Nass R, Gross A, Devinsky O
New York University Medical Center, Department of Neurology, NYC 10016, USA.
Dev Med Child Neurol. 1998 Jul;40(7):453-8. doi: 10.1111/j.1469-8749.1998.tb15395.x.
The electroencephalographic abnormalities seen in Landau-Kleffner syndrome (LKS) (language deterioration) are non-specific, and consist of a variety of epileptiform discharge patterns including continuous slow spike-wave discharges during sleep, focal sharp waves with spikes, and centrotemporal (rolandic) spikes. Similarly, the EEG abnormalities seen in autistic epileptiform regression (language and social/behavioral deterioration) are non-specific and overlap with those seen in LKS. By contrast, distinct epilepsy syndromes in otherwise normal children occur in the EEG-defined benign focal epilepsies of childhood. Occipital spikes or spike-wave present either in the older child with visual symptoms and headache or in the younger child with autonomic symptoms followed by brief or prolonged partial motor seizures. Seven young children (five from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures, whose EEGs revealed occipital spikes or spike-wave characteristic of the benign epilepsies, are reported. Although occipital spikes are commonly seen in young children as an age-dependent EEG-defined benign focal epilepsy, their high frequency in this population with cognitive difficulties suggests a possible causal relation. The effects of the epileptiform discharge on cognitive functioning presumably reflect extension into temporal and parietal lobes, rather than occipital disturbances per se.
在Landau-Kleffner综合征（LKS，语言退化）中所见的脑电图异常是非特异性的，包括多种癫痫样放电模式，如睡眠期间的持续性慢棘波放电、伴有棘波的局灶性尖波以及中央颞区（罗兰区）棘波。同样，在自闭症癫痫样退化（语言和社交/行为退化）中所见的脑电图异常也是非特异性的，且与LKS中所见的异常有重叠。相比之下，在其他方面正常的儿童中，明确的癫痫综合征发生在脑电图定义的儿童良性局灶性癫痫中。枕叶棘波或棘慢波出现在有视觉症状和头痛的较大儿童中，或出现在有自主神经症状随后出现短暂或延长的部分运动性发作的较小儿童中。本文报告了7名临床诊断为自闭症或自闭症退化且可能或确诊有癫痫发作的幼儿（42例连续病例中的5例），他们的脑电图显示出良性癫痫所特有的枕叶棘波或棘慢波。虽然枕叶棘波在幼儿中常见，是一种脑电图定义的与年龄相关的良性局灶性癫痫，但它们在这群有认知困难的儿童中出现的高频率提示可能存在因果关系。癫痫样放电对认知功能的影响大概反映了其扩展到颞叶和顶叶，而非枕叶本身的紊乱。
