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目的探讨结节性硬化症(TSC)在6个月以下小婴儿及新生儿脑内病变MRI表现的特点。资料与方法回顾性分析14例6个月以下小婴儿及新生儿TSC的MRI脑内病变的表现特点。结果14例均发现室管膜下结节,共161个,范围1～22个/例,均位于侧脑室,室间孔和前角者86个,占53.4%,大多数表现为T1WI呈高信号,T2WI呈低信号。13例发现脑白质异常信号,表现为线样、点状T1WI高信号,T2WI低信号,线样病变长轴与侧脑室壁垂直。10例发现皮质结节,共116个,范围1～21个/例,T1WI呈高信号者占91.4%。T2WI呈低信号者占75.2%。14例中7例共发现8处经套膜发育不良,7处累及额叶,1处累及额、顶、颞、岛叶。5处病灶信号均匀,T1WI上呈高信号,T2WI上呈低信号。3处病灶呈高低混杂信号。室管膜下巨细胞瘤1例,位于室间孔区,直径14mm,T1WI呈高信号,T2WI呈低信号。结论小婴儿及新生儿TSC脑内病变MRI信号和形态均与年长儿和成人有所不同,认识其MRI特征对TSC的早期诊断至关重要。
Objective To investigate the MRI findings of tuberous sclerosis (TSC) in infants and newborn infants younger than 6 months. Materials and Methods Retrospective analysis of MRI features of brain lesions in 14 infants younger than 6 months and neonates with TSC. Results All the subventricular nodules were found in 14 cases. There were 161 nodules, ranging from 1 to 22 in each case, with 86 (53.4%) located in lateral ventricle, interventricular foramen and anterior horn, most of which showed high T1WI Signal, T2WI showed a low signal. Thirteen patients found abnormal signals of white matter, showing linear, punctate T1WI high signal and T2WI low signal. The long axis of linear lesion was perpendicular to lateral ventricular wall. A total of 116 cortical nodules were found in 10 cases, ranging from 1 to 21 cases / case, with 91.4% of patients presenting with high signal intensity on T1WI. T2WI was low signal accounted for 75.2%. Of the 14 cases, 8 were found to have dysplasia of the mantle, 7 of them had frontal lobe involvement, and 1 involved the frontal, parietal, temporal and insula. 5 lesions signal uniformity, T1WI was high signal, T2WI was low signal. 3 lesions were mixed signal level. Subependymoma giant cell tumor in 1 case, located in the intercavity area, diameter 14mm, T1WI was high signal, T2WI showed low signal. Conclusion MRI signals and morphology of brain lesions in young infants and neonates with TSC are different from those in older children and adults. It is of great importance to recognize the MRI features in early diagnosis of TSC.