儿童烟雾病脑电图及临床特征

Electroencephalogram and clinical characteristics of moyamoya disease in children

目的分析儿童烟雾病(MMD)的脑电图(EEG)特征及临床表现,探讨EEG在儿童MMD临床诊断中的价值。方法收集2012年1月至2019年6月在华中科技大学同济医学院附属武汉儿童医院行EEG检查高度提示脑血管疾病,经头颅磁共振成像(MRI)及磁共振血管成像(MRA)确诊为MMD的12例患儿,回顾性分析其EEG及临床资料。结果12例患儿中男9例,女3例;起病年龄3.0~8.7岁。12例患儿中,10例为发作性一侧或双侧肢体无力或乏力,1例为发作性摔倒,1例为发作性姿势异常。12例患儿EEG背景活动基本正常,发作间期EEG:8例正常,1例为界线性,3例异常(1例为双侧前头部较多量慢波,觉醒期显著;1例为双侧额极多量不规则δ活动,觉醒期明显;1例为稍多量多灶性δ慢波,右侧额极稍突出)。过度换气(HV)诱发试验均异常,4例出现慢波重建:HV停止0.5~1.0 min出现局灶性δ慢波(多为前头部)进展为弥散性慢波,症状缓解后为长时间存在的局灶性慢波(多为前头部),共持续5~9 min;8例在HV中出现不对称慢波且延迟消失:HV开始1.5~2.0 min出现局灶性慢波(多为前头部),进展为弥散性慢波,症状缓解后长时间存在局灶性慢波(多为前头部),共持续5~10 min。其中11例在HV中出现短暂脑缺血症状,与主诉症状基本一致,症状持续时间多短于EEG慢波消失时间,1例未出现明显伴随症状。3例患儿头颅MRI提示大脑局部软化灶或异常信号,主要为额、顶叶及基底核区。1例液体衰减反转恢复序列上双侧大脑半球脑沟内高信号影。12例头颅MRA均提示MMD。结论儿童MMD临床症状多为发作性肢体无力;慢波重建,或不对称慢波出现且延迟消失高度提示MMD;这2种异常脑电模式的持续时间较长,多为前头部局灶性δ慢波演进展为弥散性慢波,最后限局于前头部。

Objective To analyze the characteristics of electroencephalogram(EEG)and clinical manifestations of children with moyamoya disease(MMD),and to explore the value of EEG in the clinical diagnosis of childhood MMD.Methods Twelve children indicated as cerebrovascular diseases by EEG and later diagnosed with MMD by head magnetic resonance imaging(MRI)and magnetic resonance angiography(MRA)in Wuhan Children′s Hospital,Tongji Medical College,Huazhong University of Science and Technology from January 2012 to June 2019 were enrolled in this study.Their EEG and clinical data were retrospectively analyzed.Results Nine cases were male and 3 cases were female,with the onset age of 3.0-8.7 years old.Ten cases were taken to the hospital because of paroxysmal limb weakness on one side or both sides,1 case was because of paroxysmal fall,and 1 case was because of paroxysmal posture abnormalities.EEG background activities of 12 cases were normal.Interictal EEG was normal in 8 cases,borderline in 1 case,and abnormal in 3 cases(1 case had many slow waves in bilateral anterior head,and its number significantly increased during the awakening period;1 case had a great many irregular delta slow waves on bilateral prefrontal cortex,showing significant aggravation during the awakening period;1 case had many multifocal delta slow waves especially in the right frontopolar region).The hyperventilation(HV)induction test was abnormal,and rebuild-up of slow waves was noted in 4 cases.The focal delta slow waves(especially in the anterior head)developed to diffuse slow waves after stopping HV for 0.5 to 1.0 minutes,and lasted for 5 to 9 minutes after relief of symptoms.The delayed disappearance of asymmetric focal slow waves was noted in 8 cases.Focal slow waves(especially in anterior head)developed to diffuse slow waves at 1.5 to 2.0 minutes after HV initiation,and lasted for 5 to 10 minutes in total.Among them,1 case pre-sented no obvious accompanying symptoms during HV,while 11 cases showed transient ischemic symptoms that were basically consistent with the chief complaint.The duration of symptoms was much shorter than the appearance of slow waves in EEG.Besides,brain MRI of 3 cases suggested local malacia lesions or abnormal signals,especially in parietal and basal ganglia areas.The high signal shadow was observed in the sulcus of bilateral cerebral hemispheres on the fluid attenuating inversion recovery sequence of one case.All of 12 cases were proved to MMD by head MRA.Conclusions Clinical symptoms of children with MMD are paroxysmal limb weakness.Rebuild-up of slow waves or the delayed disappea-rance of focal slow waves is highly suggestive of MMD.These 2 kinds of abnormal EEG patterns sustain over long periods of time.Focal slow waves(especially in anterior head)develop to diffuse slow waves,and lastly decrease to focal slow waves in anterior head.