MOYAMOYA syndrome is an intriguing cerebrovascular condition that affects children and is characterized by diffuse narrowing of large-caliber arteries to the brain and extensive collateralization resulting in a variety of ischemic symptoms derived from hypoperfusion. The disease is typically progressive and may have hemorrhagic complications. The incidence of moyamoya varies widely, but it is much more common in Asians than in whites. Its etiology is diverse, and its features are heterogeneous. Although relatively uncommon, because of the unusual cerebrovascular anatomy of those affected, an understanding of the pathobiology of moyamoya syndrome and its appropriate management may furnish insight into other more common occlusive cerebrovascular diseases. Management of moyamoya syndrome is indeed difficult and is approached with the goal of maintaining or restoring adequate perfusion.
Whitaker JN. Management of Moyamoya Syndrome. Arch Neurol. 2001;58(1):132. doi:10.1001/archneur.58.1.132
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