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皮质发育不良导致的难治性部分性癫癎临床特征分析 被引量:19

Retrospective study on clinical features of intractable epilepsy with malformations of cortical development
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摘要 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 discuss the clinical, neuroimaging and electroencephalogram characteristics of patients with malformations of cortical development (MCD) undergoing surgical resection for intractable epilepsy. Methods A series of 46 patients with a histopathology of MCD were found out according to Palmini' s new criterion of MCD. Medical history, neuroimaging, non-invasive or invasive video-EEG recording and surgical outcome data were analyzed retrospectively. Results Twenty-three patients were conducted with temporal lobe resection, the other 23 with extra-temporal lobe resection. The 46 cases were divided into different pathologic subtypes according to the new classification of MCD: 10 Mild MCD, 25 FCD-1, 5 FCD-2, 4 dysembryoplastic neuroepithelial tumors (DNT) and 2 gangliogliomas. FCD-1 was the most common pathologic subtype in this series of patients with MCD. MRI showed focal cortical lesions in 12 patients (26. 1% ),hippocampal sclerosis in 10 patients (21.7%), and normal in 24 persons (52. 2% ). Regional epileptiform discharge appeared in 60. 9% of the patients, and repetitive or rhythmic epileptiform in 45.7% of the patients in interictal EEG. 76. 1% of the patients undergone surgery achieved good outcome, and 69. 6% of whom were finally seizure free. Conclusions MCD accounts approximately for 50% of the patients with intractable epilepsy with normal MRI, and FCD-1 is the most common subtype of MCD. Repetitive or rhythmic epileptiform is often seen in interictal EEG of MCD patients.
出处 《中华神经科杂志》 CAS CSCD 北大核心 2006年第3期148-151,共4页 Chinese Journal of Neurology
关键词 部分性癫癎 大脑皮质 神经系统畸形 皮质发育不良 Epilepsies, partial Cerebral cortex Nervous system malformations
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参考文献11

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