Kumar V, Prasad B K
Dept. of Pediatrics, National Medical College & Teaching Hospital, Birgunj, Nepal.
Kathmandu Univ Med J (KUMJ). 2004 Oct-Dec;2(4):372-4.
Sturge - Weber syndrome (SWS) is a rare disorder that occur with a frequency of approximately 1 per 50,0001. It isa neurocutaneous syndrome, characterized by a facial vascular birthmark and neurological abnormalities. The hallmark is intracranial vascular angioma, most often involving the occipital and posterior parietal lobes, but it can also affect the other cortical regions. An ipsilateral facial cutaneous vascular malformation (port wine nevus)usually affects the upper face. Other clinical findings associated with SWS are seizures, glaucoma, hemiparesis,mental retardation and delayed developmental milestones. This article reports a case of 8 years old boy who presented with weakness of right half of body since birth, mental retardation and delayed developmental milestones. Clinical examination revealed deep purple nevus on left lower face, and less power of left upper & lower limbs.X-ray skull showed calcification. C.T. Scan of brain revealed curvilinear calcification with focal atrophy.
斯特奇-韦伯综合征（SWS）是一种罕见疾病，发病率约为每50000人中有1例。它是一种神经皮肤综合征，其特征为面部血管性胎记和神经学异常。其标志是颅内血管性血管瘤，最常累及枕叶和顶叶后部，但也可影响其他皮质区域。同侧面部皮肤血管畸形（葡萄酒色斑痣）通常累及上半面部。与SWS相关的其他临床发现包括癫痫、青光眼、偏瘫、智力迟钝和发育里程碑延迟。本文报告一例8岁男孩，自出生以来出现右半身无力、智力迟钝和发育里程碑延迟。临床检查发现左下半面部有深紫色痣，左上肢和下肢肌力减弱。头颅X线显示有钙化。脑部CT扫描显示曲线状钙化伴局灶性萎缩。
