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; 何丽; 王伟秀; 任庆云

中国临床医学影像杂志 ›› 2014, Vol. 25 ›› Issue (8) : 538-541.

论著

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

作者信息 +

Clinical and MRI diagnosis of hemimegalencephaly: report of 5 cases with literature review

LIU Zhai1, HE Li1, WANG Wei-xiu2, REN Qing-yun1

Author information +

文章历史 +

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。

Abstract

Objective: 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.

导出引用

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]. 中国临床医学影像杂志. 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[J]. Journal of China Clinic Medical Imaging. 2014, 25(8): 538-541

中图分类号： Ｒ745.1 Ｒ445.2