Juan Cao, Guocheng Yang, Shoujun Xu, Pengyue Tang, Yue Wang, Yingying Shan, Yongxian Chen, Peng He
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引用次数: 2
摘要
目的:探讨斯特奇-韦伯综合征患儿的临床病理特征，分析癫痫发作时脑膜血管瘤病的分布面积与大脑皮质钙化程度、脑萎缩程度的相关性。方法:对10例SWS合并FCD IIIc患儿进行组织病理学和免疫组化诊断。采用Spearman相关分析计算SWS与FCD IIIc和儿童癫痫发作的关系。结果:10例SWS患儿脑膜血管瘤面积与脑萎缩程度呈显著正相关(r = 0.783, p = 0.007)。小脑膜血管瘤的分布、皮质钙化程度、脑萎缩程度与癫痫无显著相关性。结论:SWS可能伴有FCD iii型。SWS小脑膜血管瘤病变的脑叶越广，脑萎缩越明显。
Clinicopathological Analysis of Sturge-Weber Syndrome with Focal Cortical Dysplasia FCD IIIc.
Objective: To investigate the clinicopathological features of children with Sturge-Weber syndrome and to analyze the correlation between the distribution area of leptomeningeal angiomatosis, the degree of cerebral cortical calcification, and the degree of cerebral atrophy associated with epileptic seizures. Methods: 10 children were diagnosed with SWS with FCD IIIc by histopathology and immunohistochemistry. Spearman correlation analysis was used to calculate the association of SWS with FCD IIIc and seizures in children. Results: The leptomeningeal angiomatosis area was markedly positively correlated with the degree of brain atrophy in 10 children with SWS (r = 0.783, p = 0.007). The distribution of leptomeningeal hemangiomatosis, the degree of cortical calcification, and brain atrophy were not significantly correlated with epilepsy. Conclusion: SWS may be accompanied by FCD IIIc. The more extensive the cerebral lobes of leptomeningeal angiomatosis in SWS, the more pronounced the brain atrophy.
期刊介绍：
Fetal and Pediatric Pathology is an established bimonthly international journal that publishes data on diseases of the developing embryo, newborns, children, and adolescents. The journal publishes original and review articles and reportable case reports.
The expanded scope of the journal encompasses molecular basis of genetic disorders; molecular basis of diseases that lead to implantation failures; molecular basis of abnormal placentation; placentology and molecular basis of habitual abortion; intrauterine development and molecular basis of embryonic death; pathogenisis and etiologic factors involved in sudden infant death syndrome; the underlying molecular basis, and pathogenesis of diseases that lead to morbidity and mortality in newborns; prenatal, perinatal, and pediatric diseases and molecular basis of diseases of childhood including solid tumors and tumors of the hematopoietic system; and experimental and molecular pathology.
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