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CADASIL — SCE Neurology MCQ

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HardStrokeCADASILSCE Neurology

A 48-year-old woman has recurrent lacunar strokes, migraine with aura and progressive executive dysfunction. Her father developed dementia in his 50s. MRI shows confluent anterior temporal pole and external capsule white-matter hyperintensities with multiple lacunes. There is no hypertension or diabetes. What is the most likely underlying mechanism?

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Correct answer: C — NOTCH3-related small-vessel arteriopathy

NOTCH3-related small-vessel arteriopathy is the best answer. The family history, migraine with aura, lacunar strokes and anterior temporal/external capsule MRI pattern indicate CADASIL due to NOTCH3-related arteriopathy. Amyloid angiopathy causes lobar haemorrhage in older patients; mitochondrial disease has multisystem clues; cystatin C amyloid is rare with haemorrhage; ABCD1 causes adrenoleukodystrophy. Clinical pearl: Anterior temporal pole white-matter disease is a useful imaging discriminator.

Reference: Neurology 2022 Curriculum; GeneReviews CADASIL; NICE NG128