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Moyamoya disease — SCE Neurology MCQ

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EasyStrokeMoyamoya diseaseSCE Neurology

A 22-year-old man presented with recurrent TIAs precipitated by hyperventilation since adolescence. On examination, there was mild bilateral pyramidal weakness and cognitive slowing. Initial investigations showed: MRA showed terminal internal carotid stenosis with basal collaterals. What is the most likely underlying mechanism?

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Correct answer: A — progressive steno-occlusive arteriopathy with collateralisation

progressive steno-occlusive arteriopathy with collateralisation is the best answer because the vignette describes Moyamoya disease: bilateral distal ICA stenosis with collateral vessels suggests moyamoya. Large-vessel thromboembolism is plausible in a neighbouring presentation, but the chronology, examination or investigation pattern does not match the key discriminator here. Functional abnormality of motor prediction networks and JC virus infection of oligodendrocytes are less appropriate because they would require different localisation, timing or test findings; Raised intracranial pressure from impaired CSF dynamics would fit a different syndrome. Clinical pearl: atherosclerosis is less typical in a young patient with basal collaterals.

Reference: NICE NG128; National Clinical Guideline for Stroke; DVLA Assessing fitness to drive