Krsek P, Maton B, Jayakar P, Dean P, Korman B, Rey G, Dunoyer C, Pacheco-Jacome E, Morrison G, Ragheb J, Vinters H V, Resnick T, Duchowny M
Department of Pediatric Neurology, Charles University, Second Medical School, Motol University Hospital, V Uvalu 84, CZ 15006 Prague 5, Czech Republic.
Neurology. 2009 Jan 20;72(3):217-23. doi: 10.1212/01.wnl.0000334365.22854.d3. Epub 2008 Nov 12.
Focal cortical dysplasia (FCD) is recognized as the major cause of focal intractable epilepsy in childhood. Various factors influencing postsurgical seizure outcome in pediatric patients with FCD have been reported.
To analyze different variables in relation to seizure outcome in order to identify prognostic factors for selection of pediatric patients with FCD for epilepsy surgery.
A cohort of 149 patients with histologically confirmed mild malformations of cortical development or FCD with at least 2 years of postoperative follow-up was retrospectively studied; 113 subjects had at least 5 years of postoperative follow-up. Twenty-eight clinical, EEG, MRI, neuropsychological, surgical, and histopathologic parameters were evaluated.
The only significant predictor of surgical success was completeness of surgical resection, defined as complete removal of the structural MRI lesion (if present) and the cortical region exhibiting prominent ictal and interictal abnormalities on intracranial EEG. Unfavorable surgical outcomes are mostly caused by overlap of dysplastic and eloquent cortical regions. There were nonsignificant trends toward better outcomes in patients with normal intelligence, after hemispherectomy and with FCD type II. Other factors such as age at seizure onset, duration of epilepsy, seizure frequency, associated pathologies including hippocampal sclerosis, extent of EEG and MRI abnormalities, as well as extent and localization of resections did not influence outcome. Twenty-five percent of patients changed Engel's class of seizure outcome after the second postoperative year.
The ability to define and fully excise the entire region of dysplastic cortex is the most powerful variable influencing outcome in pediatric patients with focal cortical dysplasia.
局灶性皮质发育不良（FCD）被认为是儿童局灶性难治性癫痫的主要病因。已有报道多种影响FCD患儿术后癫痫发作结局的因素。
分析与癫痫发作结局相关的不同变量，以确定用于选择FCD患儿进行癫痫手术的预后因素。
回顾性研究一组149例经组织学证实为轻度皮质发育畸形或FCD且术后至少随访2年的患者；113例患者术后至少随访5年。评估了28项临床、脑电图（EEG）、磁共振成像（MRI）、神经心理学、手术及组织病理学参数。
手术成功的唯一显著预测因素是手术切除的完整性，定义为结构MRI病变（若存在）及颅内EEG显示发作期和发作间期明显异常的皮质区域被完全切除。手术效果不佳主要是由于发育异常皮质区域与功能区皮质重叠。智力正常、接受大脑半球切除术及II型FCD患者的结局有向好的非显著趋势。其他因素如癫痫发作起始年龄、癫痫病程、发作频率、包括海马硬化在内的相关病变、EEG和MRI异常程度以及切除范围和部位均不影响结局。25%的患者在术后第二年之后改变了恩格尔癫痫发作结局分级。
明确并完全切除发育异常皮质的整个区域的能力是影响FCD患儿结局的最有力变量。
