Type II focal cortical dysplasia: Electroclinical phenotype and surgical outcome related to imaging

Type II focal cortical dysplasia: Electroclinical phenotype and surgical outcome related to imaging
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DOI:
10.1111/j.1528-1167.2011.03363.x
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发表时间:
2012-02-01
期刊:
影响因子:
5.6
通讯作者:
Devaux, Bertrand
Devaux, Bertrand
中科院分区:
医学1区
文献类型:
--
作者:
Chassoux, Francine;Landre, Elisabeth;Devaux, Bertrand

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目的:II型局灶性皮质发育不良(TTFCD)是一种高度致痫性病变,可通过手术治愈,约有三分之一的病例被磁共振成像(MRI)漏诊。对这些MRI阴性患者的电临床表现知之甚少,经常报告手术结局不佳。我们比较了MRI阴性和MRI阳性病例的临床和神经生理学特征,以更好地确定候选人为surgery.Methods:在连续62例TTFCD患者(38例男性,24例女性; 7-52岁; 22名儿童)手术治疗顽固性癫痫,25(40%)呈阴性MRI结果。我们比较了癫痫病史;癫痫发作的类型、频率和分布;神经学检查认知和精神障碍;发作间期脑电图(EEG)和立体脑电图(SEEG)数据,氟脱氧葡萄糖正电子发射断层扫描(FDG-PET)数据,神经病理学结果; MRI阴性组和MRI阳性组的手术结果。严重的部分性癫痫开始于儿童期,癫痫发作频率高,包括癫痫持续状态,刻板的癫痫发作提示精确的大脑定位,颞外位置和功能区的参与是特征性的,在两组中发现相似。在EEG上,每组中约有40%的患者出现伪节律活动。SEEG记录显示了两组TTFCD的典型特征。FDG-PET在84%的MRI阴性病例中具有定位价值,在65%的MRI阳性病例中有助于描绘发育不良的皮质。影像学和神经生理学数据的结合使我们能够进行安全和有限的切除,在超过一半的病例中仅限于单个脑回。此外,在大多数MRI阳性病例中,甚至在一些选定的MRI阴性病例中,我们能够避免侵入性监测。两组中具有良好手术结局的患者比例相当(MRI阴性病例为88%,MRI阳性病例为94%)。两组之间的主要差异是MRI阴性组的睡眠相关癫痫发生率显著较高(p = 0.028)。这一表型特征提供了一个新的参数TTFCD在MRI阴性extratemporal epilepsy.Significance:这些结果使我们认为,儿童或成人患者的电临床数据表明TTFCD,是非常适合手术,特别是隐源性睡眠相关癫痫。
Purpose: Type II focal cortical dysplasia (TTFCD), a highly epileptogenic lesion with severe epilepsy curable by surgery, is missed by magnetic resonance imaging (MRI) in about one third of cases. Little is known about the electroclinical presentation in these MRI-negative patients and a poor surgical outcome is frequently reported. We compared the clinical and neurophysiologic features in MRI-negative and MRI-positive cases in order to better identify candidates for surgery.Methods: Among 62 consecutive TTFCD patients (38 male, 24 female; 7-52 years old; 22 children) operated for intractable epilepsy, 25 (40%) presented negative MRI findings. We compared the history of epilepsy; the type, frequency, and distribution of seizures; neurologic examination cognitive and psychiatric impairment; interictalictal electroencephalography (EEG) and stereo-EEG (SEEG) data, fluorodeoxyglucose positron emission tomography (FDG-PET) data, neuropathologic findings; and surgical outcome in the MRI-negative and the MRI-positive groups.Key Findings: Severe partial epilepsy beginning in childhood, high seizure frequency including status epilepticus, stereotyped seizures suggestive of precise brain localization, extratemporal location and functional area involvement were characteristic and similarly found in both groups. On EEG, pseudorhythmic activity was found in about 40% of patients in each group. SEEG recordings demonstrated the typical pattern characterizing TTFCD in both groups. FDG-PET had a localization value in 84% of the MRI-negative cases and helped to delineate the dysplastic cortex in 65% of the MRI-positive cases. The combination of imaging and neurophysiologic data allowed us to perform safe and restricted resections, limited to a single gyrus in more than half of all cases. In addition, we were able to avoid invasive monitoring in most MRI-positive cases and even in some selected MRI-negative cases. The proportion of patients with a favorable surgical outcome was comparable in both groups (88% in MRI-negative and 94% in MRI-positive cases). The main difference between the groups was a significantly higher frequency of sleep-related epilepsy in the MRI-negative group (p = 0.028). This phenotypic characteristic provides a new argument for TTFCD in MRI-negative extratemporal epilepsy.Significance: These results lead us to consider that children or adult patients in whom electroclinical data suggest TTFCD, are highly suitable for surgery, especially for cryptogenic sleep-related epilepsy.