moyamoya disease
Pronunciation: /ˈmɔɪəˌmɔɪə/
Progressive cerebrovascular disorder characterized by bilateral stenosis of terminal internal carotid arteries with abnormal collateral vessel networks.
Full Definition
Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by bilateral stenosis or occlusion of the terminal portions of the internal carotid arteries and their proximal branches. The condition is named after the Japanese term for 'puff of smoke,' describing the characteristic angiographic appearance of the abnormal collateral vessel networks (moyamoya vessels) that develop to compensate for the compromised blood flow. This disease can present with ischemic strokes, transient ischemic attacks, or hemorrhagic strokes, particularly in children and young adults. Diagnosis is confirmed through conventional angiography or MR angiography, and treatment often involves surgical revascularization procedures.
Usage
Usage note: Do not italicize despite Japanese origin; distinguish from moyamoya syndrome (unilateral or secondary forms).
In Context
- "The angiogram revealed bilateral moyamoya disease with extensive collateral formation." — Diagnostic report
- "Moyamoya disease should be considered in young patients presenting with recurrent strokes." — Clinical guideline