A 30-year-old Japanese woman has recurrent transient ischemic attacks precipitated by hyperventilation. Catheter digital subtraction angiography shows bilateral stenosis and occlusion of the supraclinoid internal carotid arteries with a hazy netlike network of dilated deep collaterals at the base of the brain. This angiographic appearance is termed:
- A Puff of smoke appearance ✓
- B String and pearl sign
- C Snowstorm appearance
- D Salt and pepper sign
Explanation
Moyamoya disease is a progressive idiopathic steno-occlusive arteriopathy of the distal internal carotids and their proximal branches. Enlarged lenticulostriate and thalamoperforator collaterals produce the classic 'puff of smoke' (moyamoya means haze like cigarette smoke in Japanese) on angiography. String and pearl sign describes alternating stenosis and dilatation in fibromuscular dysplasia of the renal arteries.
Reference: Grainger and Allison's Diagnostic Radiology, 7th ed.
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Written and medically reviewed by the StethoPrep medical team.