O'Regan M E, Brown J K, Goodwin G M, Clarke M
Department of Paediatric Neurology, Royal Hospital for Sick Children, Edinburgh, UK.
Dev Med Child Neurol. 1998 Aug;40(8):508-16. doi: 10.1111/j.1469-8749.1998.tb15408.x.
A series of 25 children, 13 females and 12 males, who had an acquired communication disorder together with epilepsy, but did not fulfil the strict criteria of the Landau-Kleffner syndrome, was studied. All children had a clinical neurological evaluation, speech and language assessment, an awake and sleep EEG, cranial MRI, SPET scan, and audiometry. Clinical seizures were most often polymorphic in type (17 of 25). Atypical absences were the commonest individual seizure type occurring in 15 cases. All patients had an unequivocal epileptiform EEG. Normal sleep phenomena were only observed in 10 cases, enhancement of epileptiform activity in sleep was seen in 16. Cranial MRI was abnormal in six and normal in 19 cases. The SPET scans were abnormal in 22 of 25 children. The language deficits were classified neurologically as receptive aphasia, 24 of 25; expressive aphasia, 20 of 25; nominal aphasia, eight of 25; articulatory dyspraxia, 10 of 25; and auditory agnosia, nine of 25. It is hypothesized that the loss of communication skills is due to an encephalopathy secondary to the persistent epileptic discharge and manifests as a hypometabolic area on the SPET scan.
对一系列25名儿童进行了研究，其中13名女性，12名男性，他们患有后天性沟通障碍并伴有癫痫，但不符合Landau-Kleffner综合征的严格标准。所有儿童均接受了临床神经学评估、言语和语言评估、清醒和睡眠脑电图、头颅MRI、单光子发射计算机断层扫描（SPET）以及听力测定。临床发作类型最常见的是多形性（25例中的17例）。非典型失神是最常见的个体发作类型，有15例出现。所有患者脑电图均有明确的癫痫样放电。仅10例观察到正常睡眠现象，16例出现睡眠中癫痫样活动增强。头颅MRI检查6例异常，19例正常。25名儿童中有22名的SPET扫描结果异常。语言缺陷在神经学上分类为感受性失语，25例中有24例；表达性失语，25例中有20例；命名性失语，25例中有8例；构音障碍，25例中有10例；听觉失认，25例中有9例。据推测，沟通技能的丧失是由于持续性癫痫放电继发的脑病所致，并在SPET扫描上表现为代谢减低区。
