Fig. 2. Recurrent infarcts with progression of intracranial stenosis. A 32-year-old female experienced recurrent left middle cerebral artery (MCA) infarcts (three times) within 2 years. Serial time-of-flight magnetic resonance angiography (MRA) showed the progression of stenosis in the left MCA. Transfemoral cerebral angiography (TFCA) showed no stenosis in the distal internal carotid artery or basal collaterals suggestive of moyamoya disease (MMD) (arrow). However, high-resolution (HR) MRI revealed a smaller outer diameter, concentric enhancement, and the absence of focal plaques in the stenotic segment (circle). A genetic investigation revealed that the RNF213 mutation was associated with MMD (p.Arg4810Lys). CE: contrast enhanced.