Chaudhary S C, Sonkar S K, Kumar Vivek, Golchha Sandeep
Department of Medicine, C.S.M. Medical University, Lucknow, Uttar Pradesh.
J Assoc Physicians India. 2011 May;59:327-9.
Sturge Weber Syndrome also called as encephalotrigeminal angiomatosis is a sporadically occurring neurocutaneous syndrome, characterized by vascular malformation with capillary venous angiomas that involve face, choroid of eye and leptomeninges with resulting neurological and orbital manifestations. We hereby report a young unmarried girl who diagnosed as a case of SWS on the basis of Port wine stain since birth, past history of seizures since the age of four years and at this time presented with status epilepticus, mental retardation (I.Q.--30 to 35), EEG abnormality and characteristic imaging findings. This may be a common condition for paediatrician and neurologist but as a physician we usually do not see it that often.
斯特奇-韦伯综合征也称为脑三叉神经血管瘤病，是一种散发性神经皮肤综合征，其特征为伴有毛细血管静脉血管瘤的血管畸形，累及面部、脉络膜和软脑膜，从而导致神经和眼眶表现。我们在此报告一名年轻未婚女孩，她自出生就有葡萄酒色斑，4岁起有癫痫发作史，此次因癫痫持续状态、智力发育迟缓（智商30至35）、脑电图异常及特征性影像学表现而被诊断为斯特奇-韦伯综合征。这对儿科医生和神经科医生来说可能是一种常见病症，但作为内科医生，我们通常并不经常见到。
