Liu Wenyu, An Dongmei, Xiao Jiahe, Li Jinmei, Hao Nanya, Zhou Dong
Departments of Neurology, West China Hospital, Sichuan University, No. 37 Guo Xue Alley, Chengdu 610041, China.
Departments of Radiology, West China Hospital, Sichuan University, No. 37 Guo Xue Alley, Chengdu 610041, China.
Seizure. 2015 Nov;32:92-9. doi: 10.1016/j.seizure.2015.09.009. Epub 2015 Sep 25.
Malformations of cortical development (MCDs) are abnormalities of the cerebral cortex that arise from abnormal formation of the cortical plate, and have become increasingly identified as an important etiology for refractory epilepsy. Little is known about the spectrum, distribution and clinical features of MCDs, especially in resource-limited regions. This study investigates the distribution of MCDs and compares the clinical features and long-term prognosis between the two forms of MCDs: Simple and Multiple.
One hundred and fifty epilepsy patients (138 adults, 12 pediatric patients) with radiologically diagnosed MCDs were identified at a tertiary epilepsy center in western China. Patients were divided into three subtypes according to the Barkovich classification. They were further divided into either Simple or Multiple MCD forms based on whether they had a single type of MCDs or other co-existing developmental brain abnormalities.
The most common type of MCD is focal cortical dysplasia. We found perinatal insults more common in group III patients. Multiple MCD was identified in 36 of 150 patients, and was associated with higher rates of delayed milestones (p=0.005), cognitive impairment (p=0.023) and neurological deficits (p=0.002) compared to Simple MCD. Extra-temporal epilepsy was more commonly seen among individuals with Multiple MCD (p=0.017). Participants with Multiple MCD were younger at time of seizure onset (p=0.003) and at assessment (p=0.002), had a lower seizure-free rate (p=0.033) and had worse outcomes overall. Patients with heterotopias were more commonly associated with other abnormalities.
MCDs are a critical cause of epilepsy and pose a big challenge for resource-limited countries. Imaging techniques are crucial in diagnosing and classifying cortical deformities. Multiple malformations lead to more severe clinical features and worse prognosis. Identifying and classifying MCDs can help physicians to better estimate patient prognosis and seek the best, individualized therapeutic options.
皮质发育畸形（MCDs）是由于皮质板异常形成而导致的大脑皮质异常，并且越来越多地被认为是难治性癫痫的一个重要病因。关于MCDs的谱系、分布及临床特征，人们了解甚少，尤其是在资源有限的地区。本研究调查MCDs的分布情况，并比较两种形式的MCDs（单纯型和复合型）之间的临床特征及长期预后。
在中国西部的一家三级癫痫中心，确定了150例经影像学诊断为MCDs的癫痫患者（138例成人，12例儿童患者）。根据Barkovich分类法，患者被分为三个亚型。根据是否患有单一类型的MCDs或其他并存的脑发育异常，他们被进一步分为单纯型或复合型MCDs。
最常见的MCD类型是局灶性皮质发育不良。我们发现围产期损伤在III型患者中更为常见。150例患者中有36例被诊断为复合型MCD，与单纯型MCD相比，其发育迟缓（p = 0.005）、认知障碍（p = 0.023）和神经功能缺损（p = 0.002）的发生率更高。复合型MCD患者中颞叶外癫痫更为常见（p = 0.017）。复合型MCD患者的癫痫发作起始年龄（p = 0.003）和评估时年龄（p = 0.002）更小，无癫痫发作率更低（p = 0.033），总体预后更差。异位症患者更常与其他异常相关。
MCDs是癫痫的一个关键病因，对资源有限的国家构成了巨大挑战。成像技术在诊断和分类皮质畸形方面至关重要。多种畸形会导致更严重的临床特征和更差的预后。识别和分类MCDs有助于医生更好地评估患者预后，并寻求最佳的个体化治疗方案。
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