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Quiz Prize Rules Current Rankings DIAGNOSIS:
Sturge-Weber Syndrome
The images show an enlarged choroid plexus with left cerebral atrophy, parieto-occpital leptomeningeal enhancement and enlarged and enhancing subependymal and medullary veins.
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.
MRI features of Sturge- Weber syndrome include enlargement and abnormally avid enhancement of the choroid plexus with ipsilateral leptomeningeal enhancement usually in the parietooccipital cortex. T2W images are used to detect areas of gliosis and cerebral atrophy likely related to chronic ischemia. Gradient imaging may help to look for gyral calcifications. CT scan findings includeintracranial, dense gyriform calcifications, which more commonly affect the parieto-occipital cortical areas or the choroid plexus, diffuse high attenuation of the superficial and deep white matter, presumably due to microcalcifications, gyriform enhancement after the administration of iodinated contrast material, reflecting pial angiomatosis, brain atrophy as a consequence of vascular steal phenomena of the pial angioma on the surrounding cortical structures and thickening of the calvaria as an indirect feature of loss of the brain substance .
References:
1. Anne G Osborn, Diagnostic Neuroradiology, Chapter 5, Disorders of Histogenesis: Neurocutaneous Syndromes.
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PubMed References for Sturge-Weber Syndrome
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