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: Umberto Aguglia, Maria Adele Latella, Franco Cafarelli, Emilio Le Piane, Antonio Gangemi, Angelo Labate, Antonio Gambardella, Aldo Quattrone
• Journal: Journal of the neurological sciences (J Neurol Sci) Vol. 264 Issue 1-2 Pg. 168-72 (Jan 15 2008) ISSN: 0022-510X [Print] Netherlands
• PMID: 17720199 (Publication Type: Case Reports, Journal Article)

Topics

• Adult
• Cerebral Angiography
• Cerebral Arteries (abnormalities, diagnostic imaging, physiopathology)
• Cerebral Hemorrhage (diagnosis, etiology, physiopathology)
• Cerebral Veins (abnormalities, diagnostic imaging, physiopathology)
• Female
• Humans
• Intracranial Arteriovenous Malformations (diagnostic imaging, pathology, physiopathology)
• Magnetic Resonance Imaging
• Remission, Spontaneous
• Seizures (diagnosis, etiology, physiopathology)
• Sturge-Weber Syndrome (complications)
• Temporal Lobe (blood supply, diagnostic imaging, pathology)
• Tomography, X-Ray Computed