title: MR-imaging findings in children with Sturge-Weber syndrome.
abstract: Intracranial extent and distribution of leptomeningeal angiomatosis, visualized by magnetic resonance imaging (MRI) with Gadolinium-DTPA (Gd-DTPA) enhancement, is demonstrated in four children with Sturge-Weber syndrome (SWS). Aged 7, 9, 11 and 19 months, they presented with cutaneous, neurologic and ocular symptoms at the time of MRI examination. Angiomatous alteration of the skull, atypically located and congested intracerebral and basal veins as well as intracerebral changes secondary to the leptomeningeal angiomatosis are demonstrated with T2 weighted images. Gd-DTPA enhanced T1 weighted images exhibit clearly the regional distribution of angiomatosis in the skull, meninges and within the brain. Before calcifications in children with SWS are detectable by CT, MRI is the method of choice to detect intracranial involvement. Enhancement with Gd-DTPA improves the diagnostic value of MRI, before neurological symptoms appear. Follow-up studies with Gd-DTPA enhanced MRI can be applied to recognize thrombotic changes of leptomeningeal angiomatosis as well as subsequent intracerebral impairment.
authors.affiliation: Children's Hospital, Kaiserin Auguste Victoria Haus, Berlin.
authors.forename: J
authors.initials: J
authors.lastname: Sperner
authors.forename: I
authors.initials: I
authors.lastname: Schmauser
authors.forename: R
authors.initials: R
authors.lastname: Bittner
authors.forename: H
authors.initials: H
authors.lastname: Henkes
authors.forename: C
authors.initials: C
authors.lastname: Bassir
authors.forename: C
authors.initials: C
authors.lastname: Sprung
authors.forename: D
authors.initials: D
authors.lastname: Scheffner
authors.forename: R
authors.initials: R
authors.lastname: Felix
mesh_terms: D001284:Atrophy; D001921:Brain / Q000473:pathology; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging; D002540:Cerebral Cortex / Q000473:pathology; D002550:Cerebral Veins / Q000473:pathology; D004453:Echoencephalography; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013341:Sturge-Weber Syndrome / Q000175:diagnosis* / Q000000981:diagnostic imaging