长程视频脑电监测在下丘脑错构瘤诊断治疗中的应用(附5例报告)

The application of long term video electroencephalogram in diagnose and localizaton of hypothalamic hamartoma(report of 5 cases)

目的探讨长程视频脑电监测在下丘脑错构瘤诊断治疗中的应用意义。方法回顾性分析5例下丘脑错构瘤长程视频脑电(VEEG)特征,VEEG结合磁共振成像(MRI)、发作间期正电子发射计算机断层扫描(PET)检查诊断定位。结果发作间期清醒平静状态脑电图表现:双侧波形均呈不对称表现,双侧存在广泛单发性棘慢波或者双侧广泛不规则θ或δ波,一侧波幅优势,主要为一侧额叶优势,2例左侧优势,3例右侧优势,优势侧别同MRI显示的错构瘤侧别一致;睡眠期脑电图表现:存在基本睡眠标志波形与睡眠周期,间有较多量棘慢波或多棘慢波,存在形式同间期清醒平静状态;5例均捕获临床发作过程,共计13次,其中痴笑发作8次、痴笑发作继发全身强直阵挛5次,发作期脑电图表现:3例为去同步化低电压数秒后EEG混合肌电干扰,2例以肌电伪差为主。MRI结果:下丘脑脚间池处部位可见占位性改变,位于左侧半球2例、右侧半球3例。PET结果:MRI所提示的占位性改变区域均显示低代谢。5例均手术彻底去除错构瘤,随访5例患者术后均无痴笑发作或继发全身强直阵挛。结论长程视频脑电监测结合MRI及PET检查对下丘脑错构瘤诊断定位准确性高,手术治疗下丘脑错构瘤是最佳选择。

Objective To investigate the applied significance of long term video electroencephalogram（VEEG） monitoring in diagnosis and localization of hypothalamic hamartoma（HH）. Methods To review and study the results of VEEG monitoring of 5 cases who were diagnosed hypothalamic hamartoma , to use VEEG combined with magnetic resonance imaging（MRI） and positron emission tomography（PET） in diagnosis and localization of epileptogenic foci. Results EEG in interictal period ： EEG waves of bilateral are asymmetric , wide single spike and wave complex or wide irregular θor δ waves are existed in double sides. Amplitude of abnormal waves showsadvantaged at one side, especially at frontal lob, in this group, 2 cases shows advantaged at left side and 3 cases at right side. The advantaged sides in EEG are as same as the sides of which show abnormal signs （hypothalamic hamartomas） in MRI. EEG of sleeping： It shows basic standard sleeping waves and periods in all the 5 cases, complexed with spike and wave complex, ployspike complex, the existing forms are as same as wake up and calmly state as above. 13 seizures were catched in 5 cases by VEEG,included 8 seizures of gelastic seizure（GS） and 5 seizures of secondary generalized tonic-clonic seizure from GS. EEG of the beginning of clinical onset：Desynchronization low voltage continued several seconds, and then EEG wave complexed with electromyography appeared in 3 cases, The main character is EEG wave complexed with electromyography in 2 case. Outcome of MRI .It shows abnormal signs occupying lesion in cisterna interpeduneularis in all 5 cases, Outcome of PET.It shows low metabolism in the area of abnormal signs in MRI. 5 cases were all performed resection of hypothalamic hamartoma completely, 5 cases obtained seizure free. Conclusion Using Long term VEEG combined with MRI.PET can diagnose hypothalamic haraartoma correctly as well as localize of epileptogenic focus accurately, resection of hypothalamic hamartoma is the best choice for patients.