Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA; Department of Mathematics and Statistics, Boston University, Boston, MA 02215, USA.
Epilepsy Behav. 2023 Jul;144:109254. doi: 10.1016/j.yebeh.2023.109254. Epub 2023 May 18.
Self-limited epilepsy with centrotemporal spikes is a transient developmental epilepsy with a seizure onset zone localized to the centrotemporal cortex that commonly impacts aspects of language function. To better understand the relationship between these anatomical findings and symptoms, we characterized the language profile and white matter microstructural and macrostructural features in a cohort of children with SeLECTS.
Children with active SeLECTS (n = 13), resolved SeLECTS (n = 12), and controls (n = 17) underwent high-resolution MRIs including diffusion tensor imaging sequences and multiple standardized neuropsychological measures of language function. We identified the superficial white matter abutting the inferior rolandic cortex and superior temporal gyrus using a cortical parcellation atlas and derived the arcuate fasciculus connecting them using probabilistic tractography. We compared white matter microstructural characteristics (axial, radial and mean diffusivity, and fractional anisotropy) between groups in each region, and tested for linear relationships between diffusivity metrics in these regions and language scores on neuropsychological testing.
We found significant differences in several language modalities in children with SeLECTS compared to controls. Children with SeLECTS performed worse on assessments of phonological awareness (p = 0.045) and verbal comprehension (p = 0.050). Reduced performance was more pronounced in children with active SeLECTS compared to controls, namely, phonological awareness (p = 0.028), verbal comprehension (p = 0.028), and verbal category fluency (p = 0.031), with trends toward worse performance also observed in verbal letter fluency (p = 0.052), and the expressive one-word picture vocabulary test (p = 0.068). Children with active SeLECTS perform worse than children with SeLECTS in remission on tests of verbal category fluency (p = 0.009), verbal letter fluency (p = 0.006), and the expressive one-word picture vocabulary test (p = 0.045). We also found abnormal superficial white matter microstructure in centrotemporal ROIs in children with SeLECTS, characterized by increased diffusivity and fractional anisotropy compared to controls (AD p = 0.014, RD p = 0.028, MD p = 0.020, and FA p = 0.024). Structural connectivity of the arcuate fasciculus connecting perisylvian cortical regions was lower in children with SeLECTS (p = 0.045), and in the arcuate fasciculus children with SeLECTS had increased diffusivity (AD p = 0.007, RD p = 0.006, MD p = 0.016), with no difference in fractional anisotropy (p = 0.22). However, linear tests comparing white matter microstructure in areas constituting language networks and language performance did not withstand correction for multiple comparisons in this sample, although a trend was seen between FA in the arcuate fasciculus and verbal category fluency (p = 0.047) and the expressive one-word picture vocabulary test (p = 0.036).
We found impaired language development in children with SeLECTS, particularly in those with active SeLECTS, as well as abnormalities in the superficial centrotemporal white matter as well as the fibers connecting these regions, the arcuate fasciculus. Although relationships between language performance and white matter abnormalities did not pass correction for multiple comparisons, taken together, these results provide evidence of atypical white matter maturation in fibers involved in language processing, which may contribute to the aspects of language function that are commonly affected by the disorder.
具有中央颞区棘波的自限性癫痫是一种具有局灶性的发育性癫痫,其发作区定位于中央颞叶皮质,通常会影响语言功能的各个方面。为了更好地了解这些解剖学发现与症状之间的关系,我们对患有 SeLECTS 的儿童队列进行了语言特征分析,并对其白质微观结构和宏观结构特征进行了研究。
活跃性 SeLECTS 患儿(n=13)、缓解性 SeLECTS 患儿(n=12)和对照组儿童(n=17)接受了高分辨率 MRI 检查,包括弥散张量成像序列和多项语言功能的标准化神经心理学测试。我们使用皮质分区图谱识别毗邻下额回皮质和上颞回的表浅白质,并使用概率追踪技术确定连接它们的弓状束。我们比较了各组之间这些区域的白质微观结构特征(轴向弥散度、径向弥散度、平均弥散度和各向异性分数),并测试了这些区域弥散度指标与神经心理学测试中语言评分之间的线性关系。
我们发现与对照组相比,患有 SeLECTS 的儿童在几个语言模态上存在显著差异。患有 SeLECTS 的儿童在语音意识(p=0.045)和言语理解(p=0.050)评估方面表现较差。与对照组相比,活动性 SeLECTS 患儿的表现更差,具体表现为语音意识(p=0.028)、言语理解(p=0.028)和言语类别流畅性(p=0.031),在言语字母流畅性(p=0.052)和表达性单字图片词汇测试(p=0.068)方面也存在表现趋势较差的情况。与缓解性 SeLECTS 患儿相比,活动性 SeLECTS 患儿在言语类别流畅性(p=0.009)、言语字母流畅性(p=0.006)和表达性单字图片词汇测试(p=0.045)方面的表现更差。我们还发现患有 SeLECTS 的儿童中央颞区 ROI 中的表浅白质微观结构异常,与对照组相比,表现为弥散度和各向异性分数增加(AD p=0.014、RD p=0.028、MD p=0.020 和 FA p=0.024)。连接颞叶皮质区的弓状束的结构连接在 SeLECTS 患儿中较低(p=0.045),并且在弓状束中,SeLECTS 患儿的弥散度增加(AD p=0.007、RD p=0.006、MD p=0.016),但各向异性分数没有差异(p=0.22)。然而,在该样本中,对构成语言网络的白质微观结构和语言表现进行线性比较的测试,没有通过多重比较的校正,尽管在弓状束的 FA 与言语类别流畅性(p=0.047)和表达性单字图片词汇测试(p=0.036)之间存在趋势。
我们发现患有 SeLECTS 的儿童语言发育受损,尤其是活动性 SeLECTS 患儿,以及中央颞区表浅白质以及连接这些区域的纤维(弓状束)异常。尽管语言表现与白质异常之间的关系没有通过多重比较的校正,但总的来说,这些结果提供了证据,表明与语言处理相关的纤维的白质成熟度异常,这可能导致该疾病常见的语言功能方面受到影响。