Noli Daniel, Bartuluchi Marcelo, González Federico Sanchez, Kaltenmeier María Carolina, Cersosimo Ricardo, Rugilo Carlos, Princich Juan P, Lubieniecki Fabiana, Pomata Hugo, Caraballo Roberto
Neurology Unit-Epilepsy Group, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Combate de los Pozos 1881, CP 1245, Buenos Aires, Argentina.
Childs Nerv Syst. 2013 Nov;29(11):2079-87. doi: 10.1007/s00381-013-2165-x. Epub 2013 Jul 7.
The aim of this study was to analyze the electroclinical features and surgical outcome of 31 pediatric patients with focal cortical dysplasia (FCD) type II.
We conducted a retrospective, descriptive study of 31 patients with FCD type II followed between 1998 and 2011. We included patients with FCD type II confirmed by histopathological examination with abnormal magnetic resonance imaging and at least 1 year of follow-up.
All patients had severe focal epilepsy; in infancy, four of them had also had epileptic spasms, associated with hypsarrhythmia in three. Focal status epilepticus occurred in five patients (16 %) and epilepsia partialis continua in one (3.2 %). Seizures occurred during sleep in 20 (64.5 %) and in clusters in 19 (61.3 %) patients. Neurological examination showed a mild motor deficit in seven (22.8 %) patients. Interictal abnormalities were characterized by rhythmic spikes and polyspike discharges, increasing during sleep in 13 (41.9 %) patients. Average time of follow-up after surgery was 4.7 years with a median time of 4 years and a range from 1 to 9 years. Engel classification class I was found in 20 (67.7 %) and class II in 3 cases (9.6 %). There were no significant changes after an average time of follow-up of 4.7 years.
Our results confirm that surgery is the best treatment option for pediatric patients with refractory focal epilepsy due to type II FCD. A statistically significant correlation was found between a good prognosis and age at epilepsy onset older than 2 years.
本研究旨在分析31例II型局灶性皮质发育不良（FCD）患儿的电临床特征及手术疗效。
我们对1998年至2011年间随访的31例II型FCD患者进行了一项回顾性描述性研究。纳入经组织病理学检查确诊、磁共振成像异常且随访至少1年的II型FCD患者。
所有患者均患有严重的局灶性癫痫；婴儿期，其中4例还发生过癫痫痉挛，3例伴有高峰节律紊乱。5例患者（16%）出现局灶性癫痫持续状态，1例（3.2%）出现持续性部分性癫痫。20例（64.5%）患者在睡眠中发作，19例（61.3%）患者发作呈簇状。神经系统检查显示7例（22.8%）患者有轻度运动功能缺损。发作间期异常表现为节律性棘波和多棘波放电，13例（41.9%）患者在睡眠中增多。术后平均随访时间为4.7年，中位时间为4年，范围为1至9年。Engel分级I级的有20例（67.7%），II级的有3例（9.6%）。平均随访4.7年后无显著变化。
我们的结果证实，手术是治疗II型FCD所致难治性局灶性癫痫患儿的最佳选择。发现癫痫发作起始年龄大于2岁与良好预后之间存在统计学显著相关性。
