Fischbein N J, Barkovich A J, Wu Y, Berg B O
Neuroradiology. 1998 Mar;40(3):177-80. doi: 10.1007/s002340050563.
Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by a facial nevus flammeus associated with seizures, developmental delay, and, often, with hemiparesis and hemianopia. On MRI, the most characteristic finding has been reported to be leptomeningeal enhancement, believed to represent leakage of contrast medium through the anomalous pial vessels that characterize the disease. We present a case of SWS with no evidence of leptomeningeal enhancement. This case illustrates that leptomeningeal enhancement need not be present in SWS, and the absence of this characteristic finding does not preclude the diagnosis.
斯特奇-韦伯综合征（SWS）是一种神经皮肤综合征，其特征为面部葡萄酒色斑，常伴有癫痫发作、发育迟缓，还常常伴有偏瘫和偏盲。据报道，在磁共振成像（MRI）上，最具特征性的表现是软脑膜强化，据信这代表造影剂通过该病特有的异常软膜血管渗漏。我们报告一例无软脑膜强化证据的SWS病例。该病例表明，SWS不一定会出现软脑膜强化，缺乏这一特征性表现并不排除诊断。
