Department of Neurosurgery, Research Institute of Clinical Medicine of Chonbuk National University Hospital, Chonbuk National University, Jeonju, South Korea.
Acta Neurochir (Wien). 2013 Sep;155(9):1717-24. doi: 10.1007/s00701-013-1791-0. Epub 2013 Jun 23.
Schizencephaly is a developmental anomaly of the brain that is sometimes associated with intractable seizures. Patients that suffer from medically refractory seizures may be considered for surgical treatment.
Five patients with intractable epilepsy associated with schizencephaly were studied. Evaluation methods included medical history assessment, neurological examination, magnetic resonance imaging (MRI) with three-dimensional (3D) surface rendering, positron emission tomography (PET), video-electroencephalogram (EEG) monitoring with surface electrodes and subdural grid electrodes, sodium amobarbital test, and neuropsychological assessments. Topectomy was performed close to the schizencephalic cleft in two patients, and at an area distant from the cleft in one under the guidance of electrocorticography (ECoG). Temporal lobectomy was performed in two patients.
MRI revealed unilateral schizencephaly in all five patients. Video-EEG monitoring recorded simple partial seizures in two patients and complex partial seizures in three patients. The epileptogenic zone was localized close to the schizencephalic cleft in two patients, distant to the cleft in one patient, and in the temporal lobe in two patients. Postoperatively, one of two patients with temporal lobectomy and one of three patients with topectomy were seizure-free at 1-year follow-up. Three patients experienced marked seizure reduction but were not seizure-free at 1 year; however, at the follow-up periods of 2.5 years and 6.5 years, two of these three patients were seizure free. One patient who underwent temporal lobectomy began to have very brief losses of consciousness lasting 1-3 s (3-4 times per year). Neurological complications included temporary upper monoparesis in one and hemiparesis in one.
The abnormal cortex lining schizencephalic clefts and cortical tissues near the cleft may be epileptogenic. Areas distant to the cleft may also be the source of seizures. Careful evaluation should be performed to define the epileptogenic zone in patients with intractable epilepsy associated with schizencephaly, and meticulous resection of the epileptogenic zone can lead to good seizure control.
脑裂畸形是一种脑发育异常，有时与难治性癫痫有关。患有药物难治性癫痫的患者可能需要考虑手术治疗。
研究了 5 例伴有脑裂畸形的难治性癫痫患者。评估方法包括病史评估、神经检查、三维表面重建磁共振成像（MRI）、正电子发射断层扫描（PET）、表面电极和硬膜下网格电极视频脑电图（EEG）监测、戊巴比妥钠测试和神经心理学评估。在 2 例患者中，在脑电图（ECoG）指导下，在靠近脑裂的部位进行了脑裂切开术，在 1 例患者中，在远离脑裂的部位进行了脑裂切开术。在 2 例患者中进行了颞叶切除术。
MRI 显示 5 例患者均为单侧脑裂畸形。视频-EEG 监测记录了 2 例患者的简单部分性发作和 3 例患者的复杂部分性发作。2 例患者的致痫灶靠近脑裂，1 例患者的致痫灶远离脑裂，2 例患者的致痫灶在颞叶。术后，2 例颞叶切除术患者和 3 例脑裂切开术患者中有 1 例在 1 年随访时无癫痫发作。3 例患者癫痫发作明显减少，但 1 年内无癫痫发作；然而，在 2.5 年和 6.5 年的随访期，这 3 例中有 2 例无癫痫发作。1 例接受颞叶切除术的患者开始出现持续 1-3 s（每年 3-4 次）的短暂意识丧失。神经并发症包括 1 例暂时性单侧单瘫和 1 例偏瘫。
脑裂裂隙内的异常皮层和裂隙附近的皮层组织可能是致痫灶。远离裂隙的区域也可能是癫痫发作的来源。对于伴有脑裂畸形的药物难治性癫痫患者，应进行仔细评估以确定致痫灶，并进行精细的致痫灶切除，以获得良好的癫痫控制效果。
