977 Jul;56(1):46-54. doi: 10.1111/j.1600-0404.1977.tb01408.x.
The syndrome of progressive aphasia and epilepsy in childhood is delineated. Four cases are presented, three of which show the typical gradual onset, fluctuating course and EEG changes with bilateral foci of spike and spike-wave activity, and, finally, normalization after some years of the EEG. Also the aphasia tends to subside, but often with considerable delay in relation to the other features. Neuropsychological assessment showed varying residual defects mainly on language functions. The aetiology is unknown. No pathoanatomical data on this disorder has hitherto been published. In one of the present cases a cortical biopsy was performed on suspicion of a left temporal tumour. The biopsy showed changes indicative of a slow virus infection. It is therefore suggested that the newly established language function in children particularly is vulnerable to a subchronic viral encephalitis affecting both hemispheres.
儿童进行性失语症和癫痫综合征的特征已被明确描述。本文报告了4例病例，其中3例表现出典型的起病隐匿、病程波动，脑电图显示双侧棘波和棘慢波活动灶，最终脑电图在数年后恢复正常。失语症也倾向于缓解，但相对于其他特征，往往延迟很久。神经心理学评估显示主要在语言功能方面存在不同程度的残留缺陷。病因不明。迄今为止，尚未发表关于这种疾病的病理解剖学数据。在本病例中的1例中，因怀疑左颞叶肿瘤而进行了皮质活检。活检显示有提示慢病毒感染的改变。因此有人提出，儿童新建立的语言功能尤其容易受到影响双侧半球的亚慢性病毒性脑炎的损害。
