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IR@PKUHSC  > 北京大学第一临床医学院  > 医学影像科  > 期刊论文
学科主题: 影像医学
题名:
中国人福山型先天性肌营养不良的MRI特征
其他题名: MRI findings in Fukuyama-type congenital muscular dystrophy of Chinese
作者: 程双娟1; 杨海坡1; 肖江喜1; 李飞宇1; 熊晖1; 王硕1
关键词: 肌营养不良 ; ; 磁共振成像 ; Muscular dystrophies ; Brain ; Magnetic resonance imaging
刊名: 中华放射学杂志
发表日期: 2015
DOI: 10.3760/cma.j.issn.1005-1201.2015.04.017
卷: 49, 期:4, 页:310-312
收录类别: 中国科技核心期刊 ; 中文核心期刊 ; CSCD
文章类型: Journal Article
摘要: 目的 探讨中国人福山型先天性肌营养不良(FCMD)的MRI特征.方法 回顾性分析我院基因检测确诊为FCMD的3例患儿的临床及影像资料.3例患儿中女2例、男1例,行MR检查时的年龄分别是0.5、2.3、5.0岁.临床主要表现为为肌力、肌张力低下和严重的发育迟滞.由2名资深的神经放射医师对MRI影像进行评价,包括大脑皮层发育异常、小脑发育异常、大脑白质异常及脑干改变等.结果 3例患儿均有额叶多小脑回改变,小脑皮层下多发小囊状灶呈长T1、长T2信号,3例大脑白质信号异常,其中2例伴小脑结构的改变及脑干细小、1例伴颞极皮层下白质囊变及侧脑室的发育异常.结论 FCMD具有典型MRI表现,结合临床症状及生化检测可以做出初步诊断. Objective To investigate the MRI features of Fukuyama-type congenital muscular dystrophy(FCMD) in Chinese.Methods The MRI and clinical data of 3 patients with FCMD which had been diagnosed by gene analysis were retrospectively analyzed.Two females and one male were included in this study,and they underwent MR examination at 0.5,2.3,5.0 years old respectively.The main clinical manifestations were muscular hypotonia and severe developmental delay.Abnormalities on MR images were analyzed and recorded by two experienced radiologists.Results Unlayerdpoly microgyria involved in frontal lobes,numerous intraparenchymal cysts at the peripheral hemispheres and prolonged T1 and T2 signal in the white matter were found in all the 3 cases.Disorganized cerebellar folia,lissencephaly of cerebral cortices,flattened pons were detected in 2 cases.Cystic region of white matter incerebral cortices and enlarged fourth ventricle could be seen in one case.Conclusion There are typical MR imaging featuresof FCMD,and preliminary diagnosis can be made by the combination with clinical symptoms and biochemical analysis.
语种: 中文
原文出处: 查看原文
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内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/42542
Appears in Collections:北京大学第一临床医学院_医学影像科_期刊论文

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作者单位: 1.100034,北京大学第一医院医学影像科
2.100034,北京大学第一医院儿科
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