Résumé
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.
Sujets)
Enfant , Humains , Mâle , Angiomatose , Cortex cérébral , Choroïde , Glaucome , Hémangiome , Hémianopsie , Hydrophtalmie , Déficience intellectuelle , Syndromes neurocutanés , Naevus , Parésie , Crises épileptiques , Syndrome de Sturge-WeberRésumé
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.