Sturge-Weber综合征患儿的癫痫发作与脑皮层钙化和萎缩的关联性

The correlation of seizure and cortical calcification and atrophy in Sturge-Weber syndrome

目的 应用磁敏感加权成像(SWI)技术探讨Sturge-Weber综合征(SWS)患儿癫痫发作与头颅MRI提示的脑皮层钙化和萎缩的相关性.方法 对2004年至2013年间在美国韦恩州立大学医学中心确诊为Sturge-Weber综合征的14例患儿进行MRI检查,其中男6例,女8例,平均年龄4.15岁±2.84岁(0.8~10.0岁).所有患者的病变部位均为单侧大脑半球.MR扫描序列包括:矢状位及轴位T1WI、轴位FSE T2WI、SWI及增强后轴位T1WI.观察所有图像以确认脑内病变位置及范围;利用SPIN软件观察钙化情况并进行评分.在T2WI上测量病变累及层面两侧脑半球的面积并计算得到脑萎缩比值(BAR);记录每个患儿的临床资料:首次癫痫发作年龄、癫痫发作频率(SSF)及癫痫持续时间以及IQ评分.应用社会科学统计软件包SPSS 17.0版进行数据分析:对钙化评分、脑萎缩比值与临床状况进行Spearman相关分析.结果 14例SWS患儿患侧脑实质均发生不同程度脑萎缩,7例出现皮层钙化,BAR与钙化程度呈显著的正相关(r =0.78,P=0.00).BAR与首次癫痫发作年龄存在明显负相关性(r =-0.77,P=0.00).同时,BAR与患儿的IQ评分呈明显负相关(r=-0.54,P<0.05),即萎缩程度越重则患儿智力水平越低.有明显钙化(评分=2)的患儿IQ评分均值比无皮层钙化的(评分=0)明显降低(55±42,96±13,P=0.03).结论 Sturge-Weber综合征脑萎缩程度与癫痫发作时间有关联性,首次癫痫发作越早,脑萎缩越明显;患儿脑萎缩、皮层钙化的程度也与其智力水平具有明确的关系.

Objective To investigate the correlation between Sturge-Weber syndrome (SWS) in children with epilepsy and calcification and brain atrophy of cerebral cortex using susceptibility weighted imaging(SWI). Methods Fourteen patients (8 girls and 6 boys; age range, 0.8–10.0 years; mean age, 4.15±2.84 years) with Sturge-Weber syndrome were recruited and performed MRI examination. All patients with unilateral lesions were located in one side of the cerebral hemispheres. MR protocols included axial and sagittal T1-weighted, axial fast spin-echo (FSE) T2-weighted, SWI and contrasted T1-weighted. All raw data of SWI were processed by using the Signal Process in Neuroimage (SPIN) software. After determining the location and realm of SWS lesions, the calciifcation scores of the affected and brain atrophy ratio (BAR) were measured and calculated. The clinical data of each child were recorded, including the age of first seizure, seizure frequency (Score of seizure frequency, SSF),duration of epilepsy and IQ scores. The correlations of calcification scores of the affected and BAR and seizure variables (age of ifrst seizure, SSF and duration of epilepsy) were analyzed by Spearman correlation analysis in SPSS 17.0 software.Result In all, the affected brain parenchyma showed different extent brain atrophy, and then 7 cases with cortex calciifcation. A signiifcantly positive correlation between BAR and calciifcation was found (r =0.78,P=0.00). There was a signiifcantly negative correlations between BAR and ifrst seizure age (r =-0.77,P=0.00). Moreover, a signiifcantly negative correlation (r=0.54,P<0.05) between BAR and IQ scores was also found, meaning the severer atrophy of brain, the lower intelligent level of children. The IQ scores of children with obvious calciifcation (score=2) is signiifcantlydecreased than without calciifcation (score=0) (55±42,96±13,P=0.03).Conclusions There is a relationship between the degree of brain atrophy and the age ofifrst seizure in SWS. And the earlier epileptic attack, the more obvious brain atrophy. There is a speciifc relationship between the degree of brain atrophy, cortex calciifcation and intelligence level in children.