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Case No. 187
0000-00-00~0000-00-00
출제자 : 관리자
Hit : 366
| Period | 0000-00-00~0000-00-00 |
|---|---|
| Diagnosis | |
| Clinical information | 28세 여자. 태어날 때부터 왼쪽 얼굴에 자주빛 반점이 있음. 진단은? |
| Discussion | Sturge-Weber syndrome Sturge-Weber syndrome (SWS) is a neurocutaneous disorder characterized by angiomatosis of the skin, eye, and meninges. It occurs sporadically affecting both sexes equally, but familial cases are reported. The expression of the syndrome is variable but typically manifests as facial port-wine stain, ocular choroidal hemangiomas, and cerebral pial angiomatosis. Glaucoma and neurologic complications, including seizures and developmental delay, are common. The major pathologic abnormality in SWS is a meningeal angioma in the pia mater, possibly due to persistent primordial sinusoidal vascular channels. This leads to a lack of superficial cortical venous drainage resulting in chronic venous hypertension and ischemia. MR findings include serpentine leptomeningeal enhancement, pial angiomatosis of subarachnoid space. Cortical and subcortical gyriform calcification is seen on CT, most often occipital, parietal, frontal and temporal lobes. Enlargement and calcification of the choroid plexus on the attected side is well documented. Cranial asymmetry can occure due to hemiatrophy. References 1. C. Juhasz E.M. et al. Multimodality Imaging of Cortical and White Matter Abnormalities in Sturge-Weber Syndrome. AJNR Am. J. Neuroradiol. 2007 May;28:900-6. 2. Herron J, Darrah R, Quaghebeur G. Intra-cranial manifestations of the neurocutaneous syndromes. Clin Radiol. 2000 Feb;55(2):82-98. |
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