Pop Quiz #73

Sturge-Weber syndrome

The correct answer is C. Tram-track calcifications of the temporal and occipital cortex. 

Sturge-Weber syndrome is a sporadic disorder characterized by a facial capillary malformation in a trigeminal nerve distribution. Patients with Sturge-Weber may have cerebral atrophy, ipsilateral vascular malformations of the leptomeninges, seizures, and glaucoma. The classic radiologic finding is tram-track calcifications of the temporal and occipital cortex.

Dural calcifications are seen in Papillon-Lefevre syndrome.  Calcification of the falx cerebri is seen in Gorlin’s Syndrome.  Osteopatha Striata is seen in Goltz syndrome.  Osteopoikilosis is seen in Buschke Ollendorf syndrome.

Reference:

Thomas-Sohl KA, Vaslow DF, Maria BL. Sturge-Weber syndrome: a review. Pediatr Neurol. 2004 May;30(5):303-10. PMID: 15165630

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