DIFFUSE CORTICAL DYSPLASIA, OR THE DOUBLE CORTEX SYNDROME - THE CLINICAL AND EPILEPTIC SPECTRUM IN 10 PATIENTS
DIFFUSE CORTICAL DYSPLASIA, OR THE DOUBLE CORTEX SYNDROME - THE CLINICAL AND EPILEPTIC SPECTRUM IN 10 PATIENTS
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DOI：
10.1212/wnl.41.10.1656
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发表时间：
1991-10-01
期刊：
影响因子：
9.9
通讯作者：
ROBITAILLE, Y
中科院分区：
文献类型：
--
作者：
PALMINI, A;ANDERMANN, F;ROBITAILLE, Y
现在可以通过现代神经影像技术，特别是高分辨率 MRI 来识别与癫痫相关的弥漫性神经元迁移障碍。我们报道了 10 名患者，他们最近描述了 MRI 图像，显示皮质套下存在连续或广泛的带状异位，呈现出“双皮质”的外观。他们患有癫痫症，几乎所有人都有智力障碍。癫痫病的性质和严重程度各不相同。患者可能会出现婴儿痉挛症、Lennox-Gastaut 综合征或其他形式的继发性全身性或多灶性癫痫。对药物治疗的反应是可变的。胼胝体切开术可能会大大减少跌倒攻击，减少 60%。精神发育迟滞通常是轻度或中度，很少有严重的。它与癫痫综合征的类型相关，并且在异位上皮质更混乱的患者中更大。通过 MRI 识别该实体对于癫痫病的正确诊断、治疗策略的规划和预后非常重要。
Diffuse neuronal migration disorders associated with epilepsy can now be recognized by modem neuroimaging techniques, particularly high-resolution MRI. We report 10 patients with a recently described MRI picture of continuous or generalized band heterotopia underlying the cortical mantle, giving the appearance of a "double cortex." They have epilepsy, and almost all have mental retardation. The epileptic disorder varies in nature and degree of severity. Patients may present with infantile spasms, a Lennox-Gastaut syndrome, or other forms of secondary generalized or multifocal epilepsy. Response to medical treatment is variable. Callosotomy may lead to considerable reduction of drop attacks, present in 60%. Mental retardation is usually mild or moderate, and only rarely severe. It correlates with the type of epileptic syndrome, and is greater in patients with more disorganized cortex overlying the heterotopia. Recognition of this entity by MRI is important for appropriate diagnosis of the epileptic disorder, planning of therapeutic strategy, and prognosis.