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目的探讨振幅整合脑电图(a EEG)在新生儿甲基丙二酸尿症患儿监测中的应用价值。方法回顾性分析2013年12月至2015年3月我院新生儿重症监护病房确诊的甲基丙二酸尿症患儿a EEG及临床资料,分析其变化特点及临床预后。结果 13例甲基丙二酸尿症患儿a EEG背景呈连续正常电压8例,不连续电压2例,持续低电压3例;癫疒间性活动记录5例,暴发抑制4例;睡眠觉醒周期7例成熟,1例不成熟,5例无睡眠觉醒周期;a EEG波形正常7例,轻度异常1例,重度异常5例;5例a EEG重度异常患儿全部死亡(其中4例放弃后死亡),其余8例随访,6月龄时发育商(DQ)≥85分4例,<85分4例。a EEG波形异常程度、睡眠觉醒周期与甲基丙二酸尿症患儿近期预后相关(r=0.904,P<0.001)。结论新生儿甲基丙二酸尿症发病早,病情重,常侵犯脑神经,a EEG监测示电压连续性差、暴发抑制、睡眠觉醒周期消失可早期准确识别脑损伤,且a EEG严重程度与近期预后相关。
Objective To investigate the value of amplitude integrated electroencephalogram (aEGEG) in the monitoring of neonatal methadoneuria in children. Methods The EEG and clinical data of children with methylmalonic aciduria diagnosed in neonatal intensive care unit in our hospital from December 2013 to March 2015 were analyzed retrospectively. The changes and clinical prognosis were analyzed. Results In 13 children with methylmalonic aciduria, the background of a EEG showed continuous normal voltage in 8 cases, discontinuous voltage in 2 cases and continuous low voltage in 3 cases, 5 cases of epileptic seizure activity and 4 cases of outbreak suppression, 7 cases were mature, 1 case was immature and 5 cases had no sleep arousal cycle. There were 7 cases with normal EEG waveform, 1 case with mild abnormality and 5 cases with severe abnormality. All 5 cases with severe EEG abnormality died (4 cases gave up After death). The remaining 8 patients were followed up. At 6 months of age, DQ ≥85 points and 4 cases <85 points in 4 cases. a EEG waveform anomalies, sleep arousal cycle and the recent prognosis of children with methylmalonic acid (r = 0.904, P <0.001). Conclusion Neonatal methylmalonic aciduria is early onset and severe in severity. It often invades the cranial nerves. EEG monitoring shows poor continuity of voltage, suppression of outbreak, disappearance of sleep awakening period, accurate and early identification of brain injury, Prognosis related.