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Journal: University Journal of Medicine and Medical Specialities (Vol.3, No. 5)

Publication Date:

Authors : ;

Page : 75-77

Keywords : Neurocutaneous syndrome; Phakomatoses; Meningofacial angiomatosis;

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The Sturge-Weber syndrome, also called encephalotrigeminal angiomatosis, is a neurocutaneous disorder with angiomas involving the leptomeninges and skin of the face and often of the eye. The skin lesion is typically an ipsilateral facial nevus (port-wine stain). The constellation of symptoms and signs also include intracranial calcifications, contralateral hemiparesis, hemiatrophy and homonymous hemianopia, seizures, glaucoma and, in many cases, mental retardation. We hereby report a 12-year-old boy, who was diagnosed as a case of Sturge - Weber syndrome on the basis of long-standing seizures, port-wine nevus on the right side of the face along the distribution of trigeminal nerve, right eye glaucoma, left sided hemiatrophy with spastic hemiparesis, mental retardation, EEG abnormality and characteristic imaging findings.'

Last modified: 2017-11-06 15:52:42