"Sturge-Weber syndrome, also known as encephalotrigeminal angiomatosis, is a rare neurocutaneous disorder. It is clinically characterized by epilepsy, progressive mental retardation, and facial telangiectatic nevi, often in the distribution of a trigeminal nerve division. The pathogenesis is believed to be related to a vascular steal phenomenon secondary to an extensive cortical pial angiomatous malformation. Approximately 75%–90% of patients with Sturge-Weber syndrome have epilepsy".
"Unenhanced computed tomographic scan shows subcortical white matter and gray matter calcifications in the left parietal lobe (arrowhead)
Computed tomographic features of Sturge-Weber syndrome include
(a) intracranial dense gyriform calcifications, which more commonly affect the parieto-occipital cortical areas or the choroid plexus;
(b) diffuse high attenuation of the superficial and deep white matter, presumably due to microcalcifications;
(c) gyriform enhancement after the administration of iodinated contrast material, reflecting pial angiomatosis;
(d) brain atrophy as a consequence of vascular steal phenomena of the pial angioma on the surrounding cortical structures; and
(e) thickening of the calvaria as an indirect feature of loss of the brain substance ".
Axial contrast-enhanced T1-weighted MR image shows leptomeningeal enhancement in the left temporo-occipital lobe
"MR imaging is considered to be the standard of reference for the imaging of Sturge-Weber syndrome. One of the most important signs is leptomeningeal enhancement with gadolinium-based contrast agents . On T1-weighted MR images, enlargement and abnormally avid enhancement of the ipsilateral choroid plexus may also be seen . T2-weighted images are used to detect areas of gliosis and cerebral atrophy likely related to chronic ischemia . Gradient-recalled echo images are particularly sensitive for the detection of calcifications."
http://radiographics.rsna.org/content/28/4/1079.full#sec-7
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