Pedespan J M, Loiseau H, Vital A, Marchal C, Fontan D, Rougier A
Service de Neurologie Infantile, Hôpital Pellegrin, Bordeaux, France.
Epilepsia. 1995 Jan;36(1):37-40. doi: 10.1111/j.1528-1157.1995.tb01662.x.
A case of early epileptic encephalopathy (EIEE) with suppression-bursts or Ohtahara's syndrome, associated with focal cortical dysplasia is reported. Infantile spasms and brief tonic unilateral seizures began on the fifth day of life. Interictal EEG demonstrated an asymmetrical "suppression-burst" pattern with no wake or sleep cycling. Seizures were refractory to all antiepileptic drug (AED) and steroid therapy. Magnetic resonance imaging (MRI) showed right frontotemporal cortical thickening. After three weeks of an ineffective medical treatment a preoperative evaluation with single photon emission computed tomography (SPECT) and electrocorticography (ECoG) was performed to characterize epileptogenic focus. Surgical resection of the precentral area was performed at age 1 month. Neuropathologic examinations confirmed diagnosis of focal cortical dysplasia by identifying cytoarchitectural disarray and ectopic neurons located deep in subcortical white matter. During follow-up, 1-year postoperative the child had a single febrile seizure. Neurologic examination showed minor developmental delay and slight left-sided weakness.
本文报告了一例早期癫痫性脑病（EIEE），伴有抑制爆发或大田原综合征，与局灶性皮质发育异常相关。婴儿痉挛和短暂的单侧强直性发作始于出生后第5天。发作间期脑电图显示不对称的“抑制爆发”模式，无清醒或睡眠周期。癫痫发作对所有抗癫痫药物（AED）和类固醇治疗均无效。磁共振成像（MRI）显示右侧额颞叶皮质增厚。在药物治疗无效三周后，进行了单光子发射计算机断层扫描（SPECT）和皮质脑电图（ECoG）的术前评估，以确定致痫灶。患儿1个月大时进行了中央前区手术切除。神经病理学检查通过识别皮质结构紊乱和位于皮质下白质深处的异位神经元，确诊为局灶性皮质发育异常。随访期间，术后1年患儿出现一次热性惊厥。神经系统检查显示有轻度发育迟缓及轻微左侧肢体无力。
