Chen Hsin-Hung, Chen Chien, Hung Sheng-Che, Liang Sheng-Yuan, Lin Shih-Chieh, Hsu Ting-Rong, Yeh Tzu-Chen, Yu Hsiang-Yu, Lin Chun-Fu, Hsu Sanford P C, Liang Muh-Lii, Yang Tsui-Fen, Chu Lee-Shing, Lin Yung-Yang, Chang Kai-Ping, Kwan Shang-Yeong, Ho Donald M, Wong Tai-Tong, Shih Yang-Hsin
Department of Neurosurgery, The Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan,
Childs Nerv Syst. 2014 Nov;30(11):1885-95. doi: 10.1007/s00381-014-2463-y. Epub 2014 Oct 9.
Focal cortical dysplasia (FCD) is a specific malformation of cortical development harboring intrinsic epileptogenicity, and most of the patients develop drug-resistant epilepsy in early childhood. The detrimental effects of early and frequent seizures on cognitive function in children are significant clinical issues. In this study, we evaluate the effects of early surgical intervention of FCD on epilepsy outcome and cognitive development.
From 2006 to 2013, 30 children younger than 18 years old underwent resective surgery for FCDs at Taipei Veterans General Hospital. The mean age at surgery was 10.0 years (range 1.7 to 17.6 years). There were 21 boys and 9 girls. In this retrospective clinical study, seizure outcome, cognitive function, and quality of life were evaluated. To evaluate the effects to outcomes on early interventions, the patients were categorized into four groups according to age of seizure onset, duration of seizure before surgery, and severity of cognitive deficits.
Eleven of 22 (50 %) patients demonstrated developmental delay preoperatively. The Engel seizure outcome achievements were class I in 21 (70 %), class II in 2 (7 %), class III in 6 (20 %), and class IV in 1 (3 %) patients. The locations of FCDs resected were in the frontal lobe in 18 cases, temporal lobe in 7, parietal lobe in 2, and in bilobes including frontoparietal lobe in 2 and parieto-occipital lobes in 1. Eight cases that had FCDs involved in the rolandic cortex presented hemiparesis before surgical resection. Motor function in four of them improved after operation. The histopathological types of FCDs were type Ia in 1, type Ib in 7, type IIa in 7, type IIb in 12, and type III in 3 patients. FCDs were completely resected in 20 patients. Eighteen (90 %) of them were seizure free (p < 0.001) with three patients that received more than one surgery to accomplish complete resection. The patients who had early seizure onset, no significant cognitive function deficit, and early surgical intervention with complete resection in less than 2 years of seizure duration showed best outcomes on seizure control, cognitive function, and quality of life.
Delay in cognitive development and poor quality of life is common in children treated for FCDs. Early surgical intervention and complete resection of the lesion help for a better seizure control, cognitive function development, and quality of life. FCDs involved eloquent cortex may not prohibit complete resection for better outcomes.
局灶性皮质发育不良（FCD）是一种具有内在致痫性的皮质发育特异性畸形，大多数患者在幼儿期就会发展为药物难治性癫痫。儿童早期频繁发作对认知功能的有害影响是重大的临床问题。在本研究中，我们评估了FCD早期手术干预对癫痫结局和认知发育的影响。
2006年至2013年，30名18岁以下儿童在台北荣民总医院接受了FCD切除术。手术时的平均年龄为10.0岁（范围1.7至17.6岁）。其中有21名男孩和9名女孩。在这项回顾性临床研究中，评估了癫痫结局、认知功能和生活质量。为了评估早期干预对结局的影响，根据癫痫发作起始年龄、手术前癫痫发作持续时间和认知缺陷严重程度将患者分为四组。
22名患者中有11名（50%）术前表现出发育迟缓。恩格尔癫痫结局分级为I级的患者有21名（70%），II级的有2名（7%），III级的有6名（20%），IV级的有1名（3%）。切除的FCD部位，额叶18例，颞叶7例，顶叶2例，双叶（包括额顶叶2例和顶枕叶1例）。8例累及中央沟周围皮质的FCD患者在手术切除前出现偏瘫。其中4例术后运动功能改善。FCD的组织病理学类型，Ia型1例，Ib型7例，IIa型7例，IIb型12例，III型3例。20例患者的FCD被完全切除。其中18例（90%）无癫痫发作（p<0.001），3例患者接受了不止一次手术以完成完全切除。癫痫发作起始早、无明显认知功能缺陷且在癫痫发作持续时间不到2年时进行早期手术干预并完全切除的患者在癫痫控制、认知功能和生活质量方面显示出最佳结局。
接受FCD治疗的儿童中，认知发育延迟和生活质量差很常见。早期手术干预和病变完全切除有助于更好地控制癫痫发作、促进认知功能发育和提高生活质量。累及功能区皮质的FCD可能并不妨碍完全切除以获得更好的结局。
