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Moyamoya Disease — RACP Paediatrics MCQ

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HardNeurology & DevelopmentMoyamoya DiseaseRACP Paediatrics

A 4-year-old child presents with hemiplegia, headache, and seizure. MRI with MRA shows bilateral narrowing of the internal carotid arteries with a 'puff of smoke' appearance on angiography. What is the diagnosis?

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Correct answer: D — Moyamoya disease

Moyamoya disease causes progressive stenosis of the bilateral distal internal carotid arteries with collateral vessel formation (giving the characteristic 'puff of smoke' angiographic appearance). It presents with ischaemic or haemorrhagic stroke. Surgical revascularisation (direct or indirect bypass) is the definitive treatment.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Paediatric Stroke