Résumé
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.