October 17, 2016 E-Pearl of the Week: Moyamoya disease and Moymoya syndrome

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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.


  1. Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease: disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969; 20: 288-299.
  2. 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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