成年人无颜面部血管瘤斯特奇-韦伯综合征一例

Adult Sturge-Weber syndrome without facial hemangioma:report of one case

斯特奇-韦伯综合征（Sturge-Weber syndrome,SWS）以一侧面部三叉神经分布区血管瘤、癫痫、智能减退、对侧偏瘫及青光眼为主要临床特征.本文报道一例成年首次发病的无颜面部血管瘤的SWS（SWSⅢ型）病例.37岁出现偏头痛后反复发作,47岁时首次癫痫发作,随后出现失语、左上肢软瘫;头颅CT显示右侧顶枕部钙化,CT血管成像显示右侧颞叶及枕叶血管畸形,MRI显示右侧大脑软脑膜强化明显.采用药物控制癫痫发作,其他症状也改善.

Sturge-Weber syndrome （SWS） is characterized by angiomas affecting the ophthalmic division of the trigeminal nerve, epilepsy, intellectual impairment, hemiplegia and glaucoma. We report a patient who developed SWS without facial hemangioma （SWS type Ⅲ） in his adulthood. The patient presented with repeated episodes of headache since age 37 year. He manifested first attack of seizure at the age 47 year followed by aphasia and right upper limb palsy. Brain CT scan revealed fight parietal-occipital calcification, brain CT angiography showed right temporal lobe and occipital lobe vascular malformation, and MRI showed leptomeningeal enhancement in the riht cerebral piamater. The seizure was controlled with antiepileptic drugs and reviewed in routine follow up.