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Sturge-Weber syndrome — RACP Paediatrics MCQ

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ModerateNeurologySturge-Weber syndromeRACP Paediatrics

A newborn has a port-wine stain (capillary malformation) in the V1 distribution of the right trigeminal nerve. At 4 months, the child develops seizures. MRI shows leptomeningeal enhancement over the right parieto-occipital cortex with underlying cortical atrophy. What is the most likely diagnosis?

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Correct answer: B — Sturge-Weber syndrome

Sturge-Weber syndrome is characterised by a facial port-wine stain in the V1 trigeminal distribution with ipsilateral leptomeningeal angioma (vascular malformation). It presents with seizures (often refractory), hemiparesis contralateral to the lesion, and glaucoma. CT may show tramline (gyriform) calcification. Low-dose aspirin may be beneficial.

Reference: RCH Melbourne – 2023 – Neurocutaneous CPG; RACP Paediatric Curriculum – Neurology