Tuber Brain Proportion Determines Epilepsy Onset in Children With Tuberous Sclerosis Complex

作者全名："Zhang, Fuyi; Xie, Lingling; He, Xiaoya; Song, Xiaojie; Zheng, Helin; Wang, Longlun; Jiang, Li"

作者地址："[Zhang, Fuyi; Xie, Lingling; Song, Xiaojie; Jiang, Li] Chongqing Med Univ, Dept Neurol, Childrens Hosp,Chongqing Key Lab Pediat, Natl Clin Res Ctr Child Hlth & Disorders,Minist E, Chongqing, Peoples R China; [He, Xiaoya; Zheng, Helin; Wang, Longlun] Chongqing Med Univ, Dept Radiol, Childrens Hosp, Chongqing, Peoples R China"

通信作者："Jiang, L (通讯作者)，Chongqing Med Univ, Dept Neurol, Childrens Hosp, 136 Zhongshan Er Rd, Chongqing 400014, Peoples R China."

来源：PEDIATRIC NEUROLOGY

ESI学科分类：NEUROSCIENCE & BEHAVIOR

WOS号：WOS:000765946200008

JCR分区：Q1

影响因子：3.8

年份：2022

卷号：129

期号：　

开始页：39

结束页：45

文献类型：Article

关键词：Tuberous sclerosis complex; Magnetic resonance imaging; Tuber brain proportion; Epilepsy; Severity; Lesion burden

摘要："Background: Tuberous sclerosis complex (TSC) is a rare autosomal dominant disorder characterized by epilepsy and structural abnormalities of the brain. Little research has been done to explore the rela-tionship between the tuber brain proportion (TBP) and epilepsy. We investigated several quantitative cerebral lesions including TBP on magnetic resonance imaging (MRI) and their impact on the onset age, seizure mode, and antiseizure treatment effectiveness of epilepsy in children with TSC. Methods: We reviewed the clinical characteristics and MRI information of 44 children with TSC who had experienced epileptic seizures. Supratentorial tubers were quantitatively manually measured to calculate the TBP. The numbers of cortical/subcortical cyst-like tubers, diffuse lesions, subependymal nodules, and subependymal giant cell astrocytomas were also evaluated. Results: Twelve children (27.3%) had experienced infantile spasms, thirteen children (29.5%) had early-onset epilepsy, and twenty-seven patients (64.3%) had a significant reduction in the frequency of sei-zures after antiseizure treatments. The median TBP was 9.2%, and diffuse lesions (range: 0-2) and cortical cyst-like lesions (range: 0-17) were seen in seven and seventeen children, respectively. The values of TBP (P < 0.001), diffuse lesions (P < 0.001), and cortical cyst-like tubers (P < 0.001) were all associated with early-onset epilepsy. The values of TBP (P = 0.004) and cortical cyst-like tuber (P < 0.001) were asso-ciated with the occurrence of infantile spasms. The values of TBP (P = 0.01), diffuse lesions (P = 0.04), and cortical cyst-like tubers (P = 0.004) were negatively associated with the effectiveness of antiseizure treatments. There was no significant correlation between subcortical cyst-like tuber, subependymal nodule, subependymal giant cell astrocytoma, and epilepsy severity. Conclusions: Increasing abnormality of the cerebral hemispheres, as shown by quantitative MRI analysis including TBP, cortical cyst-like tubers, and diffuse lesions, is associated with measures of more severe epilepsy due to TSC. The values of TBP demonstrate strong significance for early-onset epilepsy. (c) 2021 Elsevier Inc. All rights reserved."

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