Sturge-Weber syndrome accompanied with multiple congenital intracranial lesions.
Sturge-Weber syndrome is one of the neurocutaneous syndromes. It is a rare, nonfamiliar disease that is characterized by facial port-wine stain, leptomeningeal angiomatosis, choroidal angioma, buphthalmos, intracranial calcification, cerebral atrophy, mental retardation, glaucoma, seizures and hemiparesis. CT and MR are complementary in the evaluation of this disease. Epilepsy is an essential feature of Sturge-Weber syndrome and it has a major significance for prognosis and treatment. We report a 2-year-old boy with Sturge-Weber syndrome who had in addition an intracranial lipoma, a temporal arachnoid cyst and a porencephalic cyst. This combination of intracranial lesions with Sturge-Weber syndrome has not been previously reported.
Department of Neurosurgery, Abant Izzet Baysal University, Bolu, Turkey. ruchanergun@hotmail.com
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D000015:Abnormalities, Multiple / Q000175:diagnosis*; D000927:Anticonvulsants / Q000627:therapeutic use; D016080:Arachnoid Cysts / Q000151:congenital* / Q000175:diagnosis; D001927:Brain Diseases / Q000151:congenital* / Q000175:diagnosis; D002114:Calcinosis / Q000151:congenital* / Q000175:diagnosis; D020863:Central Nervous System Cysts / Q000151:congenital* / Q000175:diagnosis; D020787:Central Nervous System Venous Angioma / Q000175:diagnosis; D002675:Child, Preschool; D004829:Epilepsy, Generalized / Q000188:drug therapy / Q000209:etiology*; D005500:Follow-Up Studies; D006801:Humans; D008067:Lipoma / Q000151:congenital* / Q000175:diagnosis; D008279:Magnetic Resonance Imaging; D008297:Male; D009778:Occipital Lobe / Q000002:abnormalities* / Q000473:pathology; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D013702:Temporal Lobe / Q000002:abnormalities* / Q000473:pathology; D014057:Tomography, X-Ray Computed
D000927:Anticonvulsants