Boyd S G, Harden A, Patton M A
Department of Clinical Neurophysiology, Hospital for Sick Children, London, England.
Eur J Pediatr. 1988 Jun;147(5):508-13. doi: 10.1007/BF00441976.
An EEG study has been carried out on 19 children (including siblings in 3 families) with clinical features of Angelman syndrome. The age at time of the first EEG ranged from 11 months to 11 years with the majority under 5 years. Six children had no history of seizures at the time of the first EEG. One or more of the following EEG abnormalities were seen in all patients: 1. Persistent rhythmic 4-6/s activities reaching more than 200 microV not associated with drowsiness. 2. Prolonged runs of rhythmic 2-3/s activity (200-500 microV) often more prominent anteriorly, sometimes associated with discharges (ill-defined spike/wave complexes). 3. Spikes mixed with 3-4/s components usually more than 200 microV mainly posteriorly and facilitated by, or only seen with, eye closure. Two and sometimes three of these EEG features could be present in the same record particularly at a young age. The appearance of discharges mixed with slow components on eye closure was the commonest finding seen at some stage in 17 patients (aged from 11 months to over 12 years). The EEG features of Angelman syndrome appear to be sufficiently characteristic to help identify patients at an early age before the clinical features become obvious and at a time when genetic counselling may be particularly important.
对19名具有天使综合征临床特征的儿童（包括3个家庭中的兄弟姐妹）进行了脑电图（EEG）研究。首次脑电图检查时的年龄范围为11个月至11岁，大多数在5岁以下。6名儿童在首次脑电图检查时无癫痫发作史。所有患者均出现以下一种或多种脑电图异常：1. 持续的4 - 6次/秒节律性活动，幅度超过200微伏，与嗜睡无关。2. 长时间的2 - 3次/秒节律性活动（200 - 500微伏），通常在前部更为明显，有时伴有放电（不明确的棘波/慢波复合波）。3. 与3 - 4次/秒成分混合的棘波，通常超过200微伏，主要在后部，闭眼时增强或仅在闭眼时出现。这些脑电图特征中的两种甚至三种可能出现在同一记录中，尤其是在年幼时。闭眼时出现与慢成分混合的放电是17名患者（年龄从11个月至12岁以上）在某个阶段最常见的发现。天使综合征的脑电图特征似乎具有足够的特异性，有助于在临床特征变得明显之前的早期阶段识别患者，而此时遗传咨询可能尤为重要。
