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Journal of Korean Neurosurgical Society 1985;14(2): 451-456.
A Case of Incomplete form cf Sturge-Weber Syndrome.
Suck Ho Nam, Chang Soo Rim, Ki Chan Lee, Hye Yeon Kim, Je G Chi
1Department of Neurosurgery, College of Medicine, Korea University, Korea.
2Department of Clinical Pathology, College of Medicine, Korea University, Korea.
3Department of Pathology, College of Medicine, Seoul National University, Korea.
ABSTRACT
Sturge-Weber syndrome is a neurocutaneous syndrome characterized by portwine nevus of the face with angiomatosis of the ipsilateral cerebral leptomeninges and extensive calcification in the underlying cerebral cortex. Associated features include mental retardation, generalized or focal seizures, hemiparesis, choroidal angioma, & buphthalmos or glaucoma. We experienced a case of incomplete form of Sturge-Weber syndrome in a 12 year-old boy, who had generalized seizure, a homonymous hemianopsia, intracranial calficication and leptomeningeal angiomatosis without facial nevus or mental retardation. So we presented a case with a brief review of related literatures.
Key Words: Struge-Weber syndrome; Incomplete form; Seizure
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