T1 post-gad PDW T2

Diagnosis: Sturge-Weber syndrome

Sturge-Weber syndrome is a sporadically occurring phakomotosis which is otherwise known as encephalotrigeminal angiomatosis. It is caused by the failure of development of normal cortical venous drainage. Patients typically present with seizures, focal neurologic deficit, nevus flammeus in a distribution of cranial nerve 5. They may also have a glaucoma or buphthalmos. The classic imaging findings include gyriform cortical calcification with thickening of the ipsilateral calvarium and ipsilateral hemiatrophy on CT. On MR, there may be enhancement of the pial angiomatosis with prominent, enhancing choroid plexus. Other findings include enlarged subependymal or medullary veins and non-specific foci of patchy gliosis and demyelination in the brain parenchyma.

In this case the constellation of findings here is typical of Sturge-Weber syndrome. This patient has a right facial nevus flammeus and is status post radiofrequency ablation of a portion of the trigeminal nerve for trigeminal neuralgia. That is the reason for the asymmetry of the muscles of mastication. The finding of increased T1 signal pre-gad within the occipital cortex is probably due to calcification. Other entities to consider when faced with enhancement of the pia alone, without the other stigmata of Sturge-Weber include ischemia, encephalitis or meningitis, and leptomeningeal carcinomatosis. Related Cases

Vogl TJ, Stemmler J, Bergman C, et al. MR and MR angiography of Sturge-Weber syndrome. AJNR, Mar-Apr 1993; 14(2):p417-25.

Benedikt RA, Brown DC, Walker R, et al. Sturge-Weber syndrome: cranial MR imaging with Gd-DTPA. AJNR, Mar-Apr 1993; 14(2):p409-15.

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