Introduction. Ulegyria results from perinatal hypoxic-ischemic brain injury in term infants. The specific mushroom-shaped configuration of ulegyria results from small atrophic circumvolutions at the bottom of a sulcus underlying an intact gyral apex. Clinically, ulegyria is generally associated with epilepsy. Here, we aimed to delineate the characteristics of patients with ulegyria and the epileptic seizures they experience. Material and methods. Medical records including radiology and pathology reports, video-electroencephalographic (EEG) analysis, operative notes, hospital progress and outpatient clinic notes were reviewed retrospectively in a total of 10 ulegyria patients. Results. Patients ages ranged between 24 and 58 years (mean, 32 ± 9.8 years). Past medical history was confirmed for neonatal asphyxia in 2 (20%). Neurological examination was remarkable for spastic hemiparesis in 1 (10%) patient with perisylvian ulegyria and for visual field deficits in 2 patients (20%) with occipital ulegyria. Ulegyria most commonly involved the temporoparietal region (n = 5, 50%) followed by the perisylvian area (n = 2, 20%). Except the one with bilateral perisylvian ulegyria, all patients had unilateral lesions (n = 9, 90%). Hippocampal sclerosis accompanied ulegyria in 2 patients (20%). All patients experienced epileptic seizures. Mean age at seizure onset was 8.8 ± 5.4 years (range, 2-20 years). Interictal scalp EEG and EEG-video monitoring records demonstrated temporoparietal and frontotemporal activities in 5 (50%) and 2 (20%) patients, respectively. The seizures were successfully controlled by antiepileptic medication in 8 patients (n = 8, 80%). The remaining 2 patients (%20) with concomitant hippocampal sclerosis required microsurgical resection of the seizure foci due to medically resistant seizures. Discussion. Ulegyria is easily recognized with its unique magnetic resonance imaging characteristics and clinical presentation in the majority of cases. It is highly associated with either medically resistant or medically controllable epileptic seizures. The treatment strategy depends on the age at onset and extends of the lesion that has a significant impact on the severity of the clinical picture.

介绍。Ulegyria 是足月儿围产期缺氧缺血性脑损伤的结果。ulegyria 的特定蘑菇状结构是由完整的脑回顶端下方的沟底部的小型萎缩性回旋产生的。临床上，痛风一般与癫痫有关。在这里，我们的目的是描述患有 legyria 的患者的特征和他们经历的癫痫发作。材料和方法。回顾性审查了总共 10 例 ulegyria 患者的医疗记录，包括放射学和病理学报告、视频脑电图 (EEG) 分析、手术记录、医院进展和门诊记录。结果。患者年龄介于 24 至 58 岁之间（平均 32 ± 9.8 岁）。2 例（20%）新生儿窒息的既往病史被证实。神经系统检查显示 1 名 (10%) 患有侧裂神经痛的患者痉挛性偏瘫和 2 名 (20%) 患有枕骨痛的患者的视野缺损。Ulegyria 最常累及颞顶区（n = 5, 50%），其次是外侧裂区（n = 2, 20%）。除双侧外侧裂痛患者外，所有患者均为单侧病变（n = 9, 90%）。2 名患者 (20%) 伴有海马硬化症。所有患者均出现癫痫发作。癫痫发作的平均年龄为 8.8 ± 5.4 岁（范围 2-20 岁）。发作间期头皮脑电图和脑电图视频监测记录分别显示 5 名 (50%) 和 2 名 (20%) 患者的颞顶和额颞活动。8 名患者（n = 8, 80%）通过抗癫痫药物成功控制了癫痫发作。其余 2 名合并海马硬化的患者 (%20) 因药物难治性癫痫发作需要显微手术切除癫痫病灶。讨论。在大多数情况下，Ulegyria 以其独特的磁共振成像特征和临床表现很容易被识别。它与医学上耐药或医学上可控的癫痫发作高度相关。治疗策略取决于发病年龄和病变范围，这对临床表现的严重程度有显着影响。