Clinical and radiological features in Sturge-Weber syndrome: an analysis of 8 cases

VernacularTitle:Sturge-Weber综合征的临床及影像学特点分析(附八例报道)
Author: Jie WU ¹ ; Fang HUA ; Xiaohua WANG ; Yanan WANG ; Xiaoqian LIANG ; Hao CHEN
Author Information

1. 徐州医科大学研究生院
Keywords: Sturge-Weber syndrome; Glaucoma; Epilepsy
From: Chinese Journal of Neuromedicine 2016;15(10):1026-1031
CountryChina
Language:Chinese
Abstract: Objective To explore the clinical features and radiological findings of patients with Sturge-Weber syndrome (SWS).Methods A retrospective study was performed on the course of illness,clinical data and neuroimages of 8 patients with SWS,admitted to our hospital from June 2012 to November 2015.Results The main symptoms included facial hemangioma in 7 patients,epilepsy in 5 patients,headache in 4 patients,limb hemiplegia in 3 patients,glaucoma in 3 patients,and thyroid dysfunction in one patient.Neurological imaging showed subcortical calcification,brain atrophy,gyriform enhancement and increased pial vascular tortuosity.Fundus examination revealed optic atrophy and vascular malformations of choroid.All symptoms in the patients were alleviated after treatment.Conclusions SWS should be highly suspected in young patients who present with typical glaucoma,epilepsy or hemiplegia with/without facial hemangioma.CT angiography or MR imaging (especially susceptibility-weighted imaging) is required to confirm the diagnosis.If medications fail to control seizures,surgery could be considered in some patients.