A 34-year-old woman with recurrent ischaemic strokes undergoes digital subtraction angiography, which shows severe stenosis of the terminal internal carotid arteries bilaterally with a hazy network of fine collateral vessels arising from enlarged lenticulostriate and thalamoperforating arteries. This angiographic appearance is termed:
- A String and pearl sign
- B Double lumen sign
- C Puff of smoke sign ✓
- D Snowstorm appearance
Explanation
Moyamoya disease produces progressive steno-occlusion of the distal internal carotid arteries with compensatory hypertrophy of deep perforator collaterals, giving the classic puff of smoke appearance on angiography. The string and pearl sign describes alternating stenosis and dilatation in extracranial carotid dissection, and the double lumen sign is the intimal flap of dissection, neither of which fits this picture.
Reference: Osborn's Brain, 2nd ed.
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Written and medically reviewed by the StethoPrep medical team.