Ergün R, Okten A I, Gezercan Y, Gezici A R
Department of Neurosurgery, Abant Izzet Baysal University, Bolu, Turkey.
Acta Neurochir (Wien). 2007 Aug;149(8):829-30; discussion 830. doi: 10.1007/s00701-007-1224-z. Epub 2007 Aug 1.
Sturge-Weber syndrome is one of the neurocutaneous syndromes. It is a rare, nonfamiliar disease that is characterized by facial port-wine stain, leptomeningeal angiomatosis, choroidal angioma, buphthalmos, intracranial calcification, cerebral atrophy, mental retardation, glaucoma, seizures and hemiparesis. CT and MR are complementary in the evaluation of this disease. Epilepsy is an essential feature of Sturge-Weber syndrome and it has a major significance for prognosis and treatment. We report a 2-year-old boy with Sturge-Weber syndrome who had in addition an intracranial lipoma, a temporal arachnoid cyst and a porencephalic cyst. This combination of intracranial lesions with Sturge-Weber syndrome has not been previously reported.
斯特奇-韦伯综合征是一种神经皮肤综合征。它是一种罕见的非家族性疾病，其特征为面部葡萄酒色斑、软脑膜血管瘤病、脉络膜血管瘤、牛眼、颅内钙化、脑萎缩、智力迟钝、青光眼、癫痫发作和偏瘫。CT和MR在该疾病的评估中具有互补性。癫痫是斯特奇-韦伯综合征的一个基本特征，对预后和治疗具有重要意义。我们报告一名患有斯特奇-韦伯综合征的2岁男孩，其还伴有颅内脂肪瘤、颞部蛛网膜囊肿和脑穿通畸形囊肿。这种颅内病变与斯特奇-韦伯综合征的组合此前尚未见报道。
