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The Korean Journal of Pathology 1985;19(2): 226-230.
Sturge-Weber Syndrome: Report of an incomplete form.
Hye Yeon Kim, Kap No Lee, Seung Young Paik, Ki Chan Lee, Chang Soo Lim, Suck Ho Nam, Je G Chi
1Department of Clinical Pathology, College of Medicine, Korea University, Seoul, Korea.
2Department of Neurosurgery, College of Medicine, Korea University, Seoul, Korea.
3Department of Pathology, College of Medicine, Seoul National University, Seoul, Korea.
The Sturge Weber syndrome is an uncommon disorder characterized by port-wine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated with this syndrome are mental retardation, choroidal angioma, buphthalmus or glaucoma, seizure and hemiplegia. We studied a case of incomplete form of Sturage Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calcification and leptomeningeal angiomatosis. There are no characteristic facial nevus, mental retardation, occular changes and hemiplegia.
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