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目的探讨病理表现为皮质发育不良的难治性部分性癫的临床特征。方法回顾分析46例手术切除癫灶后,病理证实为皮质发育不良的难治性癫病例的影像学和脑电图表现以及手术疗效等资料,分析此类癫的临床特征。结果在46例中,癫灶的病理类型为轻度皮质发育不良10例、Ⅰ型局灶性皮质发育不良25例、Ⅱ型局灶性皮质发育不良5例、伴有胚胎发育障碍性神经上皮瘤和神经节胶质瘤的分别为4例和2例。通过磁共振成像(MRI)扫描可以发现局灶性皮质异常的12例(26·1%),海马硬化10例(21·7%),未见明显异常的24例(52·2%)。在发作间期,60·9%的病例为区域性的性放电,45·7%的病例表现出节律性样电活动。手术切除癫灶后,69·6%的病例发作消失。结论皮质发育不良约占MRI阴性表现的难治性癫病例的50%,以Ⅰ型局灶性皮质发育不良最为常见,发作间期脑电图常常显示节律性样电活动。
Objective To investigate the clinical features of intractable parietal epilepsy with pathological manifestations of cortical dysplasia. Methods Retrospective analysis of 46 cases of surgical removal of epileptic foci, pathologically confirmed as cortical dysplasia in patients with intractable epilepsy imaging and EEG performance and surgical data and other clinical data to analyze the clinical features of such epilepsy. Results In 46 cases, the pathological type of epilepsy was mild cortical dysplasia in 10 cases, type Ⅰ focal cortical dysplasia in 25 cases, type Ⅱ focal cortical dysplasia in 5 cases, accompanied by embryonic developmental disorders Epithelial tumors and ganglion gliomas were 4 and 2 cases, respectively. Twelve patients (26.1%) with focal cortical abnormalities, 10 (21.7%) with hippocampal sclerosis and 24 (52.2%) with no obvious abnormalities were found by MRI scans. During the interictal period, 60.9% of cases were regional discharges and 45.7% of cases showed rhythmic electrical activity. After surgical removal of epileptic foci, 69.6% of the episodes disappeared. Conclusions Cortical dysplasia accounts for about 50% of refractory epilepsy cases with MRI negative manifestations. Type I focal cortical dysplasia is the most common, and interictal EEG often shows rhythmic electrical activity.