October 17, 2016 E-Pearl of the Week: Moyamoya disease and Moymoya syndrome
October 17, 2016
Moyamoya disease and Moymoya syndrome
Moyamoya is Japanese for “hazy, like a puff of cigarette smoke.” Moyamoya vasculopathy is a chronic cerebrovascular disease involving bilateral stenosis or occlusions of the internal carotid arteries and their proximal branches.1 Cerebral angiography reveals a classic smoky appearance due to the development of prominent arterial collateral circulation. Patients without known associated risk factors have Moyamoya disease, whereas patients with known associated conditions - including Sickle Cell Disease, Neurofibromatosis Type 1, and Down's syndrome - have Moyamoya syndrome.2 Moyamoya is more common in Asia but has been observed in populations throughout the world. Medical treatment includes the use of an antiplatelet agents.
- Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969; 20: 288-299.
- R. Michael Scott, M.D., and Edward R. Smith, M.D. Moyamoya Disease and Moyamoya Syndrome N Engl J Med 2009; 360: 1226-1237.
Submitted by Steve O'Donnell from the Department of Neurology at the University of Utah in Salt Lake City, UT. Dr. O'Donnell is a member of the Resident and Fellow Section of Neurology.
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