title: Spontaneous obliteration of MRI-silent cerebral angiomatosis revealed by CT angiography in a patient with Sturge-Weber syndrome.
abstract: Type I Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by facial port wine stain and ipsilateral leptomeningeal angiomatosis. It is commonly complicated by glaucoma and neurological disturbances including seizures, hemiparesis, transient stroke-like deficits, and behavioral problems. Gadolinium-enhanced MRI is the study of choice for demonstrating the leptomeningeal angiomatosis, and is particularly useful for making the diagnosis. Cerebral hematoma and spontaneous obliteration of cerebral angiomatosis have never been reported in SWS. We describe a patient with type I SWS in which a hemorrhage in the left temporal lobe was caused by an underlying angiomatosis. No arteriovenous malformations were detected by brain CT or gadolinium-enhanced MRI, whereas a small angiomatous nidus draining into an ectasic venous collector near the hematoma was disclosed by brain CT angiography. Carotid angiography, performed 3 years after the hemorrhage, demonstrated the spontaneous obliteration of the angioma. This description expands the phenotypic spectrum of type I SWS in which the occurrence of cerebral hemorrhage and MRI-silent cerebral angiomatosis should be included, and emphasizes the need of performing a brain CT angiography in SWS patients whose routine neuroimaging studies fail to detect potentially harmful vessel malformation.
authors.affiliation: Magna Graecia University of Catanzaro, Regional Epilepsy Center, Azienda Ospedaliera Bianchi Melacrino Morelli, Reggio Cal., Italy. u.aguglia@tin.it
authors.forename: Umberto
authors.initials: U
authors.lastname: Aguglia
authors.forename: Maria Adele
authors.initials: MA
authors.lastname: Latella
authors.forename: Franco
authors.initials: F
authors.lastname: Cafarelli
authors.forename: Emilio
authors.initials: E
authors.lastname: Le Piane
authors.forename: Antonio
authors.initials: A
authors.lastname: Gangemi
authors.forename: Angelo
authors.initials: A
authors.lastname: Labate
authors.forename: Antonio
authors.initials: A
authors.lastname: Gambardella
authors.forename: Aldo
authors.initials: A
authors.lastname: Quattrone
mesh_terms: D000328:Adult; D002533:Cerebral Angiography; D002536:Cerebral Arteries / Q000002:abnormalities* / Q000000981:diagnostic imaging* / Q000503:physiopathology; D002543:Cerebral Hemorrhage / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D002550:Cerebral Veins / Q000002:abnormalities / Q000000981:diagnostic imaging / Q000503:physiopathology; D005260:Female; D006801:Humans; D002538:Intracranial Arteriovenous Malformations / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D012075:Remission, Spontaneous; D012640:Seizures / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D013341:Sturge-Weber Syndrome / Q000150:complications*; D013702:Temporal Lobe / Q000098:blood supply / Q000000981:diagnostic imaging / Q000473:pathology; D014057:Tomography, X-Ray Computed