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目的探讨结节性硬化症(TSC)的磁共振影像表现。方法收集2002年3月至2005年4月间临床证实的结节性硬化症14例,全部病例均进行MR检查,其中3例进行增强检查。不合作儿童检查前采用6.5%水合氯醛保留灌肠镇静睡眠。结果全部病例均显示大小不等(1～5mm)的室管膜下结节。皮层或皮层下结节10例,表现为脑回核心样病灶和H型病灶。白质异常信号6例,分别呈线状、楔形、不规则形。室管膜下巨细胞星形细胞瘤2例,肾血管平滑肌脂肪瘤1例。结论MRI对TSC诊断的敏感性高,是首选的影像学检查方法。室管膜下结节是TSC最重要的MR影像学表现之一,以T1加权成像(T1WI)及梯度回波序列显示效果最佳。T2加权成像(T2WI)及液体衰减反转恢复序列(FLAIR)对TSC大脑皮层或皮层下区结节、脑白质区病灶的显示优于T1WI。
Objective To investigate the magnetic resonance imaging of tuberous sclerosis (TSC). Methods Totally 14 cases of tuberous sclerosis clinically confirmed from March 2002 to April 2005 were collected. All the cases were examined by MR, of which 3 cases were examined by enhancement. Before uncooperative children check with 6.5% chloral hydrate retention enema calm sleep. Results All cases showed subependymal nodules of various sizes (1-5 mm). Cortical or subcortical nodules in 10 cases, manifested as retroccipital heart-like lesions and H-shaped lesions. Six cases of white matter abnormal signal were linear, wedge-shaped, irregular shape. Subependymal giant cell astrocytoma in 2 cases, 1 case of renal angiomyolipoma. Conclusion MRI is highly sensitive to the diagnosis of TSC and is the preferred imaging method. Subependymal nodules are one of the most important manifestations of MR imaging in TSC. T1 weighted imaging (T1WI) and gradient echocardiography show the best results. T2 weighted imaging (T2WI) and liquid attenuated inversion recovery sequence (FLAIR) showed superiority to T1WI in nodular and white matter lesions of cerebral cortex or subcortical TSC in TSC.