双侧脑室旁结节状灰质异位症患者11例影像与临床研究

摘要

目的 分析双侧脑室旁结节状灰质异位症(BPNH)的影像特征与所致癫(癎)的临床特点和疗效.方法 MRI确诊的BPNH患者11例,根据是否合并其他神经系统解剖异常分为单纯BPNH组和叠加BPNH组,随访1～10年.对照分析2组患者的临床特点和药物治疗长期预后的差异.结果 单纯BPNH组患者7例,发病年龄8～32岁,7例患者精神行为智力发育正常,癫(癎)发作均为部分性发作,4例有异位灰质结节部位的(癎)样放电,5例有发作间期的颞叶(癎)样放电,7例患者经卡马西平治疗均有效;叠加BPNH组患者4例,发病年龄3～8个月,均有早期的精神运动发育迟缓,癫(癎)发作频繁,存在抗癫(癎)药物抵抗.4例BPNH患者合并有心血管系统畸形.结论 单纯BPNH组出现癫(癎)发作的年龄较叠加BPNH组晚,患者精神行为智力发育正常,发作形式均为部分性发作,对卡马西平治疗敏感,预后好.异位灰质结节部位和颞叶的(癎)样放电是单纯BPNH组患者的脑电图特征.应注意BPNH患者其他系统疾病的诊断,早期进行治疗.%Objective To analyze the clinical characteristics,brain MRI,EEG and the anti- epileptic therapeutic efficacy of bilateral periventricular nodular heterotopia induced epilepsy.Methods According to whether there were other neural abnormalities except BPNH,the patients were divided into two groups.Seven patients had periventricular nodules only(simple BPNH)and 4 patients also presented with other cortical or cerebral malformations(BPNH plus).The clinical data were collected,and the patients were followed up for 1 year to 10 years.Results Two patterns emerged:(1)The first pattern,associated with simple BPNH,was characterized by normal intelligence and seizures occurred in the second decade of life.Partial seizures were the commonest seizure types in this study.Interictal spiking or slow wave activity was found in ectopic grey matter in four patients,and in the temporal structures in 5 patients.All of them were efiective with carbamazepine.After a long term follow up,their prognosis Was favorable. (2)The second pattern,associated with BPNH plus,was characterized by mental retardation and seizures that began during the first decade.Seizures of the BPNH plus were intractable.Four of the BPNH also had malformations of the cardiovascular system. Conclusions Simple BPNH patients have normal intelligence and partial seizures which begin during the second decade of life. The seizures are effective with carbamazepine and never become frequent and tend to become very rare or disappear.Interictal spiking activity accords with ectopic grey matter,and spiking in the temporal structures are two EEG characteristics of the simple BPNH patients.The results also suggest that we should pay attention to malformations of others systems besides neural system.