moyamoya disease
Pronunciation: moy-ah-moy-ah
Progressive cerebrovascular disorder characterized by bilateral stenosis of terminal internal carotid arteries with development of collateral vessel networks. Creates characteristic 'puff of smoke' appearance on angiography.
Full Definition
Moyamoya disease is a chronic, progressive cerebrovascular disorder characterized by bilateral stenosis or occlusion of the terminal portions of the internal carotid arteries and their proximal branches. The condition leads to the development of an abnormal vascular network at the base of the brain, consisting of dilated perforating arteries that create a characteristic 'puff of smoke' appearance on conventional angiography. This collateral circulation attempts to compensate for the reduced blood flow from the primary vessels. The disease predominantly affects children and young adults, with higher prevalence in East Asian populations. Imaging evaluation requires multiple modalities including conventional angiography, CT angiography, MR angiography, and perfusion studies.
Usage
Usage note: Japanese term meaning 'puff of smoke'; no hyphenation required.
In Context
- "The conventional angiography revealed bilateral moyamoya disease with extensive collateral formation." — Neuroradiology report