儿童线粒体脑肌病中枢神经系统CT与MRI表现

发布时间：2018-12-06 12:27

【摘要】：目的探讨儿童线粒体脑肌病(ME)中枢神经系统CT与MRI表现特点。方法搜集32例经临床和病理证实的ME患儿CT和MRI资料,分析其中枢神经系统影像学表现。结果 32例中,脑内共48个病灶,45个位于基底节区、丘脑和脑干,颞、顶、枕叶皮层及皮层下白质和小脑半球。病变分布呈多发性和游走性。CT表现为尾状核、豆状核和大脑半球点片状钙化,脑实质呈低密度灶。MRI表现为大片长T1、长T2信号病灶,DWI可以鉴别脑水肿类型。1H-MRS病变区出现异常乳酸峰。静脉增强扫描脑内病灶均无强化。MRA或MRV可表现为对应病变区域的动脉或静脉的狭窄或消失。脊髓灰质出现对称性片状长T2信号灶或脊髓变细。结论 ME在CT及MRI图像上具有一定的特征性,主要表现为脑和脊髓的片状异常密度或信号,结合DWI及MRS表现和相关临床资料,可以对ME做出正确诊断。
[Abstract]:Objective to investigate the CT and MRI features of (ME) in children with mitochondrial encephalomyopathy. Methods the data of CT and MRI in 32 children with ME confirmed by clinic and pathology were collected and the imaging findings of central nervous system were analyzed. Results there were 48 lesions in the brain, 45 of which were located in basal ganglia, thalamus and brainstem, temporal, parietal, occipital cortex, subcortical white matter and cerebellar hemisphere. The lesions were multiple and mobile. CT showed calcification of the caudate nucleus, lentiform nucleus and cerebral hemispheres, and low density of cerebral parenchyma. MRI showed a large lesion of long T _ 1 and long T _ 2 signal intensity. DWI can distinguish the type of brain edema. Abnormal lactate peak appears in the lesion area of 1H-MRS. No enhancement was found in the cerebral lesions on intravenous contrast enhanced scan. MRA or MRV showed stenosis or disappearance of the artery or vein corresponding to the lesion area. Symmetrical lamellar long T 2 signal foci appeared in the gray matter of the spinal cord or the spinal cord became thinner. Conclusion ME has some characteristics on CT and MRI images, mainly manifested as flaky abnormal density or signal of brain and spinal cord. Combined with DWI and MRS findings and related clinical data, ME can be correctly diagnosed.
【作者单位】：首都医科大学附属北京儿童医院影像中心;
【基金】：首都卫生发展科研专项(编号:首发2011-2009-03)
【分类号】：R748;R816.92;R445.2

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