Department of Pediatrics, International St. Mary's Hospital, Catholic Kwandong University, College of Medicine, Incheon, Republic of Korea.
Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea.
Seizure. 2020 Nov;82:50-55. doi: 10.1016/j.seizure.2020.09.019. Epub 2020 Sep 23.
The observation of mild malformation of cortical development (mMCD) has yet to have a major clinical impact due to the lack of clinical and research data. We characterized the clinical features, surgical outcomes, and postoperative seizure control patterns in pediatric patients with mMCD.
We examined 40 patients with isolated mMCD who underwent resective surgery during a 10-year period.
The median age at seizure onset was 1.2 years, and the median age at surgery was 7.9 years. Twenty-seven patients (67.5%) presented with childhood-onset epileptic encephalopathy (21 Lennox-Gastaut syndrome, 6 West syndrome), and 13 patients (32.5%) presented with intractable focal epilepsy (10 extratemporal lesions, 3 temporal lesions). Twenty-one patients (52.5%) showed "suspected focal cortical malformation" on MRI, whereas 16 patients (40.0%) and 3 patients (7.5%) showed normal MRI findings or mild brain atrophy, respectively. The most common surgical procedures were two lobar resections (18 patients, 45.0%), followed by unilobar resections (12 patients, 30.0%) and resections exceeding two lobar boundaries (10 patients, 25.0%). As a final surgical outcome, 24 patients (60.0%) were ILAE Class 1-3. Discontinuation of all AEDs was possible for 36.8% of ILAE Class 1 patients. Regarding the seizure control pattern, fluctuating seizure control was observed most frequently (21 patients, 52.5%).
Our results suggest that mMCD is an important pathological finding in children related to a significant degree of epileptogenicity, and resective surgery can have positive outcomes. However, these patients showed unstable postoperative seizure control patterns with a high rate of late recurrence, suggesting difficulties in the surgical treatment of intractable epilepsy.
由于缺乏临床和研究数据，轻度皮质发育不良（mMCD）的观察尚未对临床产生重大影响。我们对接受 mMCD 切除术的儿科患者的临床特征、手术结果和术后癫痫控制模式进行了描述。
我们回顾了 10 年间接受切除术的 40 例孤立性 mMCD 患者。
癫痫发作的中位年龄为 1.2 岁，手术的中位年龄为 7.9 岁。27 例（67.5%）患者表现为儿童起病的癫痫性脑病（21 例 Lennox-Gastaut 综合征，6 例 West 综合征），13 例（32.5%）患者表现为难治性局灶性癫痫（10 例颞叶外病变，3 例颞叶病变）。21 例（52.5%）患者 MRI 显示“可疑局灶性皮质发育不良”，16 例（40.0%）和 3 例（7.5%）患者 MRI 正常或轻度脑萎缩。最常见的手术方式是两叶切除术（18 例，45.0%），其次是单叶切除术（12 例，30.0%）和超过两叶边界的切除术（10 例，25.0%）。作为最终手术结果，24 例（60.0%）患者 ILAE 分级为 1-3 级。1 级患者中有 36.8%可停用所有抗癫痫药物。就癫痫控制模式而言，波动的癫痫控制最为常见（21 例，52.5%）。
我们的结果表明，mMCD 是一种与高度致痫性相关的重要儿童病理发现，切除术可获得良好的效果。然而，这些患者术后癫痫控制模式不稳定，复发率较高，提示难治性癫痫的手术治疗存在困难。
