A 7 year old child presents with focal neurologic deficits.The following appearance on cerebral angiography is seen in which of the following conditions:
High-Yield Explanation
- Puff of smoke appearance on cerebral angiography suggests the diagnosis of Moyamoya disease. - Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked aeries at the base of the brain in an area called the basal ganglia. - The name "moyamoya" means "puff of smoke" in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. - In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks (TIA, commonly referred to as "mini-strokes"), frequently accompanied by muscular weakness or paralysis affecting one side of the body. Other options: Takayasu is characterized by segmental inflammatory panaeritis resulting in stenosis of aoa and its branches causing weak peripheral pulses. Takayasu's aeritis (also known as aoic arch syndrome, nonspecific aooaeritis, and pulseless disease) An aeriovenous malformation(AVM) is an abnormal tangle of blood vessels connecting aeries and veins, which disrupts normal blood flow and oxygen circulation