Extractions: A congenital syndrome characterized by a port-wine nevus covering portions of the face and cranium (in the distribution of the ophthalmic division of the TRIGEMINAL NERVE) and angiomas of the meninges and choroid. Clinical manifestations include the onset of focal SEIZURES, progressive hemiparesis, GLAUCOMA, hemianopsia, and cognitive deficits in the first decade of life. By age two years, skull radiographs reveal "tramline calcifications" of the margins of the occipital and parietal lobes. Pathologically cortical neurons are replaced by glial tissue that undergoes calcification. (From Adams et al., Principles of Neurology, 6th ed, pp1018-9) Synonyms and Source Vocabularies:
Entrez PubMed The case of a 21year-old woman who was affected by both encephalofacialangiomatosis (Sturge-Weber http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=3
Log In Problems SturgeWeber syndrome, also known as encephalofacial/encephalotrigeminalangiomatosis, is characterized by a facial port wine nevus and leptomeningeal http://www.medscape.com/viewarticle/438132_print
Extractions: Sturge-Weber Syndrome Definition: A congenital syndrome characterized by a port-wine nevus covering portions of the face and cranium (in the distribution of the ophthalmic division of the TRIGEMINAL NERVE ) and angiomas of the meninges and choroid. Clinical manifestations include the onset of focal SEIZURES , progressive hemiparesis, GLAUCOMA , hemianopsia, and cognitive deficits in the first decade of life. By age two years, skull radiographs reveal "tramline calcifications" of the margins of the occipital and parietal lobes. Pathologically cortical neurons are replaced by glial tissue that undergoes calcification. (From Adams et al., Principles of Neurology, 6th ed, pp1018-9)
Portal Toolkit Invalid Site URL Novotny EJ, Urich H. The coincidence of neurocutaneous melanosis and encephalofacialangiomatosis. Clin. Neuropathol. 1986; 5 24651. http://ppv.ovid.com/pt/re/aujd/fulltext.00000936-200105000-00011.htm
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