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Arch Pediatr Adolesc Med. 2009;163(2):180.

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Denouement and Comment: Moyamoya Disease

Moyamoya disease is a chronic cerebrovascular condition in which internal carotid artery or its terminal branches occlude progressively. New vessels develop, which bypass the stenotic arteries of the circle of Willis, creating a rich network of small fragile arteries. The dense angiographic appearance of newly formed collaterals is the hallmark of moyamoya, a Japanese term meaning "hazy, like a puff of smoke."

Moyamoya disease is rare, with an incidence of 0.35 to 0.54 per 100 000 people in Asia,^1-3 whereas its incidence is much lower in Western countries.³ It has a female predominance of 1.8:1,¹ which is less evident at younger ages.⁴

Secondary moyamoya disease has been associated with neurofibromatosis type 1, Down syndrome, fibromuscular dysplasia, congenital heart disease, cranial radiotherapy, intrathecal chemotherapy, sickle cell disease,⁵ and others. However, most cases are idiopathic, and a genetic origin may be present, as indicated by a higher prevalence in people of Asian descent³ . . . [Full Text of this Article]

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Arch Pediatr Adolesc Med. 2009;163(2):179.
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