Moyamoya Disease

**Definition:** A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.

**Tree numbers:** - C10.228.140.300.200.600 - C10.228.140.300.510.200.737 - C14.907.137.615 - C14.907.253.123.620 - C14.907.253.560.200.737

**Synonyms:** - Moya-Moya Disease - Cerebrovascular Moyamoya Disease - Moyamoya Syndrome - Progressive Intracranial Occlusive Arteropathy (Moyamoya)