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。
Purpose To explore brain MRI features in newborn infants with incontinentia pigmenti, and to enhance the understanding and diagnosis level of this disease. Materials and Methods A retrospective analysis on MRI data of 13 neonates with incontinentia pigmenti admitted to Hunan Children＇s Hospital from January 2009 to December 2016 and further confirmed by clinical, pathology and gene diagnosis were conducted. Such conventional MRI sequences as T1WI and T2WI as well as DWI and SWI sequences were incorporated. Results Five out of 13 newborn infants with incontinentia pigmenti were abnormal in the MRI features. In the conventional sequence, slightly short T1 signals in bilateral frontal lobe and right parietal occipital cortex were identified in 3 cases, right basal ganglia hemorrhage in 1 case. Slightly short T1 and long T2 signals in bilateral basal ganglia were observed in 1 case; 1 case of subarachnoid hemorrhage and 1 case of bilateral lateral ventriculomegaly were noticed as well. The lesion sites of 4 cases were observed high density signal in DWI findings, three of which were widely distributed in abnormal signals. Lobes deep in bilateral cerebral hemispheres and subcortical white matter, bilateral basal ganglia, thalamus, internal capsule, corpus callosum, brain stem and cerebellum were the most affected areas. DWI could detect lesion area earlier and more accurate than conventional sequences. Conclusion Incontinentia pigmenti among newborn infants demonstrates a high incidence. MRI examination should be adopted the moment neurological symptoms occur. Incontinentia pigmenti is relatively distinct in DWI findings, and hence is helpful for early detection of lesions and evaluation of clinical prognosis.
出处
《中国医学影像学杂志》
CSCD
北大核心
2017年第9期651-654,共4页
Chinese Journal of Medical Imaging
关键词
色素沉着异常
中枢神经系统
磁共振成像
婴儿
新生
Pigmentation disorders
Central nervous system
Magnetic resonance imaging
Infant, newborn
