Kuzniecky R, Andermann F, Tampieri D, Melanson D, Olivier A, Leppik I
Department of Neurology and Neurosurgery, McGill University, Montreal, Canada.
Ann Neurol. 1989 Jun;25(6):547-54. doi: 10.1002/ana.410250604.
The neuronal migration disorders comprise several morphological entities that are recognizable during life using current imaging techniques. We studied 4 patients who had a characteristic bilateral central rolandic and sylvian macrogyria. The patients had pseudobulbar palsy with oromotor incoordination and developmental delay and were mildly retarded. Minor seizures developed between the ages of 8 and 9 years. Subsequently, atonic drop attacks became the predominant epileptic pattern. Epileptogenic electrographic abnormalities were secondary generalized or multifocal. The lesions were detected by computed tomography and magnetic resonance imaging in all patients. Bilateral symmetrical areas of thick cortex surrounding a large sulcus were seen. This syndrome consists of specific clinical, imaging, electroencephalographic, and epileptic features. It can be suspected clinically and confirmed by imaging studies. Callosotomy in two patients helped the intractable seizures.
神经元迁移障碍包括几种形态学实体,使用当前的成像技术在生命过程中可识别。我们研究了4例具有特征性双侧中央前回和外侧裂巨脑回的患者。这些患者有假性延髓麻痹伴口运动不协调和发育迟缓,且轻度智力低下。在8至9岁之间出现轻微癫痫发作。随后,失张力跌倒发作成为主要的癫痫发作形式。致痫性脑电图异常为继发性全身性或多灶性。所有患者均通过计算机断层扫描和磁共振成像检测到病变。可见围绕大沟的双侧对称厚皮质区域。该综合征具有特定的临床、影像学、脑电图和癫痫特征。临床上可怀疑,并通过影像学研究确诊。两名患者行胼胝体切开术对难治性癫痫发作有帮助。