Research Center for Automatic Control of Nancy (CRAN), University of Lorraine, CNRS, UMR 7039, Vandoeuvre, France.
Department of Neurology, Central University Hospital (CHU) Nancy, Nancy, France.
Ann Neurol. 2017 Nov;82(5):781-794. doi: 10.1002/ana.25081. Epub 2017 Nov 11.
We aimed to (1) assess the concordance between various polymicrogyria (PMG) types and the associated epileptogenic zone (EZ), as defined by stereoelectroencephalography (SEEG), and (2) determine the postsurgical seizure outcome in PMG-related drug-resistant epilepsy.
We retrospectively analyzed 58 cases: 49 had SEEG and 39 corticectomy or hemispherotomy.
Mean age at SEEG or surgery was 28.3 years (range, 2-50). PMG was bilateral in 9 (16%) patients and unilateral in 49, including 17 (29%) unilobar, 12 (21%) multilobar, 15 (26%) perisylvian, and only 5 (9%) hemispheric. Twenty-eight (48%) patients additionally had schizencephaly, heterotopia, or focal cortical dysplasia. The SEEG-determined EZ was fully concordant with the PMG in only 8 (16%) cases, partially concordant in 74%, and discordant in 10%. The EZ included remote cortical areas in 21 (43%) cases and was primarily localized in those in 5 (10%), all related to the mesial temporal structures. All but 1 PMG patient with corticectomy or hemispherotomy had a unilateral PMG. At last follow-up (mean, 4.6 years; range, 1-16), 28 (72%) patients remained seizure free. Shorter epilepsy duration to surgery was an independent predictor of seizure freedom.
PMG-related drug-resistant epilepsy warrants a comprehensive presurgical evaluation, including SEEG investigations in most cases, given that the EZ may only partially overlap with the PMG or include solely remote cortical areas. Seizure freedom is feasible in a large proportion of patients. PMG extent should not deter from exploring the possibility of epilepsy surgery. Our data support the early consideration of epilepsy surgery in this patient group. Ann Neurol 2017;82:781-794.
（1）评估各种多微小脑回畸形（PMG）类型与立体脑电图（SEEG）定义的致痫区（EZ）之间的一致性，以及（2）确定 PMG 相关耐药性癫痫的术后癫痫发作结果。
我们回顾性分析了 58 例病例：49 例行 SEEG 检查，39 例行皮层切除术或半球切开术。
SEEG 或手术时的平均年龄为 28.3 岁（范围：2-50 岁）。9 例（16%）患者为双侧 PMG，49 例为单侧 PMG，其中 17 例（29%）为单叶，12 例（21%）为多叶，15 例（26%）为围侧裂，仅 5 例（9%）为半球性。28 例（48%）患者还存在裂脑畸形、异位或局灶性皮质发育不良。仅 8 例（16%）病例的 SEEG 确定的 EZ 与 PMG 完全一致，74%的病例部分一致，10%的病例不一致。EZ 包括 21 例（43%）远程皮质区，主要定位于其中 5 例（10%），均与内侧颞叶结构有关。除 1 例外，所有行皮层切除术或半球切开术的 PMG 患者均为单侧 PMG。末次随访（平均 4.6 年；范围 1-16 年）时，28 例（72%）患者无癫痫发作。手术至癫痫发作的时间较短是无癫痫发作的独立预测因素。
PMG 相关耐药性癫痫需要进行全面的术前评估，包括大多数情况下的 SEEG 检查，因为 EZ 可能仅与 PMG 部分重叠或仅包括远程皮质区。很大一部分患者可以无癫痫发作。PMG 程度不应阻止探索癫痫手术的可能性。我们的数据支持在该患者群体中早期考虑癫痫手术。
Ann Neurol 2017;82:781-794.
