新生儿色素失禁症的脑部MRI表现

发布时间：2018-07-24 20:46

【摘要】：目的探讨新生儿色素失禁症的脑部MRI表现,提高对本病的认识和诊断水平。资料与方法回顾性分析2009年1月-2016年12月湖南省儿童医院收治,并经临床、病理及基因证实的13例新生儿色素失禁症的MRI特征,包括T1WI、T2WI常规序列及扩散加权成像(DWI)、磁敏感加权成像序列。结果 13例患儿中,5例脑部MRI表现异常。常规序列中,双侧额叶、右侧顶枕叶皮层少许短T1信号3例,右侧基底节出血1例,双侧基底节少许稍短T1长T2信号1例,蛛网膜下腔出血、双侧脑室扩张各1例。DWI出现高信号病灶4例,其中3例异常信号呈广泛性分布,受累部位主要包括双侧大脑半球各叶深部及皮层下白质、双侧基底节、丘脑、内囊、胼胝体、脑干、小脑。DWI较常规序列可更早、更敏感地显示病变范围。结论新生儿色素失禁症脑部病变发病率高,当出现神经系统症状时应尽早行MRI检查。本病DWI具有一定的特征性,有助于病变的早期发现及临床预后评估。
[Abstract]:Objective to explore the MRI findings of neonatal pigmentary incontinence and to improve the level of understanding and diagnosis of this disease. Data and Methods Retrospective analysis of the MRI characteristics of 13 cases of neonatal pigmentary incontinence admitted in Hunan Children's Hospital in December, -2016, January 2009, which were confirmed by clinical, pathological and gene, including T1WI, T2WI routine sequence and diffusion weighting. Imaging (DWI), magnetic sensitive weighted imaging sequence. Results of 13 children, 5 cases of brain MRI showed abnormal performance. In the routine sequence, 3 cases of bilateral frontal lobes, right occipital cortex a little short T1 signal, right basal ganglia hemorrhage 1 cases, bilateral basal ganglia slightly short T1 long T2 signal 1 cases, arachnoid hemorrhage, bilateral ventricle dilatation each 1 cases.DWI high signal In 4 cases, 3 cases of abnormal signals were widely distributed, including the deep and subcortical white matter in the bilateral cerebral hemispheres, bilateral basal ganglia, thalamus, internal capsule, corpus callosum, brain stem, and cerebellar.DWI, which were earlier and more sensitive to the lesion range. Conclusion the incidence of brain lesions in neonatal pigmented incontinence is high. MRI should be performed as early as possible in the presence of nervous system symptoms. DWI is characteristic of this disease and is helpful for early detection and prognosis evaluation.
【作者单位】：湖南省儿童医院放射科;
【分类号】：R445.2;R722.1

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