Sturge-Weber综合征临床分析及影像诊断

Clinical and imaging features of Sturge-Weber syndrome

目的探讨Sturge-Weber综合征的临床特点及CT、MRI表现及诊断。方法归纳8例Sturge-Weber综合征患者的临床表现、影像学特点,进行回顾性分析,并复习相关文献。结果 8例均有脑内软脑膜血管瘤,其中3例伴有颜面部血管瘤,均位于三叉神经眼支分布区(均累及单侧);7例表现为癫痫发作,其中5例伴有头痛,1例伴有头晕及一侧肢体乏力;1例表现为单纯反复头痛;均无智力低下或青光眼。影像学检查可清晰显示脑萎缩、脑回样钙化、软脑膜广泛强化伴静脉畸形。结论 Sturge-Weber综合征是一种少见疾病,根据典型临床特点及CT、MRI等影像学检查有助于确诊。

Objective To determine the clinical and imaging features of Sturge-Weber syndrome （SWS）. Methods The clinical and imaging features of 8 patients with Sturge-Weber syndrome were reviewed. Results Leptomeningeal angiomatosis was found in all patients,accompanied by unilateral facial hemangioma （3） in the distribution of the ophthalmic division of the trigeminal nerve. Of 7 patients with epilepsy , 5 were accompanied by headache , 1 by dizziness and unilateral muscle weakness , 1 by recurring simple headache. There was no mental retardation or glaucoma in any of the patients...CT and MRI clearly showed brain atrophy, gyriform calcification, diffuse enhancement of pia mater accompanied by cerebral venous malformation. Conclusion Sturge-Weber syndrome has typical clinical and imaging features on CT and MRI for diagnosis.