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Sturge-Weber Syndrome — SCE Neurology MCQ

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EasyEpilepsy & Seizure DisordersSturge-Weber SyndromeSCE Neurology

A 12-year-old girl (seen in transition clinic) has a history of seizures since age 3, developmental delay, and a facial port-wine stain (naevus flammeus) affecting the right forehead and upper eyelid in the V1 distribution. MRI brain shows an enhancing leptomeningeal angioma over the right occipital cortex with underlying cortical calcification. What is the diagnosis?

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Correct answer: A — Sturge-Weber syndrome

Sturge-Weber syndrome (encephalotrigeminal angiomatosis) is a neurocutaneous syndrome characterised by a facial port-wine stain in the V1 (ophthalmic) trigeminal distribution, ipsilateral leptomeningeal angioma, and ipsilateral glaucoma. CT shows characteristic 'tram-track' calcification; MRI shows leptomeningeal enhancement. Seizures are common and often drug-resistant. A: NF1 has café-au-lait spots and neurofibromas. B: TSC has facial angiofibromas and cortical tubers. D: Klippel-Trenaunay involves limb hypertrophy. E: VHL has haemangioblastomas.

Reference: ABN Neurocutaneous Syndromes Guidelines; NICE NG217