Ricci S, Cusmai R, Fariello G, Fusco L, Vigevano F
Section of Neurophysiology, Bambino Gesù Children's Hospital, Rome, Italy.
Arch Neurol. 1992 Jan;49(1):61-4. doi: 10.1001/archneur.1992.00530250065017.
Band heterotopia, or "double cortex," is a neuronal migration disorder that consists of a symmetrical subcortical neuronal band. The overlying cortex may be normal or macrogyric. We describe two severely mentally retarded girls, aged 14 and 18 years, who had band heterotopia and Lennox-Gastaut syndrome. Band heterotopia was evident in both hemispheres as a subcortical symmetrical layer isointense with gray matter on magnetic resonance T1- and T2-weighted images. Both patients had atonic seizures, atypical absences, and tonic seizures. The electroencephalograms in both cases showed frequent generalized paroxysms and slow background activity. The association of a Lennox-Gastaut syndrome with double cortex in these two patients and in a previously reported autopsy-confirmed case suggests that this malformation may be responsible for other similar cases.
带状灰质异位症，即“双皮质征”，是一种神经元迁移障碍疾病，表现为对称的皮质下神经元带。其上方的皮质可能正常或呈巨脑回畸形。我们描述了两名严重智力发育迟缓的女孩，年龄分别为14岁和18岁，她们患有带状灰质异位症和Lennox-Gastaut综合征。在磁共振T1加权和T2加权图像上，双侧半球均可见明显的带状灰质异位症，表现为皮质下对称层，与灰质等信号。两名患者均有失张力发作、不典型失神发作和强直发作。两例患者的脑电图均显示频繁的全身性阵发活动和缓慢的背景活动。这两名患者以及之前一例经尸检证实的病例中Lennox-Gastaut综合征与双皮质征的关联表明，这种畸形可能是其他类似病例的病因。
