St. Johns's Medical College & Hospital, Bangalore, India.
Seizure. 2010 Apr;19(3):147-52. doi: 10.1016/j.seizure.2010.01.005. Epub 2010 Feb 9.
Malformations of cortical development (MCDs) are increasingly recognized as important cause of epilepsy, especially refractory epilepsy. In developing countries like India, where the facilities for sophisticated imaging are not easily accessible to all, the prevalence and the types of cortical malformations are largely unknown. Hence this preliminary study has been undertaken to examine the relation between epilepsy and malformations of cortical development in a resource-limited setting.
To study various types of malformations of cortical development (MCDs) associated with epilepsy and to correlate with their clinical semiology.
The study was conducted in a tertiary care neurological center in south India. Cohort included all patients with epilepsy associated with cortical malformation on neuroimaging.
Neuroimaging data of all patients with epilepsy were evaluated for a 5-year period from 1998 to 2003, for the presence of cortical malformations. The case records of those patients with cortical malformations were taken from the medical records department and the clinical and electrophysiological data were analyzed.
We are reporting 34 cases of MCDs evaluated during the 5-year period. The mean age of the cohort was 15.1 (+/-12.2) years, with a range from 3 months to 45 years and 52.9% were males. Mean age at seizure onset was 7.2 years (+/-7.8), with a mean duration of seizure of 8.1 years (+/-7.7). Delayed motor and mental milestones were present in 15 patients (44.1%) and positive family history of seizure/epilepsy was seen in 9 patients (26.5%). Cortical malformations were most often associated with partial seizures (19/34, 55.9%). The most common type of seizure was complex partial seizure, seen in 12 patients (35.3%). Majority had very frequent, uncontrolled seizures with 16 (47.1%) patients having a seizure frequency of more than one per day. Heterotopias were seen in 14 patients (41.2%), in isolation in 5 (14.7%) patients and in combination with other malformations in 9 (26.5%) patients. Pachygyria was present as an isolated anomaly in five (14.7%) patients and combined with other abnormalities in eight (23.5%) patients. Cortical dysplasia was seen in 5 (14.7%) patients, hemimegalencephaly in two patients, polymicrogyria in two patients, lissencephaly and schizencephaly were seen in one patient each. EEG demonstrated focal epileptiform discharges in 59.1%, while generalized epileptiform discharges were seen in 22.7% of patients. Twenty-seven out of 34 (79.4%) patients had refractory/difficult to treat epilepsy.
Malformations of cortical development are a heterogeneous group of disorders, associated with developmental delay and refractory seizures but seizures usually do not have pathognomonic semiologic features. Possibility of MCDs should be considered during the evaluation of refractory epilepsy cases.
皮质发育畸形（MCD）越来越被认为是癫痫的重要原因，尤其是难治性癫痫。在印度等发展中国家，复杂的成像设备不容易普及，皮质畸形的患病率和类型在很大程度上尚不清楚。因此，进行了这项初步研究，以检查资源有限环境中皮质发育畸形与癫痫之间的关系。
研究与癫痫相关的各种类型的皮质发育畸形（MCD），并与它们的临床症状学相关联。
该研究在印度南部的一家三级护理神经科中心进行。队列包括所有在神经影像学上有皮质畸形相关癫痫的患者。
对 1998 年至 2003 年期间的 5 年神经影像学数据进行评估，以确定是否存在皮质畸形。从病历部门获取有皮质畸形的患者的病历记录，并分析临床和电生理数据。
在 5 年期间，我们报告了 34 例 MCD 病例。队列的平均年龄为 15.1（+/-12.2）岁，年龄范围为 3 个月至 45 岁，52.9%为男性。癫痫发作的平均年龄为 7.2（+/-7.8）岁，癫痫发作的平均持续时间为 8.1（+/-7.7）年。15 名患者（44.1%）有运动和精神发育迟缓，9 名患者（26.5%）有癫痫/癫痫家族史。皮质畸形最常与部分性癫痫发作相关（19/34，55.9%）。最常见的癫痫发作类型是复杂部分性发作，12 名患者（35.3%）有这种发作。大多数患者癫痫发作非常频繁，无法控制，16 名患者（47.1%）每天发作超过一次。14 名患者（41.2%）有异位症，5 名患者（14.7%）孤立性异位症，9 名患者（26.5%）与其他畸形并存。无脑回畸形作为一种孤立性异常存在于 5 名患者（14.7%）中，存在于 8 名患者（23.5%）中与其他异常并存。皮质发育不良在 5 名患者（14.7%）中可见，2 名患者有偏瘫，2 名患者有巨脑回畸形，1 名患者有无脑回畸形和脑裂畸形。脑电图显示 59.1%有局灶性癫痫样放电，22.7%的患者有全身性癫痫样放电。34 名患者中有 27 名（79.4%）有难治性/难以治疗的癫痫。
皮质发育畸形是一组异质性疾病，与发育迟缓和难治性癫痫相关，但癫痫发作通常没有特异性的症状学特征。在评估难治性癫痫病例时，应考虑到 MCD 的可能性。
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