半侧巨脑畸形临床及磁共振成像特点分析

发布时间：2018-10-10 13:25

【摘要】：目的分析半侧巨脑畸形(HME)的临床及磁共振成像(MRI)特点,以提高对HME的认识。方法回顾性分析2008—2012年河北医科大学第一医院收治的HME患者的临床及MRI表现。结果 6例HME临床表现为癫痫4例,偏侧肢体肥大3例,精神运动发育迟滞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4例,右侧大脑半球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T2加权像(T2WI)高信号。1例患儿行弥散张量成像(DTI)检查,显示通过胼胝体膝部及压部的半球间脑白质纤维束在双侧额叶及枕叶分布不对称。结论 HME典型的临床表现为癫痫及精神运动发育迟滞;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 analyze the clinical features and magnetic resonance imaging (MRI) features of hemiencephaly (HME) in order to improve the understanding of HME. Methods the clinical and MRI manifestations of HME patients admitted in the first Hospital of Hebei Medical University from 2008 to 2012 were analyzed retrospectively. Results the clinical manifestations of HME were epilepsy in 4 cases, hypertrophy of hemilimb in 3 cases, mental and motor retardation in 2 cases. MRI plain scan showed enlargement of unilateral cerebral hemisphere, thickening of cerebral cortex and blur of gray and white matter junction. The lesions were located in the left hemisphere in 4 cases and in the right hemisphere in 2 cases. The lateral ventricle of the lesion showed obvious enlargement, in which 1 case showed the enlargement of the posterior horn of the lateral ventricle, 2 cases showed the extension of the anterior horn of the lateral ventricle, and the other 2 cases showed the extension of the anterior horn of the lateral ventricle. High signal intensity was found in 3 cases of lesions in the white matter of white matter. 1 case was examined by diffusion-Zhang Liang (DTI), and 1 case was examined by diffusion-enhanced Zhang Liang imaging, and 1 case had high signal intensity on T 2 weighted imaging (T2WI) in the white matter. The distribution of hemispheric diencephalic white matter bundles across the genu and superior parts of the corpus callosum was asymmetrical in the bilateral frontal and occipital lobes. Conclusion the typical clinical manifestations of HME are epilepsy and psychomotor retardation, and MRI is characterized by diffuse enlargement of unilateral cerebral hemisphere, enlargement of lateral ventricle, thickening of cerebral cortex and abnormal signal intensity of long T2 in white matter.
【作者单位】：河北医科大学第一医院放射科;
【分类号】：R445.2;R741

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