2014, Vol. 25 Issue (8): 538-541    DOI:
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半侧巨脑畸形的临床及MRI诊断(附5例报告并文献复习)
刘 斋1,何 丽1,王伟秀2,任庆云1
1. 河北医科大学第一医院放射科,河北 石家庄 050031;2. 河北省儿童医院,河北 石家庄 050031
Clinical and MRI diagnosis of hemimegalencephaly: report of 5 cases with literature review
LIU Zhai1, HE Li1, WANG Wei-xiu2, REN Qing-yun1
1. Department of Radiology, the First Hospital of Hebei Medical University, Shijiazhuang 050031, China;2. Children’s Hospital of Hebei Province, Shijiazhuang 050031, China
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摘要 目的:分析半侧巨脑畸形(HME)的临床及MRI特点,以提高对HME的认识。方法:回顾性分析5例HME的临床及MRI表现,并复习文献。结果:5例HME临床表现为癫痫3例、偏侧肢体肥大3例、精神发育迟滞2例。MRI平扫均表现为一侧大脑半球增大、大脑皮质增厚、灰白质交界模糊,病变位于左侧大脑半球3例、右侧2例。病变侧侧脑室均表现为明显扩大,其中1例以侧脑室后角扩大为主,2例表现为侧脑室前角伸直,指向前上方。3例病变侧脑白质内出现斑片状T2WI高信号。结论:HME的MRI表现具有特征性,表现为一侧大脑半球弥漫性增大,侧脑室扩大,脑皮质增厚,脑白质内可见长T2异常信号。
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关键词 神经系统畸形磁共振成像    
AbstractObjective: To investigate the clinical and MRI features of hemimegalencephaly(HME). Methods: The clinical and MRI findings of 5 cases with HME were reviewed retrospectively, at the same time literature review was carried out. Results: Epilepsy was found in 3 cases, hemi-hypertrophy of extremities found in 3 cases, mental retardation in 2 cases. MRI showed enlargement of the affected cerebral hemisphere, apparent thickening of the grey matter, and poor grey/white differentiation in all 5 cases. Involvement of left cerebral hemisphere in 3 cases and right hemisphere 2 cases. Enlargement of the ipsilateral ventricle was seen in all cases, straightening of the forntal horn of the ipsilateral ventricle in 2 cases and colpocephaly in 1 case. Three cases had hyper-intensity in the white matter of the affected hemisphere. Conclusion: There is characteristic MRI features of HME. MRI demonstrates diffuse hypertrophy of the affected hemisphere, ipsilateral ventricle dilatation, thickened cortex, and hyperintensity of the white matter.
Key wordsNervous system malformations    Magnetic resonance imaging
收稿日期: 2014-02-21     
:  R745.1  
  R445.2  
通讯作者: 任庆云   
引用本文:   
刘 斋;何 丽;王伟秀;任庆云. 半侧巨脑畸形的临床及MRI诊断(附5例报告并文献复习)[J]. , 2014, 25(8): 538-541.
LIU Zhai;HE Li;WANG Wei-xiu;REN Qing-yun. Clinical and MRI diagnosis of hemimegalencephaly: report of 5 cases with literature review. , 2014, 25(8): 538-541.
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